Case Reports

Patients presenting to the ED with injuries due to accidental self-injection with an epinephrine pen typically receive treatment to alleviate symptoms and reduce the potential of digital ischemia leading to gangrene and loss of tissue and function. Although there is no consensus or set guidelines in the literature regarding the management protocol of such cases, many reports support pharmacological intervention. There are, however, other reports that advocate conservative, nonpharmaceutical management (eg, immersing the affected digit in warm water) or an observation-only approach.

We present the first case report in Saudi Arabia of digital ischemia due to accidental injection of an epinephrine autoinjector, along with a review of the literature and management recommendations.

Case

A 28-year-old woman presented to the ED in significant pain and discomfort 20 minutes after she accidentally injected the entire contents of her aunt’s epinephrine autoinjector (0.3 mg of 1:1000) into her right thumb. The patient, who was in significant pain and discomfort, stated that she was unable to remove the injector needle, which was firmly embedded in the bone of the palmer aspect of the distal phalanx in a manner similar to that of an intraosseous injection (Figure 1).

The patient’s vital signs and oxygen saturation on presentation were within normal limits. The emergency physician successfully removed the embedded needle through moderate countertraction. On examination, the patient’s right thumb was pale and cold, and had poor capillary refill (Figure 2). Due to concerns of the potential for digital tissue ischemia leading to tissue loss and gangrene, warm, moist compresses were applied to the affected thumb, followed by 2% topical nitroglycerin paste, after which the thumb was covered with an occlusive dressing. Since there was no improvement in circulation after 20 minutes, an infiltrate of 5 mg (0.5 mL of 10 mg/mL) of phentolamine (α-agonist) mixed with 2.5 mL of 2% lidocaine was injected at the puncture site and base of the right thumb.¹ Hyperemia developed immediately at both injection sites, and the patient’s right thumb returned to a normal color and sensation 1 hour later, with a return to normal capillary refill. She remained in stable condition and was discharged home. Prior to discharge, the patient was educated on the proper handling and administration of an epinephrine autoinjector.

Discussion

Epinephrine is an ὰ- and β-adrenergic agonist that binds to the ὰ-adrenergic receptors of blood vessels, causing an increase in vascular resistance and vasoconstriction. Although the plasma half-life of epinephrine is approximately 2 to 3 minutes, subcutaneous or intramuscular injection resulting in local vasoconstriction may delay absorption; therefore, the effects of epinephrine may last much longer than its half-life.

The incidence of accidental injection from an epinephrine autoinjector is estimated to be 1 per 50,000 units dispensed.² To date, there are no established treatment guidelines on managing cases of digital injection. An online PubMed and Google Scholar search of the literature found one systematic review,³ four observational studies,^4-7 seven case series,^8-14 and several case reports^1,15-33 on the subject. Most of the patients in the published retrospective studies (71%) were treated conservatively with warming of the affected hand and observation, and the majority of patients in the case reports (87%) were treated pharmacologically, most commonly with topical nitroglycerin and phentolamine.^1,3-34 All of the patients in both the retrospective studies and case reports had restoration of perfusion without necrosis, irrespective of treatment modality. However, patients who were managed conservatively or who were treated with topical nitroglycerin required a longer duration of stay in the ED, suffered from severe reperfusion pain, and in some cases, had a longer time to complete recovery (≥10 weeks).⁸

Pharmaceutical and Nonpharmaceutical Management

Phentolamine. Phentolamine is a nonselective ὰ-adrenergic antagonist that binds to ὰ1 and ὰ2 receptors of blood vessels, resulting in a decrease in peripheral vascular resistance and vasodilation. Phentolamine directly antagonizes the effect of epinephrine by blocking the ὰ-adrenergic receptors, which in our patient resulted in immediate return of digital circulation and full resolution of symptoms.

 

Topical Nitroglycerin. Nitroglycerin is a nitrate vasodilator that when metabolically converted to nitric oxide, results in smooth muscle relaxation, venodilation, and arteriodilation. Patients suffering from digital ischemia and vasoconstriction may be treated with topical nitroglycerin paste to reverse ischemia by causing smooth muscle relaxation of digital blood vessels. Conservative Management. As previously noted, not all cases of digital epinephrine injection are treated pharmacologically. Some patients are not treated, but kept in observation until the ischemic effects of epinephrine have resolved. Likewise, some patients are treated conservatively with warm water compresses or by fully immersing the affected digit in warm water to facilitate reversal of vasoconstriction and ischemia.^3,8

Treatment Efficacy

In 2007, Fitzcharles-Bowe et al⁸ published a review of 59 cases of digital injection with high-dose epinephrine from 1989 to 2005. In this review, 32 of the 59 patients received no treatment, 25 patients received pharmacological treatment and in two patients, the treatment was unknown. Phentolamine was the most commonly used pharmacological agent (15 of 25 cases or 60%). Although none of the patients experienced digital necrosis, those treated with a local infiltration of phentolamine experienced a faster resolution of symptoms and normalization of perfusion. In 2004, Turner¹ reported a case of a 10-year-old boy who was treated with phentolamine following an accidental injection of epinephrine into his left hand. While circulation returned to the affected digit within 5 minutes of receiving the phentolamine injection, the patient continued to experience reduced sensation in the digit 6 weeks later.⁸

Interestingly, one of the coauthors of the Fitzcharles-Bowe et al⁸ report intentionally injected three of the digits of his left hand (middle, ring, and small fingers) at the same time with high-dose epinephrine to carefully observe and document the outcomes. All three of the digits became very pale and cool, with decreased sensation. The author treated himself conservatively (observation-only). He experienced spontaneous return of circulation in two of the digits within 6 to 10 hours. Although there was some spontaneous return of circulation to the third digit after 13 hours, the author noted prolonged, intense reperfusion pain 4 hours after return of circulation. He also suffered from neuropraxia in the third digit, which did not fully resolve until 10 weeks after the injury.⁸

A review of the literature shows phentolamine to be a safe and effective treatment for patients presenting with digital ischemia, with no long-term adverse effects or complications. Moreover, phentolamine appears to be safe and effective for use in both adult and pediatric patients.^3,8,35-38

Accidental Injection Prevention

Some of the cases of accidental epinephrine injection are due to user error. For example, a novice user may be holding the incorrect end of the injector in his or her hand when attempting to administer/deploy the device, resulting in premature dislodgement of the needle.³⁹

Although, most of the autoinjector devices available today are user-friendly, we believe the addition of a safety feature such as a trigger or safety-lock may further help to reduce accidents. The European Medicines Agency recommends that all patients and caregivers receive training on the proper handling and administration of epinephrine autoinjectors, citing this as the most important factor to ensure successful use of an epinephrine autoinjector and reduce accidental injury.⁴⁰ The patient in this case had not received any formal education or training regarding autoinjector use prior to this incident.

Safety of Lidocaine-Containing Epinephrine in Digital Anesthesia

Aside from cases of accidental digital epinephrine injection, clinicians have traditionally been taught to avoid using lidocaine with epinephrine for digital anesthesia. However, since the introduction of commercial lidocaine with epinephrine in 1948, there are no case reports of digital gangrene from commercially available lidocaine-epinephrine formulations.^41,42 In a multicenter prospective study by Lalonde et al⁴³ of 3,110 consecutive cases of elective injection of low-dose epinephrine in the hand, the authors concluded the likelihood of finger infarction is remote, particularly with possible phentolamine rescue therapy. Moreover, lidocaine-containing epinephrine (1%-2%) has a much lower concentration of epinephrine per mL of solution (5-10 mcg/mL) and appears to be safe for digital use.

Conclusion

This case describes the presentation and treatment of accidental digital injection of epinephrine, highlighting and supporting the benefits of local infiltration with phentolamine and observation until full recovery of perfusion. Local treatment with phentolamine not only facilitates recovery and return of capillary refill, but also shortens the duration of symptoms and alleviates vasoconstriction. In less severe cases, watchful waiting and observation may be appropriate and effective.

This case also underscores the importance of patient and caregiver education on the proper handling and administration of epinephrine autoinjectors to decrease the incidence of accidental injection.

Patients presenting to the ED with injuries due to accidental self-injection with an epinephrine pen typically receive treatment to alleviate symptoms and reduce the potential of digital ischemia leading to gangrene and loss of tissue and function. Although there is no consensus or set guidelines in the literature regarding the management protocol of such cases, many reports support pharmacological intervention. There are, however, other reports that advocate conservative, nonpharmaceutical management (eg, immersing the affected digit in warm water) or an observation-only approach.

We present the first case report in Saudi Arabia of digital ischemia due to accidental injection of an epinephrine autoinjector, along with a review of the literature and management recommendations.

Case

A 28-year-old woman presented to the ED in significant pain and discomfort 20 minutes after she accidentally injected the entire contents of her aunt’s epinephrine autoinjector (0.3 mg of 1:1000) into her right thumb. The patient, who was in significant pain and discomfort, stated that she was unable to remove the injector needle, which was firmly embedded in the bone of the palmer aspect of the distal phalanx in a manner similar to that of an intraosseous injection (Figure 1).

The patient’s vital signs and oxygen saturation on presentation were within normal limits. The emergency physician successfully removed the embedded needle through moderate countertraction. On examination, the patient’s right thumb was pale and cold, and had poor capillary refill (Figure 2). Due to concerns of the potential for digital tissue ischemia leading to tissue loss and gangrene, warm, moist compresses were applied to the affected thumb, followed by 2% topical nitroglycerin paste, after which the thumb was covered with an occlusive dressing. Since there was no improvement in circulation after 20 minutes, an infiltrate of 5 mg (0.5 mL of 10 mg/mL) of phentolamine (α-agonist) mixed with 2.5 mL of 2% lidocaine was injected at the puncture site and base of the right thumb.¹ Hyperemia developed immediately at both injection sites, and the patient’s right thumb returned to a normal color and sensation 1 hour later, with a return to normal capillary refill. She remained in stable condition and was discharged home. Prior to discharge, the patient was educated on the proper handling and administration of an epinephrine autoinjector.

Discussion

Epinephrine is an ὰ- and β-adrenergic agonist that binds to the ὰ-adrenergic receptors of blood vessels, causing an increase in vascular resistance and vasoconstriction. Although the plasma half-life of epinephrine is approximately 2 to 3 minutes, subcutaneous or intramuscular injection resulting in local vasoconstriction may delay absorption; therefore, the effects of epinephrine may last much longer than its half-life.

The incidence of accidental injection from an epinephrine autoinjector is estimated to be 1 per 50,000 units dispensed.² To date, there are no established treatment guidelines on managing cases of digital injection. An online PubMed and Google Scholar search of the literature found one systematic review,³ four observational studies,^4-7 seven case series,^8-14 and several case reports^1,15-33 on the subject. Most of the patients in the published retrospective studies (71%) were treated conservatively with warming of the affected hand and observation, and the majority of patients in the case reports (87%) were treated pharmacologically, most commonly with topical nitroglycerin and phentolamine.^1,3-34 All of the patients in both the retrospective studies and case reports had restoration of perfusion without necrosis, irrespective of treatment modality. However, patients who were managed conservatively or who were treated with topical nitroglycerin required a longer duration of stay in the ED, suffered from severe reperfusion pain, and in some cases, had a longer time to complete recovery (≥10 weeks).⁸

Pharmaceutical and Nonpharmaceutical Management

Phentolamine. Phentolamine is a nonselective ὰ-adrenergic antagonist that binds to ὰ1 and ὰ2 receptors of blood vessels, resulting in a decrease in peripheral vascular resistance and vasodilation. Phentolamine directly antagonizes the effect of epinephrine by blocking the ὰ-adrenergic receptors, which in our patient resulted in immediate return of digital circulation and full resolution of symptoms.

 

Topical Nitroglycerin. Nitroglycerin is a nitrate vasodilator that when metabolically converted to nitric oxide, results in smooth muscle relaxation, venodilation, and arteriodilation. Patients suffering from digital ischemia and vasoconstriction may be treated with topical nitroglycerin paste to reverse ischemia by causing smooth muscle relaxation of digital blood vessels. Conservative Management. As previously noted, not all cases of digital epinephrine injection are treated pharmacologically. Some patients are not treated, but kept in observation until the ischemic effects of epinephrine have resolved. Likewise, some patients are treated conservatively with warm water compresses or by fully immersing the affected digit in warm water to facilitate reversal of vasoconstriction and ischemia.^3,8

Treatment Efficacy

In 2007, Fitzcharles-Bowe et al⁸ published a review of 59 cases of digital injection with high-dose epinephrine from 1989 to 2005. In this review, 32 of the 59 patients received no treatment, 25 patients received pharmacological treatment and in two patients, the treatment was unknown. Phentolamine was the most commonly used pharmacological agent (15 of 25 cases or 60%). Although none of the patients experienced digital necrosis, those treated with a local infiltration of phentolamine experienced a faster resolution of symptoms and normalization of perfusion. In 2004, Turner¹ reported a case of a 10-year-old boy who was treated with phentolamine following an accidental injection of epinephrine into his left hand. While circulation returned to the affected digit within 5 minutes of receiving the phentolamine injection, the patient continued to experience reduced sensation in the digit 6 weeks later.⁸

Interestingly, one of the coauthors of the Fitzcharles-Bowe et al⁸ report intentionally injected three of the digits of his left hand (middle, ring, and small fingers) at the same time with high-dose epinephrine to carefully observe and document the outcomes. All three of the digits became very pale and cool, with decreased sensation. The author treated himself conservatively (observation-only). He experienced spontaneous return of circulation in two of the digits within 6 to 10 hours. Although there was some spontaneous return of circulation to the third digit after 13 hours, the author noted prolonged, intense reperfusion pain 4 hours after return of circulation. He also suffered from neuropraxia in the third digit, which did not fully resolve until 10 weeks after the injury.⁸

A review of the literature shows phentolamine to be a safe and effective treatment for patients presenting with digital ischemia, with no long-term adverse effects or complications. Moreover, phentolamine appears to be safe and effective for use in both adult and pediatric patients.^3,8,35-38

Accidental Injection Prevention

Some of the cases of accidental epinephrine injection are due to user error. For example, a novice user may be holding the incorrect end of the injector in his or her hand when attempting to administer/deploy the device, resulting in premature dislodgement of the needle.³⁹

Although, most of the autoinjector devices available today are user-friendly, we believe the addition of a safety feature such as a trigger or safety-lock may further help to reduce accidents. The European Medicines Agency recommends that all patients and caregivers receive training on the proper handling and administration of epinephrine autoinjectors, citing this as the most important factor to ensure successful use of an epinephrine autoinjector and reduce accidental injury.⁴⁰ The patient in this case had not received any formal education or training regarding autoinjector use prior to this incident.

Safety of Lidocaine-Containing Epinephrine in Digital Anesthesia

Aside from cases of accidental digital epinephrine injection, clinicians have traditionally been taught to avoid using lidocaine with epinephrine for digital anesthesia. However, since the introduction of commercial lidocaine with epinephrine in 1948, there are no case reports of digital gangrene from commercially available lidocaine-epinephrine formulations.^41,42 In a multicenter prospective study by Lalonde et al⁴³ of 3,110 consecutive cases of elective injection of low-dose epinephrine in the hand, the authors concluded the likelihood of finger infarction is remote, particularly with possible phentolamine rescue therapy. Moreover, lidocaine-containing epinephrine (1%-2%) has a much lower concentration of epinephrine per mL of solution (5-10 mcg/mL) and appears to be safe for digital use.

Conclusion

This case describes the presentation and treatment of accidental digital injection of epinephrine, highlighting and supporting the benefits of local infiltration with phentolamine and observation until full recovery of perfusion. Local treatment with phentolamine not only facilitates recovery and return of capillary refill, but also shortens the duration of symptoms and alleviates vasoconstriction. In less severe cases, watchful waiting and observation may be appropriate and effective.

This case also underscores the importance of patient and caregiver education on the proper handling and administration of epinephrine autoinjectors to decrease the incidence of accidental injection.

Patients presenting to the ED with injuries due to accidental self-injection with an epinephrine pen typically receive treatment to alleviate symptoms and reduce the potential of digital ischemia leading to gangrene and loss of tissue and function. Although there is no consensus or set guidelines in the literature regarding the management protocol of such cases, many reports support pharmacological intervention. There are, however, other reports that advocate conservative, nonpharmaceutical management (eg, immersing the affected digit in warm water) or an observation-only approach.

We present the first case report in Saudi Arabia of digital ischemia due to accidental injection of an epinephrine autoinjector, along with a review of the literature and management recommendations.

Case

A 28-year-old woman presented to the ED in significant pain and discomfort 20 minutes after she accidentally injected the entire contents of her aunt’s epinephrine autoinjector (0.3 mg of 1:1000) into her right thumb. The patient, who was in significant pain and discomfort, stated that she was unable to remove the injector needle, which was firmly embedded in the bone of the palmer aspect of the distal phalanx in a manner similar to that of an intraosseous injection (Figure 1).

The patient’s vital signs and oxygen saturation on presentation were within normal limits. The emergency physician successfully removed the embedded needle through moderate countertraction. On examination, the patient’s right thumb was pale and cold, and had poor capillary refill (Figure 2). Due to concerns of the potential for digital tissue ischemia leading to tissue loss and gangrene, warm, moist compresses were applied to the affected thumb, followed by 2% topical nitroglycerin paste, after which the thumb was covered with an occlusive dressing. Since there was no improvement in circulation after 20 minutes, an infiltrate of 5 mg (0.5 mL of 10 mg/mL) of phentolamine (α-agonist) mixed with 2.5 mL of 2% lidocaine was injected at the puncture site and base of the right thumb.¹ Hyperemia developed immediately at both injection sites, and the patient’s right thumb returned to a normal color and sensation 1 hour later, with a return to normal capillary refill. She remained in stable condition and was discharged home. Prior to discharge, the patient was educated on the proper handling and administration of an epinephrine autoinjector.

Discussion

Epinephrine is an ὰ- and β-adrenergic agonist that binds to the ὰ-adrenergic receptors of blood vessels, causing an increase in vascular resistance and vasoconstriction. Although the plasma half-life of epinephrine is approximately 2 to 3 minutes, subcutaneous or intramuscular injection resulting in local vasoconstriction may delay absorption; therefore, the effects of epinephrine may last much longer than its half-life.

The incidence of accidental injection from an epinephrine autoinjector is estimated to be 1 per 50,000 units dispensed.² To date, there are no established treatment guidelines on managing cases of digital injection. An online PubMed and Google Scholar search of the literature found one systematic review,³ four observational studies,^4-7 seven case series,^8-14 and several case reports^1,15-33 on the subject. Most of the patients in the published retrospective studies (71%) were treated conservatively with warming of the affected hand and observation, and the majority of patients in the case reports (87%) were treated pharmacologically, most commonly with topical nitroglycerin and phentolamine.^1,3-34 All of the patients in both the retrospective studies and case reports had restoration of perfusion without necrosis, irrespective of treatment modality. However, patients who were managed conservatively or who were treated with topical nitroglycerin required a longer duration of stay in the ED, suffered from severe reperfusion pain, and in some cases, had a longer time to complete recovery (≥10 weeks).⁸

Pharmaceutical and Nonpharmaceutical Management

Phentolamine. Phentolamine is a nonselective ὰ-adrenergic antagonist that binds to ὰ1 and ὰ2 receptors of blood vessels, resulting in a decrease in peripheral vascular resistance and vasodilation. Phentolamine directly antagonizes the effect of epinephrine by blocking the ὰ-adrenergic receptors, which in our patient resulted in immediate return of digital circulation and full resolution of symptoms.

 

Topical Nitroglycerin. Nitroglycerin is a nitrate vasodilator that when metabolically converted to nitric oxide, results in smooth muscle relaxation, venodilation, and arteriodilation. Patients suffering from digital ischemia and vasoconstriction may be treated with topical nitroglycerin paste to reverse ischemia by causing smooth muscle relaxation of digital blood vessels. Conservative Management. As previously noted, not all cases of digital epinephrine injection are treated pharmacologically. Some patients are not treated, but kept in observation until the ischemic effects of epinephrine have resolved. Likewise, some patients are treated conservatively with warm water compresses or by fully immersing the affected digit in warm water to facilitate reversal of vasoconstriction and ischemia.^3,8

Treatment Efficacy

In 2007, Fitzcharles-Bowe et al⁸ published a review of 59 cases of digital injection with high-dose epinephrine from 1989 to 2005. In this review, 32 of the 59 patients received no treatment, 25 patients received pharmacological treatment and in two patients, the treatment was unknown. Phentolamine was the most commonly used pharmacological agent (15 of 25 cases or 60%). Although none of the patients experienced digital necrosis, those treated with a local infiltration of phentolamine experienced a faster resolution of symptoms and normalization of perfusion. In 2004, Turner¹ reported a case of a 10-year-old boy who was treated with phentolamine following an accidental injection of epinephrine into his left hand. While circulation returned to the affected digit within 5 minutes of receiving the phentolamine injection, the patient continued to experience reduced sensation in the digit 6 weeks later.⁸

Interestingly, one of the coauthors of the Fitzcharles-Bowe et al⁸ report intentionally injected three of the digits of his left hand (middle, ring, and small fingers) at the same time with high-dose epinephrine to carefully observe and document the outcomes. All three of the digits became very pale and cool, with decreased sensation. The author treated himself conservatively (observation-only). He experienced spontaneous return of circulation in two of the digits within 6 to 10 hours. Although there was some spontaneous return of circulation to the third digit after 13 hours, the author noted prolonged, intense reperfusion pain 4 hours after return of circulation. He also suffered from neuropraxia in the third digit, which did not fully resolve until 10 weeks after the injury.⁸

A review of the literature shows phentolamine to be a safe and effective treatment for patients presenting with digital ischemia, with no long-term adverse effects or complications. Moreover, phentolamine appears to be safe and effective for use in both adult and pediatric patients.^3,8,35-38

Accidental Injection Prevention

Some of the cases of accidental epinephrine injection are due to user error. For example, a novice user may be holding the incorrect end of the injector in his or her hand when attempting to administer/deploy the device, resulting in premature dislodgement of the needle.³⁹

Although, most of the autoinjector devices available today are user-friendly, we believe the addition of a safety feature such as a trigger or safety-lock may further help to reduce accidents. The European Medicines Agency recommends that all patients and caregivers receive training on the proper handling and administration of epinephrine autoinjectors, citing this as the most important factor to ensure successful use of an epinephrine autoinjector and reduce accidental injury.⁴⁰ The patient in this case had not received any formal education or training regarding autoinjector use prior to this incident.

Safety of Lidocaine-Containing Epinephrine in Digital Anesthesia

Aside from cases of accidental digital epinephrine injection, clinicians have traditionally been taught to avoid using lidocaine with epinephrine for digital anesthesia. However, since the introduction of commercial lidocaine with epinephrine in 1948, there are no case reports of digital gangrene from commercially available lidocaine-epinephrine formulations.^41,42 In a multicenter prospective study by Lalonde et al⁴³ of 3,110 consecutive cases of elective injection of low-dose epinephrine in the hand, the authors concluded the likelihood of finger infarction is remote, particularly with possible phentolamine rescue therapy. Moreover, lidocaine-containing epinephrine (1%-2%) has a much lower concentration of epinephrine per mL of solution (5-10 mcg/mL) and appears to be safe for digital use.

Conclusion

This case describes the presentation and treatment of accidental digital injection of epinephrine, highlighting and supporting the benefits of local infiltration with phentolamine and observation until full recovery of perfusion. Local treatment with phentolamine not only facilitates recovery and return of capillary refill, but also shortens the duration of symptoms and alleviates vasoconstriction. In less severe cases, watchful waiting and observation may be appropriate and effective.

This case also underscores the importance of patient and caregiver education on the proper handling and administration of epinephrine autoinjectors to decrease the incidence of accidental injection.

Purulent pericarditis is an infection within the pericardial space rarely seen in the modern antibiotic era. Most cases are secondary to another infectious process of bacterial, viral, fungal, or parasitic origin.^1,2 Predisposing factors include malignancy, chronic kidney disease, immunosuppression, diabetes mellitus, and alcohol misuse disorder.¹ Although purulent pericarditis has been described extensively in the literature, it is a challenging diagnosis if it is not initially considered within the differential diagnosis repertoire.^1-4 Most authors agree that this may be because it has become an infrequent diagnosis.^1,2 In addition, purulent pericarditis may have an atypical presentation when compared with a classic case of pericarditis.^2,3 The authors believe that this forgotten entity will be revisited through this case.

Case Presentation

A 66-year-old-man was transferred to Veterans Affairs Caribbean Healthcare System (VACHS) from a community hospital with a diagnosis of community-acquired pneumonia (CAP) and bilateral pleural effusions. Four days prior to arrival at the community hospital, the patient had developed diffuse, watery diarrhea, which resolved in 3 days. After resolution of diarrhea, he began experiencing shortness of breath on exertion that progressed to onset at rest. The patient reported no fever, chills, nausea, vomiting, cough, or contact with others who were not healthy. He had a history of alcohol misuse without liver cirrhosis and reported no chronic diseases or use of medications. The patient had no history of tuberculosis exposure or pneumococcal vaccination, and had a negative interferon gamma release assay.

On admission to the community hospital, the patient was treated for CAP with ceftriaxone and azithromycin. On hospital day 3, the patient developed hypoxemia and an altered mental status. He was started on supplemental oxygen and transferred to the intensive care unit (ICU). Antibiotic therapy consequently was changed to levofloxacin and meropenem. However, no clinical improvement was noted on the following days.

On hospital day 7, the patient developed acute respiratory failure that required mechanical ventilation while being transferred to VACHS via air ambulance. His vital signs on arrival were the following: temperature, 97° F; heart rate, 86 beats/min; blood pressure, 103/61 mm Hg; respiratory rate, 14 breaths/min and SaO₂ of 97%, measured while he breathed supplemental oxygen at an FiO₂ of 0.4. 

Laboratory results revealed a white blood cell count (WBC) of 11.2 × 103/µL, with 92% neutrophils, 4% bands, 1% lymphocytes; lactic acid, 1.3 mmol/L; and high sensitivity C-reactive protein, 43.1 mg/L. A chest radiograph demonstrated bibasilar pulmonary opacities, bilateral pleural effusions, and cardiomegaly (Figure 1).

Hours after arrival, the patient developed sinus tachycardia and hypotension. A bedside 2D echocardiogram demonstrated a large pericardial effusion with diastolic collapse of the right atrium (Figure 2). 

Aggressive fluid resuscitation with normal saline and inotropic support with dopamine were started as an immediate percutaneous pericardiocentesis was performed with drainage of 400 mL of frank purulent fluid (Figure 3).

After percutaneous pericardiocentesis was completed, a temporary catheter was placed into the pericardial space using the Seldinger technique in order to aspirate fluid as needed.

The patient’s clinical condition improved following drainage of pericardial fluid, with no further need for inotropic support. Antibiotic therapy was changed to vancomycin and meropenem. Initial microbiologic samples from pericardial fluid demonstrated Gram-positive diplococci, suggestive of Streptococcus pneumoniae (S pneumoniae) (Figure 4). Other diagnostic pericardial fluid test results included: WBC count 25,330 cmm, with 99% neutrophils and 1% lymphocytes; total protein, 3.8 mg/dL; glucose, < 2.0 mg/dL,LDH, > 2,500 U/L, potassium hydroxide preparation. The tests found no fungus, and the acid fast bacilli smear revealed no Bacillus. However, the pericardial fluid culture failed to demonstrate growth of any organism. Blood cultures also were negative.

The patient underwent anterior thoracotomy with partial pericardiectomy, and a pericardial tube was left in place connected to drainage. During the procedure, an abundant amount of fibrinous tissue was evacuated from the pericardial space (Figure 5). 

The patient’s status improved the following day, and inotropic support once again was stopped. Bilateral pleural tubes were placed for evacuation of loculated effusions. Laboratory results from left-sided fluid were suggestive of complicated parapneumonic effusion with a WBC count of 6,683 cmm and glucose, 12 mg/dL. Right-sided pleural fluid was consistent with exudate with a WBC count of 2,006 cmm; pH 7.49; LDH, 1450 U/L; and glucose, 66 mg/dL. Gram stain and cultures of pleural fluid were negative.

The patient was extubated, pericardial and pleural tubes were removed, and he was transferred to the internal medicine ward 24 days after admission to the ICU. He received in-patient physical rehabilitation while completing a 6-week course of IV antibiotics (vancomycin and meropenem). After completion of therapy, the patient received the pneumococcal polysaccharide vaccination, and an echocardiography was repeated. No significant re-accumulation of pericardial effusion or constrictive pattern was evidenced. The patient was discharged to his out-of-state home, and follow-up was consequently lost.

 

Discussion

Purulent pericarditis is an infection localized within the pericardial space. Most cases are secondary to an infectious process elsewhere, which could be of bacterial, viral, fungal, or parasitic etiology.¹ Five mechanisms could lead the infecting organism to infect the pericardial space; contiguous spread from intrathoracic site, hematogenous spread, extension from myocardial site, perforating injury or surgery, and extension from a subdiaphragmatic site.¹ Predisposing factors for the development of this condition include malignancy, chronic kidney disease, immunosuppression, diabetes mellitus, and alcohol misuse. Pericarditis is an infection localized within the pericardial space.

Purulent pericarditis has become a rare entity in the antibiotic era.² Prior to the development of antibiotics, most cases were secondary to S pneumoniae.^1,2,5,6 As per Cilloniz and colleagues, about 40% to 50% of all cases of purulent pericarditis are caused by Gram-positive bacteria, mostly S pneumoniae.⁵ In this case study, bacterial culture did not reveal growth of an organism—most likely because the patient had received antibiotics elsewhere. However, Gram-positive cocci were seen within the initial pericardial aspirate. This organism was suspected to have spread contiguously from a pulmonary focus, which also led to pleural effusions.

Since the patient in this case study had no history of thoracic surgery, malignancy, or other immunosuppression, the patient’s history of alcohol misuse was the only predisposing factor for development of purulent pericarditis. Contrary to the common presentation of pericarditis, purulent pericarditis may not have the common clinical findings, such as chest pain, pericardial friction rub, and distended neck veins.^2,3 Furthermore, according to Parikh and colleagues, about 35% of affected patients may have a normal electrocardiogram.² Hence, the diagnosis of purulent pericarditis often is missed because the classic signs of pericarditis are often absent, and other nonspecific symptoms are attributed to initial underlying infection.⁷

A high index of suspicion is needed to diagnose purulent pericarditis. Once a diagnosis is made, initial treatment should consist of prompt drainage of pericardial fluid combined with systemic antibiotic therapy. Vancomycin and a third-generation cephalosporin may be started empirically until results of pericardial fluid cultures become available.³ Drainage can be achieved by pericardiocentesis, pericardiotomy, or pericardiectomy (partial or total).¹ In cases of hemodynamic instability due to cardiac tamponade, sonographically guided pericardiocentesis should be undertaken and an indwelling pericardial catheter left in place.¹ Although this is the simplest and fastest method of evacuation, it may not be effective when dealing with thick, fibrinous fluid. In such cases, intrapericardial fibrinolysis may be considered. This approach may be undertaken early in the process, after drainage insertion, or as salvage therapy, when there has been incomplete evacuation of purulent material or open surgical drainage is not available.

Streptokinase, urokinase, and tissue plasminogen activator have been used for intrapericadial fibrinolysis.¹ However, there is no definite data on dosage or frequency at which these medications should be administered. No matter the therapeutic approach, effective drainage of the pericardial fluid is crucial to avoid the development of pericardial constriction. Constrictive pericarditis occurs when fibrosis and adhesions create a dense pericardium that encases the heart. This causes impaired ventricular filling that can lead eventually to heart failure.⁴ Pericardiectomy is the definitive treatment for constrictive pericarditis.

Conclusion

Although purulent pericarditis has become a rare diagnosis since the development of antibiotics, knowledge of how to identify it is essential since mortality reaches 100% if the diagnosis is missed.⁴ Even when the condition is promptly diagnosed and treated, mortality is 40%, mainly due to cardiac tamponade, septic shock, or constriction.¹ The case presented here illustrates the clinical features associated with this condition. Knowing these features can translate in a successful patient outcome.

Purulent pericarditis is an infection within the pericardial space rarely seen in the modern antibiotic era. Most cases are secondary to another infectious process of bacterial, viral, fungal, or parasitic origin.^1,2 Predisposing factors include malignancy, chronic kidney disease, immunosuppression, diabetes mellitus, and alcohol misuse disorder.¹ Although purulent pericarditis has been described extensively in the literature, it is a challenging diagnosis if it is not initially considered within the differential diagnosis repertoire.^1-4 Most authors agree that this may be because it has become an infrequent diagnosis.^1,2 In addition, purulent pericarditis may have an atypical presentation when compared with a classic case of pericarditis.^2,3 The authors believe that this forgotten entity will be revisited through this case.

Case Presentation

A 66-year-old-man was transferred to Veterans Affairs Caribbean Healthcare System (VACHS) from a community hospital with a diagnosis of community-acquired pneumonia (CAP) and bilateral pleural effusions. Four days prior to arrival at the community hospital, the patient had developed diffuse, watery diarrhea, which resolved in 3 days. After resolution of diarrhea, he began experiencing shortness of breath on exertion that progressed to onset at rest. The patient reported no fever, chills, nausea, vomiting, cough, or contact with others who were not healthy. He had a history of alcohol misuse without liver cirrhosis and reported no chronic diseases or use of medications. The patient had no history of tuberculosis exposure or pneumococcal vaccination, and had a negative interferon gamma release assay.

On admission to the community hospital, the patient was treated for CAP with ceftriaxone and azithromycin. On hospital day 3, the patient developed hypoxemia and an altered mental status. He was started on supplemental oxygen and transferred to the intensive care unit (ICU). Antibiotic therapy consequently was changed to levofloxacin and meropenem. However, no clinical improvement was noted on the following days.

On hospital day 7, the patient developed acute respiratory failure that required mechanical ventilation while being transferred to VACHS via air ambulance. His vital signs on arrival were the following: temperature, 97° F; heart rate, 86 beats/min; blood pressure, 103/61 mm Hg; respiratory rate, 14 breaths/min and SaO₂ of 97%, measured while he breathed supplemental oxygen at an FiO₂ of 0.4. 

Laboratory results revealed a white blood cell count (WBC) of 11.2 × 103/µL, with 92% neutrophils, 4% bands, 1% lymphocytes; lactic acid, 1.3 mmol/L; and high sensitivity C-reactive protein, 43.1 mg/L. A chest radiograph demonstrated bibasilar pulmonary opacities, bilateral pleural effusions, and cardiomegaly (Figure 1).

Hours after arrival, the patient developed sinus tachycardia and hypotension. A bedside 2D echocardiogram demonstrated a large pericardial effusion with diastolic collapse of the right atrium (Figure 2). 

Aggressive fluid resuscitation with normal saline and inotropic support with dopamine were started as an immediate percutaneous pericardiocentesis was performed with drainage of 400 mL of frank purulent fluid (Figure 3).

After percutaneous pericardiocentesis was completed, a temporary catheter was placed into the pericardial space using the Seldinger technique in order to aspirate fluid as needed.

The patient’s clinical condition improved following drainage of pericardial fluid, with no further need for inotropic support. Antibiotic therapy was changed to vancomycin and meropenem. Initial microbiologic samples from pericardial fluid demonstrated Gram-positive diplococci, suggestive of Streptococcus pneumoniae (S pneumoniae) (Figure 4). Other diagnostic pericardial fluid test results included: WBC count 25,330 cmm, with 99% neutrophils and 1% lymphocytes; total protein, 3.8 mg/dL; glucose, < 2.0 mg/dL,LDH, > 2,500 U/L, potassium hydroxide preparation. The tests found no fungus, and the acid fast bacilli smear revealed no Bacillus. However, the pericardial fluid culture failed to demonstrate growth of any organism. Blood cultures also were negative.

The patient underwent anterior thoracotomy with partial pericardiectomy, and a pericardial tube was left in place connected to drainage. During the procedure, an abundant amount of fibrinous tissue was evacuated from the pericardial space (Figure 5). 

The patient’s status improved the following day, and inotropic support once again was stopped. Bilateral pleural tubes were placed for evacuation of loculated effusions. Laboratory results from left-sided fluid were suggestive of complicated parapneumonic effusion with a WBC count of 6,683 cmm and glucose, 12 mg/dL. Right-sided pleural fluid was consistent with exudate with a WBC count of 2,006 cmm; pH 7.49; LDH, 1450 U/L; and glucose, 66 mg/dL. Gram stain and cultures of pleural fluid were negative.

The patient was extubated, pericardial and pleural tubes were removed, and he was transferred to the internal medicine ward 24 days after admission to the ICU. He received in-patient physical rehabilitation while completing a 6-week course of IV antibiotics (vancomycin and meropenem). After completion of therapy, the patient received the pneumococcal polysaccharide vaccination, and an echocardiography was repeated. No significant re-accumulation of pericardial effusion or constrictive pattern was evidenced. The patient was discharged to his out-of-state home, and follow-up was consequently lost.

 

Discussion

Purulent pericarditis is an infection localized within the pericardial space. Most cases are secondary to an infectious process elsewhere, which could be of bacterial, viral, fungal, or parasitic etiology.¹ Five mechanisms could lead the infecting organism to infect the pericardial space; contiguous spread from intrathoracic site, hematogenous spread, extension from myocardial site, perforating injury or surgery, and extension from a subdiaphragmatic site.¹ Predisposing factors for the development of this condition include malignancy, chronic kidney disease, immunosuppression, diabetes mellitus, and alcohol misuse. Pericarditis is an infection localized within the pericardial space.

Purulent pericarditis has become a rare entity in the antibiotic era.² Prior to the development of antibiotics, most cases were secondary to S pneumoniae.^1,2,5,6 As per Cilloniz and colleagues, about 40% to 50% of all cases of purulent pericarditis are caused by Gram-positive bacteria, mostly S pneumoniae.⁵ In this case study, bacterial culture did not reveal growth of an organism—most likely because the patient had received antibiotics elsewhere. However, Gram-positive cocci were seen within the initial pericardial aspirate. This organism was suspected to have spread contiguously from a pulmonary focus, which also led to pleural effusions.

Since the patient in this case study had no history of thoracic surgery, malignancy, or other immunosuppression, the patient’s history of alcohol misuse was the only predisposing factor for development of purulent pericarditis. Contrary to the common presentation of pericarditis, purulent pericarditis may not have the common clinical findings, such as chest pain, pericardial friction rub, and distended neck veins.^2,3 Furthermore, according to Parikh and colleagues, about 35% of affected patients may have a normal electrocardiogram.² Hence, the diagnosis of purulent pericarditis often is missed because the classic signs of pericarditis are often absent, and other nonspecific symptoms are attributed to initial underlying infection.⁷

A high index of suspicion is needed to diagnose purulent pericarditis. Once a diagnosis is made, initial treatment should consist of prompt drainage of pericardial fluid combined with systemic antibiotic therapy. Vancomycin and a third-generation cephalosporin may be started empirically until results of pericardial fluid cultures become available.³ Drainage can be achieved by pericardiocentesis, pericardiotomy, or pericardiectomy (partial or total).¹ In cases of hemodynamic instability due to cardiac tamponade, sonographically guided pericardiocentesis should be undertaken and an indwelling pericardial catheter left in place.¹ Although this is the simplest and fastest method of evacuation, it may not be effective when dealing with thick, fibrinous fluid. In such cases, intrapericardial fibrinolysis may be considered. This approach may be undertaken early in the process, after drainage insertion, or as salvage therapy, when there has been incomplete evacuation of purulent material or open surgical drainage is not available.

Streptokinase, urokinase, and tissue plasminogen activator have been used for intrapericadial fibrinolysis.¹ However, there is no definite data on dosage or frequency at which these medications should be administered. No matter the therapeutic approach, effective drainage of the pericardial fluid is crucial to avoid the development of pericardial constriction. Constrictive pericarditis occurs when fibrosis and adhesions create a dense pericardium that encases the heart. This causes impaired ventricular filling that can lead eventually to heart failure.⁴ Pericardiectomy is the definitive treatment for constrictive pericarditis.

Conclusion

Although purulent pericarditis has become a rare diagnosis since the development of antibiotics, knowledge of how to identify it is essential since mortality reaches 100% if the diagnosis is missed.⁴ Even when the condition is promptly diagnosed and treated, mortality is 40%, mainly due to cardiac tamponade, septic shock, or constriction.¹ The case presented here illustrates the clinical features associated with this condition. Knowing these features can translate in a successful patient outcome.

Purulent pericarditis is an infection within the pericardial space rarely seen in the modern antibiotic era. Most cases are secondary to another infectious process of bacterial, viral, fungal, or parasitic origin.^1,2 Predisposing factors include malignancy, chronic kidney disease, immunosuppression, diabetes mellitus, and alcohol misuse disorder.¹ Although purulent pericarditis has been described extensively in the literature, it is a challenging diagnosis if it is not initially considered within the differential diagnosis repertoire.^1-4 Most authors agree that this may be because it has become an infrequent diagnosis.^1,2 In addition, purulent pericarditis may have an atypical presentation when compared with a classic case of pericarditis.^2,3 The authors believe that this forgotten entity will be revisited through this case.

Case Presentation

A 66-year-old-man was transferred to Veterans Affairs Caribbean Healthcare System (VACHS) from a community hospital with a diagnosis of community-acquired pneumonia (CAP) and bilateral pleural effusions. Four days prior to arrival at the community hospital, the patient had developed diffuse, watery diarrhea, which resolved in 3 days. After resolution of diarrhea, he began experiencing shortness of breath on exertion that progressed to onset at rest. The patient reported no fever, chills, nausea, vomiting, cough, or contact with others who were not healthy. He had a history of alcohol misuse without liver cirrhosis and reported no chronic diseases or use of medications. The patient had no history of tuberculosis exposure or pneumococcal vaccination, and had a negative interferon gamma release assay.

On admission to the community hospital, the patient was treated for CAP with ceftriaxone and azithromycin. On hospital day 3, the patient developed hypoxemia and an altered mental status. He was started on supplemental oxygen and transferred to the intensive care unit (ICU). Antibiotic therapy consequently was changed to levofloxacin and meropenem. However, no clinical improvement was noted on the following days.

On hospital day 7, the patient developed acute respiratory failure that required mechanical ventilation while being transferred to VACHS via air ambulance. His vital signs on arrival were the following: temperature, 97° F; heart rate, 86 beats/min; blood pressure, 103/61 mm Hg; respiratory rate, 14 breaths/min and SaO₂ of 97%, measured while he breathed supplemental oxygen at an FiO₂ of 0.4. 

Laboratory results revealed a white blood cell count (WBC) of 11.2 × 103/µL, with 92% neutrophils, 4% bands, 1% lymphocytes; lactic acid, 1.3 mmol/L; and high sensitivity C-reactive protein, 43.1 mg/L. A chest radiograph demonstrated bibasilar pulmonary opacities, bilateral pleural effusions, and cardiomegaly (Figure 1).

Hours after arrival, the patient developed sinus tachycardia and hypotension. A bedside 2D echocardiogram demonstrated a large pericardial effusion with diastolic collapse of the right atrium (Figure 2). 

Aggressive fluid resuscitation with normal saline and inotropic support with dopamine were started as an immediate percutaneous pericardiocentesis was performed with drainage of 400 mL of frank purulent fluid (Figure 3).

After percutaneous pericardiocentesis was completed, a temporary catheter was placed into the pericardial space using the Seldinger technique in order to aspirate fluid as needed.

The patient’s clinical condition improved following drainage of pericardial fluid, with no further need for inotropic support. Antibiotic therapy was changed to vancomycin and meropenem. Initial microbiologic samples from pericardial fluid demonstrated Gram-positive diplococci, suggestive of Streptococcus pneumoniae (S pneumoniae) (Figure 4). Other diagnostic pericardial fluid test results included: WBC count 25,330 cmm, with 99% neutrophils and 1% lymphocytes; total protein, 3.8 mg/dL; glucose, < 2.0 mg/dL,LDH, > 2,500 U/L, potassium hydroxide preparation. The tests found no fungus, and the acid fast bacilli smear revealed no Bacillus. However, the pericardial fluid culture failed to demonstrate growth of any organism. Blood cultures also were negative.

The patient underwent anterior thoracotomy with partial pericardiectomy, and a pericardial tube was left in place connected to drainage. During the procedure, an abundant amount of fibrinous tissue was evacuated from the pericardial space (Figure 5). 

The patient’s status improved the following day, and inotropic support once again was stopped. Bilateral pleural tubes were placed for evacuation of loculated effusions. Laboratory results from left-sided fluid were suggestive of complicated parapneumonic effusion with a WBC count of 6,683 cmm and glucose, 12 mg/dL. Right-sided pleural fluid was consistent with exudate with a WBC count of 2,006 cmm; pH 7.49; LDH, 1450 U/L; and glucose, 66 mg/dL. Gram stain and cultures of pleural fluid were negative.

The patient was extubated, pericardial and pleural tubes were removed, and he was transferred to the internal medicine ward 24 days after admission to the ICU. He received in-patient physical rehabilitation while completing a 6-week course of IV antibiotics (vancomycin and meropenem). After completion of therapy, the patient received the pneumococcal polysaccharide vaccination, and an echocardiography was repeated. No significant re-accumulation of pericardial effusion or constrictive pattern was evidenced. The patient was discharged to his out-of-state home, and follow-up was consequently lost.

 

Discussion

Purulent pericarditis is an infection localized within the pericardial space. Most cases are secondary to an infectious process elsewhere, which could be of bacterial, viral, fungal, or parasitic etiology.¹ Five mechanisms could lead the infecting organism to infect the pericardial space; contiguous spread from intrathoracic site, hematogenous spread, extension from myocardial site, perforating injury or surgery, and extension from a subdiaphragmatic site.¹ Predisposing factors for the development of this condition include malignancy, chronic kidney disease, immunosuppression, diabetes mellitus, and alcohol misuse. Pericarditis is an infection localized within the pericardial space.

Purulent pericarditis has become a rare entity in the antibiotic era.² Prior to the development of antibiotics, most cases were secondary to S pneumoniae.^1,2,5,6 As per Cilloniz and colleagues, about 40% to 50% of all cases of purulent pericarditis are caused by Gram-positive bacteria, mostly S pneumoniae.⁵ In this case study, bacterial culture did not reveal growth of an organism—most likely because the patient had received antibiotics elsewhere. However, Gram-positive cocci were seen within the initial pericardial aspirate. This organism was suspected to have spread contiguously from a pulmonary focus, which also led to pleural effusions.

Since the patient in this case study had no history of thoracic surgery, malignancy, or other immunosuppression, the patient’s history of alcohol misuse was the only predisposing factor for development of purulent pericarditis. Contrary to the common presentation of pericarditis, purulent pericarditis may not have the common clinical findings, such as chest pain, pericardial friction rub, and distended neck veins.^2,3 Furthermore, according to Parikh and colleagues, about 35% of affected patients may have a normal electrocardiogram.² Hence, the diagnosis of purulent pericarditis often is missed because the classic signs of pericarditis are often absent, and other nonspecific symptoms are attributed to initial underlying infection.⁷

A high index of suspicion is needed to diagnose purulent pericarditis. Once a diagnosis is made, initial treatment should consist of prompt drainage of pericardial fluid combined with systemic antibiotic therapy. Vancomycin and a third-generation cephalosporin may be started empirically until results of pericardial fluid cultures become available.³ Drainage can be achieved by pericardiocentesis, pericardiotomy, or pericardiectomy (partial or total).¹ In cases of hemodynamic instability due to cardiac tamponade, sonographically guided pericardiocentesis should be undertaken and an indwelling pericardial catheter left in place.¹ Although this is the simplest and fastest method of evacuation, it may not be effective when dealing with thick, fibrinous fluid. In such cases, intrapericardial fibrinolysis may be considered. This approach may be undertaken early in the process, after drainage insertion, or as salvage therapy, when there has been incomplete evacuation of purulent material or open surgical drainage is not available.

Streptokinase, urokinase, and tissue plasminogen activator have been used for intrapericadial fibrinolysis.¹ However, there is no definite data on dosage or frequency at which these medications should be administered. No matter the therapeutic approach, effective drainage of the pericardial fluid is crucial to avoid the development of pericardial constriction. Constrictive pericarditis occurs when fibrosis and adhesions create a dense pericardium that encases the heart. This causes impaired ventricular filling that can lead eventually to heart failure.⁴ Pericardiectomy is the definitive treatment for constrictive pericarditis.

Conclusion

Although purulent pericarditis has become a rare diagnosis since the development of antibiotics, knowledge of how to identify it is essential since mortality reaches 100% if the diagnosis is missed.⁴ Even when the condition is promptly diagnosed and treated, mortality is 40%, mainly due to cardiac tamponade, septic shock, or constriction.¹ The case presented here illustrates the clinical features associated with this condition. Knowing these features can translate in a successful patient outcome.

Case

An 84-year-old man, who was a resident at a local nursing home, presented for evaluation after the nursing staff noticed an increasingly swollen mass on the patient’s left groin. The patient’s medical history was significant for bilateral aortofemoral graft surgery, dementia, hypertension, and severe peripheral artery disease (PAD). He was not on any anticoagulation or antiplatelet agents. Due to the patient’s dementia, he was unable to provide a history regarding the onset of the swelling or any other signs or symptoms.

On examination, the patient did not appear in distress. His son, who was the patient’s durable power of attorney, was likewise unable to provide a clear timeframe regarding onset of the mass. The patient had no recent history of trauma and had not undergone any recent medical procedures. Vital signs at presentation were: blood pressure, 110/70 mm Hg; heart rate, 84 beats/min; respiratory rate, 13 breaths/min; and temperature, 98.6°F. Oxygen saturation was 94% on room air.

Clinical examination revealed a pulsatile, purple left groin mass and bruit. The mass was located around the left inguinal ligament and extended down the proximal, inner thigh (Figure 1). There was no drainage or lesions from the mass. Inspection of the patient’s hip demonstrated decreased adduction, limited by the mass; otherwise, there was normal range of motion. The dorsalis pedis and posterior tibial pulses were equal and intact, and the rest of the physical examination was unremarkable.

The patient tolerated the examination without focal signs of discomfort. A Doppler ultrasound revealed findings consistent with a common femoral pseudoaneurysm (PSA) (Figure 2). For better visualization and extension, a computed tomography angiogram (CTA) was obtained, which demonstrated a PSA measuring 11.7 x 10.7 x 7.3 cm; there was no active extravasation (Figure 3).

The patient was started on intravenous normal saline while vascular surgery services was consulted for management and repair. After a discussion with the son regarding the patient’s wishes, surgical intervention was refused and the patient was conservatively managed and transitioned to hospice care.

Discussion

A true aneurysm differs from a PSA in that true aneurysms involve all three layers of the vessel wall. A PSA consists partly of the vessel wall and partly of encapsulating fibrous tissue or surrounding tissue.

Etiology

Femoral artery PSAs can be iatrogenic, for example, develop following cardiac catheterization or at the anastomotic site of previous surgery.¹ The incidence of diagnostic postcatheterization PSA ranges from 0.05% to 2%, whereas interventional postcatheterization PSA ranges from 2% to 6%.²

With the increasing number of peripheral coronary diagnostics and interventions, emergency physicians should include PSA in the differential diagnosis of patients with a recent or remote history of catheterization or bypass grafts. Less commonly, femoral PSAs are caused by non-surgical trauma or infection (ie, mycotic PSA). Patient risk factors for development of PSA include obesity, hypertension, PAD, and anticoagulation.³ Patients with femoral artery PSAs may present with a painful or painless pulsatile mass. Mass effect of the PSA can compress nearby neurovascular structures, leading to femoral neuropathies or limb edema secondary to venous obstruction.⁴ Complications of embolization or thrombosis can cause limb ischemia, neuropathy, and claudication, while rupture may present with a rapidly expanding groin hematoma. Additionally, sizeable PSAs can cause overlying skin necrosis.⁵

Imaging Studies

Diagnosis of a PSA can be made through Doppler ultrasound, which is the preferred imaging modality due to its accuracy, noninvasive nature, and low cost. Doppler ultrasound has been found to have a sensitivity of 94% and specificity of 97% in detecting PSAs. Additional imaging with CTA can provide further definition of vasculopathy.⁶ Treatment should be considered for patients with a symptomatic femoral PSA, a PSA measuring more than 3 cm, or patients who are on anticoagulation therapy. Studies have shown that observation-only and follow-up may be appropriate for patients with a PSA measuring less than 3 cm. A study by Toursarkissian et al⁷ found that the majority of PSAs smaller than 3 cm spontaneously resolved in a mean of 23 days without limb-threatening complications.

Treatment

Traditionally, open surgical repair techniques were the only treatment option for PSAs. However, in the early 1990s, the advent of new techniques such as stenting, coil insertion, ultrasound-guided compression, and ultrasound-guided thrombin injection, have developed as alternatives to open surgical repair; there has been variable success to these minimally invasive approaches.^5,8

 

Ultrasound-Guided Compression. A conservative approach to treating PSAs, ultrasound-guided compression requires sustained compression by a skilled physician. This technique is associated with significant discomfort to the patient.⁵ Ultrasound-Guided Thrombin Injection. This technique is the treatment of choice for postcatheterization PSA. However, this intervention is contraindicated in patients who have concerning features such as an infected PSA, rapid expansion, skin necrosis, or signs of limb ischemia. Additionally, ultrasound-guided thrombin injection is not appropriate for use in patients with a PSA occurring at anastomosis of a synthetic graft and native artery.⁵

Conclusion

Based on our patient’s clinical presentation and history of aortofemoral bypass surgery, we suspected a femoral PSA. While the PSA noted in our patient was sizeable, imaging studies and clinical examination showed no sign of limb ischemia or rupture.

Femoral PSAs are usually iatrogenic in nature, typically developing shortly after catheterization or a previous bypass surgery. The most serious complication of a PSA is rupture, but a thorough examination of the distal extremity is warranted to assess for limb ischemia as well. Ultrasound imaging is considered the modality of choice based on its high sensitivity and sensitivity for detecting PSAs.

Small PSAs (<3 cm) can be managed medically, but larger PSAs (>3 cm) require treatment. Newer techniques, including stenting, coil insertion, ultrasound-guided compression, and ultrasound-guided thrombin injection are alternatives to open surgical repair of larger, uncomplicated PSAs. However, urgent open surgical repair is the only option when there is evidence of a ruptured PSA, ischemia, or skin necrosis.

Case

An 84-year-old man, who was a resident at a local nursing home, presented for evaluation after the nursing staff noticed an increasingly swollen mass on the patient’s left groin. The patient’s medical history was significant for bilateral aortofemoral graft surgery, dementia, hypertension, and severe peripheral artery disease (PAD). He was not on any anticoagulation or antiplatelet agents. Due to the patient’s dementia, he was unable to provide a history regarding the onset of the swelling or any other signs or symptoms.

On examination, the patient did not appear in distress. His son, who was the patient’s durable power of attorney, was likewise unable to provide a clear timeframe regarding onset of the mass. The patient had no recent history of trauma and had not undergone any recent medical procedures. Vital signs at presentation were: blood pressure, 110/70 mm Hg; heart rate, 84 beats/min; respiratory rate, 13 breaths/min; and temperature, 98.6°F. Oxygen saturation was 94% on room air.

Clinical examination revealed a pulsatile, purple left groin mass and bruit. The mass was located around the left inguinal ligament and extended down the proximal, inner thigh (Figure 1). There was no drainage or lesions from the mass. Inspection of the patient’s hip demonstrated decreased adduction, limited by the mass; otherwise, there was normal range of motion. The dorsalis pedis and posterior tibial pulses were equal and intact, and the rest of the physical examination was unremarkable.

The patient tolerated the examination without focal signs of discomfort. A Doppler ultrasound revealed findings consistent with a common femoral pseudoaneurysm (PSA) (Figure 2). For better visualization and extension, a computed tomography angiogram (CTA) was obtained, which demonstrated a PSA measuring 11.7 x 10.7 x 7.3 cm; there was no active extravasation (Figure 3).

The patient was started on intravenous normal saline while vascular surgery services was consulted for management and repair. After a discussion with the son regarding the patient’s wishes, surgical intervention was refused and the patient was conservatively managed and transitioned to hospice care.

Discussion

A true aneurysm differs from a PSA in that true aneurysms involve all three layers of the vessel wall. A PSA consists partly of the vessel wall and partly of encapsulating fibrous tissue or surrounding tissue.

Etiology

Femoral artery PSAs can be iatrogenic, for example, develop following cardiac catheterization or at the anastomotic site of previous surgery.¹ The incidence of diagnostic postcatheterization PSA ranges from 0.05% to 2%, whereas interventional postcatheterization PSA ranges from 2% to 6%.²

With the increasing number of peripheral coronary diagnostics and interventions, emergency physicians should include PSA in the differential diagnosis of patients with a recent or remote history of catheterization or bypass grafts. Less commonly, femoral PSAs are caused by non-surgical trauma or infection (ie, mycotic PSA). Patient risk factors for development of PSA include obesity, hypertension, PAD, and anticoagulation.³ Patients with femoral artery PSAs may present with a painful or painless pulsatile mass. Mass effect of the PSA can compress nearby neurovascular structures, leading to femoral neuropathies or limb edema secondary to venous obstruction.⁴ Complications of embolization or thrombosis can cause limb ischemia, neuropathy, and claudication, while rupture may present with a rapidly expanding groin hematoma. Additionally, sizeable PSAs can cause overlying skin necrosis.⁵

Imaging Studies

Diagnosis of a PSA can be made through Doppler ultrasound, which is the preferred imaging modality due to its accuracy, noninvasive nature, and low cost. Doppler ultrasound has been found to have a sensitivity of 94% and specificity of 97% in detecting PSAs. Additional imaging with CTA can provide further definition of vasculopathy.⁶ Treatment should be considered for patients with a symptomatic femoral PSA, a PSA measuring more than 3 cm, or patients who are on anticoagulation therapy. Studies have shown that observation-only and follow-up may be appropriate for patients with a PSA measuring less than 3 cm. A study by Toursarkissian et al⁷ found that the majority of PSAs smaller than 3 cm spontaneously resolved in a mean of 23 days without limb-threatening complications.

Treatment

Traditionally, open surgical repair techniques were the only treatment option for PSAs. However, in the early 1990s, the advent of new techniques such as stenting, coil insertion, ultrasound-guided compression, and ultrasound-guided thrombin injection, have developed as alternatives to open surgical repair; there has been variable success to these minimally invasive approaches.^5,8

 

Ultrasound-Guided Compression. A conservative approach to treating PSAs, ultrasound-guided compression requires sustained compression by a skilled physician. This technique is associated with significant discomfort to the patient.⁵ Ultrasound-Guided Thrombin Injection. This technique is the treatment of choice for postcatheterization PSA. However, this intervention is contraindicated in patients who have concerning features such as an infected PSA, rapid expansion, skin necrosis, or signs of limb ischemia. Additionally, ultrasound-guided thrombin injection is not appropriate for use in patients with a PSA occurring at anastomosis of a synthetic graft and native artery.⁵

Conclusion

Based on our patient’s clinical presentation and history of aortofemoral bypass surgery, we suspected a femoral PSA. While the PSA noted in our patient was sizeable, imaging studies and clinical examination showed no sign of limb ischemia or rupture.

Femoral PSAs are usually iatrogenic in nature, typically developing shortly after catheterization or a previous bypass surgery. The most serious complication of a PSA is rupture, but a thorough examination of the distal extremity is warranted to assess for limb ischemia as well. Ultrasound imaging is considered the modality of choice based on its high sensitivity and sensitivity for detecting PSAs.

Small PSAs (<3 cm) can be managed medically, but larger PSAs (>3 cm) require treatment. Newer techniques, including stenting, coil insertion, ultrasound-guided compression, and ultrasound-guided thrombin injection are alternatives to open surgical repair of larger, uncomplicated PSAs. However, urgent open surgical repair is the only option when there is evidence of a ruptured PSA, ischemia, or skin necrosis.

Case

An 84-year-old man, who was a resident at a local nursing home, presented for evaluation after the nursing staff noticed an increasingly swollen mass on the patient’s left groin. The patient’s medical history was significant for bilateral aortofemoral graft surgery, dementia, hypertension, and severe peripheral artery disease (PAD). He was not on any anticoagulation or antiplatelet agents. Due to the patient’s dementia, he was unable to provide a history regarding the onset of the swelling or any other signs or symptoms.

On examination, the patient did not appear in distress. His son, who was the patient’s durable power of attorney, was likewise unable to provide a clear timeframe regarding onset of the mass. The patient had no recent history of trauma and had not undergone any recent medical procedures. Vital signs at presentation were: blood pressure, 110/70 mm Hg; heart rate, 84 beats/min; respiratory rate, 13 breaths/min; and temperature, 98.6°F. Oxygen saturation was 94% on room air.

Clinical examination revealed a pulsatile, purple left groin mass and bruit. The mass was located around the left inguinal ligament and extended down the proximal, inner thigh (Figure 1). There was no drainage or lesions from the mass. Inspection of the patient’s hip demonstrated decreased adduction, limited by the mass; otherwise, there was normal range of motion. The dorsalis pedis and posterior tibial pulses were equal and intact, and the rest of the physical examination was unremarkable.

The patient tolerated the examination without focal signs of discomfort. A Doppler ultrasound revealed findings consistent with a common femoral pseudoaneurysm (PSA) (Figure 2). For better visualization and extension, a computed tomography angiogram (CTA) was obtained, which demonstrated a PSA measuring 11.7 x 10.7 x 7.3 cm; there was no active extravasation (Figure 3).

The patient was started on intravenous normal saline while vascular surgery services was consulted for management and repair. After a discussion with the son regarding the patient’s wishes, surgical intervention was refused and the patient was conservatively managed and transitioned to hospice care.

Discussion

A true aneurysm differs from a PSA in that true aneurysms involve all three layers of the vessel wall. A PSA consists partly of the vessel wall and partly of encapsulating fibrous tissue or surrounding tissue.

Etiology

Femoral artery PSAs can be iatrogenic, for example, develop following cardiac catheterization or at the anastomotic site of previous surgery.¹ The incidence of diagnostic postcatheterization PSA ranges from 0.05% to 2%, whereas interventional postcatheterization PSA ranges from 2% to 6%.²

With the increasing number of peripheral coronary diagnostics and interventions, emergency physicians should include PSA in the differential diagnosis of patients with a recent or remote history of catheterization or bypass grafts. Less commonly, femoral PSAs are caused by non-surgical trauma or infection (ie, mycotic PSA). Patient risk factors for development of PSA include obesity, hypertension, PAD, and anticoagulation.³ Patients with femoral artery PSAs may present with a painful or painless pulsatile mass. Mass effect of the PSA can compress nearby neurovascular structures, leading to femoral neuropathies or limb edema secondary to venous obstruction.⁴ Complications of embolization or thrombosis can cause limb ischemia, neuropathy, and claudication, while rupture may present with a rapidly expanding groin hematoma. Additionally, sizeable PSAs can cause overlying skin necrosis.⁵

Imaging Studies

Diagnosis of a PSA can be made through Doppler ultrasound, which is the preferred imaging modality due to its accuracy, noninvasive nature, and low cost. Doppler ultrasound has been found to have a sensitivity of 94% and specificity of 97% in detecting PSAs. Additional imaging with CTA can provide further definition of vasculopathy.⁶ Treatment should be considered for patients with a symptomatic femoral PSA, a PSA measuring more than 3 cm, or patients who are on anticoagulation therapy. Studies have shown that observation-only and follow-up may be appropriate for patients with a PSA measuring less than 3 cm. A study by Toursarkissian et al⁷ found that the majority of PSAs smaller than 3 cm spontaneously resolved in a mean of 23 days without limb-threatening complications.

Treatment

Traditionally, open surgical repair techniques were the only treatment option for PSAs. However, in the early 1990s, the advent of new techniques such as stenting, coil insertion, ultrasound-guided compression, and ultrasound-guided thrombin injection, have developed as alternatives to open surgical repair; there has been variable success to these minimally invasive approaches.^5,8

 

Ultrasound-Guided Compression. A conservative approach to treating PSAs, ultrasound-guided compression requires sustained compression by a skilled physician. This technique is associated with significant discomfort to the patient.⁵ Ultrasound-Guided Thrombin Injection. This technique is the treatment of choice for postcatheterization PSA. However, this intervention is contraindicated in patients who have concerning features such as an infected PSA, rapid expansion, skin necrosis, or signs of limb ischemia. Additionally, ultrasound-guided thrombin injection is not appropriate for use in patients with a PSA occurring at anastomosis of a synthetic graft and native artery.⁵

Conclusion

Based on our patient’s clinical presentation and history of aortofemoral bypass surgery, we suspected a femoral PSA. While the PSA noted in our patient was sizeable, imaging studies and clinical examination showed no sign of limb ischemia or rupture.

Femoral PSAs are usually iatrogenic in nature, typically developing shortly after catheterization or a previous bypass surgery. The most serious complication of a PSA is rupture, but a thorough examination of the distal extremity is warranted to assess for limb ischemia as well. Ultrasound imaging is considered the modality of choice based on its high sensitivity and sensitivity for detecting PSAs.

Small PSAs (<3 cm) can be managed medically, but larger PSAs (>3 cm) require treatment. Newer techniques, including stenting, coil insertion, ultrasound-guided compression, and ultrasound-guided thrombin injection are alternatives to open surgical repair of larger, uncomplicated PSAs. However, urgent open surgical repair is the only option when there is evidence of a ruptured PSA, ischemia, or skin necrosis.

Phlegmasia cerulea dolens (PCD), a life-threatening complication of deep venous thrombosis (DVT), is characterized by massive iliofemoral thrombus that extends to the collateral veins, leading to fluid sequestration and elevated compartment pressures that ultimately compromise arterial flow. Phlegmasia cerulea dolens can rapidly progress to compartment syndrome and gangrene.^1,2 The affected limbs of patients with PCD can be hypoxic and appear purple in color due to substantial lack of blood flow, with diminished or absent pulses. Risk factors for PCD include malignancy, hypercoagulable states, venous stasis, contraceptive agents, inferior vena cava (IVC) filter, aneurysm, history of DVT, trauma, heparin-induced thrombocytopenia, femoral vein catheterization, antiphospholipid syndrome, or pregnancy.^3-6 Failure to treat PCD early and aggressively carries an amputation rate of up to 50% and a mortality rate of up to 40%.⁴

We present the case of a patient with PCD, whose condition rapidly deteriorated despite prompt diagnosis and treatment.

Case

A 58-year-old woman presented to the ED with a 1-day history of back and leg pain and difficulty walking. When asked about the severity of her pain, she rated her leg pain at 10 on a scale of 0 to 10. The patient’s history was significant for DVT and pulmonary embolism (PE), for which a Greenfield IVC had been placed and for which she was on prophylactic warfarin therapy. The patient stated that she had been taken off warfarin several weeks prior to presentation in preparation for an elective colonoscopy and dental procedure, but had restarted the warfarin therapy 2 days prior to presentation. She had no history of diabetes mellitus or renal disease.

Initial vital signs at presentation were: blood pressure, 120/91 mm Hg; heart rate, 110 beats/min; respiratory rate, 24 breaths/min; and temperature, 96.6°F. Oxygen saturation was 100% on a nonrebreather mask.

On examination, the patient was alert and oriented to person, time, and place, but appeared dyspneic. An electrocardiogram revealed sinus tachycardia. On physical examination, lung sounds were clear to auscultation bilaterally with good air movement, and the abdomen was soft and nontender with normal bowel sounds. The dorsalis pedis and posterior tibial pulses were absent bilaterally, lower extremity capillary refill was 3 seconds, and the legs appeared mildly erythematous and cool to touch. No speech or neurological deficits were present.

Laboratory evaluation was remarkable for metabolic acidosis, venous pH, 7.11; bicarbonate, 11.7; partial pressure of carbon dioxide, 37.6; lactic acid, 8.8 mEq/L leukocytosis, 24,900 u/L; glucose, 296 mg/dL; creatinine, 2.41 mg/dL; and international normalized ratio, 1.36.

Before additional laboratory studies and imaging could be obtained, the patient developed altered mental status, hypotension, and paralysis of the lower extremities. She was orally intubated for airway protection and was given a total of 4 L of normal saline intravenously (IV) for hypotension and acidosis; sodium bicarbonate for metabolic acidosis; norepinephrine for hypotension; fentanyl for pain; and ondansetron for nausea. A central line and arterial line were placed for administering medication and hemodynamic monitoring.

Computed tomography (CT) angiography of the chest, abdomen, and pelvis demonstrated multiple subsegmental bilateral PE with no arterial pathology (Figure 1). Beside ultrasound revealed extensive bilateral DVTs involving the superficial and common femoral veins (Figure 2). The patient’s bilateral DVTs, arterial compromise, and leg cyanosis led to the diagnosis of PCD.

Critical care and vascular surgery services were consulted, and the patient was admitted to the intensive care unit. Since the patient was too unstable to undergo thrombectomy, she was given IV tissue plasminogen activator. Despite aggressive pharmacological treatment, the patient’s condition continued to deteriorate. On hospital day 2, the patient’s family changed the patient’s code status to do-not-resuscitate/comfort-care only; she died shortly thereafter.

Discussion

This case illustrates the severity and complications of PCD and the rapidity with which this condition can deteriorate. At the time of ED presentation, the patient had already developed bilateral PCD, metabolic acidosis, and bilateral PE. Unfortunately, due to decreased venous return, decreased cardiac output, and severe shock, she quickly became unstable and progressed rapidly to multisystem organ failure leading to death.

Risk Factors

A prior patient history DVT and an IVC filter are both significant risk factors for the progression of DVT to PCD;^3,6 however, in this case, IVC filter failed to prevent emboli from reaching the lungs. Extensive thrombi led to severely decreased venous return and cardiac output, causing life-threatening shock, ischemia, and metabolic acidosis. A lactic acid level taken on hospital day 2 was elevated at 19 mEq/L, demonstrating the severity, morbidity, and progression of PCD.

 

Signs and Symptoms

The three cardinal signs that lead to a clinical diagnosis of PCD are edema, pain, and violaceous discoloration or skin mottling.³ Although most commonly found in the lower extremity, PCD can occur in any limb due to occlusion of venous outflow.⁷ Unfortunately, a clinical diagnosis of PCD is not often made until the venous occlusion becomes severe enough to impair arterial flow and cause venous gangrene, tissue ischemia, shock, and death.⁸

Although IVC filters are designed to prevent life-threatening PE, there are risk factors associated with their use. Whether placed recently or decades prior, urgent investigation, such as immediate CT scan, should be undertaken in patients presenting with DVT-like symptoms who have a history of an IVC filter, to ensure the filter has not shifted from its original placement and is not occluding the IVC.

Conclusion

Phlegmasia cerulea dolens is an uncommon vascular emergency, but one that has a high-morbidity and high-mortality rate. This case demonstrates the importance of early diagnosis, aggressive treatment, and the severe complications that can develop in PCD.

There are cases in the literature where patients diagnosed with PCD had a successful outcome with pharmacological or surgical intervention such as thrombectomy. Treatment for PCD is most effective when instituted early in onset. As seen in our patient, the tendency for rapid deterioration in PCD can limit potentially lifesaving therapeutic options, decreasing the chances of a successful outcome. Emergency physicians, therefore, must be aware of the high-mortality rate associated with this disorder and the possibility of rapid progression from stable to critical condition.

Phlegmasia cerulea dolens (PCD), a life-threatening complication of deep venous thrombosis (DVT), is characterized by massive iliofemoral thrombus that extends to the collateral veins, leading to fluid sequestration and elevated compartment pressures that ultimately compromise arterial flow. Phlegmasia cerulea dolens can rapidly progress to compartment syndrome and gangrene.^1,2 The affected limbs of patients with PCD can be hypoxic and appear purple in color due to substantial lack of blood flow, with diminished or absent pulses. Risk factors for PCD include malignancy, hypercoagulable states, venous stasis, contraceptive agents, inferior vena cava (IVC) filter, aneurysm, history of DVT, trauma, heparin-induced thrombocytopenia, femoral vein catheterization, antiphospholipid syndrome, or pregnancy.^3-6 Failure to treat PCD early and aggressively carries an amputation rate of up to 50% and a mortality rate of up to 40%.⁴

We present the case of a patient with PCD, whose condition rapidly deteriorated despite prompt diagnosis and treatment.

Case

A 58-year-old woman presented to the ED with a 1-day history of back and leg pain and difficulty walking. When asked about the severity of her pain, she rated her leg pain at 10 on a scale of 0 to 10. The patient’s history was significant for DVT and pulmonary embolism (PE), for which a Greenfield IVC had been placed and for which she was on prophylactic warfarin therapy. The patient stated that she had been taken off warfarin several weeks prior to presentation in preparation for an elective colonoscopy and dental procedure, but had restarted the warfarin therapy 2 days prior to presentation. She had no history of diabetes mellitus or renal disease.

Initial vital signs at presentation were: blood pressure, 120/91 mm Hg; heart rate, 110 beats/min; respiratory rate, 24 breaths/min; and temperature, 96.6°F. Oxygen saturation was 100% on a nonrebreather mask.

On examination, the patient was alert and oriented to person, time, and place, but appeared dyspneic. An electrocardiogram revealed sinus tachycardia. On physical examination, lung sounds were clear to auscultation bilaterally with good air movement, and the abdomen was soft and nontender with normal bowel sounds. The dorsalis pedis and posterior tibial pulses were absent bilaterally, lower extremity capillary refill was 3 seconds, and the legs appeared mildly erythematous and cool to touch. No speech or neurological deficits were present.

Laboratory evaluation was remarkable for metabolic acidosis, venous pH, 7.11; bicarbonate, 11.7; partial pressure of carbon dioxide, 37.6; lactic acid, 8.8 mEq/L leukocytosis, 24,900 u/L; glucose, 296 mg/dL; creatinine, 2.41 mg/dL; and international normalized ratio, 1.36.

Before additional laboratory studies and imaging could be obtained, the patient developed altered mental status, hypotension, and paralysis of the lower extremities. She was orally intubated for airway protection and was given a total of 4 L of normal saline intravenously (IV) for hypotension and acidosis; sodium bicarbonate for metabolic acidosis; norepinephrine for hypotension; fentanyl for pain; and ondansetron for nausea. A central line and arterial line were placed for administering medication and hemodynamic monitoring.

Computed tomography (CT) angiography of the chest, abdomen, and pelvis demonstrated multiple subsegmental bilateral PE with no arterial pathology (Figure 1). Beside ultrasound revealed extensive bilateral DVTs involving the superficial and common femoral veins (Figure 2). The patient’s bilateral DVTs, arterial compromise, and leg cyanosis led to the diagnosis of PCD.

Critical care and vascular surgery services were consulted, and the patient was admitted to the intensive care unit. Since the patient was too unstable to undergo thrombectomy, she was given IV tissue plasminogen activator. Despite aggressive pharmacological treatment, the patient’s condition continued to deteriorate. On hospital day 2, the patient’s family changed the patient’s code status to do-not-resuscitate/comfort-care only; she died shortly thereafter.

Discussion

This case illustrates the severity and complications of PCD and the rapidity with which this condition can deteriorate. At the time of ED presentation, the patient had already developed bilateral PCD, metabolic acidosis, and bilateral PE. Unfortunately, due to decreased venous return, decreased cardiac output, and severe shock, she quickly became unstable and progressed rapidly to multisystem organ failure leading to death.

Risk Factors

A prior patient history DVT and an IVC filter are both significant risk factors for the progression of DVT to PCD;^3,6 however, in this case, IVC filter failed to prevent emboli from reaching the lungs. Extensive thrombi led to severely decreased venous return and cardiac output, causing life-threatening shock, ischemia, and metabolic acidosis. A lactic acid level taken on hospital day 2 was elevated at 19 mEq/L, demonstrating the severity, morbidity, and progression of PCD.

 

Signs and Symptoms

The three cardinal signs that lead to a clinical diagnosis of PCD are edema, pain, and violaceous discoloration or skin mottling.³ Although most commonly found in the lower extremity, PCD can occur in any limb due to occlusion of venous outflow.⁷ Unfortunately, a clinical diagnosis of PCD is not often made until the venous occlusion becomes severe enough to impair arterial flow and cause venous gangrene, tissue ischemia, shock, and death.⁸

Although IVC filters are designed to prevent life-threatening PE, there are risk factors associated with their use. Whether placed recently or decades prior, urgent investigation, such as immediate CT scan, should be undertaken in patients presenting with DVT-like symptoms who have a history of an IVC filter, to ensure the filter has not shifted from its original placement and is not occluding the IVC.

Conclusion

Phlegmasia cerulea dolens is an uncommon vascular emergency, but one that has a high-morbidity and high-mortality rate. This case demonstrates the importance of early diagnosis, aggressive treatment, and the severe complications that can develop in PCD.

There are cases in the literature where patients diagnosed with PCD had a successful outcome with pharmacological or surgical intervention such as thrombectomy. Treatment for PCD is most effective when instituted early in onset. As seen in our patient, the tendency for rapid deterioration in PCD can limit potentially lifesaving therapeutic options, decreasing the chances of a successful outcome. Emergency physicians, therefore, must be aware of the high-mortality rate associated with this disorder and the possibility of rapid progression from stable to critical condition.

Phlegmasia cerulea dolens (PCD), a life-threatening complication of deep venous thrombosis (DVT), is characterized by massive iliofemoral thrombus that extends to the collateral veins, leading to fluid sequestration and elevated compartment pressures that ultimately compromise arterial flow. Phlegmasia cerulea dolens can rapidly progress to compartment syndrome and gangrene.^1,2 The affected limbs of patients with PCD can be hypoxic and appear purple in color due to substantial lack of blood flow, with diminished or absent pulses. Risk factors for PCD include malignancy, hypercoagulable states, venous stasis, contraceptive agents, inferior vena cava (IVC) filter, aneurysm, history of DVT, trauma, heparin-induced thrombocytopenia, femoral vein catheterization, antiphospholipid syndrome, or pregnancy.^3-6 Failure to treat PCD early and aggressively carries an amputation rate of up to 50% and a mortality rate of up to 40%.⁴

We present the case of a patient with PCD, whose condition rapidly deteriorated despite prompt diagnosis and treatment.

Case

A 58-year-old woman presented to the ED with a 1-day history of back and leg pain and difficulty walking. When asked about the severity of her pain, she rated her leg pain at 10 on a scale of 0 to 10. The patient’s history was significant for DVT and pulmonary embolism (PE), for which a Greenfield IVC had been placed and for which she was on prophylactic warfarin therapy. The patient stated that she had been taken off warfarin several weeks prior to presentation in preparation for an elective colonoscopy and dental procedure, but had restarted the warfarin therapy 2 days prior to presentation. She had no history of diabetes mellitus or renal disease.

Initial vital signs at presentation were: blood pressure, 120/91 mm Hg; heart rate, 110 beats/min; respiratory rate, 24 breaths/min; and temperature, 96.6°F. Oxygen saturation was 100% on a nonrebreather mask.

On examination, the patient was alert and oriented to person, time, and place, but appeared dyspneic. An electrocardiogram revealed sinus tachycardia. On physical examination, lung sounds were clear to auscultation bilaterally with good air movement, and the abdomen was soft and nontender with normal bowel sounds. The dorsalis pedis and posterior tibial pulses were absent bilaterally, lower extremity capillary refill was 3 seconds, and the legs appeared mildly erythematous and cool to touch. No speech or neurological deficits were present.

Laboratory evaluation was remarkable for metabolic acidosis, venous pH, 7.11; bicarbonate, 11.7; partial pressure of carbon dioxide, 37.6; lactic acid, 8.8 mEq/L leukocytosis, 24,900 u/L; glucose, 296 mg/dL; creatinine, 2.41 mg/dL; and international normalized ratio, 1.36.

Before additional laboratory studies and imaging could be obtained, the patient developed altered mental status, hypotension, and paralysis of the lower extremities. She was orally intubated for airway protection and was given a total of 4 L of normal saline intravenously (IV) for hypotension and acidosis; sodium bicarbonate for metabolic acidosis; norepinephrine for hypotension; fentanyl for pain; and ondansetron for nausea. A central line and arterial line were placed for administering medication and hemodynamic monitoring.

Computed tomography (CT) angiography of the chest, abdomen, and pelvis demonstrated multiple subsegmental bilateral PE with no arterial pathology (Figure 1). Beside ultrasound revealed extensive bilateral DVTs involving the superficial and common femoral veins (Figure 2). The patient’s bilateral DVTs, arterial compromise, and leg cyanosis led to the diagnosis of PCD.

Critical care and vascular surgery services were consulted, and the patient was admitted to the intensive care unit. Since the patient was too unstable to undergo thrombectomy, she was given IV tissue plasminogen activator. Despite aggressive pharmacological treatment, the patient’s condition continued to deteriorate. On hospital day 2, the patient’s family changed the patient’s code status to do-not-resuscitate/comfort-care only; she died shortly thereafter.

Discussion

This case illustrates the severity and complications of PCD and the rapidity with which this condition can deteriorate. At the time of ED presentation, the patient had already developed bilateral PCD, metabolic acidosis, and bilateral PE. Unfortunately, due to decreased venous return, decreased cardiac output, and severe shock, she quickly became unstable and progressed rapidly to multisystem organ failure leading to death.

Risk Factors

A prior patient history DVT and an IVC filter are both significant risk factors for the progression of DVT to PCD;^3,6 however, in this case, IVC filter failed to prevent emboli from reaching the lungs. Extensive thrombi led to severely decreased venous return and cardiac output, causing life-threatening shock, ischemia, and metabolic acidosis. A lactic acid level taken on hospital day 2 was elevated at 19 mEq/L, demonstrating the severity, morbidity, and progression of PCD.

 

Signs and Symptoms

The three cardinal signs that lead to a clinical diagnosis of PCD are edema, pain, and violaceous discoloration or skin mottling.³ Although most commonly found in the lower extremity, PCD can occur in any limb due to occlusion of venous outflow.⁷ Unfortunately, a clinical diagnosis of PCD is not often made until the venous occlusion becomes severe enough to impair arterial flow and cause venous gangrene, tissue ischemia, shock, and death.⁸

Although IVC filters are designed to prevent life-threatening PE, there are risk factors associated with their use. Whether placed recently or decades prior, urgent investigation, such as immediate CT scan, should be undertaken in patients presenting with DVT-like symptoms who have a history of an IVC filter, to ensure the filter has not shifted from its original placement and is not occluding the IVC.

Conclusion

Phlegmasia cerulea dolens is an uncommon vascular emergency, but one that has a high-morbidity and high-mortality rate. This case demonstrates the importance of early diagnosis, aggressive treatment, and the severe complications that can develop in PCD.

There are cases in the literature where patients diagnosed with PCD had a successful outcome with pharmacological or surgical intervention such as thrombectomy. Treatment for PCD is most effective when instituted early in onset. As seen in our patient, the tendency for rapid deterioration in PCD can limit potentially lifesaving therapeutic options, decreasing the chances of a successful outcome. Emergency physicians, therefore, must be aware of the high-mortality rate associated with this disorder and the possibility of rapid progression from stable to critical condition.

Facial paralysis is a common medical complaint—one that has fascinated ancient and contemporary physicians alike.¹ An idiopathic facial nerve paresis involving the lower motor neuron was described in 1821 by Sir Charles Bell. This entity became known as a Bell’s palsy, the hallmark of which was weakness or complete paralysis of the muscles of one side of the face, with no sparing of the muscles of the forehead. However, not all facial paralysis is due to Bell’s palsy.

We present a case of a patient with a Bell’s palsy mimic to facilitate and guide the differential diagnosis and distinguish conditions from the classical presentation that Bell first described to the more concerning symptoms that may not be immediately obvious. Our case further underscores the importance of performing a thorough assessment to determine the presence of other neurological findings.

Case

A 61-year-old woman presented to the ED for evaluation of right facial droop and sensation of “room spinning.” The patient stated both symptoms began approximately 36 hours prior to presentation, upon awakening. She further noted that the right side of her face felt “funny” and numb.

The patient denied any headache, neck or chest pain, extremity numbness, or weakness, but stated that she felt like she was going to fall toward her right side whenever she attempted to walk. The patient’s medical history was significant for hypertension, for which she was taking losartan. Her surgical history was notable for a left oophorectomy secondary to an ovarian cyst. Regarding the social history, the patient admitted to smoking 90 packs of cigarettes per year, but denied alcohol or illicit drug use.

Upon arrival at the ED, the patient’s vital signs were: blood pressure, 164/86 mm Hg: pulse, 89 beats/min; respiratory rate, 18 breaths/min; and temperature, 98.6°F. Oxygen saturation was 98% on room air.

Physical examination revealed the patient had a right facial droop consistent with right facial palsy. She was unable to wrinkle her right forehead or fully close her right eye. There were no field cuts on confrontation. The patient’s speech was noticeable for a mild dysarthria. The motor examination revealed mild weakness of the left upper extremity and impaired right facial sensation. There were no rashes noted on the face, head, or ears. The patient had slightly impaired hearing in the right ear, which was new in onset. The remainder of the physical examination was unremarkable.

Although the patient exhibited the classic signs of Bell’s palsy, including complete paralysis of the muscles of one side of the face, inability to wrinkle the muscle of the right forehead, and inability to fully close the right eye, she also had concerning symptoms of vertigo, dysarthria, and contralateral upper extremity weakness.

A computed tomography (CT) scan of the head was ordered, which revealed a large mass lesion centered in the right petrous apex, with an associated large component extending medially into the right cerebellopontine angle (CPA) that caused a mass effect on the adjacent brainstem (Figures 1a and 1b).

Upon these findings, the patient was transferred to another facility for neurosurgical evaluation. Magnetic resonance imaging (MRI) studies performed at the receiving hospital demonstrated a large expansile heterogeneous mass lesion centered in the right petrous apex with an associated large, probable hemorrhagic soft-tissue component extending medially into the right CPA, causing a mass effect on the adjacent brainstem and mild obstructive hydrocephalus (Figures 2a and 2b).

The patient was given dexamethasone 10 mg intravenously and taken to the operating room for a right suboccipital craniotomy with subtotal tumor removal. Intraoperative high-voltage stimulation of the fifth to eighth cranial nerves showed no response, indicating significant impairment.

While there were no intraoperative complications, the patient had significant postoperative dysphagia and resultant aspiration. A tracheostomy and percutaneous endoscopic gastrostomy tube were subsequently placed. Results of a biopsy taken during surgery identified an atypical meningioma. The patient remained in the hospital for 4 weeks, after which she was discharged to a long-term care (LTC) and rehabilitation facility.

A repeat CT scan taken 2 months after surgery demonstrated absence of the previously identified large mass (Figure 1b). Three months after discharge from the LTC-rehabilitation facility, MRI of the brain showed continued interval improvement of the previously noted mass centered in the right petrous apex (Figures 3a and 3b).

 

Discussion

Accounts of facial paralysis and facial nerve disorders have been noted throughout history and include accounts of the condition by Hippocrates.¹ Bell’s palsy was named after surgeon Sir Charles Bell, who described a peripheral-nerve paralysis of the facial nerve in 1821. Bell’s work helped to elucidate the anatomy and functional role of the facial nerve.^1,2

Signs and Symptoms

The classic presentation of Bell’s palsy is weakness or complete paralysis of the muscles of one side of the face, with no sparing of the muscles of the forehead. The eyelid on the affected side generally does not close, which can result in ocular irritation due to ineffective lubrication.

A scoring system has been developed by House and Brackmann which grades the degree impairment based on such characteristics as facial muscle function and eye closure.^3,4 Approximately 96% of patients with a Bell’s palsy will improve to a House-Brackmann score of 2 or better within 1 year from diagnosis,⁵ and 85% of patients with Bell’s palsy will show at least some improvement within 3 weeks of onset (Table).² Although the classic description of Bell’s palsy notes the condition as idiopathic, there is an increasing body of evidence in the literature showing a link to herpes simplex virus 1.^5-7

Ramsey-Hunt Syndrome

The relationship between Bell’s palsy and Ramsey-Hunt syndrome is complex and controversial. Ramsey-Hunt syndrome is a constellation of possible complications from varicella-virus infection. Symptoms of Ramsey-Hunt syndrome include facial paralysis, tinnitus, hearing loss, vertigo, hyperacusis (increased sensitivity to certain frequencies and volume ranges of sound), and decreased ocular tearing.⁸ Due to the nature of symptoms associated with Ramsey-Hunt syndrome, it is apparent that the condition involves more than the seventh cranial nerve. In fact, studies have shown that Ramsey-Hunt syndrome can affect the fifth, sixth, eighth, and ninth cranial nerves.⁸

Ramsey-Hunt syndrome, which can present in the absence of cutaneous rash (referred to as zoster sine herpete), is estimated to occur in 8% to 20% of unilateral facial nerve palsies in adult patients.^8,9 Regardless of the etiology of Bell’s palsy, a review of the literature makes it clear that facial nerve paralysis is not synonymous with Bell’s palsy.¹⁰ In one example, Yetter et al¹⁰ describe the case of a patient who, though initially diagnosed with Bell’s palsy, ultimately was found to have a facial palsy due to a parotid gland malignancy.

Likewise, Stomeo¹¹ describes a case of a patient with facial paralysis and profound ipsilateral hearing loss who ultimately was found to have a mucoepithelial carcinoma of the parotid gland. In their report, the authors note that approximately 80% of facial nerve paralysis is due to Bell’s palsy, while 5% is due to malignancy.

In another report, Clemis¹² describes a case in which a patient who initially was diagnosed with Bell’s palsy eventually was found to have an adenoid cystic carcinoma of the parotid. Thus, the authors appropriately emphasize in their report that “all that palsies is not Bell’s.”

Differential Diagnosis

Historical factors, including timing and duration of symptom onset, help to distinguish a Bell’s palsy from other disorders that can mimic this condition. In their study, Brach VanSwewaringen¹³ highlight the fact that “not all facial paralysis is Bell’s palsy.” In their review, the authors describe clues to help distinguish conditions that mimic Bell’s palsy. For example, maximal weakness from Bell’s Palsy typically occurs within 3 to 7 days from symptom onset, and that a more gradual onset of symptoms, with slow or negligible improvement over 6 to 12 months, is more indicative of a space-occupying lesion than Bell’s palsy.¹³It is, however, important to note that although the patient in our case had a central lesion, she experienced an acute onset of symptoms.

The presence of additional symptoms may also suggest an alternative diagnosis. Brach and VanSwearingen¹³ further noted that symptoms associated with the eighth nerve, such as vertigo, tinnitus, and hearing loss may be found in patients with a CPA tumor. In patients with larger tumors, ninth and 10th nerve symptoms, including the impaired hearing noted in our patient, may be present. Some patients with ninth and 10th nerve symptoms may perceive a sense of facial numbness, but actual sensory changes in the facial nerve distribution are unlikely in Bell’s palsy. Gustatory changes, however, are consistent with Bell’s palsy.

Ear pain is consistent with Bell’s palsy and is a signal to be vigilant for the possible emergence of an ear rash, which would suggest the diagnosis of herpes zoster oticus along the trajectory of Ramsey-Hunt syndrome. Facial pain in the area of the facial nerve is inconsistent with Bell’s palsy, while hyperacusis is consistent with Bell’s palsy. Hearing loss is an eighth nerve symptom that is inconsistent with Bell’s palsy.

Similarly, there are physical examination findings that can help distinguish a true Bell’s palsy from a mimic. Changes in tear production are consistent with Bell’s palsy, but imbalance and disequilibrium are not.¹⁴

As previously noted, the patient in this case had difficulty walking and felt as if she was falling toward her right side.

One way to organize the causes of facial paralysis has been proposed by Adour et al.¹⁵ In this system, etiologies are listed as either acute paralysis or chronic, progressive paralysis. Acute paralysis (ie, the sudden onset of symptoms with maximal severity within 2 weeks), of which Bell’s palsy is the most common, can be seen in cases of polyneuritis.

A new case of Bell’s palsy has been estimated to occur in the United States every 10 minutes.⁸ Guillain-Barré syndrome and Lyme disease are also in this category, as is Ramsey-Hunt syndrome. Patients with Lyme disease may have a history of a tick bite or rash.¹⁴

Trauma can also cause acute facial nerve paralysis (eg, blunt trauma-associated facial fracture, penetrating trauma, birth trauma). Unilateral central facial weakness can have a neurological cause, such as a lesion to the contralateral cortex, subcortical white matter, or internal capsule.^2,15 Otitis media can sometimes cause facial paralysis.¹⁶ A cholesteatoma can cause acute facial paralysis.² Malignancies cause 5% of all cases of facial paralysis. Primary parotid tumors of various types are in this category. Metastatic disease from breast, lung, skin, colon, and kidney may cause facial paralysis. As our case illustrates, CPA tumors can cause facial paralysis.¹⁵ It is important to also note that a patient can have both a Bell’s palsy and a concurrent disease. There are a number of case reports in the literature that describe acute onset of facial paralysis as a presenting symptom of malignancy.¹⁷ In addition, there are cases wherein a neurological finding on imaging, such as an acoustic neuroma, was presumed to be the cause of facial paralysis, yet the patient’s symptoms resolved in a manner consistent with Bell’s palsy.¹⁸

For example, Lagman et al¹⁹ described a patient in which a CPA lipoma was presumed to be the cause of the facial paralysis, but the eventual outcome showed the lipoma to have been an incidentaloma.

Conclusion

This case demonstrates a presenting symptom of facial palsy and the presence of a CPA tumor. The presence of vertigo along with other historical and physical examination findings inconsistent with Bell’s palsy prompted the CT scan of the head. A review of the literature suggests a number of important findings in patients with facial palsy to assist the clinician in distinguishing true Bell’s palsy from other diseases that can mimic this condition. This case serves as a reminder of the need to perform a thorough and diligent workup to determine the presence or absence of other neurologic findings prior to closing on the diagnosis of Bell’s palsy.

Facial paralysis is a common medical complaint—one that has fascinated ancient and contemporary physicians alike.¹ An idiopathic facial nerve paresis involving the lower motor neuron was described in 1821 by Sir Charles Bell. This entity became known as a Bell’s palsy, the hallmark of which was weakness or complete paralysis of the muscles of one side of the face, with no sparing of the muscles of the forehead. However, not all facial paralysis is due to Bell’s palsy.

We present a case of a patient with a Bell’s palsy mimic to facilitate and guide the differential diagnosis and distinguish conditions from the classical presentation that Bell first described to the more concerning symptoms that may not be immediately obvious. Our case further underscores the importance of performing a thorough assessment to determine the presence of other neurological findings.

Case

A 61-year-old woman presented to the ED for evaluation of right facial droop and sensation of “room spinning.” The patient stated both symptoms began approximately 36 hours prior to presentation, upon awakening. She further noted that the right side of her face felt “funny” and numb.

The patient denied any headache, neck or chest pain, extremity numbness, or weakness, but stated that she felt like she was going to fall toward her right side whenever she attempted to walk. The patient’s medical history was significant for hypertension, for which she was taking losartan. Her surgical history was notable for a left oophorectomy secondary to an ovarian cyst. Regarding the social history, the patient admitted to smoking 90 packs of cigarettes per year, but denied alcohol or illicit drug use.

Upon arrival at the ED, the patient’s vital signs were: blood pressure, 164/86 mm Hg: pulse, 89 beats/min; respiratory rate, 18 breaths/min; and temperature, 98.6°F. Oxygen saturation was 98% on room air.

Physical examination revealed the patient had a right facial droop consistent with right facial palsy. She was unable to wrinkle her right forehead or fully close her right eye. There were no field cuts on confrontation. The patient’s speech was noticeable for a mild dysarthria. The motor examination revealed mild weakness of the left upper extremity and impaired right facial sensation. There were no rashes noted on the face, head, or ears. The patient had slightly impaired hearing in the right ear, which was new in onset. The remainder of the physical examination was unremarkable.

Although the patient exhibited the classic signs of Bell’s palsy, including complete paralysis of the muscles of one side of the face, inability to wrinkle the muscle of the right forehead, and inability to fully close the right eye, she also had concerning symptoms of vertigo, dysarthria, and contralateral upper extremity weakness.

A computed tomography (CT) scan of the head was ordered, which revealed a large mass lesion centered in the right petrous apex, with an associated large component extending medially into the right cerebellopontine angle (CPA) that caused a mass effect on the adjacent brainstem (Figures 1a and 1b).

Upon these findings, the patient was transferred to another facility for neurosurgical evaluation. Magnetic resonance imaging (MRI) studies performed at the receiving hospital demonstrated a large expansile heterogeneous mass lesion centered in the right petrous apex with an associated large, probable hemorrhagic soft-tissue component extending medially into the right CPA, causing a mass effect on the adjacent brainstem and mild obstructive hydrocephalus (Figures 2a and 2b).

The patient was given dexamethasone 10 mg intravenously and taken to the operating room for a right suboccipital craniotomy with subtotal tumor removal. Intraoperative high-voltage stimulation of the fifth to eighth cranial nerves showed no response, indicating significant impairment.

While there were no intraoperative complications, the patient had significant postoperative dysphagia and resultant aspiration. A tracheostomy and percutaneous endoscopic gastrostomy tube were subsequently placed. Results of a biopsy taken during surgery identified an atypical meningioma. The patient remained in the hospital for 4 weeks, after which she was discharged to a long-term care (LTC) and rehabilitation facility.

A repeat CT scan taken 2 months after surgery demonstrated absence of the previously identified large mass (Figure 1b). Three months after discharge from the LTC-rehabilitation facility, MRI of the brain showed continued interval improvement of the previously noted mass centered in the right petrous apex (Figures 3a and 3b).

 

Discussion

Accounts of facial paralysis and facial nerve disorders have been noted throughout history and include accounts of the condition by Hippocrates.¹ Bell’s palsy was named after surgeon Sir Charles Bell, who described a peripheral-nerve paralysis of the facial nerve in 1821. Bell’s work helped to elucidate the anatomy and functional role of the facial nerve.^1,2

Signs and Symptoms

The classic presentation of Bell’s palsy is weakness or complete paralysis of the muscles of one side of the face, with no sparing of the muscles of the forehead. The eyelid on the affected side generally does not close, which can result in ocular irritation due to ineffective lubrication.

A scoring system has been developed by House and Brackmann which grades the degree impairment based on such characteristics as facial muscle function and eye closure.^3,4 Approximately 96% of patients with a Bell’s palsy will improve to a House-Brackmann score of 2 or better within 1 year from diagnosis,⁵ and 85% of patients with Bell’s palsy will show at least some improvement within 3 weeks of onset (Table).² Although the classic description of Bell’s palsy notes the condition as idiopathic, there is an increasing body of evidence in the literature showing a link to herpes simplex virus 1.^5-7

Ramsey-Hunt Syndrome

The relationship between Bell’s palsy and Ramsey-Hunt syndrome is complex and controversial. Ramsey-Hunt syndrome is a constellation of possible complications from varicella-virus infection. Symptoms of Ramsey-Hunt syndrome include facial paralysis, tinnitus, hearing loss, vertigo, hyperacusis (increased sensitivity to certain frequencies and volume ranges of sound), and decreased ocular tearing.⁸ Due to the nature of symptoms associated with Ramsey-Hunt syndrome, it is apparent that the condition involves more than the seventh cranial nerve. In fact, studies have shown that Ramsey-Hunt syndrome can affect the fifth, sixth, eighth, and ninth cranial nerves.⁸

Ramsey-Hunt syndrome, which can present in the absence of cutaneous rash (referred to as zoster sine herpete), is estimated to occur in 8% to 20% of unilateral facial nerve palsies in adult patients.^8,9 Regardless of the etiology of Bell’s palsy, a review of the literature makes it clear that facial nerve paralysis is not synonymous with Bell’s palsy.¹⁰ In one example, Yetter et al¹⁰ describe the case of a patient who, though initially diagnosed with Bell’s palsy, ultimately was found to have a facial palsy due to a parotid gland malignancy.

Likewise, Stomeo¹¹ describes a case of a patient with facial paralysis and profound ipsilateral hearing loss who ultimately was found to have a mucoepithelial carcinoma of the parotid gland. In their report, the authors note that approximately 80% of facial nerve paralysis is due to Bell’s palsy, while 5% is due to malignancy.

In another report, Clemis¹² describes a case in which a patient who initially was diagnosed with Bell’s palsy eventually was found to have an adenoid cystic carcinoma of the parotid. Thus, the authors appropriately emphasize in their report that “all that palsies is not Bell’s.”

Differential Diagnosis

Historical factors, including timing and duration of symptom onset, help to distinguish a Bell’s palsy from other disorders that can mimic this condition. In their study, Brach VanSwewaringen¹³ highlight the fact that “not all facial paralysis is Bell’s palsy.” In their review, the authors describe clues to help distinguish conditions that mimic Bell’s palsy. For example, maximal weakness from Bell’s Palsy typically occurs within 3 to 7 days from symptom onset, and that a more gradual onset of symptoms, with slow or negligible improvement over 6 to 12 months, is more indicative of a space-occupying lesion than Bell’s palsy.¹³It is, however, important to note that although the patient in our case had a central lesion, she experienced an acute onset of symptoms.

The presence of additional symptoms may also suggest an alternative diagnosis. Brach and VanSwearingen¹³ further noted that symptoms associated with the eighth nerve, such as vertigo, tinnitus, and hearing loss may be found in patients with a CPA tumor. In patients with larger tumors, ninth and 10th nerve symptoms, including the impaired hearing noted in our patient, may be present. Some patients with ninth and 10th nerve symptoms may perceive a sense of facial numbness, but actual sensory changes in the facial nerve distribution are unlikely in Bell’s palsy. Gustatory changes, however, are consistent with Bell’s palsy.

Ear pain is consistent with Bell’s palsy and is a signal to be vigilant for the possible emergence of an ear rash, which would suggest the diagnosis of herpes zoster oticus along the trajectory of Ramsey-Hunt syndrome. Facial pain in the area of the facial nerve is inconsistent with Bell’s palsy, while hyperacusis is consistent with Bell’s palsy. Hearing loss is an eighth nerve symptom that is inconsistent with Bell’s palsy.

Similarly, there are physical examination findings that can help distinguish a true Bell’s palsy from a mimic. Changes in tear production are consistent with Bell’s palsy, but imbalance and disequilibrium are not.¹⁴

As previously noted, the patient in this case had difficulty walking and felt as if she was falling toward her right side.

One way to organize the causes of facial paralysis has been proposed by Adour et al.¹⁵ In this system, etiologies are listed as either acute paralysis or chronic, progressive paralysis. Acute paralysis (ie, the sudden onset of symptoms with maximal severity within 2 weeks), of which Bell’s palsy is the most common, can be seen in cases of polyneuritis.

A new case of Bell’s palsy has been estimated to occur in the United States every 10 minutes.⁸ Guillain-Barré syndrome and Lyme disease are also in this category, as is Ramsey-Hunt syndrome. Patients with Lyme disease may have a history of a tick bite or rash.¹⁴

Trauma can also cause acute facial nerve paralysis (eg, blunt trauma-associated facial fracture, penetrating trauma, birth trauma). Unilateral central facial weakness can have a neurological cause, such as a lesion to the contralateral cortex, subcortical white matter, or internal capsule.^2,15 Otitis media can sometimes cause facial paralysis.¹⁶ A cholesteatoma can cause acute facial paralysis.² Malignancies cause 5% of all cases of facial paralysis. Primary parotid tumors of various types are in this category. Metastatic disease from breast, lung, skin, colon, and kidney may cause facial paralysis. As our case illustrates, CPA tumors can cause facial paralysis.¹⁵ It is important to also note that a patient can have both a Bell’s palsy and a concurrent disease. There are a number of case reports in the literature that describe acute onset of facial paralysis as a presenting symptom of malignancy.¹⁷ In addition, there are cases wherein a neurological finding on imaging, such as an acoustic neuroma, was presumed to be the cause of facial paralysis, yet the patient’s symptoms resolved in a manner consistent with Bell’s palsy.¹⁸

For example, Lagman et al¹⁹ described a patient in which a CPA lipoma was presumed to be the cause of the facial paralysis, but the eventual outcome showed the lipoma to have been an incidentaloma.

Conclusion

This case demonstrates a presenting symptom of facial palsy and the presence of a CPA tumor. The presence of vertigo along with other historical and physical examination findings inconsistent with Bell’s palsy prompted the CT scan of the head. A review of the literature suggests a number of important findings in patients with facial palsy to assist the clinician in distinguishing true Bell’s palsy from other diseases that can mimic this condition. This case serves as a reminder of the need to perform a thorough and diligent workup to determine the presence or absence of other neurologic findings prior to closing on the diagnosis of Bell’s palsy.

Facial paralysis is a common medical complaint—one that has fascinated ancient and contemporary physicians alike.¹ An idiopathic facial nerve paresis involving the lower motor neuron was described in 1821 by Sir Charles Bell. This entity became known as a Bell’s palsy, the hallmark of which was weakness or complete paralysis of the muscles of one side of the face, with no sparing of the muscles of the forehead. However, not all facial paralysis is due to Bell’s palsy.

We present a case of a patient with a Bell’s palsy mimic to facilitate and guide the differential diagnosis and distinguish conditions from the classical presentation that Bell first described to the more concerning symptoms that may not be immediately obvious. Our case further underscores the importance of performing a thorough assessment to determine the presence of other neurological findings.

Case

A 61-year-old woman presented to the ED for evaluation of right facial droop and sensation of “room spinning.” The patient stated both symptoms began approximately 36 hours prior to presentation, upon awakening. She further noted that the right side of her face felt “funny” and numb.

The patient denied any headache, neck or chest pain, extremity numbness, or weakness, but stated that she felt like she was going to fall toward her right side whenever she attempted to walk. The patient’s medical history was significant for hypertension, for which she was taking losartan. Her surgical history was notable for a left oophorectomy secondary to an ovarian cyst. Regarding the social history, the patient admitted to smoking 90 packs of cigarettes per year, but denied alcohol or illicit drug use.

Upon arrival at the ED, the patient’s vital signs were: blood pressure, 164/86 mm Hg: pulse, 89 beats/min; respiratory rate, 18 breaths/min; and temperature, 98.6°F. Oxygen saturation was 98% on room air.

Physical examination revealed the patient had a right facial droop consistent with right facial palsy. She was unable to wrinkle her right forehead or fully close her right eye. There were no field cuts on confrontation. The patient’s speech was noticeable for a mild dysarthria. The motor examination revealed mild weakness of the left upper extremity and impaired right facial sensation. There were no rashes noted on the face, head, or ears. The patient had slightly impaired hearing in the right ear, which was new in onset. The remainder of the physical examination was unremarkable.

Although the patient exhibited the classic signs of Bell’s palsy, including complete paralysis of the muscles of one side of the face, inability to wrinkle the muscle of the right forehead, and inability to fully close the right eye, she also had concerning symptoms of vertigo, dysarthria, and contralateral upper extremity weakness.

A computed tomography (CT) scan of the head was ordered, which revealed a large mass lesion centered in the right petrous apex, with an associated large component extending medially into the right cerebellopontine angle (CPA) that caused a mass effect on the adjacent brainstem (Figures 1a and 1b).

Upon these findings, the patient was transferred to another facility for neurosurgical evaluation. Magnetic resonance imaging (MRI) studies performed at the receiving hospital demonstrated a large expansile heterogeneous mass lesion centered in the right petrous apex with an associated large, probable hemorrhagic soft-tissue component extending medially into the right CPA, causing a mass effect on the adjacent brainstem and mild obstructive hydrocephalus (Figures 2a and 2b).

The patient was given dexamethasone 10 mg intravenously and taken to the operating room for a right suboccipital craniotomy with subtotal tumor removal. Intraoperative high-voltage stimulation of the fifth to eighth cranial nerves showed no response, indicating significant impairment.

While there were no intraoperative complications, the patient had significant postoperative dysphagia and resultant aspiration. A tracheostomy and percutaneous endoscopic gastrostomy tube were subsequently placed. Results of a biopsy taken during surgery identified an atypical meningioma. The patient remained in the hospital for 4 weeks, after which she was discharged to a long-term care (LTC) and rehabilitation facility.

A repeat CT scan taken 2 months after surgery demonstrated absence of the previously identified large mass (Figure 1b). Three months after discharge from the LTC-rehabilitation facility, MRI of the brain showed continued interval improvement of the previously noted mass centered in the right petrous apex (Figures 3a and 3b).

 

Discussion

Accounts of facial paralysis and facial nerve disorders have been noted throughout history and include accounts of the condition by Hippocrates.¹ Bell’s palsy was named after surgeon Sir Charles Bell, who described a peripheral-nerve paralysis of the facial nerve in 1821. Bell’s work helped to elucidate the anatomy and functional role of the facial nerve.^1,2

Signs and Symptoms

The classic presentation of Bell’s palsy is weakness or complete paralysis of the muscles of one side of the face, with no sparing of the muscles of the forehead. The eyelid on the affected side generally does not close, which can result in ocular irritation due to ineffective lubrication.

A scoring system has been developed by House and Brackmann which grades the degree impairment based on such characteristics as facial muscle function and eye closure.^3,4 Approximately 96% of patients with a Bell’s palsy will improve to a House-Brackmann score of 2 or better within 1 year from diagnosis,⁵ and 85% of patients with Bell’s palsy will show at least some improvement within 3 weeks of onset (Table).² Although the classic description of Bell’s palsy notes the condition as idiopathic, there is an increasing body of evidence in the literature showing a link to herpes simplex virus 1.^5-7

Ramsey-Hunt Syndrome

The relationship between Bell’s palsy and Ramsey-Hunt syndrome is complex and controversial. Ramsey-Hunt syndrome is a constellation of possible complications from varicella-virus infection. Symptoms of Ramsey-Hunt syndrome include facial paralysis, tinnitus, hearing loss, vertigo, hyperacusis (increased sensitivity to certain frequencies and volume ranges of sound), and decreased ocular tearing.⁸ Due to the nature of symptoms associated with Ramsey-Hunt syndrome, it is apparent that the condition involves more than the seventh cranial nerve. In fact, studies have shown that Ramsey-Hunt syndrome can affect the fifth, sixth, eighth, and ninth cranial nerves.⁸

Ramsey-Hunt syndrome, which can present in the absence of cutaneous rash (referred to as zoster sine herpete), is estimated to occur in 8% to 20% of unilateral facial nerve palsies in adult patients.^8,9 Regardless of the etiology of Bell’s palsy, a review of the literature makes it clear that facial nerve paralysis is not synonymous with Bell’s palsy.¹⁰ In one example, Yetter et al¹⁰ describe the case of a patient who, though initially diagnosed with Bell’s palsy, ultimately was found to have a facial palsy due to a parotid gland malignancy.

Likewise, Stomeo¹¹ describes a case of a patient with facial paralysis and profound ipsilateral hearing loss who ultimately was found to have a mucoepithelial carcinoma of the parotid gland. In their report, the authors note that approximately 80% of facial nerve paralysis is due to Bell’s palsy, while 5% is due to malignancy.

In another report, Clemis¹² describes a case in which a patient who initially was diagnosed with Bell’s palsy eventually was found to have an adenoid cystic carcinoma of the parotid. Thus, the authors appropriately emphasize in their report that “all that palsies is not Bell’s.”

Differential Diagnosis

Historical factors, including timing and duration of symptom onset, help to distinguish a Bell’s palsy from other disorders that can mimic this condition. In their study, Brach VanSwewaringen¹³ highlight the fact that “not all facial paralysis is Bell’s palsy.” In their review, the authors describe clues to help distinguish conditions that mimic Bell’s palsy. For example, maximal weakness from Bell’s Palsy typically occurs within 3 to 7 days from symptom onset, and that a more gradual onset of symptoms, with slow or negligible improvement over 6 to 12 months, is more indicative of a space-occupying lesion than Bell’s palsy.¹³It is, however, important to note that although the patient in our case had a central lesion, she experienced an acute onset of symptoms.

The presence of additional symptoms may also suggest an alternative diagnosis. Brach and VanSwearingen¹³ further noted that symptoms associated with the eighth nerve, such as vertigo, tinnitus, and hearing loss may be found in patients with a CPA tumor. In patients with larger tumors, ninth and 10th nerve symptoms, including the impaired hearing noted in our patient, may be present. Some patients with ninth and 10th nerve symptoms may perceive a sense of facial numbness, but actual sensory changes in the facial nerve distribution are unlikely in Bell’s palsy. Gustatory changes, however, are consistent with Bell’s palsy.

Ear pain is consistent with Bell’s palsy and is a signal to be vigilant for the possible emergence of an ear rash, which would suggest the diagnosis of herpes zoster oticus along the trajectory of Ramsey-Hunt syndrome. Facial pain in the area of the facial nerve is inconsistent with Bell’s palsy, while hyperacusis is consistent with Bell’s palsy. Hearing loss is an eighth nerve symptom that is inconsistent with Bell’s palsy.

Similarly, there are physical examination findings that can help distinguish a true Bell’s palsy from a mimic. Changes in tear production are consistent with Bell’s palsy, but imbalance and disequilibrium are not.¹⁴

As previously noted, the patient in this case had difficulty walking and felt as if she was falling toward her right side.

One way to organize the causes of facial paralysis has been proposed by Adour et al.¹⁵ In this system, etiologies are listed as either acute paralysis or chronic, progressive paralysis. Acute paralysis (ie, the sudden onset of symptoms with maximal severity within 2 weeks), of which Bell’s palsy is the most common, can be seen in cases of polyneuritis.

A new case of Bell’s palsy has been estimated to occur in the United States every 10 minutes.⁸ Guillain-Barré syndrome and Lyme disease are also in this category, as is Ramsey-Hunt syndrome. Patients with Lyme disease may have a history of a tick bite or rash.¹⁴

Trauma can also cause acute facial nerve paralysis (eg, blunt trauma-associated facial fracture, penetrating trauma, birth trauma). Unilateral central facial weakness can have a neurological cause, such as a lesion to the contralateral cortex, subcortical white matter, or internal capsule.^2,15 Otitis media can sometimes cause facial paralysis.¹⁶ A cholesteatoma can cause acute facial paralysis.² Malignancies cause 5% of all cases of facial paralysis. Primary parotid tumors of various types are in this category. Metastatic disease from breast, lung, skin, colon, and kidney may cause facial paralysis. As our case illustrates, CPA tumors can cause facial paralysis.¹⁵ It is important to also note that a patient can have both a Bell’s palsy and a concurrent disease. There are a number of case reports in the literature that describe acute onset of facial paralysis as a presenting symptom of malignancy.¹⁷ In addition, there are cases wherein a neurological finding on imaging, such as an acoustic neuroma, was presumed to be the cause of facial paralysis, yet the patient’s symptoms resolved in a manner consistent with Bell’s palsy.¹⁸

For example, Lagman et al¹⁹ described a patient in which a CPA lipoma was presumed to be the cause of the facial paralysis, but the eventual outcome showed the lipoma to have been an incidentaloma.

Conclusion

This case demonstrates a presenting symptom of facial palsy and the presence of a CPA tumor. The presence of vertigo along with other historical and physical examination findings inconsistent with Bell’s palsy prompted the CT scan of the head. A review of the literature suggests a number of important findings in patients with facial palsy to assist the clinician in distinguishing true Bell’s palsy from other diseases that can mimic this condition. This case serves as a reminder of the need to perform a thorough and diligent workup to determine the presence or absence of other neurologic findings prior to closing on the diagnosis of Bell’s palsy.

ABSTRACT

Soft tissue defects associated with exposed tendon pose difficult reconstructive problems because of tendon adhesions, poor range of motion, poor cosmetic appearance, and donor site morbidity. Dermal regeneration template is a skin substitute widely used in reconstructive surgery, including the occasional coverage of tendons. However, postoperative functionality of the tendons has not been well documented. We report a case of using dermal regeneration template for soft tissue reconstruction overlying tendons with loss of paratenon in a patient with Dupuytren’s contracture. Dermal regeneration template may offer an alternative option for immediate tendon coverage in the hand.

Soft tissue defects overlying exposed tendon with loss of paratenon often precipitate poor clinical outcomes because of the dichotomous demands of both closing the overlying soft-tissue defect and providing a gliding surface for the underlying tendons.¹ Although avoidance of adhesions and restoration of function are the primary goals of the procedure, satisfactory appearance is also desirable. Likewise, any form of coverage should ideally provide good vasculature required for complete healing and an early form of closure following débridement.² Simple skin grafts do not adequately meet these demands because they result in a high rate of tendon adhesions,³ and also are limited in patients with limited donor skin availability or questionable underlying wound bed viability, such as in scleroderma.

In order to reduce the frequency of tendon adhesions by creating a gliding surface, the use of interpositional materials, both artificial and biologic, has been employed with varying degrees of success, including cellophane, chitosan membrane, fibrin sealant, autogenous fascial flaps, and autogenous venous grafts.^4-7 Many of the autogenous flaps and grafts have been employed with good success.⁸ However, complications and donor site morbidity encourage alternative procedures, including the use of artificial substances.^2,8-10

We present our clinical experience with a patient who underwent successful placement of Integra (Integra LifeSciences) Dermal Regeneration Template (DRT) directly over exposed tendons with a subsequent full-thickness skin graft several weeks later. The procedures were performed per the manufacturer’s specifications, resulting in 2 stages of reconstruction. In our experience, DRT can offer immediate coverage unrestricted by wound size, and provides shorter operative time and decreased donor site and surgical morbidity compared with flap coverage, while demonstrating good cosmetic results. The patient provided written informed consent for print and electronic publication of this case report.

CASE

A 74-year-old right-handed man with Dupuytren’s contracture was evaluated for recurrent symptomatic contracture causing difficulty with daily activities. He reported palpable cords and contractures in the ring and small fingers of the right hand. He had 2 prior open surgical procedures, including palmar and digital fasciectomy of both hands. On the right hand, the ring and small fingers demonstrated 90° proximal interphalangeal (PIP) and 60° metacarpophalangeal (MCP) flexion contractures. Palpable central cords were present on the flexor surfaces of both the ring and small fingers. A well-healed surgical incision, performed 22 years earlier, was present over the palmar aspect of the ring finger.

Continue to: With consideration given...

 

With consideration given to the patient’s recurrent contracture after a prior surgical procedure, we discussed surgical excision of the diseased cords in order to eliminate the possibility of a second recurrence and maximize the gain of motion. Following discussion with the patient, we performed palmar and digital fasciectomy of the ring and small finger contractures. Postoperatively, the patient was followed closely for wound complications and vascular status. On his return to our clinic 11 days later, the patient was noted to have dehiscence of the digital wounds in the ring and small fingers (Figure 1). 

The flexor tendons of both digits were exposed with loss of paratenon, with defect sizes of 1 cm × 1.5 cm on the small finger and 1.5 cm × 2 cm on the ring finger.

STAGE 1

During the first stage, completed 14 days following the index procedure, débridement of the wounds was performed, followed by provisional DRT coverage of the tendons, secured with 5-0 nylon sutures (Figure 2). 

Next, nonadherent ADAPTIC dressings (Acelity) were placed over the DRT and affixed to the skin using gauze fluffs. On postoperative day 1, the first dressing change was performed, after which a weekly wound inspection was performed to evaluate for infection and to note the degree of vascularization of the neodermis beneath the silicone layer (Figure 3).

STAGE 2

At approximately 2 weeks after application of the DRT, a full-thickness skin graft was applied. The thickness of the graft was chosen to allow for durable coverage of the palmar skin defects. Upon successful completion of the second stage, the patient was followed and evaluated for complete wound healing. On performing an examination 14 days after surgery, the ring and small fingers demonstrated only partially healed skin graft but significantly improved range of motion (ROM), with 40° to 90° arc of motion in the PIP joint and 25° to 90° arc of motion in the MCP joint (Figure 4). Owing to their limited size, the wounds were treated with dressing changes until successful healing (Figure 5).

 

Hand therapy was instituted to achieve maximum mobility for covered soft tissue and tendons and to maximize tendon gliding. At 1-year follow-up, the skin was fully healed and the patient’s active PIP motion was 30° to 90°, active MCP motion was 0° to 90°, and grip strength was 90 lb on both sides. The tendons glided under a well-vascularized tissue at the DRT placement site, and no secondary tenolysis procedure was deemed necessary.

DISCUSSION

Soft tissue defects with exposed tendons may offer a number of challenges for coverage. The primary concern is the creation of a gliding surface and the restoration of a functional tendon without adhesions.² However, surgeons must use their own clinical judgment when choosing the method of coverage so as to minimize the effects of donor site morbidity and maximize the overall functional and cosmetic outcomes. All options must be considered while selecting a material or flap that is likely to survive in the relatively avascular tendon plane.^2,8,11 When considering the reconstructive ladder, skin grafts may not represent a viable option in the presence of a nonvascularized wound bed, such as exposed tendon or bone, where paratenon or periosteum have been damaged. That leaves the surgeon with local flaps, regional flaps, free flaps, and skin substitutes.

Continue to : Before planning closure...

 

Before planning closure, wound conditions should be optimized, including wound bed quality, vascularization, and bacterial loads. Experimental data suggest that the bacterial load should be brought down below a critical level of 10⁵ bacteria per g of tissue to allow a skin graft to take. This may be problematic from a practical standpoint because quantitative bacterial cultures take about 48 hours to obtain the result, long after a decision to graft is made. As a result, the surgeon may take an aggressive approach to wound débridement, making sure that all necrotic material has been sharply débrided prior to coverage.

As Levin¹² noted in 1993, decisions regarding repair of any soft tissue defect may follow a well-delineated ladder beginning with the primary choice of split-thickness skin grafts and ending with free flaps. When treating tissue defects in the hand complex, flaps are an excellent option as they replace like with like, allow minimal scarring and early rehabilitation. ^13,14 Nevertheless, a few general disadvantages are inherent in flap procedure: increase in operating time, risk of flap loss, and in case of free flaps, knowledge, experience, and microsurgical ability.² In reference to complications, the rate of flap loss found by Khouri and colleagues¹⁵ was 4.1% with a 12.1% chance of incurring some measured complication, including wound dehiscence, arterial insufficiency, and flap necrosis.

Likewise, some of the conventional local and free flaps, including cutaneous and muscular flaps, prove ineffective in preventing tendon adhesions, create unsightly postoperative contours, or increase the area of trauma on the wounded hand, encouraging the use of free fascial flaps.¹¹ Among the wide array of potential free fascial flaps, the temporoparietal, scapular, lateral arm, radial forearm, and free serratus fascial flaps are some of the most popular for hand defects.^8,9 However, these procedures require an additional surgical site, meticulous dissection, microsurgical technique at times, and increased operating cost and time.^2,8-10 Furthermore, free fascial flaps have demonstrated occasional partial flap loss and a decreased survival of the overlying skin graft, leading some to advocate delayed skin graft placement.^10,16,17

On the basis of these complications, Bray and colleagues¹¹ noted that the utility of free flaps may be limited in smaller clinical settings. The primary disadvantage of using DRTs is the necessity for a second operative procedure to harvest and place the skin graft. Traditionally, this is performed 2 to 3 weeks after the initial DRT application. Nevertheless, a 1-stage procedure can be performed in an outpatient setup, minimizing the burden to the patient and the medical costs, followed by secondary intention healing.

In response to critics of the 2-stage technique, Sanger and colleagues¹⁸ described single-stage use of DRT with split-thickness skin grafts with placement of an overlying wound vacuum-assisted closure to help speed incorporation of the DRT and improve survival of the immediately grafted skin. Another viable alternative is the McCash open-palm technique.¹⁹ In the open-palm technique, a Brunner zigzag incision is made in the affected digit. A transverse incision is made in the palm. A partial fasciectomy is performed in the palm and digit. After release, the digital incision is closed, and the palmar incision is left open. Although this well-studied and well-reported technique is known to reduce the risk of flap necrosis due to tension and hematoma,²⁰ its main application is in the palm, as the name implies. Because in our patient the defect was palmar-digital with exposed “white structures,” we elected to use DRT.

Continue to: Although there is still...

 

Although there is still no perfect answer for wound coverage and closure in the hand with exposed or damaged tendons, DRT certainly performs well as a primary choice by minimizing adhesions; allowing a good ROM; and providing a durable, satisfactory cosmetic outcome. Likewise, an initial treatment with DRT does not preclude later, more elaborate reconstructive efforts, such as local or free flaps, if they continue to be indicated. DRT also does not diminish the ability to revise a tendon reconstruction if a secondary procedure is necessary. In our patient, tendon revision has not been necessary. DRT gives the surgeon a minimally invasive, efficient initial alternative to more labor-intensive, potentially morbid reconstructive procedures, without sacrificing outcome. Therefore, DRT can offer an alternative procedure in the surgeon’s armamentarium for tendon coverage in complex hand defects.

ABSTRACT

Soft tissue defects associated with exposed tendon pose difficult reconstructive problems because of tendon adhesions, poor range of motion, poor cosmetic appearance, and donor site morbidity. Dermal regeneration template is a skin substitute widely used in reconstructive surgery, including the occasional coverage of tendons. However, postoperative functionality of the tendons has not been well documented. We report a case of using dermal regeneration template for soft tissue reconstruction overlying tendons with loss of paratenon in a patient with Dupuytren’s contracture. Dermal regeneration template may offer an alternative option for immediate tendon coverage in the hand.

Soft tissue defects overlying exposed tendon with loss of paratenon often precipitate poor clinical outcomes because of the dichotomous demands of both closing the overlying soft-tissue defect and providing a gliding surface for the underlying tendons.¹ Although avoidance of adhesions and restoration of function are the primary goals of the procedure, satisfactory appearance is also desirable. Likewise, any form of coverage should ideally provide good vasculature required for complete healing and an early form of closure following débridement.² Simple skin grafts do not adequately meet these demands because they result in a high rate of tendon adhesions,³ and also are limited in patients with limited donor skin availability or questionable underlying wound bed viability, such as in scleroderma.

In order to reduce the frequency of tendon adhesions by creating a gliding surface, the use of interpositional materials, both artificial and biologic, has been employed with varying degrees of success, including cellophane, chitosan membrane, fibrin sealant, autogenous fascial flaps, and autogenous venous grafts.^4-7 Many of the autogenous flaps and grafts have been employed with good success.⁸ However, complications and donor site morbidity encourage alternative procedures, including the use of artificial substances.^2,8-10

We present our clinical experience with a patient who underwent successful placement of Integra (Integra LifeSciences) Dermal Regeneration Template (DRT) directly over exposed tendons with a subsequent full-thickness skin graft several weeks later. The procedures were performed per the manufacturer’s specifications, resulting in 2 stages of reconstruction. In our experience, DRT can offer immediate coverage unrestricted by wound size, and provides shorter operative time and decreased donor site and surgical morbidity compared with flap coverage, while demonstrating good cosmetic results. The patient provided written informed consent for print and electronic publication of this case report.

CASE

A 74-year-old right-handed man with Dupuytren’s contracture was evaluated for recurrent symptomatic contracture causing difficulty with daily activities. He reported palpable cords and contractures in the ring and small fingers of the right hand. He had 2 prior open surgical procedures, including palmar and digital fasciectomy of both hands. On the right hand, the ring and small fingers demonstrated 90° proximal interphalangeal (PIP) and 60° metacarpophalangeal (MCP) flexion contractures. Palpable central cords were present on the flexor surfaces of both the ring and small fingers. A well-healed surgical incision, performed 22 years earlier, was present over the palmar aspect of the ring finger.

Continue to: With consideration given...

 

With consideration given to the patient’s recurrent contracture after a prior surgical procedure, we discussed surgical excision of the diseased cords in order to eliminate the possibility of a second recurrence and maximize the gain of motion. Following discussion with the patient, we performed palmar and digital fasciectomy of the ring and small finger contractures. Postoperatively, the patient was followed closely for wound complications and vascular status. On his return to our clinic 11 days later, the patient was noted to have dehiscence of the digital wounds in the ring and small fingers (Figure 1). 

The flexor tendons of both digits were exposed with loss of paratenon, with defect sizes of 1 cm × 1.5 cm on the small finger and 1.5 cm × 2 cm on the ring finger.

STAGE 1

During the first stage, completed 14 days following the index procedure, débridement of the wounds was performed, followed by provisional DRT coverage of the tendons, secured with 5-0 nylon sutures (Figure 2). 

Next, nonadherent ADAPTIC dressings (Acelity) were placed over the DRT and affixed to the skin using gauze fluffs. On postoperative day 1, the first dressing change was performed, after which a weekly wound inspection was performed to evaluate for infection and to note the degree of vascularization of the neodermis beneath the silicone layer (Figure 3).

STAGE 2

At approximately 2 weeks after application of the DRT, a full-thickness skin graft was applied. The thickness of the graft was chosen to allow for durable coverage of the palmar skin defects. Upon successful completion of the second stage, the patient was followed and evaluated for complete wound healing. On performing an examination 14 days after surgery, the ring and small fingers demonstrated only partially healed skin graft but significantly improved range of motion (ROM), with 40° to 90° arc of motion in the PIP joint and 25° to 90° arc of motion in the MCP joint (Figure 4). Owing to their limited size, the wounds were treated with dressing changes until successful healing (Figure 5).

 

Hand therapy was instituted to achieve maximum mobility for covered soft tissue and tendons and to maximize tendon gliding. At 1-year follow-up, the skin was fully healed and the patient’s active PIP motion was 30° to 90°, active MCP motion was 0° to 90°, and grip strength was 90 lb on both sides. The tendons glided under a well-vascularized tissue at the DRT placement site, and no secondary tenolysis procedure was deemed necessary.

DISCUSSION

Soft tissue defects with exposed tendons may offer a number of challenges for coverage. The primary concern is the creation of a gliding surface and the restoration of a functional tendon without adhesions.² However, surgeons must use their own clinical judgment when choosing the method of coverage so as to minimize the effects of donor site morbidity and maximize the overall functional and cosmetic outcomes. All options must be considered while selecting a material or flap that is likely to survive in the relatively avascular tendon plane.^2,8,11 When considering the reconstructive ladder, skin grafts may not represent a viable option in the presence of a nonvascularized wound bed, such as exposed tendon or bone, where paratenon or periosteum have been damaged. That leaves the surgeon with local flaps, regional flaps, free flaps, and skin substitutes.

Continue to : Before planning closure...

 

Before planning closure, wound conditions should be optimized, including wound bed quality, vascularization, and bacterial loads. Experimental data suggest that the bacterial load should be brought down below a critical level of 10⁵ bacteria per g of tissue to allow a skin graft to take. This may be problematic from a practical standpoint because quantitative bacterial cultures take about 48 hours to obtain the result, long after a decision to graft is made. As a result, the surgeon may take an aggressive approach to wound débridement, making sure that all necrotic material has been sharply débrided prior to coverage.

As Levin¹² noted in 1993, decisions regarding repair of any soft tissue defect may follow a well-delineated ladder beginning with the primary choice of split-thickness skin grafts and ending with free flaps. When treating tissue defects in the hand complex, flaps are an excellent option as they replace like with like, allow minimal scarring and early rehabilitation. ^13,14 Nevertheless, a few general disadvantages are inherent in flap procedure: increase in operating time, risk of flap loss, and in case of free flaps, knowledge, experience, and microsurgical ability.² In reference to complications, the rate of flap loss found by Khouri and colleagues¹⁵ was 4.1% with a 12.1% chance of incurring some measured complication, including wound dehiscence, arterial insufficiency, and flap necrosis.

Likewise, some of the conventional local and free flaps, including cutaneous and muscular flaps, prove ineffective in preventing tendon adhesions, create unsightly postoperative contours, or increase the area of trauma on the wounded hand, encouraging the use of free fascial flaps.¹¹ Among the wide array of potential free fascial flaps, the temporoparietal, scapular, lateral arm, radial forearm, and free serratus fascial flaps are some of the most popular for hand defects.^8,9 However, these procedures require an additional surgical site, meticulous dissection, microsurgical technique at times, and increased operating cost and time.^2,8-10 Furthermore, free fascial flaps have demonstrated occasional partial flap loss and a decreased survival of the overlying skin graft, leading some to advocate delayed skin graft placement.^10,16,17

On the basis of these complications, Bray and colleagues¹¹ noted that the utility of free flaps may be limited in smaller clinical settings. The primary disadvantage of using DRTs is the necessity for a second operative procedure to harvest and place the skin graft. Traditionally, this is performed 2 to 3 weeks after the initial DRT application. Nevertheless, a 1-stage procedure can be performed in an outpatient setup, minimizing the burden to the patient and the medical costs, followed by secondary intention healing.

In response to critics of the 2-stage technique, Sanger and colleagues¹⁸ described single-stage use of DRT with split-thickness skin grafts with placement of an overlying wound vacuum-assisted closure to help speed incorporation of the DRT and improve survival of the immediately grafted skin. Another viable alternative is the McCash open-palm technique.¹⁹ In the open-palm technique, a Brunner zigzag incision is made in the affected digit. A transverse incision is made in the palm. A partial fasciectomy is performed in the palm and digit. After release, the digital incision is closed, and the palmar incision is left open. Although this well-studied and well-reported technique is known to reduce the risk of flap necrosis due to tension and hematoma,²⁰ its main application is in the palm, as the name implies. Because in our patient the defect was palmar-digital with exposed “white structures,” we elected to use DRT.

Continue to: Although there is still...

 

Although there is still no perfect answer for wound coverage and closure in the hand with exposed or damaged tendons, DRT certainly performs well as a primary choice by minimizing adhesions; allowing a good ROM; and providing a durable, satisfactory cosmetic outcome. Likewise, an initial treatment with DRT does not preclude later, more elaborate reconstructive efforts, such as local or free flaps, if they continue to be indicated. DRT also does not diminish the ability to revise a tendon reconstruction if a secondary procedure is necessary. In our patient, tendon revision has not been necessary. DRT gives the surgeon a minimally invasive, efficient initial alternative to more labor-intensive, potentially morbid reconstructive procedures, without sacrificing outcome. Therefore, DRT can offer an alternative procedure in the surgeon’s armamentarium for tendon coverage in complex hand defects.

ABSTRACT

Soft tissue defects associated with exposed tendon pose difficult reconstructive problems because of tendon adhesions, poor range of motion, poor cosmetic appearance, and donor site morbidity. Dermal regeneration template is a skin substitute widely used in reconstructive surgery, including the occasional coverage of tendons. However, postoperative functionality of the tendons has not been well documented. We report a case of using dermal regeneration template for soft tissue reconstruction overlying tendons with loss of paratenon in a patient with Dupuytren’s contracture. Dermal regeneration template may offer an alternative option for immediate tendon coverage in the hand.

Soft tissue defects overlying exposed tendon with loss of paratenon often precipitate poor clinical outcomes because of the dichotomous demands of both closing the overlying soft-tissue defect and providing a gliding surface for the underlying tendons.¹ Although avoidance of adhesions and restoration of function are the primary goals of the procedure, satisfactory appearance is also desirable. Likewise, any form of coverage should ideally provide good vasculature required for complete healing and an early form of closure following débridement.² Simple skin grafts do not adequately meet these demands because they result in a high rate of tendon adhesions,³ and also are limited in patients with limited donor skin availability or questionable underlying wound bed viability, such as in scleroderma.

In order to reduce the frequency of tendon adhesions by creating a gliding surface, the use of interpositional materials, both artificial and biologic, has been employed with varying degrees of success, including cellophane, chitosan membrane, fibrin sealant, autogenous fascial flaps, and autogenous venous grafts.^4-7 Many of the autogenous flaps and grafts have been employed with good success.⁸ However, complications and donor site morbidity encourage alternative procedures, including the use of artificial substances.^2,8-10

We present our clinical experience with a patient who underwent successful placement of Integra (Integra LifeSciences) Dermal Regeneration Template (DRT) directly over exposed tendons with a subsequent full-thickness skin graft several weeks later. The procedures were performed per the manufacturer’s specifications, resulting in 2 stages of reconstruction. In our experience, DRT can offer immediate coverage unrestricted by wound size, and provides shorter operative time and decreased donor site and surgical morbidity compared with flap coverage, while demonstrating good cosmetic results. The patient provided written informed consent for print and electronic publication of this case report.

CASE

A 74-year-old right-handed man with Dupuytren’s contracture was evaluated for recurrent symptomatic contracture causing difficulty with daily activities. He reported palpable cords and contractures in the ring and small fingers of the right hand. He had 2 prior open surgical procedures, including palmar and digital fasciectomy of both hands. On the right hand, the ring and small fingers demonstrated 90° proximal interphalangeal (PIP) and 60° metacarpophalangeal (MCP) flexion contractures. Palpable central cords were present on the flexor surfaces of both the ring and small fingers. A well-healed surgical incision, performed 22 years earlier, was present over the palmar aspect of the ring finger.

Continue to: With consideration given...

 

With consideration given to the patient’s recurrent contracture after a prior surgical procedure, we discussed surgical excision of the diseased cords in order to eliminate the possibility of a second recurrence and maximize the gain of motion. Following discussion with the patient, we performed palmar and digital fasciectomy of the ring and small finger contractures. Postoperatively, the patient was followed closely for wound complications and vascular status. On his return to our clinic 11 days later, the patient was noted to have dehiscence of the digital wounds in the ring and small fingers (Figure 1). 

The flexor tendons of both digits were exposed with loss of paratenon, with defect sizes of 1 cm × 1.5 cm on the small finger and 1.5 cm × 2 cm on the ring finger.

STAGE 1

During the first stage, completed 14 days following the index procedure, débridement of the wounds was performed, followed by provisional DRT coverage of the tendons, secured with 5-0 nylon sutures (Figure 2). 

Next, nonadherent ADAPTIC dressings (Acelity) were placed over the DRT and affixed to the skin using gauze fluffs. On postoperative day 1, the first dressing change was performed, after which a weekly wound inspection was performed to evaluate for infection and to note the degree of vascularization of the neodermis beneath the silicone layer (Figure 3).

STAGE 2

At approximately 2 weeks after application of the DRT, a full-thickness skin graft was applied. The thickness of the graft was chosen to allow for durable coverage of the palmar skin defects. Upon successful completion of the second stage, the patient was followed and evaluated for complete wound healing. On performing an examination 14 days after surgery, the ring and small fingers demonstrated only partially healed skin graft but significantly improved range of motion (ROM), with 40° to 90° arc of motion in the PIP joint and 25° to 90° arc of motion in the MCP joint (Figure 4). Owing to their limited size, the wounds were treated with dressing changes until successful healing (Figure 5).

 

Hand therapy was instituted to achieve maximum mobility for covered soft tissue and tendons and to maximize tendon gliding. At 1-year follow-up, the skin was fully healed and the patient’s active PIP motion was 30° to 90°, active MCP motion was 0° to 90°, and grip strength was 90 lb on both sides. The tendons glided under a well-vascularized tissue at the DRT placement site, and no secondary tenolysis procedure was deemed necessary.

DISCUSSION

Soft tissue defects with exposed tendons may offer a number of challenges for coverage. The primary concern is the creation of a gliding surface and the restoration of a functional tendon without adhesions.² However, surgeons must use their own clinical judgment when choosing the method of coverage so as to minimize the effects of donor site morbidity and maximize the overall functional and cosmetic outcomes. All options must be considered while selecting a material or flap that is likely to survive in the relatively avascular tendon plane.^2,8,11 When considering the reconstructive ladder, skin grafts may not represent a viable option in the presence of a nonvascularized wound bed, such as exposed tendon or bone, where paratenon or periosteum have been damaged. That leaves the surgeon with local flaps, regional flaps, free flaps, and skin substitutes.

Continue to : Before planning closure...

 

Before planning closure, wound conditions should be optimized, including wound bed quality, vascularization, and bacterial loads. Experimental data suggest that the bacterial load should be brought down below a critical level of 10⁵ bacteria per g of tissue to allow a skin graft to take. This may be problematic from a practical standpoint because quantitative bacterial cultures take about 48 hours to obtain the result, long after a decision to graft is made. As a result, the surgeon may take an aggressive approach to wound débridement, making sure that all necrotic material has been sharply débrided prior to coverage.

As Levin¹² noted in 1993, decisions regarding repair of any soft tissue defect may follow a well-delineated ladder beginning with the primary choice of split-thickness skin grafts and ending with free flaps. When treating tissue defects in the hand complex, flaps are an excellent option as they replace like with like, allow minimal scarring and early rehabilitation. ^13,14 Nevertheless, a few general disadvantages are inherent in flap procedure: increase in operating time, risk of flap loss, and in case of free flaps, knowledge, experience, and microsurgical ability.² In reference to complications, the rate of flap loss found by Khouri and colleagues¹⁵ was 4.1% with a 12.1% chance of incurring some measured complication, including wound dehiscence, arterial insufficiency, and flap necrosis.

Likewise, some of the conventional local and free flaps, including cutaneous and muscular flaps, prove ineffective in preventing tendon adhesions, create unsightly postoperative contours, or increase the area of trauma on the wounded hand, encouraging the use of free fascial flaps.¹¹ Among the wide array of potential free fascial flaps, the temporoparietal, scapular, lateral arm, radial forearm, and free serratus fascial flaps are some of the most popular for hand defects.^8,9 However, these procedures require an additional surgical site, meticulous dissection, microsurgical technique at times, and increased operating cost and time.^2,8-10 Furthermore, free fascial flaps have demonstrated occasional partial flap loss and a decreased survival of the overlying skin graft, leading some to advocate delayed skin graft placement.^10,16,17

On the basis of these complications, Bray and colleagues¹¹ noted that the utility of free flaps may be limited in smaller clinical settings. The primary disadvantage of using DRTs is the necessity for a second operative procedure to harvest and place the skin graft. Traditionally, this is performed 2 to 3 weeks after the initial DRT application. Nevertheless, a 1-stage procedure can be performed in an outpatient setup, minimizing the burden to the patient and the medical costs, followed by secondary intention healing.

In response to critics of the 2-stage technique, Sanger and colleagues¹⁸ described single-stage use of DRT with split-thickness skin grafts with placement of an overlying wound vacuum-assisted closure to help speed incorporation of the DRT and improve survival of the immediately grafted skin. Another viable alternative is the McCash open-palm technique.¹⁹ In the open-palm technique, a Brunner zigzag incision is made in the affected digit. A transverse incision is made in the palm. A partial fasciectomy is performed in the palm and digit. After release, the digital incision is closed, and the palmar incision is left open. Although this well-studied and well-reported technique is known to reduce the risk of flap necrosis due to tension and hematoma,²⁰ its main application is in the palm, as the name implies. Because in our patient the defect was palmar-digital with exposed “white structures,” we elected to use DRT.

Continue to: Although there is still...

 

Although there is still no perfect answer for wound coverage and closure in the hand with exposed or damaged tendons, DRT certainly performs well as a primary choice by minimizing adhesions; allowing a good ROM; and providing a durable, satisfactory cosmetic outcome. Likewise, an initial treatment with DRT does not preclude later, more elaborate reconstructive efforts, such as local or free flaps, if they continue to be indicated. DRT also does not diminish the ability to revise a tendon reconstruction if a secondary procedure is necessary. In our patient, tendon revision has not been necessary. DRT gives the surgeon a minimally invasive, efficient initial alternative to more labor-intensive, potentially morbid reconstructive procedures, without sacrificing outcome. Therefore, DRT can offer an alternative procedure in the surgeon’s armamentarium for tendon coverage in complex hand defects.

ABSTRACT

A central distal femoral physeal bone bridge in a boy aged 5 years and 7 months was resected with a fluoroscopically guided core reamer placed through a lateral parapatellar approach. At 3-year follow-up, the boy’s leg-length discrepancy was 3.0 cm (3.9 cm preoperatively), and the physeal bone bridge did not recur. The patient had full function and no pain or other patellofemoral complaints. This technique provided direct access to the physeal bone bridge, and complete resection was performed without injury to the adjacent physeal cartilage in the medial and lateral columns of the distal femur, which is expected to grow normally in the absence of the bridge.

A physeal bone bridge is an osseous connection that forms across a physis. It may cause partial premature physeal arrest. Angular deformity and limb-length discrepancy are the main complications caused by physeal bone bridges.^1-4 The indications for the treatment of physeal bridges are well documented.^1-5 Trauma and infection are common causes of distal femoral physeal bone bridges. Arkader and colleagues⁶ showed that among different types of physeal bridges, the Salter-Harris type is significantly associated with complications, among which growth arrest is the most common and occurs in 27.4% of all patients.

The treatment of distal femoral physeal bone bridges is technically difficult and provides variable results. Poor results are reported in 13% to 40% of patients.^7-10 Procedure failure has been attributed to incomplete resection with the persistent tethering and dislodgement of the graft.¹¹ Methods with improved efficacy for the removal of central physeal bridges will help prevent reformation after treatment. We have used a novel technique that allows the direct resection of a central physeal bone bridge in the distal femur through the use of a fluoroscopically guided core reamer. This technique enables the complete removal of the bone bridge and the direct visual assessment of the remaining physis. The patient’s parents provided written informed consent for print and electronic publication of this case report.

CASE

A 3-year-old boy with a history of hemifacial microsomia presented for the evaluation of genu valgum and leg-length discrepancy. His intermalleolar distance at that time was 8 cm. A standing radiograph of his lower extremities demonstrated changes consistent with physiologic genu valgum. He had no history of knee trauma, infection, or pain.

At the age of 5 years and 7 months, the patient returned for a repeat evaluation and was noted to exhibit the progressive valgus deformity of the right leg and a leg-length discrepancy of 3.9 cm (Figure 1). 

Radiographs also showed the presence of a bone bridge of unknown etiology in the boy’s distal femoral physis. Computed tomography and magnetic resonance imaging (MRI) were used to characterize the size and location of the bone bridge, which was found to involve approximately 30% of the physis (Figures 2A-2C). 

Using the multiplier method described by Paley and colleagues,¹² the patient’s projected leg-length discrepancy, assuming complete growth arrest, was approximately 10.2 cm. The risks and benefits of physeal bone bridge resection, including the high rate of recurrence, were discussed with the patient’s family, who wished to proceed with resection.

 

Continue to: With the patient supine on the operating...

 

OPERATIVE TECHNIQUE

With the patient supine on the operating table and after the administration of general anesthesia, 3-dimensional (3-D) fluoroscopy was used to localize the bone bridge, which confirmed the fluoroscopic location that was previously visualized through preoperative 3-D imaging. The leg was elevated, and a tourniquet was applied and inflated. A lateral parapatellar approach was used to isolate the distal femoral physis anteriorly because the bone bridge was centered just lateral to the central portion of the distal femoral physis. A Kirschner wire was placed in the center of the bridge under anteroposterior and lateral fluoroscopic imaging (Figures 3A-3E). 

A series of core reamers were then introduced, starting at 10 mm diameter and increasing to 18 mm diameter before complete resection was accomplished. Irrigation was used to prevent the thermal necrosis of the physis during reaming, and lateral fluoroscopic imaging was used to prevent injury to the posterior neurovascular structures. Each time a reaming was completed, the physeal bone bed was inspected to confirm complete bone bridge resection (Figure 3C). Once 18 mm of the physis had been removed, direct visual inspection confirmed normal physis was present on all sides of the bone that remained following physeal bar resection (Figures 3D and 4A, 4B). 

The defect was irrigated with normal saline and filled with cranioplast (Figure 3E). Cranioplast (the methyl ester of methacrylic acid that easily polymerizes into polymethyl methacrylate) was chosen because the amount of adipose tissue was insufficient for harvesting for interposition given the patient’s lean body habitus. Moreover, the use of the cranioplast prevented the occurrence of exothermic reactions during curing and provided hemostasis because the cranioplast occupied the entire cavity and was strong enough to provide structural support.¹³ When partially set into a putty-like state to allow molding, the cranioplast was carefully contoured within the femoral trochlea. To protect the resection site from pathologic fracture, the patient was placed in a long-leg cast, and only protected weight-bearing with the use of a walker was allowed for 6 weeks.

OUTCOME

The patient healed uneventfully, and early range-of-motion exercises were started 6 weeks postoperatively. At 6-month follow-up, his leg-length discrepancy was 2.7 cm, and the bone bridge did not recur. At 3-year follow-up, his leg-length discrepancy was 3.0 cm, and the bone bridge did not recur. Over the 3 years postoperatively, the patient exhibited 9.8 cm of growth on his operative side and 9.5 cm on his nonoperative side (Figure 5). 

The patient has returned to full function and has had no pain, patellofemoral complaints, or complications associated with the cranioplast. He currently is being followed for his leg-length discrepancy. A contralateral epiphysiodesis is planned to equalize his leg-length discrepancy.

DISCUSSION

Given the considerable growth potential of the distal femoral physis,^1,14-16 an injury to the distal femoral physis and the formation of a physeal bone bridge can have a profound effect on a young patient in terms of leg-length discrepancy and angular deformity. Fracture from trauma or infection is a common cause of physeal bone bridges.^6,17-19 The etiology of our patient’s distal femoral physeal bone bridge is idiopathic, which is considerably less common than other etiologies, and the incidence of idiopathic physeal bone bridge formation is not well established in the literature. Hresko and Kasser²¹ identified atraumatic physeal bone bridge formations in 7 patients. Among the 13 patients with physeal bone bridges described by Broughton and colleagues,²⁰ the cause of bridge formation is unknown in 1.

Physeal bone bridges that form centrally are particularly challenging because they are difficult to visualize through a peripheral approach. A number of methods for resecting central physeal bone bridges have been described. These methods have varying degrees of success. In 1981, Langenskiöld⁷ first described the creation of a metaphyseal mirror and the use of a dental mirror for visualization. This technique, however, yielded unfavorable results in 16% of patients. Williamson and Staheli⁹ reported poor results in 23% of patients. Loraas and Schmale⁴ described the use of an endoscope, termed an osteoscope, for visualization, citing advantages of superior illumination and potential for image magnification and capture. Marsh and Polzhofer⁸ also showed this technique to have low morbidity but poor results in 13% of patients, whereas Moreta and colleagues¹⁰ reported poor results in 2 out of 5 patients. The rate of poor results of these methods may be related to the technical difficulty of using dental mirrors and arthroscopes and can be improved by highly efficient direct methods with improved visualization, such as the method described in this article.

Continue to: Proper imaging is necessary for...

 

Proper imaging is necessary for the accurate quantification of bone bridges to determine resectability and to identify the best surgical approach to resection. MRI with software for the generation of 3-D physeal maps is a reproducible method with good interobserver reliability.^22,23 Intraoperative computer-assisted imaging also is beneficial for determining the extent and location of the resection to ensure complete bone bridge removal.²⁴

To our knowledge, a direct approach through parapatellar arthrotomy for the resection of a centrally located distal femoral physeal bone bridge has not been previously described. This novel technique provided direct access to the physeal bone bridge and was performed without injuring the adjacent physeal cartilage in the medial and lateral columns of the distal femur, which may grow normally in the absence of the bridge. Instead of using a lateral or medial approach with a metaphyseal window,⁴ we directly approached this central bar through a parapatellar approach and were able to completely resect it under direct visualization. This obviated the need for an arthroscope or dental mirror. To remove the entire physeal bone bridge, we needed to resect completely from the anterior cortex to the posterior cortex. Although this technique potentially increased the risk of iatrogenic fracture, we believed that this risk would not differ greatly from that of disrupting the medial or lateral metaphysis and would be more stable with either axial and torsion load. At 3-year follow-up, the patient exhibited restored normal growth in his operative limb relative to that in his nonoperative limb, had not developed angular deformity, and had maintained his previously developed limb-length discrepancy that could be corrected with the epiphysiodesis of his opposite limb at a later date.

The limitations to this technique include the fact that it may be most effective with small-to moderate-sized central physeal bone bridges, although resection has shown good results with up to 70% physeal involvement.⁸ In this patient, the bone bridge was moderately sized (30% of the physis), centrally located, and clearly visible on fluoroscopy. These characteristics increased the technical safety and ease of the procedure. The resection of large, peripheral bridges may destabilize the distal femur. The destabilization of the distal femur, in turn, can lead to fracture. Patellofemoral mechanics may also be affected during the treatment of distal femoral physeal bone bridges. This patient has not experienced any patellofemoral dysfunction or symptoms. Given the patient’s age and significant amount of remaining growth, he will need close monitoring until he reaches skeletal maturity.

This paper will be judged for the Resident Writer’s Award.

ABSTRACT

A central distal femoral physeal bone bridge in a boy aged 5 years and 7 months was resected with a fluoroscopically guided core reamer placed through a lateral parapatellar approach. At 3-year follow-up, the boy’s leg-length discrepancy was 3.0 cm (3.9 cm preoperatively), and the physeal bone bridge did not recur. The patient had full function and no pain or other patellofemoral complaints. This technique provided direct access to the physeal bone bridge, and complete resection was performed without injury to the adjacent physeal cartilage in the medial and lateral columns of the distal femur, which is expected to grow normally in the absence of the bridge.

A physeal bone bridge is an osseous connection that forms across a physis. It may cause partial premature physeal arrest. Angular deformity and limb-length discrepancy are the main complications caused by physeal bone bridges.^1-4 The indications for the treatment of physeal bridges are well documented.^1-5 Trauma and infection are common causes of distal femoral physeal bone bridges. Arkader and colleagues⁶ showed that among different types of physeal bridges, the Salter-Harris type is significantly associated with complications, among which growth arrest is the most common and occurs in 27.4% of all patients.

The treatment of distal femoral physeal bone bridges is technically difficult and provides variable results. Poor results are reported in 13% to 40% of patients.^7-10 Procedure failure has been attributed to incomplete resection with the persistent tethering and dislodgement of the graft.¹¹ Methods with improved efficacy for the removal of central physeal bridges will help prevent reformation after treatment. We have used a novel technique that allows the direct resection of a central physeal bone bridge in the distal femur through the use of a fluoroscopically guided core reamer. This technique enables the complete removal of the bone bridge and the direct visual assessment of the remaining physis. The patient’s parents provided written informed consent for print and electronic publication of this case report.

CASE

A 3-year-old boy with a history of hemifacial microsomia presented for the evaluation of genu valgum and leg-length discrepancy. His intermalleolar distance at that time was 8 cm. A standing radiograph of his lower extremities demonstrated changes consistent with physiologic genu valgum. He had no history of knee trauma, infection, or pain.

At the age of 5 years and 7 months, the patient returned for a repeat evaluation and was noted to exhibit the progressive valgus deformity of the right leg and a leg-length discrepancy of 3.9 cm (Figure 1). 

Radiographs also showed the presence of a bone bridge of unknown etiology in the boy’s distal femoral physis. Computed tomography and magnetic resonance imaging (MRI) were used to characterize the size and location of the bone bridge, which was found to involve approximately 30% of the physis (Figures 2A-2C). 

Using the multiplier method described by Paley and colleagues,¹² the patient’s projected leg-length discrepancy, assuming complete growth arrest, was approximately 10.2 cm. The risks and benefits of physeal bone bridge resection, including the high rate of recurrence, were discussed with the patient’s family, who wished to proceed with resection.

 

Continue to: With the patient supine on the operating...

 

OPERATIVE TECHNIQUE

With the patient supine on the operating table and after the administration of general anesthesia, 3-dimensional (3-D) fluoroscopy was used to localize the bone bridge, which confirmed the fluoroscopic location that was previously visualized through preoperative 3-D imaging. The leg was elevated, and a tourniquet was applied and inflated. A lateral parapatellar approach was used to isolate the distal femoral physis anteriorly because the bone bridge was centered just lateral to the central portion of the distal femoral physis. A Kirschner wire was placed in the center of the bridge under anteroposterior and lateral fluoroscopic imaging (Figures 3A-3E). 

A series of core reamers were then introduced, starting at 10 mm diameter and increasing to 18 mm diameter before complete resection was accomplished. Irrigation was used to prevent the thermal necrosis of the physis during reaming, and lateral fluoroscopic imaging was used to prevent injury to the posterior neurovascular structures. Each time a reaming was completed, the physeal bone bed was inspected to confirm complete bone bridge resection (Figure 3C). Once 18 mm of the physis had been removed, direct visual inspection confirmed normal physis was present on all sides of the bone that remained following physeal bar resection (Figures 3D and 4A, 4B). 

The defect was irrigated with normal saline and filled with cranioplast (Figure 3E). Cranioplast (the methyl ester of methacrylic acid that easily polymerizes into polymethyl methacrylate) was chosen because the amount of adipose tissue was insufficient for harvesting for interposition given the patient’s lean body habitus. Moreover, the use of the cranioplast prevented the occurrence of exothermic reactions during curing and provided hemostasis because the cranioplast occupied the entire cavity and was strong enough to provide structural support.¹³ When partially set into a putty-like state to allow molding, the cranioplast was carefully contoured within the femoral trochlea. To protect the resection site from pathologic fracture, the patient was placed in a long-leg cast, and only protected weight-bearing with the use of a walker was allowed for 6 weeks.

OUTCOME

The patient healed uneventfully, and early range-of-motion exercises were started 6 weeks postoperatively. At 6-month follow-up, his leg-length discrepancy was 2.7 cm, and the bone bridge did not recur. At 3-year follow-up, his leg-length discrepancy was 3.0 cm, and the bone bridge did not recur. Over the 3 years postoperatively, the patient exhibited 9.8 cm of growth on his operative side and 9.5 cm on his nonoperative side (Figure 5). 

The patient has returned to full function and has had no pain, patellofemoral complaints, or complications associated with the cranioplast. He currently is being followed for his leg-length discrepancy. A contralateral epiphysiodesis is planned to equalize his leg-length discrepancy.

DISCUSSION

Given the considerable growth potential of the distal femoral physis,^1,14-16 an injury to the distal femoral physis and the formation of a physeal bone bridge can have a profound effect on a young patient in terms of leg-length discrepancy and angular deformity. Fracture from trauma or infection is a common cause of physeal bone bridges.^6,17-19 The etiology of our patient’s distal femoral physeal bone bridge is idiopathic, which is considerably less common than other etiologies, and the incidence of idiopathic physeal bone bridge formation is not well established in the literature. Hresko and Kasser²¹ identified atraumatic physeal bone bridge formations in 7 patients. Among the 13 patients with physeal bone bridges described by Broughton and colleagues,²⁰ the cause of bridge formation is unknown in 1.

Physeal bone bridges that form centrally are particularly challenging because they are difficult to visualize through a peripheral approach. A number of methods for resecting central physeal bone bridges have been described. These methods have varying degrees of success. In 1981, Langenskiöld⁷ first described the creation of a metaphyseal mirror and the use of a dental mirror for visualization. This technique, however, yielded unfavorable results in 16% of patients. Williamson and Staheli⁹ reported poor results in 23% of patients. Loraas and Schmale⁴ described the use of an endoscope, termed an osteoscope, for visualization, citing advantages of superior illumination and potential for image magnification and capture. Marsh and Polzhofer⁸ also showed this technique to have low morbidity but poor results in 13% of patients, whereas Moreta and colleagues¹⁰ reported poor results in 2 out of 5 patients. The rate of poor results of these methods may be related to the technical difficulty of using dental mirrors and arthroscopes and can be improved by highly efficient direct methods with improved visualization, such as the method described in this article.

Continue to: Proper imaging is necessary for...

 

Proper imaging is necessary for the accurate quantification of bone bridges to determine resectability and to identify the best surgical approach to resection. MRI with software for the generation of 3-D physeal maps is a reproducible method with good interobserver reliability.^22,23 Intraoperative computer-assisted imaging also is beneficial for determining the extent and location of the resection to ensure complete bone bridge removal.²⁴

To our knowledge, a direct approach through parapatellar arthrotomy for the resection of a centrally located distal femoral physeal bone bridge has not been previously described. This novel technique provided direct access to the physeal bone bridge and was performed without injuring the adjacent physeal cartilage in the medial and lateral columns of the distal femur, which may grow normally in the absence of the bridge. Instead of using a lateral or medial approach with a metaphyseal window,⁴ we directly approached this central bar through a parapatellar approach and were able to completely resect it under direct visualization. This obviated the need for an arthroscope or dental mirror. To remove the entire physeal bone bridge, we needed to resect completely from the anterior cortex to the posterior cortex. Although this technique potentially increased the risk of iatrogenic fracture, we believed that this risk would not differ greatly from that of disrupting the medial or lateral metaphysis and would be more stable with either axial and torsion load. At 3-year follow-up, the patient exhibited restored normal growth in his operative limb relative to that in his nonoperative limb, had not developed angular deformity, and had maintained his previously developed limb-length discrepancy that could be corrected with the epiphysiodesis of his opposite limb at a later date.

The limitations to this technique include the fact that it may be most effective with small-to moderate-sized central physeal bone bridges, although resection has shown good results with up to 70% physeal involvement.⁸ In this patient, the bone bridge was moderately sized (30% of the physis), centrally located, and clearly visible on fluoroscopy. These characteristics increased the technical safety and ease of the procedure. The resection of large, peripheral bridges may destabilize the distal femur. The destabilization of the distal femur, in turn, can lead to fracture. Patellofemoral mechanics may also be affected during the treatment of distal femoral physeal bone bridges. This patient has not experienced any patellofemoral dysfunction or symptoms. Given the patient’s age and significant amount of remaining growth, he will need close monitoring until he reaches skeletal maturity.

This paper will be judged for the Resident Writer’s Award.

ABSTRACT

A central distal femoral physeal bone bridge in a boy aged 5 years and 7 months was resected with a fluoroscopically guided core reamer placed through a lateral parapatellar approach. At 3-year follow-up, the boy’s leg-length discrepancy was 3.0 cm (3.9 cm preoperatively), and the physeal bone bridge did not recur. The patient had full function and no pain or other patellofemoral complaints. This technique provided direct access to the physeal bone bridge, and complete resection was performed without injury to the adjacent physeal cartilage in the medial and lateral columns of the distal femur, which is expected to grow normally in the absence of the bridge.

A physeal bone bridge is an osseous connection that forms across a physis. It may cause partial premature physeal arrest. Angular deformity and limb-length discrepancy are the main complications caused by physeal bone bridges.^1-4 The indications for the treatment of physeal bridges are well documented.^1-5 Trauma and infection are common causes of distal femoral physeal bone bridges. Arkader and colleagues⁶ showed that among different types of physeal bridges, the Salter-Harris type is significantly associated with complications, among which growth arrest is the most common and occurs in 27.4% of all patients.

The treatment of distal femoral physeal bone bridges is technically difficult and provides variable results. Poor results are reported in 13% to 40% of patients.^7-10 Procedure failure has been attributed to incomplete resection with the persistent tethering and dislodgement of the graft.¹¹ Methods with improved efficacy for the removal of central physeal bridges will help prevent reformation after treatment. We have used a novel technique that allows the direct resection of a central physeal bone bridge in the distal femur through the use of a fluoroscopically guided core reamer. This technique enables the complete removal of the bone bridge and the direct visual assessment of the remaining physis. The patient’s parents provided written informed consent for print and electronic publication of this case report.

CASE

A 3-year-old boy with a history of hemifacial microsomia presented for the evaluation of genu valgum and leg-length discrepancy. His intermalleolar distance at that time was 8 cm. A standing radiograph of his lower extremities demonstrated changes consistent with physiologic genu valgum. He had no history of knee trauma, infection, or pain.

At the age of 5 years and 7 months, the patient returned for a repeat evaluation and was noted to exhibit the progressive valgus deformity of the right leg and a leg-length discrepancy of 3.9 cm (Figure 1). 

Radiographs also showed the presence of a bone bridge of unknown etiology in the boy’s distal femoral physis. Computed tomography and magnetic resonance imaging (MRI) were used to characterize the size and location of the bone bridge, which was found to involve approximately 30% of the physis (Figures 2A-2C). 

Using the multiplier method described by Paley and colleagues,¹² the patient’s projected leg-length discrepancy, assuming complete growth arrest, was approximately 10.2 cm. The risks and benefits of physeal bone bridge resection, including the high rate of recurrence, were discussed with the patient’s family, who wished to proceed with resection.

 

Continue to: With the patient supine on the operating...

 

OPERATIVE TECHNIQUE

With the patient supine on the operating table and after the administration of general anesthesia, 3-dimensional (3-D) fluoroscopy was used to localize the bone bridge, which confirmed the fluoroscopic location that was previously visualized through preoperative 3-D imaging. The leg was elevated, and a tourniquet was applied and inflated. A lateral parapatellar approach was used to isolate the distal femoral physis anteriorly because the bone bridge was centered just lateral to the central portion of the distal femoral physis. A Kirschner wire was placed in the center of the bridge under anteroposterior and lateral fluoroscopic imaging (Figures 3A-3E). 

A series of core reamers were then introduced, starting at 10 mm diameter and increasing to 18 mm diameter before complete resection was accomplished. Irrigation was used to prevent the thermal necrosis of the physis during reaming, and lateral fluoroscopic imaging was used to prevent injury to the posterior neurovascular structures. Each time a reaming was completed, the physeal bone bed was inspected to confirm complete bone bridge resection (Figure 3C). Once 18 mm of the physis had been removed, direct visual inspection confirmed normal physis was present on all sides of the bone that remained following physeal bar resection (Figures 3D and 4A, 4B). 

The defect was irrigated with normal saline and filled with cranioplast (Figure 3E). Cranioplast (the methyl ester of methacrylic acid that easily polymerizes into polymethyl methacrylate) was chosen because the amount of adipose tissue was insufficient for harvesting for interposition given the patient’s lean body habitus. Moreover, the use of the cranioplast prevented the occurrence of exothermic reactions during curing and provided hemostasis because the cranioplast occupied the entire cavity and was strong enough to provide structural support.¹³ When partially set into a putty-like state to allow molding, the cranioplast was carefully contoured within the femoral trochlea. To protect the resection site from pathologic fracture, the patient was placed in a long-leg cast, and only protected weight-bearing with the use of a walker was allowed for 6 weeks.

OUTCOME

The patient healed uneventfully, and early range-of-motion exercises were started 6 weeks postoperatively. At 6-month follow-up, his leg-length discrepancy was 2.7 cm, and the bone bridge did not recur. At 3-year follow-up, his leg-length discrepancy was 3.0 cm, and the bone bridge did not recur. Over the 3 years postoperatively, the patient exhibited 9.8 cm of growth on his operative side and 9.5 cm on his nonoperative side (Figure 5). 

The patient has returned to full function and has had no pain, patellofemoral complaints, or complications associated with the cranioplast. He currently is being followed for his leg-length discrepancy. A contralateral epiphysiodesis is planned to equalize his leg-length discrepancy.

DISCUSSION

Given the considerable growth potential of the distal femoral physis,^1,14-16 an injury to the distal femoral physis and the formation of a physeal bone bridge can have a profound effect on a young patient in terms of leg-length discrepancy and angular deformity. Fracture from trauma or infection is a common cause of physeal bone bridges.^6,17-19 The etiology of our patient’s distal femoral physeal bone bridge is idiopathic, which is considerably less common than other etiologies, and the incidence of idiopathic physeal bone bridge formation is not well established in the literature. Hresko and Kasser²¹ identified atraumatic physeal bone bridge formations in 7 patients. Among the 13 patients with physeal bone bridges described by Broughton and colleagues,²⁰ the cause of bridge formation is unknown in 1.

Physeal bone bridges that form centrally are particularly challenging because they are difficult to visualize through a peripheral approach. A number of methods for resecting central physeal bone bridges have been described. These methods have varying degrees of success. In 1981, Langenskiöld⁷ first described the creation of a metaphyseal mirror and the use of a dental mirror for visualization. This technique, however, yielded unfavorable results in 16% of patients. Williamson and Staheli⁹ reported poor results in 23% of patients. Loraas and Schmale⁴ described the use of an endoscope, termed an osteoscope, for visualization, citing advantages of superior illumination and potential for image magnification and capture. Marsh and Polzhofer⁸ also showed this technique to have low morbidity but poor results in 13% of patients, whereas Moreta and colleagues¹⁰ reported poor results in 2 out of 5 patients. The rate of poor results of these methods may be related to the technical difficulty of using dental mirrors and arthroscopes and can be improved by highly efficient direct methods with improved visualization, such as the method described in this article.

Continue to: Proper imaging is necessary for...

 

Proper imaging is necessary for the accurate quantification of bone bridges to determine resectability and to identify the best surgical approach to resection. MRI with software for the generation of 3-D physeal maps is a reproducible method with good interobserver reliability.^22,23 Intraoperative computer-assisted imaging also is beneficial for determining the extent and location of the resection to ensure complete bone bridge removal.²⁴

To our knowledge, a direct approach through parapatellar arthrotomy for the resection of a centrally located distal femoral physeal bone bridge has not been previously described. This novel technique provided direct access to the physeal bone bridge and was performed without injuring the adjacent physeal cartilage in the medial and lateral columns of the distal femur, which may grow normally in the absence of the bridge. Instead of using a lateral or medial approach with a metaphyseal window,⁴ we directly approached this central bar through a parapatellar approach and were able to completely resect it under direct visualization. This obviated the need for an arthroscope or dental mirror. To remove the entire physeal bone bridge, we needed to resect completely from the anterior cortex to the posterior cortex. Although this technique potentially increased the risk of iatrogenic fracture, we believed that this risk would not differ greatly from that of disrupting the medial or lateral metaphysis and would be more stable with either axial and torsion load. At 3-year follow-up, the patient exhibited restored normal growth in his operative limb relative to that in his nonoperative limb, had not developed angular deformity, and had maintained his previously developed limb-length discrepancy that could be corrected with the epiphysiodesis of his opposite limb at a later date.

The limitations to this technique include the fact that it may be most effective with small-to moderate-sized central physeal bone bridges, although resection has shown good results with up to 70% physeal involvement.⁸ In this patient, the bone bridge was moderately sized (30% of the physis), centrally located, and clearly visible on fluoroscopy. These characteristics increased the technical safety and ease of the procedure. The resection of large, peripheral bridges may destabilize the distal femur. The destabilization of the distal femur, in turn, can lead to fracture. Patellofemoral mechanics may also be affected during the treatment of distal femoral physeal bone bridges. This patient has not experienced any patellofemoral dysfunction or symptoms. Given the patient’s age and significant amount of remaining growth, he will need close monitoring until he reaches skeletal maturity.

This paper will be judged for the Resident Writer’s Award.

ABSTRACT

This case report is a rare form of lymphoma recurrence which presented as tarsal tunnel syndrome. The patient had been previously treated for the malignancy and was presumed to be in remission; however, standard radiology imaging protocols failed to include the distal extremities on these scans. The patient presented to the orthopedic clinic with tarsal tunnel symptoms and a mass in the tarsal tunnel. A complete evaluation resulted in a diagnosis of recurrence of the malignancy. This case illustrates the importance of a thorough medical history and personal review of imaging studies, and how a systematic approach can produce the correct diagnosis for any unknown lesion. Furthermore, this case may prompt oncologists to consider obtaining whole-body fluorodeoxyglucose positron emission tomography computed tomography when evaluating for recurrence in patients.

Nasal-type, extranodal natural killer/T-cell lymphoma (ENKTL) is a rare form of non-Hodgkin lymphoma (NHL). Malignancies account for only 10% of NHL in Asian and South American populations. However, in Caucasians, it represents <1% of all cases. In addition, at 3:1 male to female ratio, the disease most commonly affects male patients who are 50 to 59 years old.^1-3 The etiology of this malignancy is strongly related to prior infection with Epstein-Barr virus (EBV) as EBV-encoded early small ribonucleic acid on in situ hybridization of lymphoma cells is positive in 95% of cases.^4-6

Typical sites of involvement include the nasal cavity, nasopharynx, and sinuses, causing patients to present with nasal obstruction, chronic sinusitis, or epistaxis. Additionally, ENKTL can occur primarily in the skin, gastrointestinal tract, spleen, and testis, whereas the bone marrow may be involved in 10% of cases. Although rare, unusual sites, including muscle, adrenals, and ovaries, have been published.^7,8

Staging is best performed using the T-staging system, which accounts for the extent of local tumor involvement. Higher stages, such as T3 /T4, equate to locally advanced disease and imply a worse prognosis.^9,10 Computed tomography (CT) and magnetic resonance imaging (MRI) help define local soft tissues and bony involvement. Furthermore, CT of the chest, abdomen, and pelvis as well as bone marrow biopsy are performed as part of the staging process. Lastly, fluorine-18 fluorodeoxyglucose positron emission tomography CT (18-FDG PET-CT) is often used to detect extranodal spread, define the extent of involvement, differentiate between lymphoma and inflammatory masses, and monitor for recurrence.¹¹

Treatment for local ENKTL involves concurrent chemoradiotherapy followed by 3 cycles of etoposide, ifosfamide, cisplatin, and dexamethasone, which results in a complete response rate of 80%, and is the most favorable when comparing treatment modalities.¹² Unfortunately, recurrence rates reach as high as 50%, whereas the 5-year survival rate is 59%.^13,14 For recurrent or disseminated disease, high-dose chemotherapy and hematopoietic stem cell transplantation remain as alternative treatments for patients who have undergone 2 complete remissions and can be curative in some instances.^13,15

Continue to: In summary, ENKTL is a rare form...

 

In summary, ENKTL is a rare form of NHL which classically presents in the nasal cavity; however, this type of lymphoma may present in a variety of extranodal sites.^7,8 Despite the numerous published reports on ENKTL, no study has reported either primary or recurrent ENKTL in the feet or hands. To our knowledge, this is one of the first published cases of a patient who developed a rare and recurring ENKTL in the foot and ankle. The patient provided written informed consent for print and electronic publication of this case report.

CASE

A 59-year-old Caucasian woman was referred to the orthopedic foot and ankle clinic by her primary care physician for right medial ankle pain, skin ulceration, and numbness over the plantar aspect of her right foot. Upon questioning, the patient noted that the pain and numbness were present for almost 6 months. She denied trauma to the concerned area. Previously, the patient was observed and treated elsewhere for plantar fasciitis and was prescribed a brace before being immobilized in a controlled ankle motion (CAM) boot for 6 weeks. At follow-up with her outside provider, the patient had developed skin breakdown over the medial aspect of the right ankle, and this condition was presumed to be caused by the boot. After local wound care failed to improve her skin ulceration, she returned to her primary care physician, who ordered an MRI of the area and referred her to our specialty clinic.

Upon review, the patient’s past medical history included a diagnosis of nasal-type ENKTL. Her malignancy was treated with chemoradiotherapy 2 years prior to her consultation with the foot and ankle clinic.

The patient was noted by her medical oncologist and interventional radiologist to be in complete stage 4 remission since being treated. She underwent routine MRI and CT scans of the head and neck at 6-month intervals and FDG PET-CT scans at 3-month intervals, as per institutional protocol. The examinations showed no evidence of malignancy or metabolically active disease. The last imaging study occurred 2 months prior to admission to the foot and ankle clinic.

The patient consulted her medical oncologist 1 month prior to presenting to our clinic and was noted to exhibit an “excellent response to chemoradiotherapy” and “continues to remain disease free at 2 years.” She was instructed to continue routine follow-up. However, the office notes mentioned no ankle pain and non-healing wounds.

During physical examination, the patient presented an antalgic gait on the right side. Inspection demonstrated an increased circumference of the right ankle compared with the left, with a soft, palpable mass over the medial aspect of her right ankle. A 3 cm × 2 cm, grade 2 abrasion of the skin was observed over the medial mass just posterior to her medial malleolus. Range of motion was within normal limits. The patient exhibited a palpable posterior tibial artery pulse and full strength upon muscle testing of the lower extremities. She featured a positive Tinel’s sign and discomfort over the mass itself, with the pain radiating down to the plantar aspect of her foot and diffuse numbness over the plantar aspect of the foot.

Continue to: Review of her plain radiographs...

 

Review of her plain radiographs demonstrated no bony abnormalities, fractures, nor visible deformity (Figures 1A, 1B). 

MRI of the foot and ankle was reviewed and demonstrated a large soft-tissue mass over the medial aspect of the ankle extending posteriorly to engulf the medial flexor tendons and medial neurovascular bundle (Figures 2A-2C). 

Interestingly, the radiologist’s report only mentioned a “large region of devitalized tissue underlying the known medial ankle ulcer which extended to the tibiotalar and subtalar joints and bone marrow changes in the talus most compatible with osteomyelitis.” FDG PET-CT images from the patient’s past 2 years were reviewed, and the radiologist was contacted to confirm the negative findings. Unfortunately, FDG PET-CT performs no routine imaging below the elbow or knee when evaluating for this particular malignancy (Figure 3).

At presentation, our differential diagnosis included recurrence of the malignancy, secondary malignancy, infection, and inflammatory disease. After a lengthy discussion with the patient and consultation with our institution’s musculoskeletal oncologist, the decision was made to perform a right-ankle mass biopsy and marginal excision with wound irrigation and débridement and tarsal tunnel release.

The patient was placed in the supine position with standard prepping and draping. The medial eschar was excised in an elliptical fashion, and a curvilinear, longitudinal approach was performed within the compartment to access the mass along the posteromedial aspect of the ankle. Although no evidence of infection was observed, the tissue was thickened with areas of necrosis down to the flexor retinaculum. Once the flexor retinaculum was opened, a fibrous, plaque-like mass was observed, and it was encased with flexor tendons and neurovascular structures of the tarsal tunnel. After mass excision, a complete tarsal tunnel release was performed until the neurovascular bundle was free. Irrigation and débridement of the ulcer were performed along with complicated wound closure, and the patient was placed in a well-padded postoperative splint.

Pathology was finalized as a recurrent, EBV-positive, and nasal-type ENKTL. The patient underwent bone marrow biopsy, which yielded negative results. CT of the chest, abdomen, and pelvis were negative for the disease. FDG PET-CT, which included the extremities, was performed and demonstrated increased uptake in the right ankle, consistent with the malignancy (Figure 4). 

The patient was placed on her second cycle of chemoradiotherapy and again showed complete remission. At over 1-year follow-up, the patient’s wound had completely healed, and FDG PET-CT of the extremity was negative for ENKTL recurrence (Figure 5). 

At present, the patient’s medical oncologists recommend a stem cell transplant as the patient exhibited 2 positive responses to chemoradiotherapy.

DISCUSSION

ENKTL is an uncommon form of lymphoma and is exceedingly rare in Caucasian females.^1-3 Although the patient’s primary occurrence was in the nasal cavity, recurrence in the foot and ankle must still be described.^7,8 To our knowledge, this article is one of the first published cases of a patient who developed a rare-recurrence ENKTL about the foot and ankle. Occurrence in extremities is extremely rare that the staging protocol does not include FDG PET-CT of these areas. The patient’s “negative” scans led many providers to neglect the symptoms in her right ankle until the lesion had ulcerated through the skin. If one would have relied on imaging reports and outside records alone, the diagnosis would have been delayed longer or missed all together. This case illustrates the importance of a thorough medical history and personal review of imaging studies, and how a systematic approach can produce the correct diagnosis for any unknown lesion. Furthermore, this case may prompt oncologists to consider obtaining whole-body FDG PET-CT when evaluating for recurrence in patients.

ABSTRACT

This case report is a rare form of lymphoma recurrence which presented as tarsal tunnel syndrome. The patient had been previously treated for the malignancy and was presumed to be in remission; however, standard radiology imaging protocols failed to include the distal extremities on these scans. The patient presented to the orthopedic clinic with tarsal tunnel symptoms and a mass in the tarsal tunnel. A complete evaluation resulted in a diagnosis of recurrence of the malignancy. This case illustrates the importance of a thorough medical history and personal review of imaging studies, and how a systematic approach can produce the correct diagnosis for any unknown lesion. Furthermore, this case may prompt oncologists to consider obtaining whole-body fluorodeoxyglucose positron emission tomography computed tomography when evaluating for recurrence in patients.

Nasal-type, extranodal natural killer/T-cell lymphoma (ENKTL) is a rare form of non-Hodgkin lymphoma (NHL). Malignancies account for only 10% of NHL in Asian and South American populations. However, in Caucasians, it represents <1% of all cases. In addition, at 3:1 male to female ratio, the disease most commonly affects male patients who are 50 to 59 years old.^1-3 The etiology of this malignancy is strongly related to prior infection with Epstein-Barr virus (EBV) as EBV-encoded early small ribonucleic acid on in situ hybridization of lymphoma cells is positive in 95% of cases.^4-6

Typical sites of involvement include the nasal cavity, nasopharynx, and sinuses, causing patients to present with nasal obstruction, chronic sinusitis, or epistaxis. Additionally, ENKTL can occur primarily in the skin, gastrointestinal tract, spleen, and testis, whereas the bone marrow may be involved in 10% of cases. Although rare, unusual sites, including muscle, adrenals, and ovaries, have been published.^7,8

Staging is best performed using the T-staging system, which accounts for the extent of local tumor involvement. Higher stages, such as T3 /T4, equate to locally advanced disease and imply a worse prognosis.^9,10 Computed tomography (CT) and magnetic resonance imaging (MRI) help define local soft tissues and bony involvement. Furthermore, CT of the chest, abdomen, and pelvis as well as bone marrow biopsy are performed as part of the staging process. Lastly, fluorine-18 fluorodeoxyglucose positron emission tomography CT (18-FDG PET-CT) is often used to detect extranodal spread, define the extent of involvement, differentiate between lymphoma and inflammatory masses, and monitor for recurrence.¹¹

Treatment for local ENKTL involves concurrent chemoradiotherapy followed by 3 cycles of etoposide, ifosfamide, cisplatin, and dexamethasone, which results in a complete response rate of 80%, and is the most favorable when comparing treatment modalities.¹² Unfortunately, recurrence rates reach as high as 50%, whereas the 5-year survival rate is 59%.^13,14 For recurrent or disseminated disease, high-dose chemotherapy and hematopoietic stem cell transplantation remain as alternative treatments for patients who have undergone 2 complete remissions and can be curative in some instances.^13,15

Continue to: In summary, ENKTL is a rare form...

 

In summary, ENKTL is a rare form of NHL which classically presents in the nasal cavity; however, this type of lymphoma may present in a variety of extranodal sites.^7,8 Despite the numerous published reports on ENKTL, no study has reported either primary or recurrent ENKTL in the feet or hands. To our knowledge, this is one of the first published cases of a patient who developed a rare and recurring ENKTL in the foot and ankle. The patient provided written informed consent for print and electronic publication of this case report.

CASE

A 59-year-old Caucasian woman was referred to the orthopedic foot and ankle clinic by her primary care physician for right medial ankle pain, skin ulceration, and numbness over the plantar aspect of her right foot. Upon questioning, the patient noted that the pain and numbness were present for almost 6 months. She denied trauma to the concerned area. Previously, the patient was observed and treated elsewhere for plantar fasciitis and was prescribed a brace before being immobilized in a controlled ankle motion (CAM) boot for 6 weeks. At follow-up with her outside provider, the patient had developed skin breakdown over the medial aspect of the right ankle, and this condition was presumed to be caused by the boot. After local wound care failed to improve her skin ulceration, she returned to her primary care physician, who ordered an MRI of the area and referred her to our specialty clinic.

Upon review, the patient’s past medical history included a diagnosis of nasal-type ENKTL. Her malignancy was treated with chemoradiotherapy 2 years prior to her consultation with the foot and ankle clinic.

The patient was noted by her medical oncologist and interventional radiologist to be in complete stage 4 remission since being treated. She underwent routine MRI and CT scans of the head and neck at 6-month intervals and FDG PET-CT scans at 3-month intervals, as per institutional protocol. The examinations showed no evidence of malignancy or metabolically active disease. The last imaging study occurred 2 months prior to admission to the foot and ankle clinic.

The patient consulted her medical oncologist 1 month prior to presenting to our clinic and was noted to exhibit an “excellent response to chemoradiotherapy” and “continues to remain disease free at 2 years.” She was instructed to continue routine follow-up. However, the office notes mentioned no ankle pain and non-healing wounds.

During physical examination, the patient presented an antalgic gait on the right side. Inspection demonstrated an increased circumference of the right ankle compared with the left, with a soft, palpable mass over the medial aspect of her right ankle. A 3 cm × 2 cm, grade 2 abrasion of the skin was observed over the medial mass just posterior to her medial malleolus. Range of motion was within normal limits. The patient exhibited a palpable posterior tibial artery pulse and full strength upon muscle testing of the lower extremities. She featured a positive Tinel’s sign and discomfort over the mass itself, with the pain radiating down to the plantar aspect of her foot and diffuse numbness over the plantar aspect of the foot.

Continue to: Review of her plain radiographs...

 

Review of her plain radiographs demonstrated no bony abnormalities, fractures, nor visible deformity (Figures 1A, 1B). 

MRI of the foot and ankle was reviewed and demonstrated a large soft-tissue mass over the medial aspect of the ankle extending posteriorly to engulf the medial flexor tendons and medial neurovascular bundle (Figures 2A-2C). 

Interestingly, the radiologist’s report only mentioned a “large region of devitalized tissue underlying the known medial ankle ulcer which extended to the tibiotalar and subtalar joints and bone marrow changes in the talus most compatible with osteomyelitis.” FDG PET-CT images from the patient’s past 2 years were reviewed, and the radiologist was contacted to confirm the negative findings. Unfortunately, FDG PET-CT performs no routine imaging below the elbow or knee when evaluating for this particular malignancy (Figure 3).

At presentation, our differential diagnosis included recurrence of the malignancy, secondary malignancy, infection, and inflammatory disease. After a lengthy discussion with the patient and consultation with our institution’s musculoskeletal oncologist, the decision was made to perform a right-ankle mass biopsy and marginal excision with wound irrigation and débridement and tarsal tunnel release.

The patient was placed in the supine position with standard prepping and draping. The medial eschar was excised in an elliptical fashion, and a curvilinear, longitudinal approach was performed within the compartment to access the mass along the posteromedial aspect of the ankle. Although no evidence of infection was observed, the tissue was thickened with areas of necrosis down to the flexor retinaculum. Once the flexor retinaculum was opened, a fibrous, plaque-like mass was observed, and it was encased with flexor tendons and neurovascular structures of the tarsal tunnel. After mass excision, a complete tarsal tunnel release was performed until the neurovascular bundle was free. Irrigation and débridement of the ulcer were performed along with complicated wound closure, and the patient was placed in a well-padded postoperative splint.

Pathology was finalized as a recurrent, EBV-positive, and nasal-type ENKTL. The patient underwent bone marrow biopsy, which yielded negative results. CT of the chest, abdomen, and pelvis were negative for the disease. FDG PET-CT, which included the extremities, was performed and demonstrated increased uptake in the right ankle, consistent with the malignancy (Figure 4). 

The patient was placed on her second cycle of chemoradiotherapy and again showed complete remission. At over 1-year follow-up, the patient’s wound had completely healed, and FDG PET-CT of the extremity was negative for ENKTL recurrence (Figure 5). 

At present, the patient’s medical oncologists recommend a stem cell transplant as the patient exhibited 2 positive responses to chemoradiotherapy.

DISCUSSION

ENKTL is an uncommon form of lymphoma and is exceedingly rare in Caucasian females.^1-3 Although the patient’s primary occurrence was in the nasal cavity, recurrence in the foot and ankle must still be described.^7,8 To our knowledge, this article is one of the first published cases of a patient who developed a rare-recurrence ENKTL about the foot and ankle. Occurrence in extremities is extremely rare that the staging protocol does not include FDG PET-CT of these areas. The patient’s “negative” scans led many providers to neglect the symptoms in her right ankle until the lesion had ulcerated through the skin. If one would have relied on imaging reports and outside records alone, the diagnosis would have been delayed longer or missed all together. This case illustrates the importance of a thorough medical history and personal review of imaging studies, and how a systematic approach can produce the correct diagnosis for any unknown lesion. Furthermore, this case may prompt oncologists to consider obtaining whole-body FDG PET-CT when evaluating for recurrence in patients.

ABSTRACT

This case report is a rare form of lymphoma recurrence which presented as tarsal tunnel syndrome. The patient had been previously treated for the malignancy and was presumed to be in remission; however, standard radiology imaging protocols failed to include the distal extremities on these scans. The patient presented to the orthopedic clinic with tarsal tunnel symptoms and a mass in the tarsal tunnel. A complete evaluation resulted in a diagnosis of recurrence of the malignancy. This case illustrates the importance of a thorough medical history and personal review of imaging studies, and how a systematic approach can produce the correct diagnosis for any unknown lesion. Furthermore, this case may prompt oncologists to consider obtaining whole-body fluorodeoxyglucose positron emission tomography computed tomography when evaluating for recurrence in patients.

Nasal-type, extranodal natural killer/T-cell lymphoma (ENKTL) is a rare form of non-Hodgkin lymphoma (NHL). Malignancies account for only 10% of NHL in Asian and South American populations. However, in Caucasians, it represents <1% of all cases. In addition, at 3:1 male to female ratio, the disease most commonly affects male patients who are 50 to 59 years old.^1-3 The etiology of this malignancy is strongly related to prior infection with Epstein-Barr virus (EBV) as EBV-encoded early small ribonucleic acid on in situ hybridization of lymphoma cells is positive in 95% of cases.^4-6

Typical sites of involvement include the nasal cavity, nasopharynx, and sinuses, causing patients to present with nasal obstruction, chronic sinusitis, or epistaxis. Additionally, ENKTL can occur primarily in the skin, gastrointestinal tract, spleen, and testis, whereas the bone marrow may be involved in 10% of cases. Although rare, unusual sites, including muscle, adrenals, and ovaries, have been published.^7,8

Staging is best performed using the T-staging system, which accounts for the extent of local tumor involvement. Higher stages, such as T3 /T4, equate to locally advanced disease and imply a worse prognosis.^9,10 Computed tomography (CT) and magnetic resonance imaging (MRI) help define local soft tissues and bony involvement. Furthermore, CT of the chest, abdomen, and pelvis as well as bone marrow biopsy are performed as part of the staging process. Lastly, fluorine-18 fluorodeoxyglucose positron emission tomography CT (18-FDG PET-CT) is often used to detect extranodal spread, define the extent of involvement, differentiate between lymphoma and inflammatory masses, and monitor for recurrence.¹¹

Treatment for local ENKTL involves concurrent chemoradiotherapy followed by 3 cycles of etoposide, ifosfamide, cisplatin, and dexamethasone, which results in a complete response rate of 80%, and is the most favorable when comparing treatment modalities.¹² Unfortunately, recurrence rates reach as high as 50%, whereas the 5-year survival rate is 59%.^13,14 For recurrent or disseminated disease, high-dose chemotherapy and hematopoietic stem cell transplantation remain as alternative treatments for patients who have undergone 2 complete remissions and can be curative in some instances.^13,15

Continue to: In summary, ENKTL is a rare form...

 

In summary, ENKTL is a rare form of NHL which classically presents in the nasal cavity; however, this type of lymphoma may present in a variety of extranodal sites.^7,8 Despite the numerous published reports on ENKTL, no study has reported either primary or recurrent ENKTL in the feet or hands. To our knowledge, this is one of the first published cases of a patient who developed a rare and recurring ENKTL in the foot and ankle. The patient provided written informed consent for print and electronic publication of this case report.

CASE

A 59-year-old Caucasian woman was referred to the orthopedic foot and ankle clinic by her primary care physician for right medial ankle pain, skin ulceration, and numbness over the plantar aspect of her right foot. Upon questioning, the patient noted that the pain and numbness were present for almost 6 months. She denied trauma to the concerned area. Previously, the patient was observed and treated elsewhere for plantar fasciitis and was prescribed a brace before being immobilized in a controlled ankle motion (CAM) boot for 6 weeks. At follow-up with her outside provider, the patient had developed skin breakdown over the medial aspect of the right ankle, and this condition was presumed to be caused by the boot. After local wound care failed to improve her skin ulceration, she returned to her primary care physician, who ordered an MRI of the area and referred her to our specialty clinic.

Upon review, the patient’s past medical history included a diagnosis of nasal-type ENKTL. Her malignancy was treated with chemoradiotherapy 2 years prior to her consultation with the foot and ankle clinic.

The patient was noted by her medical oncologist and interventional radiologist to be in complete stage 4 remission since being treated. She underwent routine MRI and CT scans of the head and neck at 6-month intervals and FDG PET-CT scans at 3-month intervals, as per institutional protocol. The examinations showed no evidence of malignancy or metabolically active disease. The last imaging study occurred 2 months prior to admission to the foot and ankle clinic.

The patient consulted her medical oncologist 1 month prior to presenting to our clinic and was noted to exhibit an “excellent response to chemoradiotherapy” and “continues to remain disease free at 2 years.” She was instructed to continue routine follow-up. However, the office notes mentioned no ankle pain and non-healing wounds.

During physical examination, the patient presented an antalgic gait on the right side. Inspection demonstrated an increased circumference of the right ankle compared with the left, with a soft, palpable mass over the medial aspect of her right ankle. A 3 cm × 2 cm, grade 2 abrasion of the skin was observed over the medial mass just posterior to her medial malleolus. Range of motion was within normal limits. The patient exhibited a palpable posterior tibial artery pulse and full strength upon muscle testing of the lower extremities. She featured a positive Tinel’s sign and discomfort over the mass itself, with the pain radiating down to the plantar aspect of her foot and diffuse numbness over the plantar aspect of the foot.

Continue to: Review of her plain radiographs...

 

Review of her plain radiographs demonstrated no bony abnormalities, fractures, nor visible deformity (Figures 1A, 1B). 

MRI of the foot and ankle was reviewed and demonstrated a large soft-tissue mass over the medial aspect of the ankle extending posteriorly to engulf the medial flexor tendons and medial neurovascular bundle (Figures 2A-2C). 

Interestingly, the radiologist’s report only mentioned a “large region of devitalized tissue underlying the known medial ankle ulcer which extended to the tibiotalar and subtalar joints and bone marrow changes in the talus most compatible with osteomyelitis.” FDG PET-CT images from the patient’s past 2 years were reviewed, and the radiologist was contacted to confirm the negative findings. Unfortunately, FDG PET-CT performs no routine imaging below the elbow or knee when evaluating for this particular malignancy (Figure 3).

At presentation, our differential diagnosis included recurrence of the malignancy, secondary malignancy, infection, and inflammatory disease. After a lengthy discussion with the patient and consultation with our institution’s musculoskeletal oncologist, the decision was made to perform a right-ankle mass biopsy and marginal excision with wound irrigation and débridement and tarsal tunnel release.

The patient was placed in the supine position with standard prepping and draping. The medial eschar was excised in an elliptical fashion, and a curvilinear, longitudinal approach was performed within the compartment to access the mass along the posteromedial aspect of the ankle. Although no evidence of infection was observed, the tissue was thickened with areas of necrosis down to the flexor retinaculum. Once the flexor retinaculum was opened, a fibrous, plaque-like mass was observed, and it was encased with flexor tendons and neurovascular structures of the tarsal tunnel. After mass excision, a complete tarsal tunnel release was performed until the neurovascular bundle was free. Irrigation and débridement of the ulcer were performed along with complicated wound closure, and the patient was placed in a well-padded postoperative splint.

Pathology was finalized as a recurrent, EBV-positive, and nasal-type ENKTL. The patient underwent bone marrow biopsy, which yielded negative results. CT of the chest, abdomen, and pelvis were negative for the disease. FDG PET-CT, which included the extremities, was performed and demonstrated increased uptake in the right ankle, consistent with the malignancy (Figure 4). 

The patient was placed on her second cycle of chemoradiotherapy and again showed complete remission. At over 1-year follow-up, the patient’s wound had completely healed, and FDG PET-CT of the extremity was negative for ENKTL recurrence (Figure 5). 

At present, the patient’s medical oncologists recommend a stem cell transplant as the patient exhibited 2 positive responses to chemoradiotherapy.

DISCUSSION

ENKTL is an uncommon form of lymphoma and is exceedingly rare in Caucasian females.^1-3 Although the patient’s primary occurrence was in the nasal cavity, recurrence in the foot and ankle must still be described.^7,8 To our knowledge, this article is one of the first published cases of a patient who developed a rare-recurrence ENKTL about the foot and ankle. Occurrence in extremities is extremely rare that the staging protocol does not include FDG PET-CT of these areas. The patient’s “negative” scans led many providers to neglect the symptoms in her right ankle until the lesion had ulcerated through the skin. If one would have relied on imaging reports and outside records alone, the diagnosis would have been delayed longer or missed all together. This case illustrates the importance of a thorough medical history and personal review of imaging studies, and how a systematic approach can produce the correct diagnosis for any unknown lesion. Furthermore, this case may prompt oncologists to consider obtaining whole-body FDG PET-CT when evaluating for recurrence in patients.

ABSTRACT

The lateral tibial eminence shares a close relationship with the anterior root of the lateral meniscus. Limited studies have reported traumatic injury to the anterior meniscal roots in the setting of tibial eminence fractures, and reported rates of occurrence of concomitant meniscal and chondral injuries vary widely. The purpose of this article is to describe the case of a 28-year-old woman who had a complete avulsion of the anterolateral meniscal root caused by a tibial eminence fracture with resultant malunion and root displacement. The anterolateral meniscal root was anatomically repaired following arthroscopic resection of the malunited fragment.

The lateral tibial eminence is intimately associated with the root attachment of the anterior horn of the lateral meniscus.^1-3 Previous studies have demonstrated both the close proximity of the anterior cruciate ligament (ACL) insertion to the meniscal roots and the potential for disruption in surgical interventions, such as tibial tunnel drilling in ACL reconstruction or placement of intramedullary tibial nails.^4-6 The meniscal roots play a crucial role in force distribution, and disruption of these structures has been shown to significantly increase joint contact forces. Despite the deleterious effects of this injury, limited studies have reported on traumatic injury to the meniscal roots in the setting of tibial eminence fractures.

Reported rates of occurrence of concomitant meniscal and chondral injuries occurring with tibial eminence fractures vary widely, ranging from <5% to 40%.^7,8 Although fractures to the tibial eminence are more common in children, an association between these injuries and concomitant soft tissue injuries, including meniscal, chondral, and collateral ligament injuries, in the adult population has been reported.⁷ Monto and Cameron-Donaldson⁸ used magnetic resonance imaging (MRI) to evaluate tibial eminence fractures in adults and found that 23% of study subjects had associated medial meniscus tears and 18% had lateral meniscus tears. In a similar study, Ishibashi and colleagues⁹ found that 25% of tibial eminence fractures were associated with lateral meniscus tears and 16% with medial meniscus tears.

These studies demonstrate the potential for meniscus injuries during tibial eminence fractures. However, the authors are unaware of any reports of complete tearing of the anterior horn of the lateral meniscus in association with this injury. This is an important injury to recognize and identify intraoperatively because an injury of this nature could potentially compromise the mechanical loading patterns and health of the articular cartilage of the lateral compartment of the knee. The purpose of this article is to describe a complete avulsion of the anterolateral meniscal root due to a tibial eminence fracture with resultant malunion and displacement of the root in a nonanatomical position. The patient provided written informed consent for print and electronic publication of this case report.

Continue to: A 28-year-old active woman...

 

CASE

A 28-year-old active woman presented to our clinic 22 months after sustaining a right knee tibial eminence fracture that was initially treated with extension immobilization, which resulted in a fibrous malunion. She subsequently sustained a second injury resulting in displacement of the malunion fracture fragment, and was treated at another institution 10 months prior to presentation at our clinic with arthroscopic reduction and internal fixation with a cannulated screw and washer of the tibial eminence fracture. This was followed by hardware removal 6 months prior to her office visit at our clinic. At presentation, she reported worsening right knee pain, mechanical symptoms, and loss of both flexion and extension compared with her uninjured knee. Conservative management, including activity modification, extensive physical therapy, and anti-inflammatory medication following her most recent procedure, had not resulted in improvement of her symptoms.

Physical examination revealed significantly reduced knee flexion and extension (+15°-120° on the affected side compared with 5° of hyperextension to 130° flexion of the contralateral knee). Ligamentous examination demonstrated no laxity with varus or valgus stress at 0° to 30° of flexion, negative posterior drawer, and a Grade 2 Lachman and positive pivot shift. She also exhibited pain with attempted right knee terminal extension. Radiographs and computed tomography scans were obtained and reviewed. They revealed a malunited tibial eminence fracture (Figures 1A-1D).  

The fragment was located anterior and lateral to its native location, which created a mechanical block during knee motion. Additionally, MRI demonstrated that the anterior horn of the lateral meniscus was displaced and attached to the malunited fragment (Figures 2A, 2B) as well as to nonfunctional ACL fibers.

  On the basis of the mechanical block restricting extension and the displaced anterior horn of the lateral meniscus compromising meniscal function, we recommended arthroscopic surgery. After discussion of the risks and benefits of the procedure with the patient, she provided informed consent, and it was decided that the patient would undergo arthroscopic fragment excision followed by anatomic repair of the anterior root of the lateral meniscus, and that we would proceed with ACL reconstruction in the future given her subjective instability and physical examination findings of ACL insufficiency.

Arthroscopic assessment of the right knee demonstrated the large osseous fragment located in the anterolateral aspect of the joint with the displaced anterior horn of the lateral meniscus attached as well as significant anterior impingement limiting knee extension. Probing of the anterolateral meniscal root in the lateral compartment showed abundant surrounding scar tissue with an abnormal attachment, representing a chronic root avulsion. A mechanical shaver was used to débride the scar tissue and expose the malunited fragment, followed by complete osseous fragment excision with a high-speed burr (Figure 3). 

The knee was taken through full range of motion (ROM) from 5° of hyperextension to 130° of flexion with arthroscopic confirmation of no further anterior impingement.

A soft tissue anterolateral meniscal root repair was performed by creating a 2-cm to 3-cm incision on the anterolateral tibia, just distal to the medial aspect of the Gerdy tubercle. To best restore the footprint of the repair and increase the potential for biologic healing, 2 transtibial tunnels were created at the location of the root attachment. An ACL aiming device with a cannulated sleeve was used to drill 2 bony tunnels approximately 5 mm apart, exiting at the anatomic root footprint. The drill pins were removed, leaving the 2 cannulas in place for later suture passage. A suture-passing device was used to pass 2 separate sutures through the detached meniscal root. 

A looped passing wire was directed up the previously placed cannulas, and 1 suture was shuttled down each tunnel. The sutures were securely tied down over a bony bridge with a cortical fixation button on the anterolateral tibia. This was visualized arthroscopically to ensure proper positioning and tension of the root to its native footprint (Figure 4).  A comparison of preoperative and postoperative anteroposterior and lateral knee radiographs is shown in Figures 5A, 5B.

Continue to: Postoperatively, the patient was placed...

 

Postoperatively, the patient was placed on a non-weight-bearing protocol for her operative lower extremity for 6 weeks. A brace locked in extension was used for the same period of time (being removed only for physical therapy exercises). Enoxaparin was used for the first 2 weeks for deep vein thrombosis prophylaxis, followed by aspirin for an additional 4 weeks. Physical therapy was started on postoperative day 1 to begin working on early passive ROM exercises. Knee flexion was limited to 0° to 90° of flexion for the first 2 weeks and then progressed as tolerated.

DISCUSSION

This article describes a rare case of a patient with lateral meniscal anterior root avulsion in the setting of a tibial eminence fracture with subsequent malunion and root displacement. In a case such as this, delineation of the true extent of the injury is difficult because the anterior meniscal root can be torn, displaced, and nonanatomically scarred to surrounding soft tissues, making MRI interpretation challenging. Clinically, patients can present with a wide range of symptoms, including pain, mechanical symptoms, instability, and loss of knee motion.¹⁰

The anterior root of the lateral meniscus has been reported to be attached anterior to the lateral tibial eminence and adjacent to the insertion of the ACL. Fibrous connections extending from the anterior horn of the lateral meniscus attachment to the lateral tibial eminence are constant.¹¹ Furumatsu and colleagues¹² demonstrated the existence of dense fibers linking the anterior root of the lateral meniscus with the lateral aspect of the ACL tibial insertion. Acknowledging the close relationship of these structures is key to comprehending the importance of evaluating the anterior horn of the lateral meniscus in cases of tibial eminence fractures at the initial time of injury. Failure to diagnose this pathology can lead to poor clinical outcomes and early degenerative changes of the knee.

Tibial intercondylar eminence avulsion fractures are most likely to occur in children and adolescents, and are equivalent to an ACL tear in adults.¹³ When tibial eminence fractures occur in an older cohort, they are often combined with lesions of the menisci, capsule, or collateral ligaments.¹⁴ The initial injury in our patient demonstrated concomitant anterior root injury that progressed with time to nonanatomical healing of the root, leading to altered biomechanics. Surgical techniques available for meniscal root repair are broadly divided into transosseous suture repairs and suture anchor repairs.¹⁰ The transtibial pullout technique using 2 transtibial bone tunnels as described in this report is the senior author’s (RFL) preference because it provides a strong construct with minimal displacement of the repaired meniscus.^15-17

This article describes a complete avulsion of the anterolateral meniscal root caused by a tibial eminence fracture with resultant malunion and displacement of the root in a nonanatomic position. Anterior meniscal root tears have been reported to result in altered biomechanics and force transmission across the knee, and therefore, anatomic repair of the anterior root is indicated.

ABSTRACT

The lateral tibial eminence shares a close relationship with the anterior root of the lateral meniscus. Limited studies have reported traumatic injury to the anterior meniscal roots in the setting of tibial eminence fractures, and reported rates of occurrence of concomitant meniscal and chondral injuries vary widely. The purpose of this article is to describe the case of a 28-year-old woman who had a complete avulsion of the anterolateral meniscal root caused by a tibial eminence fracture with resultant malunion and root displacement. The anterolateral meniscal root was anatomically repaired following arthroscopic resection of the malunited fragment.

The lateral tibial eminence is intimately associated with the root attachment of the anterior horn of the lateral meniscus.^1-3 Previous studies have demonstrated both the close proximity of the anterior cruciate ligament (ACL) insertion to the meniscal roots and the potential for disruption in surgical interventions, such as tibial tunnel drilling in ACL reconstruction or placement of intramedullary tibial nails.^4-6 The meniscal roots play a crucial role in force distribution, and disruption of these structures has been shown to significantly increase joint contact forces. Despite the deleterious effects of this injury, limited studies have reported on traumatic injury to the meniscal roots in the setting of tibial eminence fractures.

Reported rates of occurrence of concomitant meniscal and chondral injuries occurring with tibial eminence fractures vary widely, ranging from <5% to 40%.^7,8 Although fractures to the tibial eminence are more common in children, an association between these injuries and concomitant soft tissue injuries, including meniscal, chondral, and collateral ligament injuries, in the adult population has been reported.⁷ Monto and Cameron-Donaldson⁸ used magnetic resonance imaging (MRI) to evaluate tibial eminence fractures in adults and found that 23% of study subjects had associated medial meniscus tears and 18% had lateral meniscus tears. In a similar study, Ishibashi and colleagues⁹ found that 25% of tibial eminence fractures were associated with lateral meniscus tears and 16% with medial meniscus tears.

These studies demonstrate the potential for meniscus injuries during tibial eminence fractures. However, the authors are unaware of any reports of complete tearing of the anterior horn of the lateral meniscus in association with this injury. This is an important injury to recognize and identify intraoperatively because an injury of this nature could potentially compromise the mechanical loading patterns and health of the articular cartilage of the lateral compartment of the knee. The purpose of this article is to describe a complete avulsion of the anterolateral meniscal root due to a tibial eminence fracture with resultant malunion and displacement of the root in a nonanatomical position. The patient provided written informed consent for print and electronic publication of this case report.

Continue to: A 28-year-old active woman...

 

CASE

A 28-year-old active woman presented to our clinic 22 months after sustaining a right knee tibial eminence fracture that was initially treated with extension immobilization, which resulted in a fibrous malunion. She subsequently sustained a second injury resulting in displacement of the malunion fracture fragment, and was treated at another institution 10 months prior to presentation at our clinic with arthroscopic reduction and internal fixation with a cannulated screw and washer of the tibial eminence fracture. This was followed by hardware removal 6 months prior to her office visit at our clinic. At presentation, she reported worsening right knee pain, mechanical symptoms, and loss of both flexion and extension compared with her uninjured knee. Conservative management, including activity modification, extensive physical therapy, and anti-inflammatory medication following her most recent procedure, had not resulted in improvement of her symptoms.

Physical examination revealed significantly reduced knee flexion and extension (+15°-120° on the affected side compared with 5° of hyperextension to 130° flexion of the contralateral knee). Ligamentous examination demonstrated no laxity with varus or valgus stress at 0° to 30° of flexion, negative posterior drawer, and a Grade 2 Lachman and positive pivot shift. She also exhibited pain with attempted right knee terminal extension. Radiographs and computed tomography scans were obtained and reviewed. They revealed a malunited tibial eminence fracture (Figures 1A-1D).  

The fragment was located anterior and lateral to its native location, which created a mechanical block during knee motion. Additionally, MRI demonstrated that the anterior horn of the lateral meniscus was displaced and attached to the malunited fragment (Figures 2A, 2B) as well as to nonfunctional ACL fibers.

  On the basis of the mechanical block restricting extension and the displaced anterior horn of the lateral meniscus compromising meniscal function, we recommended arthroscopic surgery. After discussion of the risks and benefits of the procedure with the patient, she provided informed consent, and it was decided that the patient would undergo arthroscopic fragment excision followed by anatomic repair of the anterior root of the lateral meniscus, and that we would proceed with ACL reconstruction in the future given her subjective instability and physical examination findings of ACL insufficiency.

Arthroscopic assessment of the right knee demonstrated the large osseous fragment located in the anterolateral aspect of the joint with the displaced anterior horn of the lateral meniscus attached as well as significant anterior impingement limiting knee extension. Probing of the anterolateral meniscal root in the lateral compartment showed abundant surrounding scar tissue with an abnormal attachment, representing a chronic root avulsion. A mechanical shaver was used to débride the scar tissue and expose the malunited fragment, followed by complete osseous fragment excision with a high-speed burr (Figure 3). 

The knee was taken through full range of motion (ROM) from 5° of hyperextension to 130° of flexion with arthroscopic confirmation of no further anterior impingement.

A soft tissue anterolateral meniscal root repair was performed by creating a 2-cm to 3-cm incision on the anterolateral tibia, just distal to the medial aspect of the Gerdy tubercle. To best restore the footprint of the repair and increase the potential for biologic healing, 2 transtibial tunnels were created at the location of the root attachment. An ACL aiming device with a cannulated sleeve was used to drill 2 bony tunnels approximately 5 mm apart, exiting at the anatomic root footprint. The drill pins were removed, leaving the 2 cannulas in place for later suture passage. A suture-passing device was used to pass 2 separate sutures through the detached meniscal root. 

A looped passing wire was directed up the previously placed cannulas, and 1 suture was shuttled down each tunnel. The sutures were securely tied down over a bony bridge with a cortical fixation button on the anterolateral tibia. This was visualized arthroscopically to ensure proper positioning and tension of the root to its native footprint (Figure 4).  A comparison of preoperative and postoperative anteroposterior and lateral knee radiographs is shown in Figures 5A, 5B.

Continue to: Postoperatively, the patient was placed...

 

Postoperatively, the patient was placed on a non-weight-bearing protocol for her operative lower extremity for 6 weeks. A brace locked in extension was used for the same period of time (being removed only for physical therapy exercises). Enoxaparin was used for the first 2 weeks for deep vein thrombosis prophylaxis, followed by aspirin for an additional 4 weeks. Physical therapy was started on postoperative day 1 to begin working on early passive ROM exercises. Knee flexion was limited to 0° to 90° of flexion for the first 2 weeks and then progressed as tolerated.

DISCUSSION

This article describes a rare case of a patient with lateral meniscal anterior root avulsion in the setting of a tibial eminence fracture with subsequent malunion and root displacement. In a case such as this, delineation of the true extent of the injury is difficult because the anterior meniscal root can be torn, displaced, and nonanatomically scarred to surrounding soft tissues, making MRI interpretation challenging. Clinically, patients can present with a wide range of symptoms, including pain, mechanical symptoms, instability, and loss of knee motion.¹⁰

The anterior root of the lateral meniscus has been reported to be attached anterior to the lateral tibial eminence and adjacent to the insertion of the ACL. Fibrous connections extending from the anterior horn of the lateral meniscus attachment to the lateral tibial eminence are constant.¹¹ Furumatsu and colleagues¹² demonstrated the existence of dense fibers linking the anterior root of the lateral meniscus with the lateral aspect of the ACL tibial insertion. Acknowledging the close relationship of these structures is key to comprehending the importance of evaluating the anterior horn of the lateral meniscus in cases of tibial eminence fractures at the initial time of injury. Failure to diagnose this pathology can lead to poor clinical outcomes and early degenerative changes of the knee.

Tibial intercondylar eminence avulsion fractures are most likely to occur in children and adolescents, and are equivalent to an ACL tear in adults.¹³ When tibial eminence fractures occur in an older cohort, they are often combined with lesions of the menisci, capsule, or collateral ligaments.¹⁴ The initial injury in our patient demonstrated concomitant anterior root injury that progressed with time to nonanatomical healing of the root, leading to altered biomechanics. Surgical techniques available for meniscal root repair are broadly divided into transosseous suture repairs and suture anchor repairs.¹⁰ The transtibial pullout technique using 2 transtibial bone tunnels as described in this report is the senior author’s (RFL) preference because it provides a strong construct with minimal displacement of the repaired meniscus.^15-17

This article describes a complete avulsion of the anterolateral meniscal root caused by a tibial eminence fracture with resultant malunion and displacement of the root in a nonanatomic position. Anterior meniscal root tears have been reported to result in altered biomechanics and force transmission across the knee, and therefore, anatomic repair of the anterior root is indicated.

ABSTRACT

The lateral tibial eminence shares a close relationship with the anterior root of the lateral meniscus. Limited studies have reported traumatic injury to the anterior meniscal roots in the setting of tibial eminence fractures, and reported rates of occurrence of concomitant meniscal and chondral injuries vary widely. The purpose of this article is to describe the case of a 28-year-old woman who had a complete avulsion of the anterolateral meniscal root caused by a tibial eminence fracture with resultant malunion and root displacement. The anterolateral meniscal root was anatomically repaired following arthroscopic resection of the malunited fragment.

The lateral tibial eminence is intimately associated with the root attachment of the anterior horn of the lateral meniscus.^1-3 Previous studies have demonstrated both the close proximity of the anterior cruciate ligament (ACL) insertion to the meniscal roots and the potential for disruption in surgical interventions, such as tibial tunnel drilling in ACL reconstruction or placement of intramedullary tibial nails.^4-6 The meniscal roots play a crucial role in force distribution, and disruption of these structures has been shown to significantly increase joint contact forces. Despite the deleterious effects of this injury, limited studies have reported on traumatic injury to the meniscal roots in the setting of tibial eminence fractures.

Reported rates of occurrence of concomitant meniscal and chondral injuries occurring with tibial eminence fractures vary widely, ranging from <5% to 40%.^7,8 Although fractures to the tibial eminence are more common in children, an association between these injuries and concomitant soft tissue injuries, including meniscal, chondral, and collateral ligament injuries, in the adult population has been reported.⁷ Monto and Cameron-Donaldson⁸ used magnetic resonance imaging (MRI) to evaluate tibial eminence fractures in adults and found that 23% of study subjects had associated medial meniscus tears and 18% had lateral meniscus tears. In a similar study, Ishibashi and colleagues⁹ found that 25% of tibial eminence fractures were associated with lateral meniscus tears and 16% with medial meniscus tears.

These studies demonstrate the potential for meniscus injuries during tibial eminence fractures. However, the authors are unaware of any reports of complete tearing of the anterior horn of the lateral meniscus in association with this injury. This is an important injury to recognize and identify intraoperatively because an injury of this nature could potentially compromise the mechanical loading patterns and health of the articular cartilage of the lateral compartment of the knee. The purpose of this article is to describe a complete avulsion of the anterolateral meniscal root due to a tibial eminence fracture with resultant malunion and displacement of the root in a nonanatomical position. The patient provided written informed consent for print and electronic publication of this case report.

Continue to: A 28-year-old active woman...

 

CASE

A 28-year-old active woman presented to our clinic 22 months after sustaining a right knee tibial eminence fracture that was initially treated with extension immobilization, which resulted in a fibrous malunion. She subsequently sustained a second injury resulting in displacement of the malunion fracture fragment, and was treated at another institution 10 months prior to presentation at our clinic with arthroscopic reduction and internal fixation with a cannulated screw and washer of the tibial eminence fracture. This was followed by hardware removal 6 months prior to her office visit at our clinic. At presentation, she reported worsening right knee pain, mechanical symptoms, and loss of both flexion and extension compared with her uninjured knee. Conservative management, including activity modification, extensive physical therapy, and anti-inflammatory medication following her most recent procedure, had not resulted in improvement of her symptoms.

Physical examination revealed significantly reduced knee flexion and extension (+15°-120° on the affected side compared with 5° of hyperextension to 130° flexion of the contralateral knee). Ligamentous examination demonstrated no laxity with varus or valgus stress at 0° to 30° of flexion, negative posterior drawer, and a Grade 2 Lachman and positive pivot shift. She also exhibited pain with attempted right knee terminal extension. Radiographs and computed tomography scans were obtained and reviewed. They revealed a malunited tibial eminence fracture (Figures 1A-1D).  

The fragment was located anterior and lateral to its native location, which created a mechanical block during knee motion. Additionally, MRI demonstrated that the anterior horn of the lateral meniscus was displaced and attached to the malunited fragment (Figures 2A, 2B) as well as to nonfunctional ACL fibers.

  On the basis of the mechanical block restricting extension and the displaced anterior horn of the lateral meniscus compromising meniscal function, we recommended arthroscopic surgery. After discussion of the risks and benefits of the procedure with the patient, she provided informed consent, and it was decided that the patient would undergo arthroscopic fragment excision followed by anatomic repair of the anterior root of the lateral meniscus, and that we would proceed with ACL reconstruction in the future given her subjective instability and physical examination findings of ACL insufficiency.

Arthroscopic assessment of the right knee demonstrated the large osseous fragment located in the anterolateral aspect of the joint with the displaced anterior horn of the lateral meniscus attached as well as significant anterior impingement limiting knee extension. Probing of the anterolateral meniscal root in the lateral compartment showed abundant surrounding scar tissue with an abnormal attachment, representing a chronic root avulsion. A mechanical shaver was used to débride the scar tissue and expose the malunited fragment, followed by complete osseous fragment excision with a high-speed burr (Figure 3). 

The knee was taken through full range of motion (ROM) from 5° of hyperextension to 130° of flexion with arthroscopic confirmation of no further anterior impingement.

A soft tissue anterolateral meniscal root repair was performed by creating a 2-cm to 3-cm incision on the anterolateral tibia, just distal to the medial aspect of the Gerdy tubercle. To best restore the footprint of the repair and increase the potential for biologic healing, 2 transtibial tunnels were created at the location of the root attachment. An ACL aiming device with a cannulated sleeve was used to drill 2 bony tunnels approximately 5 mm apart, exiting at the anatomic root footprint. The drill pins were removed, leaving the 2 cannulas in place for later suture passage. A suture-passing device was used to pass 2 separate sutures through the detached meniscal root. 

A looped passing wire was directed up the previously placed cannulas, and 1 suture was shuttled down each tunnel. The sutures were securely tied down over a bony bridge with a cortical fixation button on the anterolateral tibia. This was visualized arthroscopically to ensure proper positioning and tension of the root to its native footprint (Figure 4).  A comparison of preoperative and postoperative anteroposterior and lateral knee radiographs is shown in Figures 5A, 5B.

Continue to: Postoperatively, the patient was placed...

 

Postoperatively, the patient was placed on a non-weight-bearing protocol for her operative lower extremity for 6 weeks. A brace locked in extension was used for the same period of time (being removed only for physical therapy exercises). Enoxaparin was used for the first 2 weeks for deep vein thrombosis prophylaxis, followed by aspirin for an additional 4 weeks. Physical therapy was started on postoperative day 1 to begin working on early passive ROM exercises. Knee flexion was limited to 0° to 90° of flexion for the first 2 weeks and then progressed as tolerated.

DISCUSSION

This article describes a rare case of a patient with lateral meniscal anterior root avulsion in the setting of a tibial eminence fracture with subsequent malunion and root displacement. In a case such as this, delineation of the true extent of the injury is difficult because the anterior meniscal root can be torn, displaced, and nonanatomically scarred to surrounding soft tissues, making MRI interpretation challenging. Clinically, patients can present with a wide range of symptoms, including pain, mechanical symptoms, instability, and loss of knee motion.¹⁰

The anterior root of the lateral meniscus has been reported to be attached anterior to the lateral tibial eminence and adjacent to the insertion of the ACL. Fibrous connections extending from the anterior horn of the lateral meniscus attachment to the lateral tibial eminence are constant.¹¹ Furumatsu and colleagues¹² demonstrated the existence of dense fibers linking the anterior root of the lateral meniscus with the lateral aspect of the ACL tibial insertion. Acknowledging the close relationship of these structures is key to comprehending the importance of evaluating the anterior horn of the lateral meniscus in cases of tibial eminence fractures at the initial time of injury. Failure to diagnose this pathology can lead to poor clinical outcomes and early degenerative changes of the knee.

Tibial intercondylar eminence avulsion fractures are most likely to occur in children and adolescents, and are equivalent to an ACL tear in adults.¹³ When tibial eminence fractures occur in an older cohort, they are often combined with lesions of the menisci, capsule, or collateral ligaments.¹⁴ The initial injury in our patient demonstrated concomitant anterior root injury that progressed with time to nonanatomical healing of the root, leading to altered biomechanics. Surgical techniques available for meniscal root repair are broadly divided into transosseous suture repairs and suture anchor repairs.¹⁰ The transtibial pullout technique using 2 transtibial bone tunnels as described in this report is the senior author’s (RFL) preference because it provides a strong construct with minimal displacement of the repaired meniscus.^15-17

This article describes a complete avulsion of the anterolateral meniscal root caused by a tibial eminence fracture with resultant malunion and displacement of the root in a nonanatomic position. Anterior meniscal root tears have been reported to result in altered biomechanics and force transmission across the knee, and therefore, anatomic repair of the anterior root is indicated.