Case Reports

Case Report

A 41-year-old man with no dermatologic history presented for a skin examination. During a full-body skin examination, a lesion was identified on the right third toe that was partially visible underneath the nail plate. The patient stated that the lesion had been present for many years and did not appear to be growing but did cause occasional pain. On examination a 1-cm verrucous, hyperkeratotic, tan papule was noted at the distal end of the nail bed causing partial onycholysis (Figure 1). It was not tender to palpation.

A shave biopsy was obtained of the visible portion of the lesion, which revealed hyperkeratosis, acanthosis, and a population of dermal spindle cells in a myxoid stroma that could not be definitively identified. Special stains were nondiagnostic. The patient was referred to dermatologic surgery for rebiopsy of the lesion after removal of the nail plate. Mature bone was seen embedded in the dermis (Figure 2), and a diagnosis of subungual exostosis was made. Radiography of the digit confirmed a bony excrescence from the tuft of the toe, and the patient was referred to orthopedic surgery for definitive excision. There was no evidence of recurrence at 1-year follow-up.

Comment

Subungual exostosis is an uncommon benign bone tumor located beneath or adjacent to the nail bed on the dorsal aspect of the distal phalanx.¹ Although it can occur on any digit, 70% to 80% of cases have arisen on the distal phalanx of the hallux.² Both sexes are equally susceptible. The majority of lesions occur during the second or third decades of life and usually are asymptomatic unless there is trauma or infection. Growth of the lesion over time can cause lifting or deformity of the nail plate and can cause slight discomfort while walking if located on the great toe.³ Common differential diagnoses include osteochondroma, wart, fibroma, paronychia, myositis ossificans, and pyogenic granuloma.^3,4 Diagnosis can be confirmed with radiography, which should be performed prior to any biopsy or invasive procedure. In our patient, initial radiography could have obviated the need for 2 biopsies prior to definitive excision. Histopathologic evaluation typically reveals mature trabecular bone (Figure 2) surrounded by a fibrocartilage cap.

Subungual exostosis begins as an area of proliferating fibrous tissue with cartilaginous metaplasia located beneath or adjacent to the nail bed on the dorsal aspect of the distal phalanx.¹ This cartilage undergoes enchondral ossification and is converted to trabecular bone. As the lesion grows and matures, the cartilaginous cap blends imperceptibly with the nail bed and comes into continuity with the underlying distal phalanx.^1,3 This process continues until the lesion fuses completely with the distal phalanx.¹ Although the cause of subungual exostosis has not been clearly established, chronic irritation, trauma, and chronic infections are considered causative factors of fibrocartilaginous metaplasia.⁴

Conclusion

Subungual exostosis is a benign bony tumor of the distal phalanx that can cause pain and onycholysis. Radiography of the affected digit is a noninvasive way to confirm the diagnosis and should be part of the initial workup of any suspicious subungual tumor. Once identified, complete removal of the exostosis by excision has been shown to be an effective treatment with few complications.

Case Report

A 41-year-old man with no dermatologic history presented for a skin examination. During a full-body skin examination, a lesion was identified on the right third toe that was partially visible underneath the nail plate. The patient stated that the lesion had been present for many years and did not appear to be growing but did cause occasional pain. On examination a 1-cm verrucous, hyperkeratotic, tan papule was noted at the distal end of the nail bed causing partial onycholysis (Figure 1). It was not tender to palpation.

A shave biopsy was obtained of the visible portion of the lesion, which revealed hyperkeratosis, acanthosis, and a population of dermal spindle cells in a myxoid stroma that could not be definitively identified. Special stains were nondiagnostic. The patient was referred to dermatologic surgery for rebiopsy of the lesion after removal of the nail plate. Mature bone was seen embedded in the dermis (Figure 2), and a diagnosis of subungual exostosis was made. Radiography of the digit confirmed a bony excrescence from the tuft of the toe, and the patient was referred to orthopedic surgery for definitive excision. There was no evidence of recurrence at 1-year follow-up.

Comment

Subungual exostosis is an uncommon benign bone tumor located beneath or adjacent to the nail bed on the dorsal aspect of the distal phalanx.¹ Although it can occur on any digit, 70% to 80% of cases have arisen on the distal phalanx of the hallux.² Both sexes are equally susceptible. The majority of lesions occur during the second or third decades of life and usually are asymptomatic unless there is trauma or infection. Growth of the lesion over time can cause lifting or deformity of the nail plate and can cause slight discomfort while walking if located on the great toe.³ Common differential diagnoses include osteochondroma, wart, fibroma, paronychia, myositis ossificans, and pyogenic granuloma.^3,4 Diagnosis can be confirmed with radiography, which should be performed prior to any biopsy or invasive procedure. In our patient, initial radiography could have obviated the need for 2 biopsies prior to definitive excision. Histopathologic evaluation typically reveals mature trabecular bone (Figure 2) surrounded by a fibrocartilage cap.

Subungual exostosis begins as an area of proliferating fibrous tissue with cartilaginous metaplasia located beneath or adjacent to the nail bed on the dorsal aspect of the distal phalanx.¹ This cartilage undergoes enchondral ossification and is converted to trabecular bone. As the lesion grows and matures, the cartilaginous cap blends imperceptibly with the nail bed and comes into continuity with the underlying distal phalanx.^1,3 This process continues until the lesion fuses completely with the distal phalanx.¹ Although the cause of subungual exostosis has not been clearly established, chronic irritation, trauma, and chronic infections are considered causative factors of fibrocartilaginous metaplasia.⁴

Conclusion

Subungual exostosis is a benign bony tumor of the distal phalanx that can cause pain and onycholysis. Radiography of the affected digit is a noninvasive way to confirm the diagnosis and should be part of the initial workup of any suspicious subungual tumor. Once identified, complete removal of the exostosis by excision has been shown to be an effective treatment with few complications.

Case Report

A 41-year-old man with no dermatologic history presented for a skin examination. During a full-body skin examination, a lesion was identified on the right third toe that was partially visible underneath the nail plate. The patient stated that the lesion had been present for many years and did not appear to be growing but did cause occasional pain. On examination a 1-cm verrucous, hyperkeratotic, tan papule was noted at the distal end of the nail bed causing partial onycholysis (Figure 1). It was not tender to palpation.

A shave biopsy was obtained of the visible portion of the lesion, which revealed hyperkeratosis, acanthosis, and a population of dermal spindle cells in a myxoid stroma that could not be definitively identified. Special stains were nondiagnostic. The patient was referred to dermatologic surgery for rebiopsy of the lesion after removal of the nail plate. Mature bone was seen embedded in the dermis (Figure 2), and a diagnosis of subungual exostosis was made. Radiography of the digit confirmed a bony excrescence from the tuft of the toe, and the patient was referred to orthopedic surgery for definitive excision. There was no evidence of recurrence at 1-year follow-up.

Comment

Subungual exostosis is an uncommon benign bone tumor located beneath or adjacent to the nail bed on the dorsal aspect of the distal phalanx.¹ Although it can occur on any digit, 70% to 80% of cases have arisen on the distal phalanx of the hallux.² Both sexes are equally susceptible. The majority of lesions occur during the second or third decades of life and usually are asymptomatic unless there is trauma or infection. Growth of the lesion over time can cause lifting or deformity of the nail plate and can cause slight discomfort while walking if located on the great toe.³ Common differential diagnoses include osteochondroma, wart, fibroma, paronychia, myositis ossificans, and pyogenic granuloma.^3,4 Diagnosis can be confirmed with radiography, which should be performed prior to any biopsy or invasive procedure. In our patient, initial radiography could have obviated the need for 2 biopsies prior to definitive excision. Histopathologic evaluation typically reveals mature trabecular bone (Figure 2) surrounded by a fibrocartilage cap.

Subungual exostosis begins as an area of proliferating fibrous tissue with cartilaginous metaplasia located beneath or adjacent to the nail bed on the dorsal aspect of the distal phalanx.¹ This cartilage undergoes enchondral ossification and is converted to trabecular bone. As the lesion grows and matures, the cartilaginous cap blends imperceptibly with the nail bed and comes into continuity with the underlying distal phalanx.^1,3 This process continues until the lesion fuses completely with the distal phalanx.¹ Although the cause of subungual exostosis has not been clearly established, chronic irritation, trauma, and chronic infections are considered causative factors of fibrocartilaginous metaplasia.⁴

Conclusion

Subungual exostosis is a benign bony tumor of the distal phalanx that can cause pain and onycholysis. Radiography of the affected digit is a noninvasive way to confirm the diagnosis and should be part of the initial workup of any suspicious subungual tumor. Once identified, complete removal of the exostosis by excision has been shown to be an effective treatment with few complications.

Case

A 34-year-old man presented to our ED via emergency medical services (EMS) following a syncopal episode. The patient stated that as he was getting ready for work earlier that morning, he experienced sudden lightheadedness and passed out, whereupon his wife immediately called EMS. The patient denied any previous history of syncope, but said he had been experiencing frequent episodes of nausea and vomiting over the past week. He also complained of a mild occipital headache that acetaminophen had failed to relieve.

The patient had been seen at a different ED 3 days earlier for nausea and vomiting. After evaluating the patient, the emergency physician (EP) at this facility felt the most likely cause of the patient’s gastrointestinal issues was related to hydralazine, his antihypertensive medication, and advised the patient to discontinue its use.

During evaluation at our ED, the patient denied fever, chills, neck stiffness, numbness, weakness, tingling of the extremities, or difficulty walking. He also denied chest pain, shortness of breath, or urinary symptoms. The patient’s medical history was significant only for hypertension; he had not taken any antihypertensive or other medications for the past 3 days, as previously instructed by the EP at the other ED. The patient denied alcohol or drug abuse.

On physical examination, the patient’s vital signs were: temperature, 98.6°F; heart rate, 58 beats/minute; blood pressure, 130/90 mm Hg; and respiratory rate, 16 breaths/minute. Oxygen saturation was 100% on room air. Examination of the head was normal and without evidence of trauma. Both pupils measured 4 mm and were equally round and reactive to light; the patient’s extraocular movements were intact. The remainder of the head, eyes, ears, nose, and throat examination was normal. The neck was supple, without masses or meningeal signs. The cardiopulmonary and abdominal examinations were all normal. On neurological examination, the patient was awake, alert, and oriented to person, place, and time. Cranial nerves II through XII were intact, and the patient had 5/5 motor strength in all four extremities and a normal gait.

Because we were concerned about the patient’s unexplained syncopal episode, we ordered laboratory tests, including a complete blood count (CBC), evaluation of electrolytes and glucose levels, and kidney function. In addition, we also ordered an electrocardiogram (ECG) and a noncontrast computed tomography (CT) scan of the head. All laboratory test results were within normal range. The ECG, however, demonstrated sinus bradycardia (approximately 58 beats/minute), a normal PR and QRS interval, a normal axis, and an incomplete right bundle branch block with tall, large, splayed upright T waves in the precordial leads (Figure). Based on the abnormal ECG results, we ordered serum cardiac marker studies, the values of which were all within normal range. The noncontrast CT scan of the head revealed a low-density posterior fossa mass compressing the fourth ventricle with secondary hydrocephalus.

The patient was placed with his head in an upright position and given 1 g/kg mannitol and 10 mg dexamethasone intravenously (IV). Neurosurgery services were consulted, and the patient underwent surgery the following morning. Surgery confirmed the presence of a hemangioblastoma. The hemangioblastoma was successfully excised, and the patient had an uneventful recovery. Interestingly, the significant T-wave changes in the precordial leads were no longer present postoperatively.

Discussion

Syncope and near-syncope are common reasons for ED visits. Syncope is a syndrome characterized by a transient, self-limited episode of loss of consciousness occurring as a result of a brief interruption of the oxygen supply to the brain.¹ This interruption is almost always due to a transient cessation of blood flow.¹ In true syncope (as opposed to seizures or hypoglycemia), the episode is characterized by a rapid onset of loss of consciousness—with or without warning symptoms.¹ It is important to determine the cause of syncope, because 7% to 23% of such patients will suffer serious outcomes within 7 to 30 days of their ED visit—either within a hospital setting or at home.²

Etiology

There are many causes of syncope. In most cases, the etiology falls under one of three broad categories: neurally mediated (or reflex mediated), orthostatic hypotensive-mediated, or cardiovascular (CV)-mediated. Less common causes of syncope include cerebrovascular injury.¹ The Table outlines both common and uncommon causes of syncope.

On presentation, our patient had several possible causes for his syncopal episode: an abnormal ECG (CV); multiple episodes of emesis (volume depletion); and headache (cerebrovascular). The EP worked up all three of these signs and symptoms simultaneously as is the appropriate protocol when evaluating an ED patient presenting with undifferentiated syncope.

Signs and Symptoms

Patients with undiagnosed brain tumors normally present with headache, seizures, nausea, vomiting, focal neurological deficits, or an altered mental status.³ Syncope is a very rare manifestation of a brain tumor³; however, our patient did complain of headache, nausea, and vomiting.

In addition to the unusual cause of the syncope, the abnormally large upright T waves make this case even more notable. T-wave changes are the most common ECG abnormality, seen in about 50% of abnormal tracings reviewed in a hospital population and in 2.4% of all ECGs.⁴

In general, T-wave changes are a result of local changes in the duration of repolarization. T-wave inversion is the most common T-wave abnormality and is typically observed in the setting of ischemia, post-ingestion of food, following an episode of tachycardia or anxiety, and autonomic dysfunction.⁵ However, in patients who have a cerebral etiology (usually hemorrhage), the T-wave changes may be either upright (as in our case) or inverted.⁵ Historically, subarachnoid hemorrhage (SAH) has been associated with ST-segment elevation and T-wave inversion. Hypothalamic stimulation and autonomic dysfunction have been linked to abnormal T-waves, but this has not been conclusively proven to be the cause of the abnormality.⁶ For all of the aforementioned reasons, the specificity for a given cause of T-wave changes is exceedingly low.⁵

Hyperacute T-wave amplitude, with prominent symmetrical T waves in at least two continuous leads, may be the earliest sign of acute transmural myocardial infarction (MI).⁷ It usually persists for only a brief time before other ECG findings of acute MI are observed. Other common causes of hyperacute T waves include hyperkalemia (usually narrow-based, and peaked), early repolarization, left ventricular hypertrophy, and acute myocarditis.⁸ Less common causes of prominent T waves include pre-excitation  syndromes, pericarditis, and scorpion stings.^9,10

Summary

It is unclear why our patient, who had a hemangioblastoma, demonstrated hyperacute T-wave abnormality on ECG. The abnormal upright T waves may have occurred secondary to the same theories regarding SAH, hypothalamic stimulation, or autonomic dysfunction. Regardless of the underlying etiology, this case serves as a reminder to the EP that not all T-wave changes on ECG are cardiac in origin. 

Case

A 34-year-old man presented to our ED via emergency medical services (EMS) following a syncopal episode. The patient stated that as he was getting ready for work earlier that morning, he experienced sudden lightheadedness and passed out, whereupon his wife immediately called EMS. The patient denied any previous history of syncope, but said he had been experiencing frequent episodes of nausea and vomiting over the past week. He also complained of a mild occipital headache that acetaminophen had failed to relieve.

The patient had been seen at a different ED 3 days earlier for nausea and vomiting. After evaluating the patient, the emergency physician (EP) at this facility felt the most likely cause of the patient’s gastrointestinal issues was related to hydralazine, his antihypertensive medication, and advised the patient to discontinue its use.

During evaluation at our ED, the patient denied fever, chills, neck stiffness, numbness, weakness, tingling of the extremities, or difficulty walking. He also denied chest pain, shortness of breath, or urinary symptoms. The patient’s medical history was significant only for hypertension; he had not taken any antihypertensive or other medications for the past 3 days, as previously instructed by the EP at the other ED. The patient denied alcohol or drug abuse.

On physical examination, the patient’s vital signs were: temperature, 98.6°F; heart rate, 58 beats/minute; blood pressure, 130/90 mm Hg; and respiratory rate, 16 breaths/minute. Oxygen saturation was 100% on room air. Examination of the head was normal and without evidence of trauma. Both pupils measured 4 mm and were equally round and reactive to light; the patient’s extraocular movements were intact. The remainder of the head, eyes, ears, nose, and throat examination was normal. The neck was supple, without masses or meningeal signs. The cardiopulmonary and abdominal examinations were all normal. On neurological examination, the patient was awake, alert, and oriented to person, place, and time. Cranial nerves II through XII were intact, and the patient had 5/5 motor strength in all four extremities and a normal gait.

Because we were concerned about the patient’s unexplained syncopal episode, we ordered laboratory tests, including a complete blood count (CBC), evaluation of electrolytes and glucose levels, and kidney function. In addition, we also ordered an electrocardiogram (ECG) and a noncontrast computed tomography (CT) scan of the head. All laboratory test results were within normal range. The ECG, however, demonstrated sinus bradycardia (approximately 58 beats/minute), a normal PR and QRS interval, a normal axis, and an incomplete right bundle branch block with tall, large, splayed upright T waves in the precordial leads (Figure). Based on the abnormal ECG results, we ordered serum cardiac marker studies, the values of which were all within normal range. The noncontrast CT scan of the head revealed a low-density posterior fossa mass compressing the fourth ventricle with secondary hydrocephalus.

The patient was placed with his head in an upright position and given 1 g/kg mannitol and 10 mg dexamethasone intravenously (IV). Neurosurgery services were consulted, and the patient underwent surgery the following morning. Surgery confirmed the presence of a hemangioblastoma. The hemangioblastoma was successfully excised, and the patient had an uneventful recovery. Interestingly, the significant T-wave changes in the precordial leads were no longer present postoperatively.

Discussion

Syncope and near-syncope are common reasons for ED visits. Syncope is a syndrome characterized by a transient, self-limited episode of loss of consciousness occurring as a result of a brief interruption of the oxygen supply to the brain.¹ This interruption is almost always due to a transient cessation of blood flow.¹ In true syncope (as opposed to seizures or hypoglycemia), the episode is characterized by a rapid onset of loss of consciousness—with or without warning symptoms.¹ It is important to determine the cause of syncope, because 7% to 23% of such patients will suffer serious outcomes within 7 to 30 days of their ED visit—either within a hospital setting or at home.²

Etiology

There are many causes of syncope. In most cases, the etiology falls under one of three broad categories: neurally mediated (or reflex mediated), orthostatic hypotensive-mediated, or cardiovascular (CV)-mediated. Less common causes of syncope include cerebrovascular injury.¹ The Table outlines both common and uncommon causes of syncope.

On presentation, our patient had several possible causes for his syncopal episode: an abnormal ECG (CV); multiple episodes of emesis (volume depletion); and headache (cerebrovascular). The EP worked up all three of these signs and symptoms simultaneously as is the appropriate protocol when evaluating an ED patient presenting with undifferentiated syncope.

Signs and Symptoms

Patients with undiagnosed brain tumors normally present with headache, seizures, nausea, vomiting, focal neurological deficits, or an altered mental status.³ Syncope is a very rare manifestation of a brain tumor³; however, our patient did complain of headache, nausea, and vomiting.

In addition to the unusual cause of the syncope, the abnormally large upright T waves make this case even more notable. T-wave changes are the most common ECG abnormality, seen in about 50% of abnormal tracings reviewed in a hospital population and in 2.4% of all ECGs.⁴

In general, T-wave changes are a result of local changes in the duration of repolarization. T-wave inversion is the most common T-wave abnormality and is typically observed in the setting of ischemia, post-ingestion of food, following an episode of tachycardia or anxiety, and autonomic dysfunction.⁵ However, in patients who have a cerebral etiology (usually hemorrhage), the T-wave changes may be either upright (as in our case) or inverted.⁵ Historically, subarachnoid hemorrhage (SAH) has been associated with ST-segment elevation and T-wave inversion. Hypothalamic stimulation and autonomic dysfunction have been linked to abnormal T-waves, but this has not been conclusively proven to be the cause of the abnormality.⁶ For all of the aforementioned reasons, the specificity for a given cause of T-wave changes is exceedingly low.⁵

Hyperacute T-wave amplitude, with prominent symmetrical T waves in at least two continuous leads, may be the earliest sign of acute transmural myocardial infarction (MI).⁷ It usually persists for only a brief time before other ECG findings of acute MI are observed. Other common causes of hyperacute T waves include hyperkalemia (usually narrow-based, and peaked), early repolarization, left ventricular hypertrophy, and acute myocarditis.⁸ Less common causes of prominent T waves include pre-excitation  syndromes, pericarditis, and scorpion stings.^9,10

Summary

It is unclear why our patient, who had a hemangioblastoma, demonstrated hyperacute T-wave abnormality on ECG. The abnormal upright T waves may have occurred secondary to the same theories regarding SAH, hypothalamic stimulation, or autonomic dysfunction. Regardless of the underlying etiology, this case serves as a reminder to the EP that not all T-wave changes on ECG are cardiac in origin. 

Case

A 34-year-old man presented to our ED via emergency medical services (EMS) following a syncopal episode. The patient stated that as he was getting ready for work earlier that morning, he experienced sudden lightheadedness and passed out, whereupon his wife immediately called EMS. The patient denied any previous history of syncope, but said he had been experiencing frequent episodes of nausea and vomiting over the past week. He also complained of a mild occipital headache that acetaminophen had failed to relieve.

The patient had been seen at a different ED 3 days earlier for nausea and vomiting. After evaluating the patient, the emergency physician (EP) at this facility felt the most likely cause of the patient’s gastrointestinal issues was related to hydralazine, his antihypertensive medication, and advised the patient to discontinue its use.

During evaluation at our ED, the patient denied fever, chills, neck stiffness, numbness, weakness, tingling of the extremities, or difficulty walking. He also denied chest pain, shortness of breath, or urinary symptoms. The patient’s medical history was significant only for hypertension; he had not taken any antihypertensive or other medications for the past 3 days, as previously instructed by the EP at the other ED. The patient denied alcohol or drug abuse.

On physical examination, the patient’s vital signs were: temperature, 98.6°F; heart rate, 58 beats/minute; blood pressure, 130/90 mm Hg; and respiratory rate, 16 breaths/minute. Oxygen saturation was 100% on room air. Examination of the head was normal and without evidence of trauma. Both pupils measured 4 mm and were equally round and reactive to light; the patient’s extraocular movements were intact. The remainder of the head, eyes, ears, nose, and throat examination was normal. The neck was supple, without masses or meningeal signs. The cardiopulmonary and abdominal examinations were all normal. On neurological examination, the patient was awake, alert, and oriented to person, place, and time. Cranial nerves II through XII were intact, and the patient had 5/5 motor strength in all four extremities and a normal gait.

Because we were concerned about the patient’s unexplained syncopal episode, we ordered laboratory tests, including a complete blood count (CBC), evaluation of electrolytes and glucose levels, and kidney function. In addition, we also ordered an electrocardiogram (ECG) and a noncontrast computed tomography (CT) scan of the head. All laboratory test results were within normal range. The ECG, however, demonstrated sinus bradycardia (approximately 58 beats/minute), a normal PR and QRS interval, a normal axis, and an incomplete right bundle branch block with tall, large, splayed upright T waves in the precordial leads (Figure). Based on the abnormal ECG results, we ordered serum cardiac marker studies, the values of which were all within normal range. The noncontrast CT scan of the head revealed a low-density posterior fossa mass compressing the fourth ventricle with secondary hydrocephalus.

The patient was placed with his head in an upright position and given 1 g/kg mannitol and 10 mg dexamethasone intravenously (IV). Neurosurgery services were consulted, and the patient underwent surgery the following morning. Surgery confirmed the presence of a hemangioblastoma. The hemangioblastoma was successfully excised, and the patient had an uneventful recovery. Interestingly, the significant T-wave changes in the precordial leads were no longer present postoperatively.

Discussion

Syncope and near-syncope are common reasons for ED visits. Syncope is a syndrome characterized by a transient, self-limited episode of loss of consciousness occurring as a result of a brief interruption of the oxygen supply to the brain.¹ This interruption is almost always due to a transient cessation of blood flow.¹ In true syncope (as opposed to seizures or hypoglycemia), the episode is characterized by a rapid onset of loss of consciousness—with or without warning symptoms.¹ It is important to determine the cause of syncope, because 7% to 23% of such patients will suffer serious outcomes within 7 to 30 days of their ED visit—either within a hospital setting or at home.²

Etiology

There are many causes of syncope. In most cases, the etiology falls under one of three broad categories: neurally mediated (or reflex mediated), orthostatic hypotensive-mediated, or cardiovascular (CV)-mediated. Less common causes of syncope include cerebrovascular injury.¹ The Table outlines both common and uncommon causes of syncope.

On presentation, our patient had several possible causes for his syncopal episode: an abnormal ECG (CV); multiple episodes of emesis (volume depletion); and headache (cerebrovascular). The EP worked up all three of these signs and symptoms simultaneously as is the appropriate protocol when evaluating an ED patient presenting with undifferentiated syncope.

Signs and Symptoms

Patients with undiagnosed brain tumors normally present with headache, seizures, nausea, vomiting, focal neurological deficits, or an altered mental status.³ Syncope is a very rare manifestation of a brain tumor³; however, our patient did complain of headache, nausea, and vomiting.

In addition to the unusual cause of the syncope, the abnormally large upright T waves make this case even more notable. T-wave changes are the most common ECG abnormality, seen in about 50% of abnormal tracings reviewed in a hospital population and in 2.4% of all ECGs.⁴

In general, T-wave changes are a result of local changes in the duration of repolarization. T-wave inversion is the most common T-wave abnormality and is typically observed in the setting of ischemia, post-ingestion of food, following an episode of tachycardia or anxiety, and autonomic dysfunction.⁵ However, in patients who have a cerebral etiology (usually hemorrhage), the T-wave changes may be either upright (as in our case) or inverted.⁵ Historically, subarachnoid hemorrhage (SAH) has been associated with ST-segment elevation and T-wave inversion. Hypothalamic stimulation and autonomic dysfunction have been linked to abnormal T-waves, but this has not been conclusively proven to be the cause of the abnormality.⁶ For all of the aforementioned reasons, the specificity for a given cause of T-wave changes is exceedingly low.⁵

Hyperacute T-wave amplitude, with prominent symmetrical T waves in at least two continuous leads, may be the earliest sign of acute transmural myocardial infarction (MI).⁷ It usually persists for only a brief time before other ECG findings of acute MI are observed. Other common causes of hyperacute T waves include hyperkalemia (usually narrow-based, and peaked), early repolarization, left ventricular hypertrophy, and acute myocarditis.⁸ Less common causes of prominent T waves include pre-excitation  syndromes, pericarditis, and scorpion stings.^9,10

Summary

It is unclear why our patient, who had a hemangioblastoma, demonstrated hyperacute T-wave abnormality on ECG. The abnormal upright T waves may have occurred secondary to the same theories regarding SAH, hypothalamic stimulation, or autonomic dysfunction. Regardless of the underlying etiology, this case serves as a reminder to the EP that not all T-wave changes on ECG are cardiac in origin. 

Traumatic eye injuries ranging from mild corneal abrasions to penetrating globe injuries are commonly seen in the ED, and ocular trauma accounts for nearly 1% of all complaints in the ED.¹ Up to 29% of facial fractures have associated eye injuries.² Emergency physicians (EPs) must be aware of possible eye injuries, including traumatic vision loss, and the indicators for emergent interventions.

Tension pneumo-orbitum following facial trauma is rarely reported. We present a case of orbital compartment syndrome (OCS) in an elderly woman who sustained an orbital floor fracture and required emergent lateral canthotomy to preserve vision.

Case

A 76-year-old woman presented to the ED for evaluation of pain, swelling, and loss of vision in the right eye. She said she had been sitting in a chair tying her shoes when she lost her balance and fell forward, striking her head and the right side of her face against the floor. She experienced no loss of consciousness and denied any neck pain, jaw pain, or dizziness. She also denied any chest pain, shortness of breath, weakness, or loss of function in either her arms or legs. She did, however, note a small nosebleed that had stopped before she arrived at the ED. The patient’s primary complaint was a possible nasal bone fracture.

Her medical history was significant for hypertension and coronary artery disease. Her medications include amitriptyline, an antihistamine, aspirin, clopidogrel, diltiazem, folic acid, furosemide, hydralazine, levothyroxine, prednisone, and zolpidem. She stated that she was allergic to amoxicillin and sulfa drugs.

The patient’s vital signs at presentation were: blood pressure (BP), 193/82 mm Hg; heart rate, 71 beats/minute; respiratory rate, 16 breaths/minute; and temperature, 97°F. She was alert, oriented, and in no distress. Her head and neck examination showed no scalp lacerations or swelling. There was, however, significant swelling and ecchymosis around the right eye and swelling and ecchymosis around the nose, with dried blood in both nares. No septal hematoma was present. The patient had tenderness to palpation over the infraorbital area and nose. No gross facial instability was present, and Battle sign was not appreciated. No jaw or dental abnormalities were noted.

The patient’s right pupil was fixed and dilated, and she could not perceive light. She did have upward and lateral movement of the eye, but was unable to look down. A minimal amount of proptosis was noted. Her intraocular pressure (IOP) was elevated at 54 mm Hg (normal range, 10-20 mm Hg). The remainder of the examination, including the neurological examination, was unremarkable.

The patient received emergent head and facial computed tomography (CT) scans. The head CT showed no acute intracranial hemorrhage, mass, or infarct. The facial CT was read as a right orbital floor fracture with intraorbital air, and a right maxillary sinus hematoma. Laboratory evaluation revealed a hematocrit of 38% and a platelet count of 544,000/mcL (normal range, 150,000-450,000/mcL). The prothrombin time was 10.9 seconds (normal range, 11-13.5 seconds); the international normalized ratio was 0.8 (normal range, 0.8-1.1); and the partial thromboplastin time was 22.5 seconds (normal range, 25-35 seconds).

Because the patient was at risk for permanent visual impairment due to increased IOP from the injury, a lateral canthotomy was immediately performed. A small amount of air was released, and the proptosis was notably reduced.

At this point, the ophthalmologist arrived and used an 18-gauge needle to explore the retrobulbar space. Two pockets of air were released, which markedly reduced the tactile pressure of the globe. Repeat tonography of the globe was 28 mm Hg. The wound was left open to drain, and the patient was started on azithromycin. She was discharged home to follow up with ophthalmology.

The patient presented to the ED 2 months later for an unrelated condition. At that time, she reported a complete return of her vision with no deficits and no noticeable scarring around the eye.

Discussion

The orbit is an enclosed space, bordered by bone laterally and posteriorly—the orbital septa superiorly and inferiorly, and the globe anteriorly.³ The lateral canthus is a combined tendon-ligament that helps attach the tarsal plates of the lids and the orbicularis oculi muscles to the lateral orbital wall and zygoma, which forms the posterior orbital wall.^3,4 The lateral canthal tendon is located beneath the lateral canthus and is comprised of the inferior and superior crus, which attaches to the inner aspect of the lateral orbital wall, forming a structure called Whitnall’s tubercle.^3,4

Other than globe injuries, the most common findings in patients with orbital trauma are periocular lacerations (96%), orbital fractures (16%), and retrobulbar hemorrhage (8%).⁵ The most common cause of retrobulbar hemorrhage is ocular trauma, but it is also observed in facial fractures, orbital surgery, retrobulbar injections, venous anomalies, atherosclerosis, intraorbital aneurysm of the ophthalmic artery, lacerated ophthalmic artery, hypertension, hemophilia, leukemia, von Willebrand disease, and straining.^3,6,7

In retrobulbar hemorrhage, an increased pressure in the orbital space can lead to optic nerve compression and vascular compromise.⁶ Important alternative diagnoses to consider include orbital cellulitis, orbital fracture, and globe rupture.³ Retrobulbar hemorrhage should be suspected in the clinical setting of exophthalmos, proptosis, diffuse subconjunctival hemorrhage, pain, visual loss or diplopia, periorbital edema, partial or complete ophthalmoplegia, resistance to retropulsion, increased IOP, a blanched ophthalmic artery on funduscopic examination, and an afferent pupillary defect.^3,4,8,9 Less commonly, periorbital crepitus and infraorbital hypoesthesia can be appreciated.³

If a patient with a retrobulbar hemorrhage is experiencing diminished vision, an emergent lateral canthotomy should be attempted. Retrobulbar hemorrhage can be difficult to diagnose in the setting of trauma. There can be damage to the optic nerve with associated edema and vision loss that is not associated with a retrobulbar hemorrhage and does not require a lateral canthotomy.^3,7 A dedicated CT scan of the orbits can aid in the diagnosis, but treatment should not be delayed.⁸

Patients with retrobulbar hemorrhage may initially present to the ED with intact visual acuity, but as the pressure behind the globe increases, vision will diminish.³ Although the medical literature has not established a definitive timeframe, it is believed that permanent visual compromise develops between 1 to 3 hours after ischemia develops.⁶ Animal studies show that visual loss due to central retinal artery ischemia may be reversible up to 100 minutes.³

Not all cases of retrobulbar hemorrhage are associated with vision loss. In patients without diminished vision, conservative treatments such as bed rest, elevation of the head of the bed, ice packs, analgesia, lowering BP, and sedatives should be attempted first.⁵ Acetazolamide and mannitol can also be considered in consultation with an ophthalmologist.

Pneumo-orbitum

The presence of pneumo-orbitum should alert the clinician to either a communication with a paranasal sinus, a gas-forming organism, or (rarely) Munchausen syndrome.¹⁰ Unlike most case presentations, most causes of pneumo-orbitum do not involve OCS and are self-limited.¹¹ Traumatic pneumo-orbitum without OCS has been reported in the literature.^12-15 However, traumatic tension pneumo-orbitum is rare.^12-16 One case report involved an elderly man with an orbital floor fracture who developed recurrent tension pneumo-orbitum after blowing his nose while intoxicated.¹² Another case involved a boy with tension pneumo-orbitum that required surgical decompression.¹⁶

In a patient who has experienced trauma, the combination of proptosis, elevated IOP, and vision loss likely represent a retrobulbar hematoma or OCS. A lateral canthotomy can help relieve IOP from either condition.^17,18 Orbital compartment syndrome can be caused by edema, emphysema, and caroticocavernous fistula, leading to increased orbital pressure and decreased perfusion.^17,18

In a review of 10 trauma patients with OCS, all cases were intubated due to the severity of the head trauma, and all had OCS due to edema.¹⁷ In a review of eight trauma patients with OCS, all had eye pain, reduced visual acuity, and proptosis.¹⁸ Most of the patients had periorbital edema, ophthalmoparesis, a relative afferent pupillary defect (as compared to a fixed and dilated pupil), and chemosis.^18,19 All of the patients with OCS required cantholysis or a lateral canthotomy.¹⁸

Lateral Canthotomy

Although EPs rarely perform lateral canthotomy, knowledge of this procedure is important, because it can prevent vision loss in the appropriate clinical setting. To perform a lateral canthotomy, the area around the affected eye is cleaned with saline irrigation.⁴ One percent or 2% lidocaine with epinephrine is then injected into the lateral canthus of the affected eye.^4,10 A straight hemostat is applied between the upper and lower lids, producing a crush injury along the site of local anesthesia for 1 to 2 minutes.^3,4,10 This is done to reduce the risk of bleeding by devitalizing the tissue.⁴ Straight scissors are then used to make a 1-cm horizontal incision from the lateral canthal tendon to the lateral orbital rim.⁴ This initial incision exposes the orbicularis muscle, orbital septum, palpebral conjunctiva, and an area called Eisler’s pocket that sits anterior to the lateral canthal tendon.³

Cantholysis can then be performed by blunt dissection.¹⁰ The inferior crus of the lateral canthus is identified either visually or by palpation, and a 1- to 2-cm inferior-posterior cut of the inferior crus accomplishes the lateral canthotomy.^3-4 After cutting the inferior crus, the lower lid should be pulled away easily, and if this does not occur, repeated attempts at cutting the inferior crus should be made.³ Pulling the lower eyelid down and away from the lateral orbital rim separates the skin and conjunctiva, aiding in visualization.⁴

After cutting the inferior crus, only a small amount of blood or air typically is expressed, but this is usually enough to prevent vision loss.³ When the procedure is performed correctly, the practitioner should be able to palpate a difference in the pressure of the globe, and tonography will show a reduced IOP. If the ocular pressure is still significantly elevated, the physician can proceed to cut the superior canthus of the lateral canthal tendon in a manner similar to cutting the inferior crus of the tendon.⁴ After the procedure is performed, urgent ophthalmologic consultation is required.

The risks of performing a lateral canthotomy include mechanical injury, hemorrhage, and infection.⁴ The incision from a lateral canthotomy generally does not need suturing and will heal without significant scarring.⁴ If the scissors are aimed superiorly instead of inferiorly for the inferior crus of the lateral canthal tendon, there is risk of injuring the levator aponeurosis leading to ptosis, as well as a small risk of injury to the lacrimal gland and lacrimal artery.³

Conclusion

Our patient demonstrates a case of traumatic OCS, a vision-threatening medical condition that requires rapid diagnosis and lateral canthotomy to lower IOP and reduce the risk of permanent vision loss. While an orbital CT scan may assist in confirming the diagnosis, treatment of IOP should not be delayed.

Traumatic eye injuries ranging from mild corneal abrasions to penetrating globe injuries are commonly seen in the ED, and ocular trauma accounts for nearly 1% of all complaints in the ED.¹ Up to 29% of facial fractures have associated eye injuries.² Emergency physicians (EPs) must be aware of possible eye injuries, including traumatic vision loss, and the indicators for emergent interventions.

Tension pneumo-orbitum following facial trauma is rarely reported. We present a case of orbital compartment syndrome (OCS) in an elderly woman who sustained an orbital floor fracture and required emergent lateral canthotomy to preserve vision.

Case

A 76-year-old woman presented to the ED for evaluation of pain, swelling, and loss of vision in the right eye. She said she had been sitting in a chair tying her shoes when she lost her balance and fell forward, striking her head and the right side of her face against the floor. She experienced no loss of consciousness and denied any neck pain, jaw pain, or dizziness. She also denied any chest pain, shortness of breath, weakness, or loss of function in either her arms or legs. She did, however, note a small nosebleed that had stopped before she arrived at the ED. The patient’s primary complaint was a possible nasal bone fracture.

Her medical history was significant for hypertension and coronary artery disease. Her medications include amitriptyline, an antihistamine, aspirin, clopidogrel, diltiazem, folic acid, furosemide, hydralazine, levothyroxine, prednisone, and zolpidem. She stated that she was allergic to amoxicillin and sulfa drugs.

The patient’s vital signs at presentation were: blood pressure (BP), 193/82 mm Hg; heart rate, 71 beats/minute; respiratory rate, 16 breaths/minute; and temperature, 97°F. She was alert, oriented, and in no distress. Her head and neck examination showed no scalp lacerations or swelling. There was, however, significant swelling and ecchymosis around the right eye and swelling and ecchymosis around the nose, with dried blood in both nares. No septal hematoma was present. The patient had tenderness to palpation over the infraorbital area and nose. No gross facial instability was present, and Battle sign was not appreciated. No jaw or dental abnormalities were noted.

The patient’s right pupil was fixed and dilated, and she could not perceive light. She did have upward and lateral movement of the eye, but was unable to look down. A minimal amount of proptosis was noted. Her intraocular pressure (IOP) was elevated at 54 mm Hg (normal range, 10-20 mm Hg). The remainder of the examination, including the neurological examination, was unremarkable.

The patient received emergent head and facial computed tomography (CT) scans. The head CT showed no acute intracranial hemorrhage, mass, or infarct. The facial CT was read as a right orbital floor fracture with intraorbital air, and a right maxillary sinus hematoma. Laboratory evaluation revealed a hematocrit of 38% and a platelet count of 544,000/mcL (normal range, 150,000-450,000/mcL). The prothrombin time was 10.9 seconds (normal range, 11-13.5 seconds); the international normalized ratio was 0.8 (normal range, 0.8-1.1); and the partial thromboplastin time was 22.5 seconds (normal range, 25-35 seconds).

Because the patient was at risk for permanent visual impairment due to increased IOP from the injury, a lateral canthotomy was immediately performed. A small amount of air was released, and the proptosis was notably reduced.

At this point, the ophthalmologist arrived and used an 18-gauge needle to explore the retrobulbar space. Two pockets of air were released, which markedly reduced the tactile pressure of the globe. Repeat tonography of the globe was 28 mm Hg. The wound was left open to drain, and the patient was started on azithromycin. She was discharged home to follow up with ophthalmology.

The patient presented to the ED 2 months later for an unrelated condition. At that time, she reported a complete return of her vision with no deficits and no noticeable scarring around the eye.

Discussion

The orbit is an enclosed space, bordered by bone laterally and posteriorly—the orbital septa superiorly and inferiorly, and the globe anteriorly.³ The lateral canthus is a combined tendon-ligament that helps attach the tarsal plates of the lids and the orbicularis oculi muscles to the lateral orbital wall and zygoma, which forms the posterior orbital wall.^3,4 The lateral canthal tendon is located beneath the lateral canthus and is comprised of the inferior and superior crus, which attaches to the inner aspect of the lateral orbital wall, forming a structure called Whitnall’s tubercle.^3,4

Other than globe injuries, the most common findings in patients with orbital trauma are periocular lacerations (96%), orbital fractures (16%), and retrobulbar hemorrhage (8%).⁵ The most common cause of retrobulbar hemorrhage is ocular trauma, but it is also observed in facial fractures, orbital surgery, retrobulbar injections, venous anomalies, atherosclerosis, intraorbital aneurysm of the ophthalmic artery, lacerated ophthalmic artery, hypertension, hemophilia, leukemia, von Willebrand disease, and straining.^3,6,7

In retrobulbar hemorrhage, an increased pressure in the orbital space can lead to optic nerve compression and vascular compromise.⁶ Important alternative diagnoses to consider include orbital cellulitis, orbital fracture, and globe rupture.³ Retrobulbar hemorrhage should be suspected in the clinical setting of exophthalmos, proptosis, diffuse subconjunctival hemorrhage, pain, visual loss or diplopia, periorbital edema, partial or complete ophthalmoplegia, resistance to retropulsion, increased IOP, a blanched ophthalmic artery on funduscopic examination, and an afferent pupillary defect.^3,4,8,9 Less commonly, periorbital crepitus and infraorbital hypoesthesia can be appreciated.³

If a patient with a retrobulbar hemorrhage is experiencing diminished vision, an emergent lateral canthotomy should be attempted. Retrobulbar hemorrhage can be difficult to diagnose in the setting of trauma. There can be damage to the optic nerve with associated edema and vision loss that is not associated with a retrobulbar hemorrhage and does not require a lateral canthotomy.^3,7 A dedicated CT scan of the orbits can aid in the diagnosis, but treatment should not be delayed.⁸

Patients with retrobulbar hemorrhage may initially present to the ED with intact visual acuity, but as the pressure behind the globe increases, vision will diminish.³ Although the medical literature has not established a definitive timeframe, it is believed that permanent visual compromise develops between 1 to 3 hours after ischemia develops.⁶ Animal studies show that visual loss due to central retinal artery ischemia may be reversible up to 100 minutes.³

Not all cases of retrobulbar hemorrhage are associated with vision loss. In patients without diminished vision, conservative treatments such as bed rest, elevation of the head of the bed, ice packs, analgesia, lowering BP, and sedatives should be attempted first.⁵ Acetazolamide and mannitol can also be considered in consultation with an ophthalmologist.

Pneumo-orbitum

The presence of pneumo-orbitum should alert the clinician to either a communication with a paranasal sinus, a gas-forming organism, or (rarely) Munchausen syndrome.¹⁰ Unlike most case presentations, most causes of pneumo-orbitum do not involve OCS and are self-limited.¹¹ Traumatic pneumo-orbitum without OCS has been reported in the literature.^12-15 However, traumatic tension pneumo-orbitum is rare.^12-16 One case report involved an elderly man with an orbital floor fracture who developed recurrent tension pneumo-orbitum after blowing his nose while intoxicated.¹² Another case involved a boy with tension pneumo-orbitum that required surgical decompression.¹⁶

In a patient who has experienced trauma, the combination of proptosis, elevated IOP, and vision loss likely represent a retrobulbar hematoma or OCS. A lateral canthotomy can help relieve IOP from either condition.^17,18 Orbital compartment syndrome can be caused by edema, emphysema, and caroticocavernous fistula, leading to increased orbital pressure and decreased perfusion.^17,18

In a review of 10 trauma patients with OCS, all cases were intubated due to the severity of the head trauma, and all had OCS due to edema.¹⁷ In a review of eight trauma patients with OCS, all had eye pain, reduced visual acuity, and proptosis.¹⁸ Most of the patients had periorbital edema, ophthalmoparesis, a relative afferent pupillary defect (as compared to a fixed and dilated pupil), and chemosis.^18,19 All of the patients with OCS required cantholysis or a lateral canthotomy.¹⁸

Lateral Canthotomy

Although EPs rarely perform lateral canthotomy, knowledge of this procedure is important, because it can prevent vision loss in the appropriate clinical setting. To perform a lateral canthotomy, the area around the affected eye is cleaned with saline irrigation.⁴ One percent or 2% lidocaine with epinephrine is then injected into the lateral canthus of the affected eye.^4,10 A straight hemostat is applied between the upper and lower lids, producing a crush injury along the site of local anesthesia for 1 to 2 minutes.^3,4,10 This is done to reduce the risk of bleeding by devitalizing the tissue.⁴ Straight scissors are then used to make a 1-cm horizontal incision from the lateral canthal tendon to the lateral orbital rim.⁴ This initial incision exposes the orbicularis muscle, orbital septum, palpebral conjunctiva, and an area called Eisler’s pocket that sits anterior to the lateral canthal tendon.³

Cantholysis can then be performed by blunt dissection.¹⁰ The inferior crus of the lateral canthus is identified either visually or by palpation, and a 1- to 2-cm inferior-posterior cut of the inferior crus accomplishes the lateral canthotomy.^3-4 After cutting the inferior crus, the lower lid should be pulled away easily, and if this does not occur, repeated attempts at cutting the inferior crus should be made.³ Pulling the lower eyelid down and away from the lateral orbital rim separates the skin and conjunctiva, aiding in visualization.⁴

After cutting the inferior crus, only a small amount of blood or air typically is expressed, but this is usually enough to prevent vision loss.³ When the procedure is performed correctly, the practitioner should be able to palpate a difference in the pressure of the globe, and tonography will show a reduced IOP. If the ocular pressure is still significantly elevated, the physician can proceed to cut the superior canthus of the lateral canthal tendon in a manner similar to cutting the inferior crus of the tendon.⁴ After the procedure is performed, urgent ophthalmologic consultation is required.

The risks of performing a lateral canthotomy include mechanical injury, hemorrhage, and infection.⁴ The incision from a lateral canthotomy generally does not need suturing and will heal without significant scarring.⁴ If the scissors are aimed superiorly instead of inferiorly for the inferior crus of the lateral canthal tendon, there is risk of injuring the levator aponeurosis leading to ptosis, as well as a small risk of injury to the lacrimal gland and lacrimal artery.³

Conclusion

Our patient demonstrates a case of traumatic OCS, a vision-threatening medical condition that requires rapid diagnosis and lateral canthotomy to lower IOP and reduce the risk of permanent vision loss. While an orbital CT scan may assist in confirming the diagnosis, treatment of IOP should not be delayed.

Traumatic eye injuries ranging from mild corneal abrasions to penetrating globe injuries are commonly seen in the ED, and ocular trauma accounts for nearly 1% of all complaints in the ED.¹ Up to 29% of facial fractures have associated eye injuries.² Emergency physicians (EPs) must be aware of possible eye injuries, including traumatic vision loss, and the indicators for emergent interventions.

Tension pneumo-orbitum following facial trauma is rarely reported. We present a case of orbital compartment syndrome (OCS) in an elderly woman who sustained an orbital floor fracture and required emergent lateral canthotomy to preserve vision.

Case

A 76-year-old woman presented to the ED for evaluation of pain, swelling, and loss of vision in the right eye. She said she had been sitting in a chair tying her shoes when she lost her balance and fell forward, striking her head and the right side of her face against the floor. She experienced no loss of consciousness and denied any neck pain, jaw pain, or dizziness. She also denied any chest pain, shortness of breath, weakness, or loss of function in either her arms or legs. She did, however, note a small nosebleed that had stopped before she arrived at the ED. The patient’s primary complaint was a possible nasal bone fracture.

Her medical history was significant for hypertension and coronary artery disease. Her medications include amitriptyline, an antihistamine, aspirin, clopidogrel, diltiazem, folic acid, furosemide, hydralazine, levothyroxine, prednisone, and zolpidem. She stated that she was allergic to amoxicillin and sulfa drugs.

The patient’s vital signs at presentation were: blood pressure (BP), 193/82 mm Hg; heart rate, 71 beats/minute; respiratory rate, 16 breaths/minute; and temperature, 97°F. She was alert, oriented, and in no distress. Her head and neck examination showed no scalp lacerations or swelling. There was, however, significant swelling and ecchymosis around the right eye and swelling and ecchymosis around the nose, with dried blood in both nares. No septal hematoma was present. The patient had tenderness to palpation over the infraorbital area and nose. No gross facial instability was present, and Battle sign was not appreciated. No jaw or dental abnormalities were noted.

The patient’s right pupil was fixed and dilated, and she could not perceive light. She did have upward and lateral movement of the eye, but was unable to look down. A minimal amount of proptosis was noted. Her intraocular pressure (IOP) was elevated at 54 mm Hg (normal range, 10-20 mm Hg). The remainder of the examination, including the neurological examination, was unremarkable.

The patient received emergent head and facial computed tomography (CT) scans. The head CT showed no acute intracranial hemorrhage, mass, or infarct. The facial CT was read as a right orbital floor fracture with intraorbital air, and a right maxillary sinus hematoma. Laboratory evaluation revealed a hematocrit of 38% and a platelet count of 544,000/mcL (normal range, 150,000-450,000/mcL). The prothrombin time was 10.9 seconds (normal range, 11-13.5 seconds); the international normalized ratio was 0.8 (normal range, 0.8-1.1); and the partial thromboplastin time was 22.5 seconds (normal range, 25-35 seconds).

Because the patient was at risk for permanent visual impairment due to increased IOP from the injury, a lateral canthotomy was immediately performed. A small amount of air was released, and the proptosis was notably reduced.

At this point, the ophthalmologist arrived and used an 18-gauge needle to explore the retrobulbar space. Two pockets of air were released, which markedly reduced the tactile pressure of the globe. Repeat tonography of the globe was 28 mm Hg. The wound was left open to drain, and the patient was started on azithromycin. She was discharged home to follow up with ophthalmology.

The patient presented to the ED 2 months later for an unrelated condition. At that time, she reported a complete return of her vision with no deficits and no noticeable scarring around the eye.

Discussion

The orbit is an enclosed space, bordered by bone laterally and posteriorly—the orbital septa superiorly and inferiorly, and the globe anteriorly.³ The lateral canthus is a combined tendon-ligament that helps attach the tarsal plates of the lids and the orbicularis oculi muscles to the lateral orbital wall and zygoma, which forms the posterior orbital wall.^3,4 The lateral canthal tendon is located beneath the lateral canthus and is comprised of the inferior and superior crus, which attaches to the inner aspect of the lateral orbital wall, forming a structure called Whitnall’s tubercle.^3,4

Other than globe injuries, the most common findings in patients with orbital trauma are periocular lacerations (96%), orbital fractures (16%), and retrobulbar hemorrhage (8%).⁵ The most common cause of retrobulbar hemorrhage is ocular trauma, but it is also observed in facial fractures, orbital surgery, retrobulbar injections, venous anomalies, atherosclerosis, intraorbital aneurysm of the ophthalmic artery, lacerated ophthalmic artery, hypertension, hemophilia, leukemia, von Willebrand disease, and straining.^3,6,7

In retrobulbar hemorrhage, an increased pressure in the orbital space can lead to optic nerve compression and vascular compromise.⁶ Important alternative diagnoses to consider include orbital cellulitis, orbital fracture, and globe rupture.³ Retrobulbar hemorrhage should be suspected in the clinical setting of exophthalmos, proptosis, diffuse subconjunctival hemorrhage, pain, visual loss or diplopia, periorbital edema, partial or complete ophthalmoplegia, resistance to retropulsion, increased IOP, a blanched ophthalmic artery on funduscopic examination, and an afferent pupillary defect.^3,4,8,9 Less commonly, periorbital crepitus and infraorbital hypoesthesia can be appreciated.³

If a patient with a retrobulbar hemorrhage is experiencing diminished vision, an emergent lateral canthotomy should be attempted. Retrobulbar hemorrhage can be difficult to diagnose in the setting of trauma. There can be damage to the optic nerve with associated edema and vision loss that is not associated with a retrobulbar hemorrhage and does not require a lateral canthotomy.^3,7 A dedicated CT scan of the orbits can aid in the diagnosis, but treatment should not be delayed.⁸

Patients with retrobulbar hemorrhage may initially present to the ED with intact visual acuity, but as the pressure behind the globe increases, vision will diminish.³ Although the medical literature has not established a definitive timeframe, it is believed that permanent visual compromise develops between 1 to 3 hours after ischemia develops.⁶ Animal studies show that visual loss due to central retinal artery ischemia may be reversible up to 100 minutes.³

Not all cases of retrobulbar hemorrhage are associated with vision loss. In patients without diminished vision, conservative treatments such as bed rest, elevation of the head of the bed, ice packs, analgesia, lowering BP, and sedatives should be attempted first.⁵ Acetazolamide and mannitol can also be considered in consultation with an ophthalmologist.

Pneumo-orbitum

The presence of pneumo-orbitum should alert the clinician to either a communication with a paranasal sinus, a gas-forming organism, or (rarely) Munchausen syndrome.¹⁰ Unlike most case presentations, most causes of pneumo-orbitum do not involve OCS and are self-limited.¹¹ Traumatic pneumo-orbitum without OCS has been reported in the literature.^12-15 However, traumatic tension pneumo-orbitum is rare.^12-16 One case report involved an elderly man with an orbital floor fracture who developed recurrent tension pneumo-orbitum after blowing his nose while intoxicated.¹² Another case involved a boy with tension pneumo-orbitum that required surgical decompression.¹⁶

In a patient who has experienced trauma, the combination of proptosis, elevated IOP, and vision loss likely represent a retrobulbar hematoma or OCS. A lateral canthotomy can help relieve IOP from either condition.^17,18 Orbital compartment syndrome can be caused by edema, emphysema, and caroticocavernous fistula, leading to increased orbital pressure and decreased perfusion.^17,18

In a review of 10 trauma patients with OCS, all cases were intubated due to the severity of the head trauma, and all had OCS due to edema.¹⁷ In a review of eight trauma patients with OCS, all had eye pain, reduced visual acuity, and proptosis.¹⁸ Most of the patients had periorbital edema, ophthalmoparesis, a relative afferent pupillary defect (as compared to a fixed and dilated pupil), and chemosis.^18,19 All of the patients with OCS required cantholysis or a lateral canthotomy.¹⁸

Lateral Canthotomy

Although EPs rarely perform lateral canthotomy, knowledge of this procedure is important, because it can prevent vision loss in the appropriate clinical setting. To perform a lateral canthotomy, the area around the affected eye is cleaned with saline irrigation.⁴ One percent or 2% lidocaine with epinephrine is then injected into the lateral canthus of the affected eye.^4,10 A straight hemostat is applied between the upper and lower lids, producing a crush injury along the site of local anesthesia for 1 to 2 minutes.^3,4,10 This is done to reduce the risk of bleeding by devitalizing the tissue.⁴ Straight scissors are then used to make a 1-cm horizontal incision from the lateral canthal tendon to the lateral orbital rim.⁴ This initial incision exposes the orbicularis muscle, orbital septum, palpebral conjunctiva, and an area called Eisler’s pocket that sits anterior to the lateral canthal tendon.³

Cantholysis can then be performed by blunt dissection.¹⁰ The inferior crus of the lateral canthus is identified either visually or by palpation, and a 1- to 2-cm inferior-posterior cut of the inferior crus accomplishes the lateral canthotomy.^3-4 After cutting the inferior crus, the lower lid should be pulled away easily, and if this does not occur, repeated attempts at cutting the inferior crus should be made.³ Pulling the lower eyelid down and away from the lateral orbital rim separates the skin and conjunctiva, aiding in visualization.⁴

After cutting the inferior crus, only a small amount of blood or air typically is expressed, but this is usually enough to prevent vision loss.³ When the procedure is performed correctly, the practitioner should be able to palpate a difference in the pressure of the globe, and tonography will show a reduced IOP. If the ocular pressure is still significantly elevated, the physician can proceed to cut the superior canthus of the lateral canthal tendon in a manner similar to cutting the inferior crus of the tendon.⁴ After the procedure is performed, urgent ophthalmologic consultation is required.

The risks of performing a lateral canthotomy include mechanical injury, hemorrhage, and infection.⁴ The incision from a lateral canthotomy generally does not need suturing and will heal without significant scarring.⁴ If the scissors are aimed superiorly instead of inferiorly for the inferior crus of the lateral canthal tendon, there is risk of injuring the levator aponeurosis leading to ptosis, as well as a small risk of injury to the lacrimal gland and lacrimal artery.³

Conclusion

Our patient demonstrates a case of traumatic OCS, a vision-threatening medical condition that requires rapid diagnosis and lateral canthotomy to lower IOP and reduce the risk of permanent vision loss. While an orbital CT scan may assist in confirming the diagnosis, treatment of IOP should not be delayed.

Acute lymphoblastic leukemia is a neoplastic proliferation of lymphoblasts in the bone marrow. Normal hematopoiesis is affected, and symptoms from anemia (fatigue, breathlessness), leukopenia (recurrent infections) or thrombocytopenia (easy bruising, mucosal bleeding) are typically described in ALL. Hepatosplenomegaly and B-symptoms (fever, weight loss, and night sweats) are frequently seen. Presence of lymphoblasts in the peripheral smear is indicative of ALL, and a bone marrow biopsy finding of >25% lymphoblasts is confirmatory. Absence of peripheral lymphoblasts in a patient with acute leukemia is known as aleukemic leukemia. Aleukemic leukemia is uncommon, and most cases have described skin lesions from lymphoblast infiltration (leukemia cutis) in addition to bone marrow involvement.¹ We report a case of aleukemic ALL in an adult presenting with unusual clinical features including bone pain, osteolytic lesions, hypercalcemia, and normal blood counts. To our knowledge, this is fifth such case ever reported in an adult patient.

Click on the PDF icon at the top of this introduction to read the full article.

Acute lymphoblastic leukemia is a neoplastic proliferation of lymphoblasts in the bone marrow. Normal hematopoiesis is affected, and symptoms from anemia (fatigue, breathlessness), leukopenia (recurrent infections) or thrombocytopenia (easy bruising, mucosal bleeding) are typically described in ALL. Hepatosplenomegaly and B-symptoms (fever, weight loss, and night sweats) are frequently seen. Presence of lymphoblasts in the peripheral smear is indicative of ALL, and a bone marrow biopsy finding of >25% lymphoblasts is confirmatory. Absence of peripheral lymphoblasts in a patient with acute leukemia is known as aleukemic leukemia. Aleukemic leukemia is uncommon, and most cases have described skin lesions from lymphoblast infiltration (leukemia cutis) in addition to bone marrow involvement.¹ We report a case of aleukemic ALL in an adult presenting with unusual clinical features including bone pain, osteolytic lesions, hypercalcemia, and normal blood counts. To our knowledge, this is fifth such case ever reported in an adult patient.

Click on the PDF icon at the top of this introduction to read the full article.

Acute lymphoblastic leukemia is a neoplastic proliferation of lymphoblasts in the bone marrow. Normal hematopoiesis is affected, and symptoms from anemia (fatigue, breathlessness), leukopenia (recurrent infections) or thrombocytopenia (easy bruising, mucosal bleeding) are typically described in ALL. Hepatosplenomegaly and B-symptoms (fever, weight loss, and night sweats) are frequently seen. Presence of lymphoblasts in the peripheral smear is indicative of ALL, and a bone marrow biopsy finding of >25% lymphoblasts is confirmatory. Absence of peripheral lymphoblasts in a patient with acute leukemia is known as aleukemic leukemia. Aleukemic leukemia is uncommon, and most cases have described skin lesions from lymphoblast infiltration (leukemia cutis) in addition to bone marrow involvement.¹ We report a case of aleukemic ALL in an adult presenting with unusual clinical features including bone pain, osteolytic lesions, hypercalcemia, and normal blood counts. To our knowledge, this is fifth such case ever reported in an adult patient.

Click on the PDF icon at the top of this introduction to read the full article.

THE CASE

An 85-year-old woman sought care at our outpatient clinic for a 9-month history of severe pruritus and crusted lesions on her face, extremities, and trunk. She had been diagnosed with hepatitis C virus (HCV) infection one year ago and was not taking any medication. The patient, who had been living with her family, had visited various clinics for her complaints and was diagnosed as having contact dermatitis and senile pruritus. She was prescribed topical mometasone furoate and moisturizers.

After 6 months of using this therapy, widespread grey-white plaques and minimal excoriation appeared on her face, scalp, and trunk. This was diagnosed as psoriasis, and the patient was prescribed topical corticosteroids, which she used for 9 months until she came to our clinic. She said the lesions regressed minimally with the topical corticosteroids, but did not fully clear.

Dermatologic examination revealed widespread erythema and grey-white, cohesive, thick, pruritic plaques on her scalp, face, trunk, and bilateral extremities (FIGURE 1). A punch biopsy specimen was taken from the border of a plaque on her trunk.

THE DIAGNOSIS

A complete blood cell count and wide biochemistry panel, including tumor markers and viral serology for human immunodeficiency virus (HIV), were normal. The patient had lymphadenopathy in her posterior cervical, bilateral preauricular, and bilateral inguinal regions.

Histopathologic examination revealed hyperkeratosis, acanthosis, and spongiotic edema in the epidermis, and vesiculation and mites in the stratum corneum. The dermal changes consisted of perivascular and diffuse cell infiltrates that were mainly mononuclear cells and eosinophilic granulocytes.

Based on the dermatologic examination and the histopathologic findings, we diagnosed the patient with crusted (Norwegian) scabies.

DISCUSSION

Crusted (Norwegian) scabies is a rare, highly contagious form of scabies that is characterized by the presence of millions of Sarcoptes scabiei var hominis mites in the epidermis.¹ This variant of scabies can affect individuals of any age, gender, or race.² It was first described by Boeck and Danielssen in 1848 in Norway and was named Norwegian scabies by von Hebra in 1862.³ In 2010, more than 200 cases of crusted scabies were reported in the literature.⁴

Crusted scabies is usually seen in immunocompromised patients, such as the elderly, those who’ve had solid organ transplantation, and those with HIV, malignancy, or malnutrition. Crusted scabies may also occur in patients with decreased sensory function (such as those with leprosy) or decreased ability to scratch, intellectual disabilities, and in those who use biologic agents or systemic/topical corticosteroids.^4-8

Crusted scabies is associated with increased morbidity and mortality, especially in children and the elderly, because of complications such as secondary bacterial infections and sepsis.^1,3 Widespread inflammation may also cause erythroderma, which can lead to metabolic disorders.

Distinguish it from other pruritic papulosquamous diseases

The differential diagnosis for crusted scabies includes psoriasis, eczema, cutaneous lymphoma, Darier disease, and adverse drug reactions.⁹ Crusted scabies can be differentiated from these other diagnoses by its clinical presentation and histopathological examination.

Crusted scabies is characterized by hyperkeratosis and wart-like crusts that are due to extreme proliferation of mites in the stratum corneum of the epidermis.² Lesions are usually localized on acral sites (especially the hands), although the entire body, including the face and the scalp, can be involved.¹ Psoriasiform or bullous pemphigoid-like eruptions have also been reported in the literature.^5,9

Our patient presented with widespread erythema and psoriasiform grey-white crusts on her scalp, face, chest, periareolar region, and extremities. In addition, she did not have an immunosuppressant disease or medication history.

However, the fact that our patient was using topical corticosteroids for so long explained the extent of her condition. Topical corticosteroids have been linked to scabies incognito.¹⁰ Topical or systemic corticosteroid use for long periods of time may alter the skin immune system by suppressing cellular immunity, thereby reducing the inflammatory response. This may lead to progression of the regular variant of scabies to crusted scabies, as our patient had.

Topical treatments, oral ivermectin proven to be effective

Topical keratolytics, permethrin 5%, lindane 1%, crotamiton 10%, sulfur ointment (5%-10%), malathion 0.5%, benzyl benzoate (10%-25%), oral ivermectin (2 doses of 200 mcg/kg/dose), and systemic antihistamines are appropriate therapies.³ While oral ivermectin is effective, it is not available in Turkey.

Because of our patient’s hepatic disorder, we opted for a topical, rather than a systemic, treatment and recommended repeated applications of topical permethrin. Repeated treatment with topical permethrin is often sufficient in patients who are unable to take systemic therapy. In fact, Binic et al⁴ reported a case in which an elderly patient with crusted scabies (who had previously been treated with systemic and topical corticosteroids) responded well to repeated topical treatment with lindane 1%, 25% benzyl benzoate, and 10% precipitated sulfur.

Our patient. We prescribed topical 5% permethrin lotion for our patient to apply to her entire body 4 times a week and advised her to wash her clothing and bed linens at 140° F. She was scheduled for biweekly check-ups. We also advised the patient’s family to use the same topical therapy 2 times per week because crusted scabies is highly contagious. One month later, our patient’s lesions had resolved (FIGURE 2).

THE TAKEAWAY

Early diagnosis and treatment of crusted scabies is important, both for the treatment of the patient and to stop the spread of the disease. Although rare, crusted scabies should be included in the differential diagnosis of long-term pruritic papulosquamous diseases, and the possibility of an atypical presentation in all patients should be considered—whether their immunity is compromised or not. Scabies should also be considered in patients with a positive family history of the disease and in those with chronic pruritus that is unresponsive to topical therapies.

THE CASE

An 85-year-old woman sought care at our outpatient clinic for a 9-month history of severe pruritus and crusted lesions on her face, extremities, and trunk. She had been diagnosed with hepatitis C virus (HCV) infection one year ago and was not taking any medication. The patient, who had been living with her family, had visited various clinics for her complaints and was diagnosed as having contact dermatitis and senile pruritus. She was prescribed topical mometasone furoate and moisturizers.

After 6 months of using this therapy, widespread grey-white plaques and minimal excoriation appeared on her face, scalp, and trunk. This was diagnosed as psoriasis, and the patient was prescribed topical corticosteroids, which she used for 9 months until she came to our clinic. She said the lesions regressed minimally with the topical corticosteroids, but did not fully clear.

Dermatologic examination revealed widespread erythema and grey-white, cohesive, thick, pruritic plaques on her scalp, face, trunk, and bilateral extremities (FIGURE 1). A punch biopsy specimen was taken from the border of a plaque on her trunk.

THE DIAGNOSIS

A complete blood cell count and wide biochemistry panel, including tumor markers and viral serology for human immunodeficiency virus (HIV), were normal. The patient had lymphadenopathy in her posterior cervical, bilateral preauricular, and bilateral inguinal regions.

Histopathologic examination revealed hyperkeratosis, acanthosis, and spongiotic edema in the epidermis, and vesiculation and mites in the stratum corneum. The dermal changes consisted of perivascular and diffuse cell infiltrates that were mainly mononuclear cells and eosinophilic granulocytes.

Based on the dermatologic examination and the histopathologic findings, we diagnosed the patient with crusted (Norwegian) scabies.

DISCUSSION

Crusted (Norwegian) scabies is a rare, highly contagious form of scabies that is characterized by the presence of millions of Sarcoptes scabiei var hominis mites in the epidermis.¹ This variant of scabies can affect individuals of any age, gender, or race.² It was first described by Boeck and Danielssen in 1848 in Norway and was named Norwegian scabies by von Hebra in 1862.³ In 2010, more than 200 cases of crusted scabies were reported in the literature.⁴

Crusted scabies is usually seen in immunocompromised patients, such as the elderly, those who’ve had solid organ transplantation, and those with HIV, malignancy, or malnutrition. Crusted scabies may also occur in patients with decreased sensory function (such as those with leprosy) or decreased ability to scratch, intellectual disabilities, and in those who use biologic agents or systemic/topical corticosteroids.^4-8

Crusted scabies is associated with increased morbidity and mortality, especially in children and the elderly, because of complications such as secondary bacterial infections and sepsis.^1,3 Widespread inflammation may also cause erythroderma, which can lead to metabolic disorders.

Distinguish it from other pruritic papulosquamous diseases

The differential diagnosis for crusted scabies includes psoriasis, eczema, cutaneous lymphoma, Darier disease, and adverse drug reactions.⁹ Crusted scabies can be differentiated from these other diagnoses by its clinical presentation and histopathological examination.

Crusted scabies is characterized by hyperkeratosis and wart-like crusts that are due to extreme proliferation of mites in the stratum corneum of the epidermis.² Lesions are usually localized on acral sites (especially the hands), although the entire body, including the face and the scalp, can be involved.¹ Psoriasiform or bullous pemphigoid-like eruptions have also been reported in the literature.^5,9

Our patient presented with widespread erythema and psoriasiform grey-white crusts on her scalp, face, chest, periareolar region, and extremities. In addition, she did not have an immunosuppressant disease or medication history.

However, the fact that our patient was using topical corticosteroids for so long explained the extent of her condition. Topical corticosteroids have been linked to scabies incognito.¹⁰ Topical or systemic corticosteroid use for long periods of time may alter the skin immune system by suppressing cellular immunity, thereby reducing the inflammatory response. This may lead to progression of the regular variant of scabies to crusted scabies, as our patient had.

Topical treatments, oral ivermectin proven to be effective

Topical keratolytics, permethrin 5%, lindane 1%, crotamiton 10%, sulfur ointment (5%-10%), malathion 0.5%, benzyl benzoate (10%-25%), oral ivermectin (2 doses of 200 mcg/kg/dose), and systemic antihistamines are appropriate therapies.³ While oral ivermectin is effective, it is not available in Turkey.

Because of our patient’s hepatic disorder, we opted for a topical, rather than a systemic, treatment and recommended repeated applications of topical permethrin. Repeated treatment with topical permethrin is often sufficient in patients who are unable to take systemic therapy. In fact, Binic et al⁴ reported a case in which an elderly patient with crusted scabies (who had previously been treated with systemic and topical corticosteroids) responded well to repeated topical treatment with lindane 1%, 25% benzyl benzoate, and 10% precipitated sulfur.

Our patient. We prescribed topical 5% permethrin lotion for our patient to apply to her entire body 4 times a week and advised her to wash her clothing and bed linens at 140° F. She was scheduled for biweekly check-ups. We also advised the patient’s family to use the same topical therapy 2 times per week because crusted scabies is highly contagious. One month later, our patient’s lesions had resolved (FIGURE 2).

THE TAKEAWAY

Early diagnosis and treatment of crusted scabies is important, both for the treatment of the patient and to stop the spread of the disease. Although rare, crusted scabies should be included in the differential diagnosis of long-term pruritic papulosquamous diseases, and the possibility of an atypical presentation in all patients should be considered—whether their immunity is compromised or not. Scabies should also be considered in patients with a positive family history of the disease and in those with chronic pruritus that is unresponsive to topical therapies.

THE CASE

An 85-year-old woman sought care at our outpatient clinic for a 9-month history of severe pruritus and crusted lesions on her face, extremities, and trunk. She had been diagnosed with hepatitis C virus (HCV) infection one year ago and was not taking any medication. The patient, who had been living with her family, had visited various clinics for her complaints and was diagnosed as having contact dermatitis and senile pruritus. She was prescribed topical mometasone furoate and moisturizers.

After 6 months of using this therapy, widespread grey-white plaques and minimal excoriation appeared on her face, scalp, and trunk. This was diagnosed as psoriasis, and the patient was prescribed topical corticosteroids, which she used for 9 months until she came to our clinic. She said the lesions regressed minimally with the topical corticosteroids, but did not fully clear.

Dermatologic examination revealed widespread erythema and grey-white, cohesive, thick, pruritic plaques on her scalp, face, trunk, and bilateral extremities (FIGURE 1). A punch biopsy specimen was taken from the border of a plaque on her trunk.

THE DIAGNOSIS

A complete blood cell count and wide biochemistry panel, including tumor markers and viral serology for human immunodeficiency virus (HIV), were normal. The patient had lymphadenopathy in her posterior cervical, bilateral preauricular, and bilateral inguinal regions.

Histopathologic examination revealed hyperkeratosis, acanthosis, and spongiotic edema in the epidermis, and vesiculation and mites in the stratum corneum. The dermal changes consisted of perivascular and diffuse cell infiltrates that were mainly mononuclear cells and eosinophilic granulocytes.

Based on the dermatologic examination and the histopathologic findings, we diagnosed the patient with crusted (Norwegian) scabies.

DISCUSSION

Crusted (Norwegian) scabies is a rare, highly contagious form of scabies that is characterized by the presence of millions of Sarcoptes scabiei var hominis mites in the epidermis.¹ This variant of scabies can affect individuals of any age, gender, or race.² It was first described by Boeck and Danielssen in 1848 in Norway and was named Norwegian scabies by von Hebra in 1862.³ In 2010, more than 200 cases of crusted scabies were reported in the literature.⁴

Crusted scabies is usually seen in immunocompromised patients, such as the elderly, those who’ve had solid organ transplantation, and those with HIV, malignancy, or malnutrition. Crusted scabies may also occur in patients with decreased sensory function (such as those with leprosy) or decreased ability to scratch, intellectual disabilities, and in those who use biologic agents or systemic/topical corticosteroids.^4-8

Crusted scabies is associated with increased morbidity and mortality, especially in children and the elderly, because of complications such as secondary bacterial infections and sepsis.^1,3 Widespread inflammation may also cause erythroderma, which can lead to metabolic disorders.

Distinguish it from other pruritic papulosquamous diseases

The differential diagnosis for crusted scabies includes psoriasis, eczema, cutaneous lymphoma, Darier disease, and adverse drug reactions.⁹ Crusted scabies can be differentiated from these other diagnoses by its clinical presentation and histopathological examination.

Crusted scabies is characterized by hyperkeratosis and wart-like crusts that are due to extreme proliferation of mites in the stratum corneum of the epidermis.² Lesions are usually localized on acral sites (especially the hands), although the entire body, including the face and the scalp, can be involved.¹ Psoriasiform or bullous pemphigoid-like eruptions have also been reported in the literature.^5,9

Our patient presented with widespread erythema and psoriasiform grey-white crusts on her scalp, face, chest, periareolar region, and extremities. In addition, she did not have an immunosuppressant disease or medication history.

However, the fact that our patient was using topical corticosteroids for so long explained the extent of her condition. Topical corticosteroids have been linked to scabies incognito.¹⁰ Topical or systemic corticosteroid use for long periods of time may alter the skin immune system by suppressing cellular immunity, thereby reducing the inflammatory response. This may lead to progression of the regular variant of scabies to crusted scabies, as our patient had.

Topical treatments, oral ivermectin proven to be effective

Topical keratolytics, permethrin 5%, lindane 1%, crotamiton 10%, sulfur ointment (5%-10%), malathion 0.5%, benzyl benzoate (10%-25%), oral ivermectin (2 doses of 200 mcg/kg/dose), and systemic antihistamines are appropriate therapies.³ While oral ivermectin is effective, it is not available in Turkey.

Because of our patient’s hepatic disorder, we opted for a topical, rather than a systemic, treatment and recommended repeated applications of topical permethrin. Repeated treatment with topical permethrin is often sufficient in patients who are unable to take systemic therapy. In fact, Binic et al⁴ reported a case in which an elderly patient with crusted scabies (who had previously been treated with systemic and topical corticosteroids) responded well to repeated topical treatment with lindane 1%, 25% benzyl benzoate, and 10% precipitated sulfur.

Our patient. We prescribed topical 5% permethrin lotion for our patient to apply to her entire body 4 times a week and advised her to wash her clothing and bed linens at 140° F. She was scheduled for biweekly check-ups. We also advised the patient’s family to use the same topical therapy 2 times per week because crusted scabies is highly contagious. One month later, our patient’s lesions had resolved (FIGURE 2).

THE TAKEAWAY

Early diagnosis and treatment of crusted scabies is important, both for the treatment of the patient and to stop the spread of the disease. Although rare, crusted scabies should be included in the differential diagnosis of long-term pruritic papulosquamous diseases, and the possibility of an atypical presentation in all patients should be considered—whether their immunity is compromised or not. Scabies should also be considered in patients with a positive family history of the disease and in those with chronic pruritus that is unresponsive to topical therapies.

THE CASE

A 63-year-old woman with a history of hyperlipidemia presented to our hospital with a swollen right hand. The patient noted that she had closed her hand in a car door one week earlier, causing minor trauma to the right third metacarpophalangeal joint. Shortly after injuring her hand, she’d sought care at an outpatient facility, where she was given a diagnosis of cellulitis and a prescription for an oral antibiotic. The swelling, however, worsened, prompting her visit to our hospital. She was admitted for further work-up and started on intravenous (IV) antibiotics.

Her family history included a sister with deep vein thrombosis (DVT) and a maternal aunt with breast cancer. The patient denied oral contraceptive use or a personal history of malignancy. On physical examination, her right hand and forearm were swollen, tender, and erythematous.

THE DIAGNOSIS

Laboratory data showed a normal complete blood count and complete metabolic panel. The patient’s sedimentation rate and C-reactive protein level were elevated at 65 mm/hr and 110 mg/L, respectively. The patient was not improving on IV antibiotics, so we performed a right upper extremity venous duplex ultrasound. The ultrasound showed an occlusive thrombus in the right ulnar and radial veins (FIGURES 1 AND 2), and we diagnosed the patient with upper extremity deep vein thrombosis (UEDVT). (The timing of the diagnosis, relative to the injury to the patient’s hand, appeared to have been coincidental.)

Further work-up revealed normal complement C3 and C4 tests, as well as negative antinuclear, anti-double stranded DNA, anti-Smith, and anticardiolipin antibody tests. Similarly, a factor II DNA analysis was negative. However, the patient was positive for the factor V Leiden heterozygous mutation.

DISCUSSION

More than 350,000 people are diagnosed with DVT or pulmonary embolism (PE) in the United States each year.¹ Up to 4% of all DVTs involve the upper extremities.² Secondary UEDVT, which occurs in patients with central venous catheters, malignancies, and thrombophilia, accounts for the majority of UEDVT cases; primary UEDVT is less common.³

Large epidemiologic studies have demonstrated that hypercoagulability is a risk factor for lower extremity DVT, but few data exist on the role of coagulation abnormalities in patients with primary UEDVT. The prevalence of clotting abnormalities in patients with primary UEDVT ranges from 8% to 43%.^4,5 Factor V Leiden is the most common cause of inherited thrombophilia. Patients with heterozygous factor V Leiden mutation have a 7-fold increased risk of venous thrombosis.⁶

Héron and colleagues⁷ reported that 16 of 51 patients with at least one clotting abnormality had primary UEDVT. Factor V Leiden was found in 5 of those patients (20%). Interestingly, 3 of the 5 carriers of the factor V Leiden mutation were older than 45 years. Our patient was 63, which was consistent with these findings.

Malignancy is also an important risk factor for UEDVT.^8,9 In patients with a DVT in an unusual location, age- and sex-appropriate cancer screenings are strongly recommended. (Our patient had undergone a colonoscopy 8 years earlier, which was normal. She’d also had a recent mammogram and Pap smear, which were normal, as well.)

It’s often difficult to distinguish between cellulitis and UEDVT

The differential diagnosis for UEDVT includes effort thrombosis (also known as Paget-Schroetter syndrome) and cellulitis.

Effort thrombosis usually occurs in young, otherwise healthy individuals and almost exclusively in the axillary and subclavian veins. Our patient’s age and a venous duplex ultrasound ruled out any thrombosis in these locations.

Distinguishing cellulitis from UEDVT based on clinical features can be difficult. In both conditions, the limb is swollen and painful and the skin is warm and erythematous. As a result, each condition is often misdiagnosed as the other.

But there are features that distinguish the 2. In patients with UEDVT, you’re likely to see limb pain and a palpable cord (a hard, thickened palpable vein along the line of the deep veins). On the other hand, patients with cellulitis tend to have more systemic symptoms, such as fever, chills, and swollen lymph nodes, as well as skin breakdown, ulcers, and pus.

The American College of Chest Physicians (ACCP) recommends that the initial evaluation for patients with suspected UEDVT be a combined modality ultrasound (compression with either Doppler or color Doppler) rather than D-dimer or venography.¹⁰ Quickly arriving at a proper diagnosis is critical, given that up to one-third of patients with UEDVT will develop a PE.⁷ Other complications include superior vena cava syndrome, septic thrombophlebitis, thoracic duct obstruction, and brachial plexopathy.¹¹

Treat with anticoagulants for no longer than 3 months

The ACCP also recommends that patients who have UEDVT that isn’t associated with a central venous catheter or with cancer be treated with anticoagulation for no longer than 3 months.¹⁰

Our patient was started on enoxaparin and warfarin. After 5 days at our hospital, she was taken off the enoxaparin and discharged home on warfarin 5 mg/d. The swelling completely resolved one week later.

THE TAKEAWAY

Ulnar and radial DVT in a patient with factor V Leiden mutation is a rare condition. UEDVT should be included in the differential diagnosis for cellulitis whenever the diagnosis is uncertain or the patient doesn’t respond to antibiotics. Factor V Leiden mutation appears to be a risk factor in UEDVT and testing for it should be considered.

THE CASE

A 63-year-old woman with a history of hyperlipidemia presented to our hospital with a swollen right hand. The patient noted that she had closed her hand in a car door one week earlier, causing minor trauma to the right third metacarpophalangeal joint. Shortly after injuring her hand, she’d sought care at an outpatient facility, where she was given a diagnosis of cellulitis and a prescription for an oral antibiotic. The swelling, however, worsened, prompting her visit to our hospital. She was admitted for further work-up and started on intravenous (IV) antibiotics.

Her family history included a sister with deep vein thrombosis (DVT) and a maternal aunt with breast cancer. The patient denied oral contraceptive use or a personal history of malignancy. On physical examination, her right hand and forearm were swollen, tender, and erythematous.

THE DIAGNOSIS

Laboratory data showed a normal complete blood count and complete metabolic panel. The patient’s sedimentation rate and C-reactive protein level were elevated at 65 mm/hr and 110 mg/L, respectively. The patient was not improving on IV antibiotics, so we performed a right upper extremity venous duplex ultrasound. The ultrasound showed an occlusive thrombus in the right ulnar and radial veins (FIGURES 1 AND 2), and we diagnosed the patient with upper extremity deep vein thrombosis (UEDVT). (The timing of the diagnosis, relative to the injury to the patient’s hand, appeared to have been coincidental.)

Further work-up revealed normal complement C3 and C4 tests, as well as negative antinuclear, anti-double stranded DNA, anti-Smith, and anticardiolipin antibody tests. Similarly, a factor II DNA analysis was negative. However, the patient was positive for the factor V Leiden heterozygous mutation.

DISCUSSION

More than 350,000 people are diagnosed with DVT or pulmonary embolism (PE) in the United States each year.¹ Up to 4% of all DVTs involve the upper extremities.² Secondary UEDVT, which occurs in patients with central venous catheters, malignancies, and thrombophilia, accounts for the majority of UEDVT cases; primary UEDVT is less common.³

Large epidemiologic studies have demonstrated that hypercoagulability is a risk factor for lower extremity DVT, but few data exist on the role of coagulation abnormalities in patients with primary UEDVT. The prevalence of clotting abnormalities in patients with primary UEDVT ranges from 8% to 43%.^4,5 Factor V Leiden is the most common cause of inherited thrombophilia. Patients with heterozygous factor V Leiden mutation have a 7-fold increased risk of venous thrombosis.⁶

Héron and colleagues⁷ reported that 16 of 51 patients with at least one clotting abnormality had primary UEDVT. Factor V Leiden was found in 5 of those patients (20%). Interestingly, 3 of the 5 carriers of the factor V Leiden mutation were older than 45 years. Our patient was 63, which was consistent with these findings.

Malignancy is also an important risk factor for UEDVT.^8,9 In patients with a DVT in an unusual location, age- and sex-appropriate cancer screenings are strongly recommended. (Our patient had undergone a colonoscopy 8 years earlier, which was normal. She’d also had a recent mammogram and Pap smear, which were normal, as well.)

It’s often difficult to distinguish between cellulitis and UEDVT

The differential diagnosis for UEDVT includes effort thrombosis (also known as Paget-Schroetter syndrome) and cellulitis.

Effort thrombosis usually occurs in young, otherwise healthy individuals and almost exclusively in the axillary and subclavian veins. Our patient’s age and a venous duplex ultrasound ruled out any thrombosis in these locations.

Distinguishing cellulitis from UEDVT based on clinical features can be difficult. In both conditions, the limb is swollen and painful and the skin is warm and erythematous. As a result, each condition is often misdiagnosed as the other.

But there are features that distinguish the 2. In patients with UEDVT, you’re likely to see limb pain and a palpable cord (a hard, thickened palpable vein along the line of the deep veins). On the other hand, patients with cellulitis tend to have more systemic symptoms, such as fever, chills, and swollen lymph nodes, as well as skin breakdown, ulcers, and pus.

The American College of Chest Physicians (ACCP) recommends that the initial evaluation for patients with suspected UEDVT be a combined modality ultrasound (compression with either Doppler or color Doppler) rather than D-dimer or venography.¹⁰ Quickly arriving at a proper diagnosis is critical, given that up to one-third of patients with UEDVT will develop a PE.⁷ Other complications include superior vena cava syndrome, septic thrombophlebitis, thoracic duct obstruction, and brachial plexopathy.¹¹

Treat with anticoagulants for no longer than 3 months

The ACCP also recommends that patients who have UEDVT that isn’t associated with a central venous catheter or with cancer be treated with anticoagulation for no longer than 3 months.¹⁰

Our patient was started on enoxaparin and warfarin. After 5 days at our hospital, she was taken off the enoxaparin and discharged home on warfarin 5 mg/d. The swelling completely resolved one week later.

THE TAKEAWAY

Ulnar and radial DVT in a patient with factor V Leiden mutation is a rare condition. UEDVT should be included in the differential diagnosis for cellulitis whenever the diagnosis is uncertain or the patient doesn’t respond to antibiotics. Factor V Leiden mutation appears to be a risk factor in UEDVT and testing for it should be considered.

THE CASE

A 63-year-old woman with a history of hyperlipidemia presented to our hospital with a swollen right hand. The patient noted that she had closed her hand in a car door one week earlier, causing minor trauma to the right third metacarpophalangeal joint. Shortly after injuring her hand, she’d sought care at an outpatient facility, where she was given a diagnosis of cellulitis and a prescription for an oral antibiotic. The swelling, however, worsened, prompting her visit to our hospital. She was admitted for further work-up and started on intravenous (IV) antibiotics.

Her family history included a sister with deep vein thrombosis (DVT) and a maternal aunt with breast cancer. The patient denied oral contraceptive use or a personal history of malignancy. On physical examination, her right hand and forearm were swollen, tender, and erythematous.

THE DIAGNOSIS

Laboratory data showed a normal complete blood count and complete metabolic panel. The patient’s sedimentation rate and C-reactive protein level were elevated at 65 mm/hr and 110 mg/L, respectively. The patient was not improving on IV antibiotics, so we performed a right upper extremity venous duplex ultrasound. The ultrasound showed an occlusive thrombus in the right ulnar and radial veins (FIGURES 1 AND 2), and we diagnosed the patient with upper extremity deep vein thrombosis (UEDVT). (The timing of the diagnosis, relative to the injury to the patient’s hand, appeared to have been coincidental.)

Further work-up revealed normal complement C3 and C4 tests, as well as negative antinuclear, anti-double stranded DNA, anti-Smith, and anticardiolipin antibody tests. Similarly, a factor II DNA analysis was negative. However, the patient was positive for the factor V Leiden heterozygous mutation.

DISCUSSION

More than 350,000 people are diagnosed with DVT or pulmonary embolism (PE) in the United States each year.¹ Up to 4% of all DVTs involve the upper extremities.² Secondary UEDVT, which occurs in patients with central venous catheters, malignancies, and thrombophilia, accounts for the majority of UEDVT cases; primary UEDVT is less common.³

Large epidemiologic studies have demonstrated that hypercoagulability is a risk factor for lower extremity DVT, but few data exist on the role of coagulation abnormalities in patients with primary UEDVT. The prevalence of clotting abnormalities in patients with primary UEDVT ranges from 8% to 43%.^4,5 Factor V Leiden is the most common cause of inherited thrombophilia. Patients with heterozygous factor V Leiden mutation have a 7-fold increased risk of venous thrombosis.⁶

Héron and colleagues⁷ reported that 16 of 51 patients with at least one clotting abnormality had primary UEDVT. Factor V Leiden was found in 5 of those patients (20%). Interestingly, 3 of the 5 carriers of the factor V Leiden mutation were older than 45 years. Our patient was 63, which was consistent with these findings.

Malignancy is also an important risk factor for UEDVT.^8,9 In patients with a DVT in an unusual location, age- and sex-appropriate cancer screenings are strongly recommended. (Our patient had undergone a colonoscopy 8 years earlier, which was normal. She’d also had a recent mammogram and Pap smear, which were normal, as well.)

It’s often difficult to distinguish between cellulitis and UEDVT

The differential diagnosis for UEDVT includes effort thrombosis (also known as Paget-Schroetter syndrome) and cellulitis.

Effort thrombosis usually occurs in young, otherwise healthy individuals and almost exclusively in the axillary and subclavian veins. Our patient’s age and a venous duplex ultrasound ruled out any thrombosis in these locations.

Distinguishing cellulitis from UEDVT based on clinical features can be difficult. In both conditions, the limb is swollen and painful and the skin is warm and erythematous. As a result, each condition is often misdiagnosed as the other.

But there are features that distinguish the 2. In patients with UEDVT, you’re likely to see limb pain and a palpable cord (a hard, thickened palpable vein along the line of the deep veins). On the other hand, patients with cellulitis tend to have more systemic symptoms, such as fever, chills, and swollen lymph nodes, as well as skin breakdown, ulcers, and pus.

The American College of Chest Physicians (ACCP) recommends that the initial evaluation for patients with suspected UEDVT be a combined modality ultrasound (compression with either Doppler or color Doppler) rather than D-dimer or venography.¹⁰ Quickly arriving at a proper diagnosis is critical, given that up to one-third of patients with UEDVT will develop a PE.⁷ Other complications include superior vena cava syndrome, septic thrombophlebitis, thoracic duct obstruction, and brachial plexopathy.¹¹

Treat with anticoagulants for no longer than 3 months

The ACCP also recommends that patients who have UEDVT that isn’t associated with a central venous catheter or with cancer be treated with anticoagulation for no longer than 3 months.¹⁰

Our patient was started on enoxaparin and warfarin. After 5 days at our hospital, she was taken off the enoxaparin and discharged home on warfarin 5 mg/d. The swelling completely resolved one week later.

THE TAKEAWAY

Ulnar and radial DVT in a patient with factor V Leiden mutation is a rare condition. UEDVT should be included in the differential diagnosis for cellulitis whenever the diagnosis is uncertain or the patient doesn’t respond to antibiotics. Factor V Leiden mutation appears to be a risk factor in UEDVT and testing for it should be considered.

Growing teratoma syndrome (GTS) is a rare condition seen in patients with germ-cell tumors (GCT) who present with enlarging masses during or after appropriate chemotherapy with normalized serum markers.1 Three defining criteria for GTS are: a persistently growing tumor mass or evolving new mass during or after chemotherapy, normalization of tumor markers, and presence of only mature teratoma in the resected specimen on the final histopathological examination.1 Growing teratomas lack the metastatic potential; however, their relentless local growth causes compression and infiltration of adjacent organs, which produces symptoms.

Click on the PDF icon at the top of this introduction to read the full article. 

Growing teratoma syndrome (GTS) is a rare condition seen in patients with germ-cell tumors (GCT) who present with enlarging masses during or after appropriate chemotherapy with normalized serum markers.1 Three defining criteria for GTS are: a persistently growing tumor mass or evolving new mass during or after chemotherapy, normalization of tumor markers, and presence of only mature teratoma in the resected specimen on the final histopathological examination.1 Growing teratomas lack the metastatic potential; however, their relentless local growth causes compression and infiltration of adjacent organs, which produces symptoms.

Click on the PDF icon at the top of this introduction to read the full article. 

Growing teratoma syndrome (GTS) is a rare condition seen in patients with germ-cell tumors (GCT) who present with enlarging masses during or after appropriate chemotherapy with normalized serum markers.1 Three defining criteria for GTS are: a persistently growing tumor mass or evolving new mass during or after chemotherapy, normalization of tumor markers, and presence of only mature teratoma in the resected specimen on the final histopathological examination.1 Growing teratomas lack the metastatic potential; however, their relentless local growth causes compression and infiltration of adjacent organs, which produces symptoms.

Click on the PDF icon at the top of this introduction to read the full article.