Case Reports

Case

A 34-year-old woman presented to the ED with severe, persistent abdominal pain that had begun 18 days earlier. She was 7 weeks pregnant and had been seen in the same ED the day before. During that visit, ultrasound had shown a single pregnancy of doubtful viability. Abdominal magnetic resonance imaging was normal. She was given multiple doses of hydromorphone. The discharge diagnosis was “missed abortion.”

Since the onset of her pain, she had been hospitalized twice elsewhere, with no clear diagnosis to explain her pain. Treatment consisted of repeat doses of hydromorphone. During the second hospitalization, a sodium level of 109 mEq/L had been corrected with hypertonic saline, and a urinary tract infection (UTI) had been treated with cephalexin.

Our patient had never experienced similar abdominal pain. Her medical history included depression and asthma. Her family history was notable for an aunt who had died of lung cancer.

On this ED visit, the patient’s vital signs were normal. On examination, she was moaning in pain and clutching her abdomen. The abdomen was tender in both lower quadrants, with guarding but no rebound. Her sodium level was 125 mEq/L; the day before it had been 132 mEq/L.

Urine dipstick testing showed 2+ glucose and 2+ bilirubin; both had been within normal range (negative) the day before. An abdominal/pelvic computed tomography scan with intravenous (IV) and oral contrast did not reveal any potential cause of the patient’s pain. Multiple doses of IV hydromorphone were given, but her pain persisted.

We revisited our patient’s family history. With prompting, she remembered that as a teenager, her mother had had an illness that caused “problems with her nerves and blood vessels and turned her urine red.” When reached by phone, the patient’s mother, who lives outside the United States, said she was familiar with the term “porphyria,” but curiously, she did not state she carried the diagnosis, and had not advised her children they could be at risk.

The patient was admitted to the intensive care unit (ICU) for treatment of hyponatremia. Her mother’s history led us to suspect porphyria, so we sent a urine sample from the ED for porphobilinogen (PBG) testing. Her urine was not red at that time (on further questioning, she remembered she had had an episode of “red urine” recently). Two days later, after the PBG result came back positive, treatment was initiated for porphyria. With further stool and serum testing, the diagnosis of acute intermittent porphyria (AIP) was made.

The patient was treated with glucose loading and hemin therapy. In the ICU, 2 ampules of 50% dextrose in water solution (D50W) was administered, and she was transferred to the hematology-oncology service for hemin therapy. Soon after, she underwent a dilation and curettage. Three weeks later, she was discharged in good condition.

Discussion

The unifying diagnostic concept is that toxins damage all components of the nervous system: intestinal, central, peripheral, and autonomic. Hence, any combination of abdominal pain, vomiting, psychiatric symptoms, vital sign instability, weakness, or sensory loss may occur.¹ To further confuse the diagnostician, the constellation of symptoms may vary with each episode.

Two critical laboratory clues are red urine—which often is mistaken for a UTI or hematuria—and hyponatremia.¹ Another porphyria hallmark is triggers. These include drugs, carbohydrate deprivation, smoking, and stress. Common chemical inciters include alcohol, ketamine, etomidate, macrodantin, nifedipine, progesterone, and phenytoin.¹ The Atkins diet (zero carbohydrates) reportedly caused an uptick in new porphyria cases.¹

Attacks usually start after puberty. Women tend to experience flares during the luteal (progesterone) phase of the menstrual cycle.¹ Acute intermittent porphyria can mimic Guillain-Barré syndrome and psychosis.² Delayed diagnosis may lead to irreversible neurological damage or death.²

Despite AIP’s complexity, initial diagnostic testing is simple: a urinary PBG level obtained during an attack is virtually 100% sensitive and specific for AIP and two other acute porphyrias: hereditary coproporphyria (HCP), and variegate porphyria (VP). A positive urine PBG mandates immediate treatment—even while awaiting porphyrin and GK delta-aminolevulinic acid (ALA) levels in stool and serum to identify which porphyria (AIP, HCP, or VP) is present. The fourth (and least common) acute porphyria, ALA dehydratase porphyria (ADP), may produce no PBG elevation and requires separate porphyrin and ALA testing to make the diagnosis.

Treatment

Treatment targets runaway heme precursor synthesis at its start and finish (Figure). Glucose-loading suppresses the initial enzyme, ALA synthase. Since the absence of normal end-product (heme) drives the enzymatic cascade, addition of IV hemin provides the substrate—and negative feedback—to stop it.

Conclusion

This case represents an example of AIP in which a patient presented with the characteristic abdominal pain and hyponatremia, complicated by the fact that she was pregnant and her urine was not red.

Intractable abdominal pain with negative imaging must prompt a search for red urine, neurological symptoms, porphyria medication triggers, and a family history of porphyria. Any constellation of findings should prompt immediate urine PBG testing.

In the appropriate clinical setting, it may be prudent to glucose-load a patient while waiting for confirmatory testing (which can take 1-2 days). Hemin therapy is best instituted by the hematology service after high urine PBG levels are confirmed.

Case

A 34-year-old woman presented to the ED with severe, persistent abdominal pain that had begun 18 days earlier. She was 7 weeks pregnant and had been seen in the same ED the day before. During that visit, ultrasound had shown a single pregnancy of doubtful viability. Abdominal magnetic resonance imaging was normal. She was given multiple doses of hydromorphone. The discharge diagnosis was “missed abortion.”

Since the onset of her pain, she had been hospitalized twice elsewhere, with no clear diagnosis to explain her pain. Treatment consisted of repeat doses of hydromorphone. During the second hospitalization, a sodium level of 109 mEq/L had been corrected with hypertonic saline, and a urinary tract infection (UTI) had been treated with cephalexin.

Our patient had never experienced similar abdominal pain. Her medical history included depression and asthma. Her family history was notable for an aunt who had died of lung cancer.

On this ED visit, the patient’s vital signs were normal. On examination, she was moaning in pain and clutching her abdomen. The abdomen was tender in both lower quadrants, with guarding but no rebound. Her sodium level was 125 mEq/L; the day before it had been 132 mEq/L.

Urine dipstick testing showed 2+ glucose and 2+ bilirubin; both had been within normal range (negative) the day before. An abdominal/pelvic computed tomography scan with intravenous (IV) and oral contrast did not reveal any potential cause of the patient’s pain. Multiple doses of IV hydromorphone were given, but her pain persisted.

We revisited our patient’s family history. With prompting, she remembered that as a teenager, her mother had had an illness that caused “problems with her nerves and blood vessels and turned her urine red.” When reached by phone, the patient’s mother, who lives outside the United States, said she was familiar with the term “porphyria,” but curiously, she did not state she carried the diagnosis, and had not advised her children they could be at risk.

The patient was admitted to the intensive care unit (ICU) for treatment of hyponatremia. Her mother’s history led us to suspect porphyria, so we sent a urine sample from the ED for porphobilinogen (PBG) testing. Her urine was not red at that time (on further questioning, she remembered she had had an episode of “red urine” recently). Two days later, after the PBG result came back positive, treatment was initiated for porphyria. With further stool and serum testing, the diagnosis of acute intermittent porphyria (AIP) was made.

The patient was treated with glucose loading and hemin therapy. In the ICU, 2 ampules of 50% dextrose in water solution (D50W) was administered, and she was transferred to the hematology-oncology service for hemin therapy. Soon after, she underwent a dilation and curettage. Three weeks later, she was discharged in good condition.

Discussion

The unifying diagnostic concept is that toxins damage all components of the nervous system: intestinal, central, peripheral, and autonomic. Hence, any combination of abdominal pain, vomiting, psychiatric symptoms, vital sign instability, weakness, or sensory loss may occur.¹ To further confuse the diagnostician, the constellation of symptoms may vary with each episode.

Two critical laboratory clues are red urine—which often is mistaken for a UTI or hematuria—and hyponatremia.¹ Another porphyria hallmark is triggers. These include drugs, carbohydrate deprivation, smoking, and stress. Common chemical inciters include alcohol, ketamine, etomidate, macrodantin, nifedipine, progesterone, and phenytoin.¹ The Atkins diet (zero carbohydrates) reportedly caused an uptick in new porphyria cases.¹

Attacks usually start after puberty. Women tend to experience flares during the luteal (progesterone) phase of the menstrual cycle.¹ Acute intermittent porphyria can mimic Guillain-Barré syndrome and psychosis.² Delayed diagnosis may lead to irreversible neurological damage or death.²

Despite AIP’s complexity, initial diagnostic testing is simple: a urinary PBG level obtained during an attack is virtually 100% sensitive and specific for AIP and two other acute porphyrias: hereditary coproporphyria (HCP), and variegate porphyria (VP). A positive urine PBG mandates immediate treatment—even while awaiting porphyrin and GK delta-aminolevulinic acid (ALA) levels in stool and serum to identify which porphyria (AIP, HCP, or VP) is present. The fourth (and least common) acute porphyria, ALA dehydratase porphyria (ADP), may produce no PBG elevation and requires separate porphyrin and ALA testing to make the diagnosis.

Treatment

Treatment targets runaway heme precursor synthesis at its start and finish (Figure). Glucose-loading suppresses the initial enzyme, ALA synthase. Since the absence of normal end-product (heme) drives the enzymatic cascade, addition of IV hemin provides the substrate—and negative feedback—to stop it.

Conclusion

This case represents an example of AIP in which a patient presented with the characteristic abdominal pain and hyponatremia, complicated by the fact that she was pregnant and her urine was not red.

Intractable abdominal pain with negative imaging must prompt a search for red urine, neurological symptoms, porphyria medication triggers, and a family history of porphyria. Any constellation of findings should prompt immediate urine PBG testing.

In the appropriate clinical setting, it may be prudent to glucose-load a patient while waiting for confirmatory testing (which can take 1-2 days). Hemin therapy is best instituted by the hematology service after high urine PBG levels are confirmed.

Case

A 34-year-old woman presented to the ED with severe, persistent abdominal pain that had begun 18 days earlier. She was 7 weeks pregnant and had been seen in the same ED the day before. During that visit, ultrasound had shown a single pregnancy of doubtful viability. Abdominal magnetic resonance imaging was normal. She was given multiple doses of hydromorphone. The discharge diagnosis was “missed abortion.”

Since the onset of her pain, she had been hospitalized twice elsewhere, with no clear diagnosis to explain her pain. Treatment consisted of repeat doses of hydromorphone. During the second hospitalization, a sodium level of 109 mEq/L had been corrected with hypertonic saline, and a urinary tract infection (UTI) had been treated with cephalexin.

Our patient had never experienced similar abdominal pain. Her medical history included depression and asthma. Her family history was notable for an aunt who had died of lung cancer.

On this ED visit, the patient’s vital signs were normal. On examination, she was moaning in pain and clutching her abdomen. The abdomen was tender in both lower quadrants, with guarding but no rebound. Her sodium level was 125 mEq/L; the day before it had been 132 mEq/L.

Urine dipstick testing showed 2+ glucose and 2+ bilirubin; both had been within normal range (negative) the day before. An abdominal/pelvic computed tomography scan with intravenous (IV) and oral contrast did not reveal any potential cause of the patient’s pain. Multiple doses of IV hydromorphone were given, but her pain persisted.

We revisited our patient’s family history. With prompting, she remembered that as a teenager, her mother had had an illness that caused “problems with her nerves and blood vessels and turned her urine red.” When reached by phone, the patient’s mother, who lives outside the United States, said she was familiar with the term “porphyria,” but curiously, she did not state she carried the diagnosis, and had not advised her children they could be at risk.

The patient was admitted to the intensive care unit (ICU) for treatment of hyponatremia. Her mother’s history led us to suspect porphyria, so we sent a urine sample from the ED for porphobilinogen (PBG) testing. Her urine was not red at that time (on further questioning, she remembered she had had an episode of “red urine” recently). Two days later, after the PBG result came back positive, treatment was initiated for porphyria. With further stool and serum testing, the diagnosis of acute intermittent porphyria (AIP) was made.

The patient was treated with glucose loading and hemin therapy. In the ICU, 2 ampules of 50% dextrose in water solution (D50W) was administered, and she was transferred to the hematology-oncology service for hemin therapy. Soon after, she underwent a dilation and curettage. Three weeks later, she was discharged in good condition.

Discussion

The unifying diagnostic concept is that toxins damage all components of the nervous system: intestinal, central, peripheral, and autonomic. Hence, any combination of abdominal pain, vomiting, psychiatric symptoms, vital sign instability, weakness, or sensory loss may occur.¹ To further confuse the diagnostician, the constellation of symptoms may vary with each episode.

Two critical laboratory clues are red urine—which often is mistaken for a UTI or hematuria—and hyponatremia.¹ Another porphyria hallmark is triggers. These include drugs, carbohydrate deprivation, smoking, and stress. Common chemical inciters include alcohol, ketamine, etomidate, macrodantin, nifedipine, progesterone, and phenytoin.¹ The Atkins diet (zero carbohydrates) reportedly caused an uptick in new porphyria cases.¹

Attacks usually start after puberty. Women tend to experience flares during the luteal (progesterone) phase of the menstrual cycle.¹ Acute intermittent porphyria can mimic Guillain-Barré syndrome and psychosis.² Delayed diagnosis may lead to irreversible neurological damage or death.²

Despite AIP’s complexity, initial diagnostic testing is simple: a urinary PBG level obtained during an attack is virtually 100% sensitive and specific for AIP and two other acute porphyrias: hereditary coproporphyria (HCP), and variegate porphyria (VP). A positive urine PBG mandates immediate treatment—even while awaiting porphyrin and GK delta-aminolevulinic acid (ALA) levels in stool and serum to identify which porphyria (AIP, HCP, or VP) is present. The fourth (and least common) acute porphyria, ALA dehydratase porphyria (ADP), may produce no PBG elevation and requires separate porphyrin and ALA testing to make the diagnosis.

Treatment

Treatment targets runaway heme precursor synthesis at its start and finish (Figure). Glucose-loading suppresses the initial enzyme, ALA synthase. Since the absence of normal end-product (heme) drives the enzymatic cascade, addition of IV hemin provides the substrate—and negative feedback—to stop it.

Conclusion

This case represents an example of AIP in which a patient presented with the characteristic abdominal pain and hyponatremia, complicated by the fact that she was pregnant and her urine was not red.

Intractable abdominal pain with negative imaging must prompt a search for red urine, neurological symptoms, porphyria medication triggers, and a family history of porphyria. Any constellation of findings should prompt immediate urine PBG testing.

In the appropriate clinical setting, it may be prudent to glucose-load a patient while waiting for confirmatory testing (which can take 1-2 days). Hemin therapy is best instituted by the hematology service after high urine PBG levels are confirmed.

Intra-articular corticosteroid injections (CSIs) have been a common treatment for osteoarthritis since the 1950s and continue to be an option for patients who prefer nonoperative management.¹ Although CSIs may improve pain secondary to osteoarthritis temporarily, they do not slow articular cartilage degradation, and many patients request multiple CSIs before total joint arthroplasty ultimately is required.^1,2 Therefore, acute and chronic side effects of CSI must be considered when repeatedly administering corticosteroids.

A postinjection flare, the most common acute side effect of intra-articular CSI, is characterized by a localized inflammatory response that can last 2 to 3 days. The flare occurs in 2% to 25% of CSI cases.^3-5 Symptoms can range from mild joint effusion to disabling pain.⁶ In the present case, a severe postinjection flare occurred after intra-articular administration of triamcinolone acetonide (Kenalog). This case is novel in that its acuity of onset, severity of symptoms, and synovial fluid analysis mimicked septic arthritis, which was ultimately ruled out with negative cultures and confirmation of triamcinolone acetonide crystals in the synovial aspirate, viewed by polarized light microscopy. To date, only one other case of an immediate (<2 hours) and severe postinjection flare in response to triamcinolone has been reported.⁷ As CSIs are often used in the nonoperative treatment of osteoarthritis, it is imperative for the treating physician to be aware of this potential side effect in order to appropriately inform the patient of this risk and guide treatment should the scenario arise. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

A 56-year-old woman with a history of hypertension, hypothyroidism, and moderate bilateral knee osteoarthritis presented with left knee pain. She had been receiving annual hylan injections for 5 years and had no adverse reactions, but the pain gradually worsened over the past 3 months. She was given an intra-articular injection of 2 mL of 1% lidocaine and 2 mL (40 mg) of triamcinolone acetonide in the left knee.

Two hours later, she experienced swelling and intense pain in the knee and was unable to ambulate. Physical examination revealed she was afebrile but was having severe pain in the knee through all range of motion. The knee had no appreciable erythema or warmth. Laboratory data were significant: White blood cell (WBC) count was 14,600, and erythrocyte sedimentation rate was 1 mm/h. The knee was aspirated with a return of 25 mL of “butterscotch”-colored fluid (Figure 1). The patient was admitted to rule out iatrogenic septic arthritis, or chronic, indolent septic arthritis acutely worsened by CSI, until synovial fluid analysis and cultures could be performed (Table 1).

She was treated overnight with a compressive wrap, elevation, ice, and nonsteroidal anti-inflammatory drugs, which provided significant pain relief. Polarized light microscopy revealed polymorphic intracellular and extracellular crystals with crystal morphology consistent with the injection of triamcinolone ester (Figure 2). Gram stain showed many WBCs but no organisms. These findings were thought to represent an exogenous crystal-induced acute inflammatory response. Given the patient’s improving clinical course, she was discharged the next morning.

Twelve days later, at clinic follow-up, she was still experiencing pain above her baseline level. Given the continued effusion, 8 mL of synovial fluid was aspirated, which appeared clear and only slightly blood-tinged. Synovial analysis showed resolution of leukocytosis, confirming a severe postinjection flare in response to triamcinolone acetonide.

Discussion

Although rare, side effects from repeated intra-articular CSIs include hypothalamic-pituitary-adrenal axis dysfunction and steroid-induced myopathy.^8,9 Acute side effects are more common and include postinjection flare, iatrogenic septic arthritis, local tissue atrophy, cartilage damage, tendon rupture, nerve atrophy, increased blood glucose, and osteonecrosis.^10,11 The present case report describes an extreme example of a postinjection flare in response to triamcinolone acetonide and summarizes the characteristics of injections that cause flares.

The physical properties of corticosteroids have a significant impact on their efficacy and on their potential for adverse events. Corticosteroid preparations can be water-soluble or water-insoluble. Most commonly, water-insoluble preparations that contain insoluble corticosteroid esters (eg, triamcinolone, methylprednisolone) are used in intra-articular injections. These form microcrystalline aggregates in solution, which require the patient’s own hydrolytic enzymes (esterases) to release the active moiety and thus have a longer duration of action. However, they are more commonly associated with postinjection flares compared with their more soluble and faster- acting counterparts (eg. dexamethasone, betamethasone).¹⁰ Microcrystalline aggregates, which are larger in size, induce a stronger inflammatory response, and in a dose-dependent manner.6A sterile inflammatory reaction to hydrocortisone, cortisone, dexamethasone, triamcinolone, and prednisolone crystals in normal joints has been previously described,6,12,13 and crystals of the various preparations have been demonstrated within leukocytes by both polarized light and electron microscopy.^12,13 Table 2 summarizes previous synovial fluid analyses after intra-articular injections of various corticosteroid preparations in normal healthy joints and in patients experiencing a postinjection flare. To date, there have been no reports of an immediate (<2 hours) and severe postinjection flare in response to triamcinolone acetonide, though there was a report of a postinjection flare in response to triamcinolone hexacetonide (Aristospan),⁷ and here the synovial fluid WBC count (30,000) was much lower.

Although many cases of corticosteroid hypersensitivity have been reported, in rare cases intra-articular administration of triamcinolone has caused anaphylactic reactions and shock.^14,15 Multiple case studies have determined that the specific excipient carboxymethylcellulose (found in many triamcinolone preparations), and not the corticosteroid itself, can cause an immunoglobulin E–mediated anaphylactic reaction.^16-18 Therefore, performing skin-prick tests for potential corticosteroids and their excipients in patients with known postinjection flares might help prevent serious adverse reactions.^18,19

The present case involved an extreme postinjection flare in response to intra-articular administration of triamcinolone acetonide. Postinjection flares are rare but significant events, and physicians using CSIs in the treatment of arthritis need to be aware of this potential reaction in order to appropriately inform patients of this risk and guide treatment should the scenario arise.

Intra-articular corticosteroid injections (CSIs) have been a common treatment for osteoarthritis since the 1950s and continue to be an option for patients who prefer nonoperative management.¹ Although CSIs may improve pain secondary to osteoarthritis temporarily, they do not slow articular cartilage degradation, and many patients request multiple CSIs before total joint arthroplasty ultimately is required.^1,2 Therefore, acute and chronic side effects of CSI must be considered when repeatedly administering corticosteroids.

A postinjection flare, the most common acute side effect of intra-articular CSI, is characterized by a localized inflammatory response that can last 2 to 3 days. The flare occurs in 2% to 25% of CSI cases.^3-5 Symptoms can range from mild joint effusion to disabling pain.⁶ In the present case, a severe postinjection flare occurred after intra-articular administration of triamcinolone acetonide (Kenalog). This case is novel in that its acuity of onset, severity of symptoms, and synovial fluid analysis mimicked septic arthritis, which was ultimately ruled out with negative cultures and confirmation of triamcinolone acetonide crystals in the synovial aspirate, viewed by polarized light microscopy. To date, only one other case of an immediate (<2 hours) and severe postinjection flare in response to triamcinolone has been reported.⁷ As CSIs are often used in the nonoperative treatment of osteoarthritis, it is imperative for the treating physician to be aware of this potential side effect in order to appropriately inform the patient of this risk and guide treatment should the scenario arise. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

A 56-year-old woman with a history of hypertension, hypothyroidism, and moderate bilateral knee osteoarthritis presented with left knee pain. She had been receiving annual hylan injections for 5 years and had no adverse reactions, but the pain gradually worsened over the past 3 months. She was given an intra-articular injection of 2 mL of 1% lidocaine and 2 mL (40 mg) of triamcinolone acetonide in the left knee.

Two hours later, she experienced swelling and intense pain in the knee and was unable to ambulate. Physical examination revealed she was afebrile but was having severe pain in the knee through all range of motion. The knee had no appreciable erythema or warmth. Laboratory data were significant: White blood cell (WBC) count was 14,600, and erythrocyte sedimentation rate was 1 mm/h. The knee was aspirated with a return of 25 mL of “butterscotch”-colored fluid (Figure 1). The patient was admitted to rule out iatrogenic septic arthritis, or chronic, indolent septic arthritis acutely worsened by CSI, until synovial fluid analysis and cultures could be performed (Table 1).

She was treated overnight with a compressive wrap, elevation, ice, and nonsteroidal anti-inflammatory drugs, which provided significant pain relief. Polarized light microscopy revealed polymorphic intracellular and extracellular crystals with crystal morphology consistent with the injection of triamcinolone ester (Figure 2). Gram stain showed many WBCs but no organisms. These findings were thought to represent an exogenous crystal-induced acute inflammatory response. Given the patient’s improving clinical course, she was discharged the next morning.

Twelve days later, at clinic follow-up, she was still experiencing pain above her baseline level. Given the continued effusion, 8 mL of synovial fluid was aspirated, which appeared clear and only slightly blood-tinged. Synovial analysis showed resolution of leukocytosis, confirming a severe postinjection flare in response to triamcinolone acetonide.

Discussion

Although rare, side effects from repeated intra-articular CSIs include hypothalamic-pituitary-adrenal axis dysfunction and steroid-induced myopathy.^8,9 Acute side effects are more common and include postinjection flare, iatrogenic septic arthritis, local tissue atrophy, cartilage damage, tendon rupture, nerve atrophy, increased blood glucose, and osteonecrosis.^10,11 The present case report describes an extreme example of a postinjection flare in response to triamcinolone acetonide and summarizes the characteristics of injections that cause flares.

The physical properties of corticosteroids have a significant impact on their efficacy and on their potential for adverse events. Corticosteroid preparations can be water-soluble or water-insoluble. Most commonly, water-insoluble preparations that contain insoluble corticosteroid esters (eg, triamcinolone, methylprednisolone) are used in intra-articular injections. These form microcrystalline aggregates in solution, which require the patient’s own hydrolytic enzymes (esterases) to release the active moiety and thus have a longer duration of action. However, they are more commonly associated with postinjection flares compared with their more soluble and faster- acting counterparts (eg. dexamethasone, betamethasone).¹⁰ Microcrystalline aggregates, which are larger in size, induce a stronger inflammatory response, and in a dose-dependent manner.6A sterile inflammatory reaction to hydrocortisone, cortisone, dexamethasone, triamcinolone, and prednisolone crystals in normal joints has been previously described,6,12,13 and crystals of the various preparations have been demonstrated within leukocytes by both polarized light and electron microscopy.^12,13 Table 2 summarizes previous synovial fluid analyses after intra-articular injections of various corticosteroid preparations in normal healthy joints and in patients experiencing a postinjection flare. To date, there have been no reports of an immediate (<2 hours) and severe postinjection flare in response to triamcinolone acetonide, though there was a report of a postinjection flare in response to triamcinolone hexacetonide (Aristospan),⁷ and here the synovial fluid WBC count (30,000) was much lower.

Although many cases of corticosteroid hypersensitivity have been reported, in rare cases intra-articular administration of triamcinolone has caused anaphylactic reactions and shock.^14,15 Multiple case studies have determined that the specific excipient carboxymethylcellulose (found in many triamcinolone preparations), and not the corticosteroid itself, can cause an immunoglobulin E–mediated anaphylactic reaction.^16-18 Therefore, performing skin-prick tests for potential corticosteroids and their excipients in patients with known postinjection flares might help prevent serious adverse reactions.^18,19

The present case involved an extreme postinjection flare in response to intra-articular administration of triamcinolone acetonide. Postinjection flares are rare but significant events, and physicians using CSIs in the treatment of arthritis need to be aware of this potential reaction in order to appropriately inform patients of this risk and guide treatment should the scenario arise.

Intra-articular corticosteroid injections (CSIs) have been a common treatment for osteoarthritis since the 1950s and continue to be an option for patients who prefer nonoperative management.¹ Although CSIs may improve pain secondary to osteoarthritis temporarily, they do not slow articular cartilage degradation, and many patients request multiple CSIs before total joint arthroplasty ultimately is required.^1,2 Therefore, acute and chronic side effects of CSI must be considered when repeatedly administering corticosteroids.

A postinjection flare, the most common acute side effect of intra-articular CSI, is characterized by a localized inflammatory response that can last 2 to 3 days. The flare occurs in 2% to 25% of CSI cases.^3-5 Symptoms can range from mild joint effusion to disabling pain.⁶ In the present case, a severe postinjection flare occurred after intra-articular administration of triamcinolone acetonide (Kenalog). This case is novel in that its acuity of onset, severity of symptoms, and synovial fluid analysis mimicked septic arthritis, which was ultimately ruled out with negative cultures and confirmation of triamcinolone acetonide crystals in the synovial aspirate, viewed by polarized light microscopy. To date, only one other case of an immediate (<2 hours) and severe postinjection flare in response to triamcinolone has been reported.⁷ As CSIs are often used in the nonoperative treatment of osteoarthritis, it is imperative for the treating physician to be aware of this potential side effect in order to appropriately inform the patient of this risk and guide treatment should the scenario arise. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

A 56-year-old woman with a history of hypertension, hypothyroidism, and moderate bilateral knee osteoarthritis presented with left knee pain. She had been receiving annual hylan injections for 5 years and had no adverse reactions, but the pain gradually worsened over the past 3 months. She was given an intra-articular injection of 2 mL of 1% lidocaine and 2 mL (40 mg) of triamcinolone acetonide in the left knee.

Two hours later, she experienced swelling and intense pain in the knee and was unable to ambulate. Physical examination revealed she was afebrile but was having severe pain in the knee through all range of motion. The knee had no appreciable erythema or warmth. Laboratory data were significant: White blood cell (WBC) count was 14,600, and erythrocyte sedimentation rate was 1 mm/h. The knee was aspirated with a return of 25 mL of “butterscotch”-colored fluid (Figure 1). The patient was admitted to rule out iatrogenic septic arthritis, or chronic, indolent septic arthritis acutely worsened by CSI, until synovial fluid analysis and cultures could be performed (Table 1).

She was treated overnight with a compressive wrap, elevation, ice, and nonsteroidal anti-inflammatory drugs, which provided significant pain relief. Polarized light microscopy revealed polymorphic intracellular and extracellular crystals with crystal morphology consistent with the injection of triamcinolone ester (Figure 2). Gram stain showed many WBCs but no organisms. These findings were thought to represent an exogenous crystal-induced acute inflammatory response. Given the patient’s improving clinical course, she was discharged the next morning.

Twelve days later, at clinic follow-up, she was still experiencing pain above her baseline level. Given the continued effusion, 8 mL of synovial fluid was aspirated, which appeared clear and only slightly blood-tinged. Synovial analysis showed resolution of leukocytosis, confirming a severe postinjection flare in response to triamcinolone acetonide.

Discussion

Although rare, side effects from repeated intra-articular CSIs include hypothalamic-pituitary-adrenal axis dysfunction and steroid-induced myopathy.^8,9 Acute side effects are more common and include postinjection flare, iatrogenic septic arthritis, local tissue atrophy, cartilage damage, tendon rupture, nerve atrophy, increased blood glucose, and osteonecrosis.^10,11 The present case report describes an extreme example of a postinjection flare in response to triamcinolone acetonide and summarizes the characteristics of injections that cause flares.

The physical properties of corticosteroids have a significant impact on their efficacy and on their potential for adverse events. Corticosteroid preparations can be water-soluble or water-insoluble. Most commonly, water-insoluble preparations that contain insoluble corticosteroid esters (eg, triamcinolone, methylprednisolone) are used in intra-articular injections. These form microcrystalline aggregates in solution, which require the patient’s own hydrolytic enzymes (esterases) to release the active moiety and thus have a longer duration of action. However, they are more commonly associated with postinjection flares compared with their more soluble and faster- acting counterparts (eg. dexamethasone, betamethasone).¹⁰ Microcrystalline aggregates, which are larger in size, induce a stronger inflammatory response, and in a dose-dependent manner.6A sterile inflammatory reaction to hydrocortisone, cortisone, dexamethasone, triamcinolone, and prednisolone crystals in normal joints has been previously described,6,12,13 and crystals of the various preparations have been demonstrated within leukocytes by both polarized light and electron microscopy.^12,13 Table 2 summarizes previous synovial fluid analyses after intra-articular injections of various corticosteroid preparations in normal healthy joints and in patients experiencing a postinjection flare. To date, there have been no reports of an immediate (<2 hours) and severe postinjection flare in response to triamcinolone acetonide, though there was a report of a postinjection flare in response to triamcinolone hexacetonide (Aristospan),⁷ and here the synovial fluid WBC count (30,000) was much lower.

Although many cases of corticosteroid hypersensitivity have been reported, in rare cases intra-articular administration of triamcinolone has caused anaphylactic reactions and shock.^14,15 Multiple case studies have determined that the specific excipient carboxymethylcellulose (found in many triamcinolone preparations), and not the corticosteroid itself, can cause an immunoglobulin E–mediated anaphylactic reaction.^16-18 Therefore, performing skin-prick tests for potential corticosteroids and their excipients in patients with known postinjection flares might help prevent serious adverse reactions.^18,19

The present case involved an extreme postinjection flare in response to intra-articular administration of triamcinolone acetonide. Postinjection flares are rare but significant events, and physicians using CSIs in the treatment of arthritis need to be aware of this potential reaction in order to appropriately inform patients of this risk and guide treatment should the scenario arise.

T-cell prolymphocytic leukemia (T-PLL) is a rare malignancy that comprises about 2% of all mature lymphoid neoplasms. Patients usually present with prominent peripheral blood lymphocytosis, splenomegaly, hepatomegaly, lymphadenopathy, B symptoms, and occasionally with skin lesions.¹ The disease follows an aggressive clinical course with rapid progression and typically has a median survival of less than 1 year. In some cases, the disease is indolent for a period of time before becoming aggressive.² In 2002, 7 years after initial diagnosis in 1995, the case discussed herein was reported as a rare, indolent form of T-PLL.³ We now present 11 additional years of follow-up of this case, during which time the patient remained asymptomatic with respect to his lymphoid neoplasm.

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

T-cell prolymphocytic leukemia (T-PLL) is a rare malignancy that comprises about 2% of all mature lymphoid neoplasms. Patients usually present with prominent peripheral blood lymphocytosis, splenomegaly, hepatomegaly, lymphadenopathy, B symptoms, and occasionally with skin lesions.¹ The disease follows an aggressive clinical course with rapid progression and typically has a median survival of less than 1 year. In some cases, the disease is indolent for a period of time before becoming aggressive.² In 2002, 7 years after initial diagnosis in 1995, the case discussed herein was reported as a rare, indolent form of T-PLL.³ We now present 11 additional years of follow-up of this case, during which time the patient remained asymptomatic with respect to his lymphoid neoplasm.

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

T-cell prolymphocytic leukemia (T-PLL) is a rare malignancy that comprises about 2% of all mature lymphoid neoplasms. Patients usually present with prominent peripheral blood lymphocytosis, splenomegaly, hepatomegaly, lymphadenopathy, B symptoms, and occasionally with skin lesions.¹ The disease follows an aggressive clinical course with rapid progression and typically has a median survival of less than 1 year. In some cases, the disease is indolent for a period of time before becoming aggressive.² In 2002, 7 years after initial diagnosis in 1995, the case discussed herein was reported as a rare, indolent form of T-PLL.³ We now present 11 additional years of follow-up of this case, during which time the patient remained asymptomatic with respect to his lymphoid neoplasm.

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

Primary bladder adenocarcinoma  is an epithelial malignancy with pure glandular differentiation, without evidence of typical urothelial (transitional cell) carcinoma. PBA is rare, accounting for 0.5%-2% of all malignant bladder neoplasms, and it is seen more frequently in men than in women and is commonly diagnosed in the sixth decade of life.^1-3 Clinical presentation includes hematuria and symptoms of bladder irritation.²

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

Primary bladder adenocarcinoma  is an epithelial malignancy with pure glandular differentiation, without evidence of typical urothelial (transitional cell) carcinoma. PBA is rare, accounting for 0.5%-2% of all malignant bladder neoplasms, and it is seen more frequently in men than in women and is commonly diagnosed in the sixth decade of life.^1-3 Clinical presentation includes hematuria and symptoms of bladder irritation.²

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

Primary bladder adenocarcinoma  is an epithelial malignancy with pure glandular differentiation, without evidence of typical urothelial (transitional cell) carcinoma. PBA is rare, accounting for 0.5%-2% of all malignant bladder neoplasms, and it is seen more frequently in men than in women and is commonly diagnosed in the sixth decade of life.^1-3 Clinical presentation includes hematuria and symptoms of bladder irritation.²

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

Total hip arthroplasty (THA) is considered to be one of the most successful orthopedic interventions of its generation.¹ In 2010, 332,000 THAs were performed in the U.S.² Although used to correct advanced joint diseases in the elderly, the THA procedure has become increasingly common in a younger population for posttraumatic fractures and conditions that lead to early onset secondary arthritis such as avascular necrosis, juvenile rheumatoid arthritis, hip dysplasia, Perthes disease, and femoro-acetabular impingement.

Current hip replacements are expected to function at least 10 to 20 years in 90% of patients.³ As increasing numbers of young patients have these procedures and as seniors continue to live longer, patients will outlast their implants. Younger and more active patients have a higher rate of revision, because the longevity of the prosthesis is usually a function of usage.³ The number of revision THAs is projected to increase 137% by 2030.⁴

Hip resurfacing has been developed as a bone preserving surgical alternative to THA. The first system for use in the U.S. received FDA approval in 2006, but concerns about the metal on metal bearing surfaces, high failure and revision rates, and early catastrophic modes of failure compared with THAs has resulted in the recall of many of these devices. Hip resurfacing may offer some advantages compared with those of a THA in a carefully selected population, but its use will not be further discussed in this case study.⁵ Periprosthetic osteolysis and aseptic loosening are 2 of the long-term consequences of THA.⁶ Bone loss is felt to be secondary to a biologic reaction to particulate debris from implants.⁶ Some patients, especially those with loosening, complete wear, or fracture, will be symptomatic with pain. However, wear and osteolysis is a silent disease unless there is mechanical failure. Other patients may not experience discomfort. Radiographic studies may reveal significant changes, which warrant the recommendation for a hip revision.

Hip revision surgery has 3 major purposes: relieving pain in the affected joint, restoring the patient’s mobility, and removing a loose or damaged prosthesis before irreversible harm is done to the joint. It’s anticipated that most primary care providers (PCPs) will encounter patients who seek advice on the need for a revision hip arthroplasty.

This case will present an asymptomatic patient who underwent a THA in 1997 at age 37, to address developmental dysplasia of the hip (DDH) and was advised to undergo a revision hip arthroplasty due to abnormal radiographic findings at age 55 years. A discussion will follow that includes a brief review of the history of THA, the materials and bearings commonly used, the presenting symptoms or radiographic changes that signal the need for a revision, and the current options available for a patient such as this.

Case Report

A man aged 55 years presented to a new orthopedic surgeon for his first orthopedic appointment in 10 years. The patient had a left metal-on-polyethylene (M-on-PE) THA 18 years prior due to early onset secondary degenerative joint disease from DDH. The patient’s M-on-PE THA was a titanium acetabular socket and femoral stem with a cobalt-chromium alloy femoral head and a polyethylene liner. The patient remained physically active with an exercise routine consisting of walking, swimming, and weight training.

The patient’s orthopedic history was notable for a right knee arthroscopy for intervention due to a torn medial and lateral meniscus, and birth history was noteworthy for a breech presentation. The physical exam was unremarkable except for a slight leg length discrepancy, but the patient did not exhibit a Trendelenburg gait.

Plain X-rays and a computed tomography (CT) scan showed eccentric PE wear and superior migration of the femoral head, which was indicative of significant PE liner wear. No significant osteolysis or periprosthetic loosening was observed on the X-rays or CT scan. He was advised that a hip revision procedure would need to be done, optimally, within the next 6 months to a year.

Discussion

Hip dysplasia represents a broad group of disorders and generally means abnormal development of the hip joint. The term is most commonly used to refer to DDH with inadequate coverage of the femoral head. In one study, 25% of hip replacements performed in patients aged ≤ 40 years were due to underlying hip dysplasia.⁷

Developmental dysplasia of the hip occurs more often in children who present in the breech position.⁸ One theory argues that packaging issues in utero may account for the increased incidence of DDH.⁹ The earliest recorded attempts at hip replacement occurred in Germany, in 1891, when ivory was used to replace the femoral heads of patients whose hip joints had been destroyed by tuberculosis.¹

The orthopedic surgeon Sir John Charnley, who worked at the Manchester Royal Infirmary, is considered the father of the modern THA.¹ His low friction arthroplasty, designed in the early 1960s is identical, in principle, to the M-on-PE prosthesis used today.¹ The PE liner used was ultrahigh molecular weight polyethylene (UHMWPE).¹

Due to the early success of the Charnley prosthesis, the M-on-PE prosthesis became the most widely used. Although PE is the most studied and understood of all acetabular liner materials, it will eventually wear and shed debris. Acetabular cup wear is the most frequent reason for mid-to-long-term revisions, especially in young and active patients.¹⁰ More active patients shed more debris.³ The PE debris instigates the release of inflammatory mediators, which results in chronic inflammation and tissue damage that erodes the supporting bone and can lead to implant loosening or fracture.⁶ Ongoing studies seek to optimize and improve properties of the UHMWPE and to develop alternative bearings. After FDA approval in 1999, highly cross-linked polyethylene liners (HXLPE) rapidly became the standard of care for THAs, at least in the U.S.¹¹ Highly cross-linked polyethylene liners are created from UHMWPE through a process of cross-linking by exposure to gamma radiation, and subsequent heat treatment to neutralize free radicals and limit oxidative degradation.¹²

In one study, the 5-year annual linear wear rate for a HXLPE liner was only 45% of that seen with the UHMWPE liner, although the qualitative wear pattern was the same.¹³ In a study that followed patients for 7 years postoperatively, the mean steady-state wear rate of the HXLPE was 0.005 mm/y compared with 0.037 mm/y for UHMWPE.¹⁴ In a long-term study (a minimum follow-up of 10 years) of 50 patients who were aged < 50 years and underwent THA using HXLPE liners, there was no radiographic evidence of osteolysis or component loosening, and liner wear was 0.020 ± 0.0047 mm/y.¹² In 2005, second-generation HXLPE liners were introduced clinically and have been shown to further reduce wear in vitro compared with both UHMWPE and first-generation HXLPE liners. Callary and colleagues calculated that the wear rates between 1 year and 5 years were all < 0.001 mm/y.¹⁵

The use of ceramic for THAs began in 1970, and ceramic heads on polyethylene (C-on-PE) liners and ceramic-on-ceramic (C-on-C) bearings have been in continual use for > 30 years in Europe. Premarket FDA approval based on European data was granted in 1983; however, the manufacturer voluntarily removed it from the market because of a high incidence of stem loosening (> 30% within 3 years in some series).¹⁶ FDA approvals came much later for C-on-PE (1989) and C-on-C (2003) bearings.

Ceramic is the hardest implant material used, and it can be concluded from many clinical and laboratory reports that C-on-PE and C-on-C combinations confer a potentially significant reduction in wear on THA bearings.¹⁶ Ceramic hips initially had 2 concerns: catastrophic shattering and squeaking. Current ceramic hips have been substantially improved, and some experts feel shattering has been essentially eliminated.¹⁶ Other experts note that ceramic brittleness remains a major concern.¹⁷ Squeaking remains a problem for some, but it usually abates over time. No study has correlated squeaking with impending failure or increased pain or disability.

While C-on-C bearings are now felt to be a good implant for young active patients, these bearings have generally not resulted in significantly lower wear rates and fewer revisions.¹⁸ High rates of wear and osteolysis have been sporadically documented over the 35-year history of ceramic implants.¹⁶ The FDA approved the first ceramic-on-metal total hip replacement system on June 13, 2011.

Metal-on-metal (M-on-M) implants have been used by some for decades, although they were not approved by the FDA until the late 1990s. However, some device recalls have brought negative attention to M-on-M implants.¹⁹ It was felt that they would generate less wear debris than PE, but reports of pseudotumors (from inflammatory mediators) and metallosis have significantly tempered enthusiasm for these products.^20,21 The wear rates are very low, estimated to be only 0.01 mm/y, but concerns about the carcinogenetic potential of systemically increased metal ions remains a possible and much debated concern.^19,22,23 In January 2013, FDA issued a safety communication on M-on-M implants.

Many experts feel that modern ceramic or metal on second-generation HXLPE represents the gold standard and the most predictable bearing choice for young, active patients.¹⁸ Others feel that the optimal choice of bearing surfaces in THA, particularly in the younger and more active patient, remains controversial.²⁴

Follow-Up

Intermittent orthopedic monitoring is recommended for all patients who have undergone a THA. The frequency of hip X-rays on follow-up appointments is left to the orthopedic surgeon. After the initial recovery, serial images every 2 to 5 years can identify progressive failure, and annual X-rays may be used for closer follow-up in high-risk patients.

Patients who experience dislocations, fractures, infections, or pain usually maintain close orthopedic follow-up. Significant wear of the prosthesis damages the socket; osteolysis can cause irreversible bone loss, fracture, and loosening. Massive acetabular bone loss is very difficult to reverse and creates major reconstruction challenges.

Figure 1A is a 2009 X-ray of a woman aged 44 years who underwent a THA after a motor vehicle accident in 1997 and who was advised to have a revision THA when seen in 2009.

Figure 3A is an X-ray of a man aged 71 years who had undergone THA 21 years earlier and had complied with routine follow-up. When his X-rays showed significant wear of the liner and some osteolysis, he was able to undergo a simple revision (Figure 3B).

Three-dimensional CT is useful for quantifying the presence and severity of osteolytic lesions, because plain radiographs may underestimate the amount of bone loss that is present.²⁵ The CT in Figure 3C shows the magnitude of osteolysis that was underestimated by the preoperative plain X-rays (Figure 3A). Computed tomography scans are crucial for surgical planning in the setting of severe acetabular bone loss.

There is a wide spectrum of signs and symptoms that can occur in the setting of acetabular component failure. Pain is a common presenting symptom. Groin pain can represent acetabular failure; thigh pain may be correlated to femoral component failure.²⁵ The clinical patient presentation ultimately depends on the underlying cause: an infection, polyethylene wear, instability, or aseptic loosening.²⁵ Leg-length discrepancy, joint deformity, location of prior incisions, functional status, and baseline neurologic status should be evaluated and documented during the preoperative evaluation as well.²⁵

Case Study Revision Options

The X-rays and CT scans for this case study patient showed that he was a possible candidate for the simplest revision surgery; an isolated liner exchange and replacement of the femoral head. When the original surgery was performed (1997), the only FDA approved PE liner was UHMWPE. To justify isolated liner exchange, the modular acetabular metallic shell also should be well-fixed and appropriately oriented.²⁶ This is evaluated both preoperatively and intraoperatively.

If found to be well fixed with an appropriate orientation and locking mechanism, the UHMWPE liner could be replaced with a HXLPE liner and a larger metal femoral head for improved wear and stability. Acetabular revision is indicted for an asymptomatic patient who has progressive osteolysis, severe wear, or bone loss that would compromise future reconstruction.

Conclusions

Over the past several decades, THA has become recognized as an effective treatment option for the reduction of pain and disability associated with hip joint disease and is associated with successful clinical outcomes. The most frequently noted recommendations for trying to increase the life expectancy of an artificial hip replacement include maintaining a normal weight, keeping leg muscles strong, and avoiding repetitive squatting and kneeling.

As the number of primary THAs has increased and the average age of those undergoing a primary THA has decreased, the need for revisions has risen. Reviews have demonstrated that the most common causes for early total hip revision, regardless of component, included infection, instability/dislocation, and fracture, whereas wear is the most common reason for mid to late revisions.

The wear of all materials used has been shown to be greatest in the most active patients.

Studies continue to identify ways to potentially prevent or reverse osteolysis from wear debris. Alendronate therapy has been shown to prevent and treat PE debris-induced periprosthetic bone loss in rats.²⁷ It also was successfully used in a case report of an asymptomatic woman aged 39 years who had rapid PE wear and aggressive periprosthetic osteolysis within just 2 years of a bilateral THA.²⁸ Other areas of research on decreasing osteolysis in THA recipients include trials with mesenchymal stem cells, bone morphogenic proteins, and gene therapy.⁶

In the U.S., 46,000 revisions were performed in 2004 and this number is expected to more than double by 2030.⁴ Primary care providers are sure to encounter patients who will be in need of a hip revision procedure. It’s important for them to make sure that their patients who have undergone a THA are periodically seen for orthopedic follow-up. Despite the long history of primary THAs, there is still not a single technique and material to suit all patient characteristics.¹ Unfortunately, the same currently applies to hip revision procedures.

Total hip arthroplasty (THA) is considered to be one of the most successful orthopedic interventions of its generation.¹ In 2010, 332,000 THAs were performed in the U.S.² Although used to correct advanced joint diseases in the elderly, the THA procedure has become increasingly common in a younger population for posttraumatic fractures and conditions that lead to early onset secondary arthritis such as avascular necrosis, juvenile rheumatoid arthritis, hip dysplasia, Perthes disease, and femoro-acetabular impingement.

Current hip replacements are expected to function at least 10 to 20 years in 90% of patients.³ As increasing numbers of young patients have these procedures and as seniors continue to live longer, patients will outlast their implants. Younger and more active patients have a higher rate of revision, because the longevity of the prosthesis is usually a function of usage.³ The number of revision THAs is projected to increase 137% by 2030.⁴

Hip resurfacing has been developed as a bone preserving surgical alternative to THA. The first system for use in the U.S. received FDA approval in 2006, but concerns about the metal on metal bearing surfaces, high failure and revision rates, and early catastrophic modes of failure compared with THAs has resulted in the recall of many of these devices. Hip resurfacing may offer some advantages compared with those of a THA in a carefully selected population, but its use will not be further discussed in this case study.⁵ Periprosthetic osteolysis and aseptic loosening are 2 of the long-term consequences of THA.⁶ Bone loss is felt to be secondary to a biologic reaction to particulate debris from implants.⁶ Some patients, especially those with loosening, complete wear, or fracture, will be symptomatic with pain. However, wear and osteolysis is a silent disease unless there is mechanical failure. Other patients may not experience discomfort. Radiographic studies may reveal significant changes, which warrant the recommendation for a hip revision.

Hip revision surgery has 3 major purposes: relieving pain in the affected joint, restoring the patient’s mobility, and removing a loose or damaged prosthesis before irreversible harm is done to the joint. It’s anticipated that most primary care providers (PCPs) will encounter patients who seek advice on the need for a revision hip arthroplasty.

This case will present an asymptomatic patient who underwent a THA in 1997 at age 37, to address developmental dysplasia of the hip (DDH) and was advised to undergo a revision hip arthroplasty due to abnormal radiographic findings at age 55 years. A discussion will follow that includes a brief review of the history of THA, the materials and bearings commonly used, the presenting symptoms or radiographic changes that signal the need for a revision, and the current options available for a patient such as this.

Case Report

A man aged 55 years presented to a new orthopedic surgeon for his first orthopedic appointment in 10 years. The patient had a left metal-on-polyethylene (M-on-PE) THA 18 years prior due to early onset secondary degenerative joint disease from DDH. The patient’s M-on-PE THA was a titanium acetabular socket and femoral stem with a cobalt-chromium alloy femoral head and a polyethylene liner. The patient remained physically active with an exercise routine consisting of walking, swimming, and weight training.

The patient’s orthopedic history was notable for a right knee arthroscopy for intervention due to a torn medial and lateral meniscus, and birth history was noteworthy for a breech presentation. The physical exam was unremarkable except for a slight leg length discrepancy, but the patient did not exhibit a Trendelenburg gait.

Plain X-rays and a computed tomography (CT) scan showed eccentric PE wear and superior migration of the femoral head, which was indicative of significant PE liner wear. No significant osteolysis or periprosthetic loosening was observed on the X-rays or CT scan. He was advised that a hip revision procedure would need to be done, optimally, within the next 6 months to a year.

Discussion

Hip dysplasia represents a broad group of disorders and generally means abnormal development of the hip joint. The term is most commonly used to refer to DDH with inadequate coverage of the femoral head. In one study, 25% of hip replacements performed in patients aged ≤ 40 years were due to underlying hip dysplasia.⁷

Developmental dysplasia of the hip occurs more often in children who present in the breech position.⁸ One theory argues that packaging issues in utero may account for the increased incidence of DDH.⁹ The earliest recorded attempts at hip replacement occurred in Germany, in 1891, when ivory was used to replace the femoral heads of patients whose hip joints had been destroyed by tuberculosis.¹

The orthopedic surgeon Sir John Charnley, who worked at the Manchester Royal Infirmary, is considered the father of the modern THA.¹ His low friction arthroplasty, designed in the early 1960s is identical, in principle, to the M-on-PE prosthesis used today.¹ The PE liner used was ultrahigh molecular weight polyethylene (UHMWPE).¹

Due to the early success of the Charnley prosthesis, the M-on-PE prosthesis became the most widely used. Although PE is the most studied and understood of all acetabular liner materials, it will eventually wear and shed debris. Acetabular cup wear is the most frequent reason for mid-to-long-term revisions, especially in young and active patients.¹⁰ More active patients shed more debris.³ The PE debris instigates the release of inflammatory mediators, which results in chronic inflammation and tissue damage that erodes the supporting bone and can lead to implant loosening or fracture.⁶ Ongoing studies seek to optimize and improve properties of the UHMWPE and to develop alternative bearings. After FDA approval in 1999, highly cross-linked polyethylene liners (HXLPE) rapidly became the standard of care for THAs, at least in the U.S.¹¹ Highly cross-linked polyethylene liners are created from UHMWPE through a process of cross-linking by exposure to gamma radiation, and subsequent heat treatment to neutralize free radicals and limit oxidative degradation.¹²

In one study, the 5-year annual linear wear rate for a HXLPE liner was only 45% of that seen with the UHMWPE liner, although the qualitative wear pattern was the same.¹³ In a study that followed patients for 7 years postoperatively, the mean steady-state wear rate of the HXLPE was 0.005 mm/y compared with 0.037 mm/y for UHMWPE.¹⁴ In a long-term study (a minimum follow-up of 10 years) of 50 patients who were aged < 50 years and underwent THA using HXLPE liners, there was no radiographic evidence of osteolysis or component loosening, and liner wear was 0.020 ± 0.0047 mm/y.¹² In 2005, second-generation HXLPE liners were introduced clinically and have been shown to further reduce wear in vitro compared with both UHMWPE and first-generation HXLPE liners. Callary and colleagues calculated that the wear rates between 1 year and 5 years were all < 0.001 mm/y.¹⁵

The use of ceramic for THAs began in 1970, and ceramic heads on polyethylene (C-on-PE) liners and ceramic-on-ceramic (C-on-C) bearings have been in continual use for > 30 years in Europe. Premarket FDA approval based on European data was granted in 1983; however, the manufacturer voluntarily removed it from the market because of a high incidence of stem loosening (> 30% within 3 years in some series).¹⁶ FDA approvals came much later for C-on-PE (1989) and C-on-C (2003) bearings.

Ceramic is the hardest implant material used, and it can be concluded from many clinical and laboratory reports that C-on-PE and C-on-C combinations confer a potentially significant reduction in wear on THA bearings.¹⁶ Ceramic hips initially had 2 concerns: catastrophic shattering and squeaking. Current ceramic hips have been substantially improved, and some experts feel shattering has been essentially eliminated.¹⁶ Other experts note that ceramic brittleness remains a major concern.¹⁷ Squeaking remains a problem for some, but it usually abates over time. No study has correlated squeaking with impending failure or increased pain or disability.

While C-on-C bearings are now felt to be a good implant for young active patients, these bearings have generally not resulted in significantly lower wear rates and fewer revisions.¹⁸ High rates of wear and osteolysis have been sporadically documented over the 35-year history of ceramic implants.¹⁶ The FDA approved the first ceramic-on-metal total hip replacement system on June 13, 2011.

Metal-on-metal (M-on-M) implants have been used by some for decades, although they were not approved by the FDA until the late 1990s. However, some device recalls have brought negative attention to M-on-M implants.¹⁹ It was felt that they would generate less wear debris than PE, but reports of pseudotumors (from inflammatory mediators) and metallosis have significantly tempered enthusiasm for these products.^20,21 The wear rates are very low, estimated to be only 0.01 mm/y, but concerns about the carcinogenetic potential of systemically increased metal ions remains a possible and much debated concern.^19,22,23 In January 2013, FDA issued a safety communication on M-on-M implants.

Many experts feel that modern ceramic or metal on second-generation HXLPE represents the gold standard and the most predictable bearing choice for young, active patients.¹⁸ Others feel that the optimal choice of bearing surfaces in THA, particularly in the younger and more active patient, remains controversial.²⁴

Follow-Up

Intermittent orthopedic monitoring is recommended for all patients who have undergone a THA. The frequency of hip X-rays on follow-up appointments is left to the orthopedic surgeon. After the initial recovery, serial images every 2 to 5 years can identify progressive failure, and annual X-rays may be used for closer follow-up in high-risk patients.

Patients who experience dislocations, fractures, infections, or pain usually maintain close orthopedic follow-up. Significant wear of the prosthesis damages the socket; osteolysis can cause irreversible bone loss, fracture, and loosening. Massive acetabular bone loss is very difficult to reverse and creates major reconstruction challenges.

Figure 1A is a 2009 X-ray of a woman aged 44 years who underwent a THA after a motor vehicle accident in 1997 and who was advised to have a revision THA when seen in 2009.

Figure 3A is an X-ray of a man aged 71 years who had undergone THA 21 years earlier and had complied with routine follow-up. When his X-rays showed significant wear of the liner and some osteolysis, he was able to undergo a simple revision (Figure 3B).

Three-dimensional CT is useful for quantifying the presence and severity of osteolytic lesions, because plain radiographs may underestimate the amount of bone loss that is present.²⁵ The CT in Figure 3C shows the magnitude of osteolysis that was underestimated by the preoperative plain X-rays (Figure 3A). Computed tomography scans are crucial for surgical planning in the setting of severe acetabular bone loss.

There is a wide spectrum of signs and symptoms that can occur in the setting of acetabular component failure. Pain is a common presenting symptom. Groin pain can represent acetabular failure; thigh pain may be correlated to femoral component failure.²⁵ The clinical patient presentation ultimately depends on the underlying cause: an infection, polyethylene wear, instability, or aseptic loosening.²⁵ Leg-length discrepancy, joint deformity, location of prior incisions, functional status, and baseline neurologic status should be evaluated and documented during the preoperative evaluation as well.²⁵

Case Study Revision Options

The X-rays and CT scans for this case study patient showed that he was a possible candidate for the simplest revision surgery; an isolated liner exchange and replacement of the femoral head. When the original surgery was performed (1997), the only FDA approved PE liner was UHMWPE. To justify isolated liner exchange, the modular acetabular metallic shell also should be well-fixed and appropriately oriented.²⁶ This is evaluated both preoperatively and intraoperatively.

If found to be well fixed with an appropriate orientation and locking mechanism, the UHMWPE liner could be replaced with a HXLPE liner and a larger metal femoral head for improved wear and stability. Acetabular revision is indicted for an asymptomatic patient who has progressive osteolysis, severe wear, or bone loss that would compromise future reconstruction.

Conclusions

Over the past several decades, THA has become recognized as an effective treatment option for the reduction of pain and disability associated with hip joint disease and is associated with successful clinical outcomes. The most frequently noted recommendations for trying to increase the life expectancy of an artificial hip replacement include maintaining a normal weight, keeping leg muscles strong, and avoiding repetitive squatting and kneeling.

As the number of primary THAs has increased and the average age of those undergoing a primary THA has decreased, the need for revisions has risen. Reviews have demonstrated that the most common causes for early total hip revision, regardless of component, included infection, instability/dislocation, and fracture, whereas wear is the most common reason for mid to late revisions.

The wear of all materials used has been shown to be greatest in the most active patients.

Studies continue to identify ways to potentially prevent or reverse osteolysis from wear debris. Alendronate therapy has been shown to prevent and treat PE debris-induced periprosthetic bone loss in rats.²⁷ It also was successfully used in a case report of an asymptomatic woman aged 39 years who had rapid PE wear and aggressive periprosthetic osteolysis within just 2 years of a bilateral THA.²⁸ Other areas of research on decreasing osteolysis in THA recipients include trials with mesenchymal stem cells, bone morphogenic proteins, and gene therapy.⁶

In the U.S., 46,000 revisions were performed in 2004 and this number is expected to more than double by 2030.⁴ Primary care providers are sure to encounter patients who will be in need of a hip revision procedure. It’s important for them to make sure that their patients who have undergone a THA are periodically seen for orthopedic follow-up. Despite the long history of primary THAs, there is still not a single technique and material to suit all patient characteristics.¹ Unfortunately, the same currently applies to hip revision procedures.

Total hip arthroplasty (THA) is considered to be one of the most successful orthopedic interventions of its generation.¹ In 2010, 332,000 THAs were performed in the U.S.² Although used to correct advanced joint diseases in the elderly, the THA procedure has become increasingly common in a younger population for posttraumatic fractures and conditions that lead to early onset secondary arthritis such as avascular necrosis, juvenile rheumatoid arthritis, hip dysplasia, Perthes disease, and femoro-acetabular impingement.

Current hip replacements are expected to function at least 10 to 20 years in 90% of patients.³ As increasing numbers of young patients have these procedures and as seniors continue to live longer, patients will outlast their implants. Younger and more active patients have a higher rate of revision, because the longevity of the prosthesis is usually a function of usage.³ The number of revision THAs is projected to increase 137% by 2030.⁴

Hip resurfacing has been developed as a bone preserving surgical alternative to THA. The first system for use in the U.S. received FDA approval in 2006, but concerns about the metal on metal bearing surfaces, high failure and revision rates, and early catastrophic modes of failure compared with THAs has resulted in the recall of many of these devices. Hip resurfacing may offer some advantages compared with those of a THA in a carefully selected population, but its use will not be further discussed in this case study.⁵ Periprosthetic osteolysis and aseptic loosening are 2 of the long-term consequences of THA.⁶ Bone loss is felt to be secondary to a biologic reaction to particulate debris from implants.⁶ Some patients, especially those with loosening, complete wear, or fracture, will be symptomatic with pain. However, wear and osteolysis is a silent disease unless there is mechanical failure. Other patients may not experience discomfort. Radiographic studies may reveal significant changes, which warrant the recommendation for a hip revision.

Hip revision surgery has 3 major purposes: relieving pain in the affected joint, restoring the patient’s mobility, and removing a loose or damaged prosthesis before irreversible harm is done to the joint. It’s anticipated that most primary care providers (PCPs) will encounter patients who seek advice on the need for a revision hip arthroplasty.

This case will present an asymptomatic patient who underwent a THA in 1997 at age 37, to address developmental dysplasia of the hip (DDH) and was advised to undergo a revision hip arthroplasty due to abnormal radiographic findings at age 55 years. A discussion will follow that includes a brief review of the history of THA, the materials and bearings commonly used, the presenting symptoms or radiographic changes that signal the need for a revision, and the current options available for a patient such as this.

Case Report

A man aged 55 years presented to a new orthopedic surgeon for his first orthopedic appointment in 10 years. The patient had a left metal-on-polyethylene (M-on-PE) THA 18 years prior due to early onset secondary degenerative joint disease from DDH. The patient’s M-on-PE THA was a titanium acetabular socket and femoral stem with a cobalt-chromium alloy femoral head and a polyethylene liner. The patient remained physically active with an exercise routine consisting of walking, swimming, and weight training.

The patient’s orthopedic history was notable for a right knee arthroscopy for intervention due to a torn medial and lateral meniscus, and birth history was noteworthy for a breech presentation. The physical exam was unremarkable except for a slight leg length discrepancy, but the patient did not exhibit a Trendelenburg gait.

Plain X-rays and a computed tomography (CT) scan showed eccentric PE wear and superior migration of the femoral head, which was indicative of significant PE liner wear. No significant osteolysis or periprosthetic loosening was observed on the X-rays or CT scan. He was advised that a hip revision procedure would need to be done, optimally, within the next 6 months to a year.

Discussion

Hip dysplasia represents a broad group of disorders and generally means abnormal development of the hip joint. The term is most commonly used to refer to DDH with inadequate coverage of the femoral head. In one study, 25% of hip replacements performed in patients aged ≤ 40 years were due to underlying hip dysplasia.⁷

Developmental dysplasia of the hip occurs more often in children who present in the breech position.⁸ One theory argues that packaging issues in utero may account for the increased incidence of DDH.⁹ The earliest recorded attempts at hip replacement occurred in Germany, in 1891, when ivory was used to replace the femoral heads of patients whose hip joints had been destroyed by tuberculosis.¹

The orthopedic surgeon Sir John Charnley, who worked at the Manchester Royal Infirmary, is considered the father of the modern THA.¹ His low friction arthroplasty, designed in the early 1960s is identical, in principle, to the M-on-PE prosthesis used today.¹ The PE liner used was ultrahigh molecular weight polyethylene (UHMWPE).¹

Due to the early success of the Charnley prosthesis, the M-on-PE prosthesis became the most widely used. Although PE is the most studied and understood of all acetabular liner materials, it will eventually wear and shed debris. Acetabular cup wear is the most frequent reason for mid-to-long-term revisions, especially in young and active patients.¹⁰ More active patients shed more debris.³ The PE debris instigates the release of inflammatory mediators, which results in chronic inflammation and tissue damage that erodes the supporting bone and can lead to implant loosening or fracture.⁶ Ongoing studies seek to optimize and improve properties of the UHMWPE and to develop alternative bearings. After FDA approval in 1999, highly cross-linked polyethylene liners (HXLPE) rapidly became the standard of care for THAs, at least in the U.S.¹¹ Highly cross-linked polyethylene liners are created from UHMWPE through a process of cross-linking by exposure to gamma radiation, and subsequent heat treatment to neutralize free radicals and limit oxidative degradation.¹²

In one study, the 5-year annual linear wear rate for a HXLPE liner was only 45% of that seen with the UHMWPE liner, although the qualitative wear pattern was the same.¹³ In a study that followed patients for 7 years postoperatively, the mean steady-state wear rate of the HXLPE was 0.005 mm/y compared with 0.037 mm/y for UHMWPE.¹⁴ In a long-term study (a minimum follow-up of 10 years) of 50 patients who were aged < 50 years and underwent THA using HXLPE liners, there was no radiographic evidence of osteolysis or component loosening, and liner wear was 0.020 ± 0.0047 mm/y.¹² In 2005, second-generation HXLPE liners were introduced clinically and have been shown to further reduce wear in vitro compared with both UHMWPE and first-generation HXLPE liners. Callary and colleagues calculated that the wear rates between 1 year and 5 years were all < 0.001 mm/y.¹⁵

The use of ceramic for THAs began in 1970, and ceramic heads on polyethylene (C-on-PE) liners and ceramic-on-ceramic (C-on-C) bearings have been in continual use for > 30 years in Europe. Premarket FDA approval based on European data was granted in 1983; however, the manufacturer voluntarily removed it from the market because of a high incidence of stem loosening (> 30% within 3 years in some series).¹⁶ FDA approvals came much later for C-on-PE (1989) and C-on-C (2003) bearings.

Ceramic is the hardest implant material used, and it can be concluded from many clinical and laboratory reports that C-on-PE and C-on-C combinations confer a potentially significant reduction in wear on THA bearings.¹⁶ Ceramic hips initially had 2 concerns: catastrophic shattering and squeaking. Current ceramic hips have been substantially improved, and some experts feel shattering has been essentially eliminated.¹⁶ Other experts note that ceramic brittleness remains a major concern.¹⁷ Squeaking remains a problem for some, but it usually abates over time. No study has correlated squeaking with impending failure or increased pain or disability.

While C-on-C bearings are now felt to be a good implant for young active patients, these bearings have generally not resulted in significantly lower wear rates and fewer revisions.¹⁸ High rates of wear and osteolysis have been sporadically documented over the 35-year history of ceramic implants.¹⁶ The FDA approved the first ceramic-on-metal total hip replacement system on June 13, 2011.

Metal-on-metal (M-on-M) implants have been used by some for decades, although they were not approved by the FDA until the late 1990s. However, some device recalls have brought negative attention to M-on-M implants.¹⁹ It was felt that they would generate less wear debris than PE, but reports of pseudotumors (from inflammatory mediators) and metallosis have significantly tempered enthusiasm for these products.^20,21 The wear rates are very low, estimated to be only 0.01 mm/y, but concerns about the carcinogenetic potential of systemically increased metal ions remains a possible and much debated concern.^19,22,23 In January 2013, FDA issued a safety communication on M-on-M implants.

Many experts feel that modern ceramic or metal on second-generation HXLPE represents the gold standard and the most predictable bearing choice for young, active patients.¹⁸ Others feel that the optimal choice of bearing surfaces in THA, particularly in the younger and more active patient, remains controversial.²⁴

Follow-Up

Intermittent orthopedic monitoring is recommended for all patients who have undergone a THA. The frequency of hip X-rays on follow-up appointments is left to the orthopedic surgeon. After the initial recovery, serial images every 2 to 5 years can identify progressive failure, and annual X-rays may be used for closer follow-up in high-risk patients.

Patients who experience dislocations, fractures, infections, or pain usually maintain close orthopedic follow-up. Significant wear of the prosthesis damages the socket; osteolysis can cause irreversible bone loss, fracture, and loosening. Massive acetabular bone loss is very difficult to reverse and creates major reconstruction challenges.

Figure 1A is a 2009 X-ray of a woman aged 44 years who underwent a THA after a motor vehicle accident in 1997 and who was advised to have a revision THA when seen in 2009.

Figure 3A is an X-ray of a man aged 71 years who had undergone THA 21 years earlier and had complied with routine follow-up. When his X-rays showed significant wear of the liner and some osteolysis, he was able to undergo a simple revision (Figure 3B).

Three-dimensional CT is useful for quantifying the presence and severity of osteolytic lesions, because plain radiographs may underestimate the amount of bone loss that is present.²⁵ The CT in Figure 3C shows the magnitude of osteolysis that was underestimated by the preoperative plain X-rays (Figure 3A). Computed tomography scans are crucial for surgical planning in the setting of severe acetabular bone loss.

There is a wide spectrum of signs and symptoms that can occur in the setting of acetabular component failure. Pain is a common presenting symptom. Groin pain can represent acetabular failure; thigh pain may be correlated to femoral component failure.²⁵ The clinical patient presentation ultimately depends on the underlying cause: an infection, polyethylene wear, instability, or aseptic loosening.²⁵ Leg-length discrepancy, joint deformity, location of prior incisions, functional status, and baseline neurologic status should be evaluated and documented during the preoperative evaluation as well.²⁵

Case Study Revision Options

The X-rays and CT scans for this case study patient showed that he was a possible candidate for the simplest revision surgery; an isolated liner exchange and replacement of the femoral head. When the original surgery was performed (1997), the only FDA approved PE liner was UHMWPE. To justify isolated liner exchange, the modular acetabular metallic shell also should be well-fixed and appropriately oriented.²⁶ This is evaluated both preoperatively and intraoperatively.

If found to be well fixed with an appropriate orientation and locking mechanism, the UHMWPE liner could be replaced with a HXLPE liner and a larger metal femoral head for improved wear and stability. Acetabular revision is indicted for an asymptomatic patient who has progressive osteolysis, severe wear, or bone loss that would compromise future reconstruction.

Conclusions

Over the past several decades, THA has become recognized as an effective treatment option for the reduction of pain and disability associated with hip joint disease and is associated with successful clinical outcomes. The most frequently noted recommendations for trying to increase the life expectancy of an artificial hip replacement include maintaining a normal weight, keeping leg muscles strong, and avoiding repetitive squatting and kneeling.

As the number of primary THAs has increased and the average age of those undergoing a primary THA has decreased, the need for revisions has risen. Reviews have demonstrated that the most common causes for early total hip revision, regardless of component, included infection, instability/dislocation, and fracture, whereas wear is the most common reason for mid to late revisions.

The wear of all materials used has been shown to be greatest in the most active patients.

Studies continue to identify ways to potentially prevent or reverse osteolysis from wear debris. Alendronate therapy has been shown to prevent and treat PE debris-induced periprosthetic bone loss in rats.²⁷ It also was successfully used in a case report of an asymptomatic woman aged 39 years who had rapid PE wear and aggressive periprosthetic osteolysis within just 2 years of a bilateral THA.²⁸ Other areas of research on decreasing osteolysis in THA recipients include trials with mesenchymal stem cells, bone morphogenic proteins, and gene therapy.⁶

In the U.S., 46,000 revisions were performed in 2004 and this number is expected to more than double by 2030.⁴ Primary care providers are sure to encounter patients who will be in need of a hip revision procedure. It’s important for them to make sure that their patients who have undergone a THA are periodically seen for orthopedic follow-up. Despite the long history of primary THAs, there is still not a single technique and material to suit all patient characteristics.¹ Unfortunately, the same currently applies to hip revision procedures.

Introduction

Coarctation of the aorta comprises approximately 5% to 8% of congenital heart defects and is often associated with valvular malformations.¹ These defects are typically diagnosed early and are managed with surgical repair, balloon angioplasty, or endovascular stent placement. However, as the following case illustrates, complications can occur in this population despite early intervention.

Case

A 15-year-old male adolescent presented to the pediatric ED after repeated blood pressure (BP) checks by the school nurse revealed consistently elevated systolic and diastolic pressures. The patient’s hypertension was associated with symptoms of intermittent headache and light-headedness. His medical history was remarkable for a congenital aortic coarctation and a bicuspid aortic valve. The patient had undergone a subclavian flap repair prior to 1 month of age, followed by a balloon dilatation 1 year later for recurrent coarctation. The rest of the patient’s medical history was unremarkable, including normal renal function. He denied illicit drug or alcohol use, sexual activity, or trauma.

On evaluation, the patient’s cardiac examination revealed a regular rate and rhythm with normally split S2; there were no rubs, murmurs, or gallops on auscultation. He had normal and equal pulses of the upper and lower extremities bilaterally. The patient presented without cyanosis. He was alert and oriented with normal upper and lower extremity reflexes. The neurological examination, including cranial nerve, strength, and gait testing, was unremarkable. The gastrointestinal examination showed a soft, nondistended abdomen, with no pulsatile masses. There was no abnormal swelling of his extremities. Although the physical examination findings were unremarkable, the patient’s vital signs were concerning as BP in his right upper extremity was as high as 208/110 mm Hg, while BP in his right leg was 130/68 mm Hg.

The patient followed up with his cardiologist, who ordered cardiac magnetic resonance imaging (MRI). The MRI showed mild narrowing of the distal aortic arch with a minimal and clinically insignificant pressure gradient. Based on the MRI findings, the patient was referred to a pediatric nephrologist, who performed a 24-hour ambulatory BP evaluation. The results of this study showed the patient to have systolic hypertension at the 95th percentile for his age and height. Based on the patient’s athletic predilection, β-blockers were avoided, and he was instead started on the angiotensin-converting enzyme (ACE) inhibitor lisinopril, along with annual follow-up cardiac evaluation.

Discussion

The authors’ initial concern for this patient was the possibility of a recurrent coarctation causing a significant pressure gradient between the upper and lower extremities with associated symptoms. A review of the literature demonstrates such an occurrence is not uncommon in this patient population, especially in patients with a history of early intervention (ie, within the first year of life).²

Causes and Incidence

One of the factors believed to contribute to recurrent coarctation is insufficient growth versus retraction of the manipulated tissues over time. The rates of recurrence vary based on the initial technique used for repair. These recurrences have been found to be approximately 6% in patients who had subclavian flap repairs; 31% for those who had balloon angioplasty alone; and approximately 20% in patients who had aortic stenting.^3-5 As seen in this case, balloon angioplasty is usually performed in patients requiring revascularization. However, up to 32% of these patients will require further intervention due to subsequent recurrence.⁶

Evaluation

Although emergency physicians (EPs) have numerous diagnostic modalities available to evaluate patients with suspected aortic coarctation, as long as the patient is in no acute distress, much of the work-up can be performed on an outpatient basis—in conjunction with the primary- and subspecialty-care team. Regarding appropriate imaging modalities, echocardiography with Doppler or 3D reconstruction of MR angiogram can be useful in detecting both anatomical abnormalities as well as the associated gradient dysfunction; computed tomography can be used for assessing the anatomy.⁷ All of these modalities can also be used to evaluate late-term complications of aortic coarctation pathology, including aortic aneurysms. To help ensure good outcome, the EP should always keep the possibility of recurrence in the differential when evaluating these patients, regardless of the number of previous interventions attempted.

Hypertension

As this case illustrates, patients with a history of coarctation repair often develop high BP. Unfortunately, up to 23% of these patients will go on to have BP above the 95th percentile.⁵ Moreover, a significant number of patients in this population will also suffer from exercise-induced hypertension, even when at-rest BP is controlled with antihypertensive medications.⁸

β-blockers, angiotensin-receptor blockers, and ACE inhibitors are considered the first-line medications for hypertension in adults and adult-sized patients with this condition.⁹

Since a high proportion of patients as young as age 7 years may develop high BP postrepair,¹⁰ the EP should discuss the initiation of an antihypertensive agent with the patient’s care team prior to discharge. It is also important to keep in mind that elevated BP is present to a significant degree even in patients without recurrent obstruction. The negative sequelae associated with uncontrolled hypertension is well known, and patients with congenital anatomical anomalies are at higher risk for such negative outcomes.

Conclusion

This case illustrates a common presentation of a teenaged patient with a chronic medical condition due to a corrected congenital cardiac defect. It also demonstrates the unique and early opportunity the EP has to evaluate and provide appropriate intervention for patients with potentially life-threatening diseases.

Patients with a history of corrective vascular surgery due to congenital heart malformations are an at-risk population. Therefore, during evaluation, the EP should always keep in mind that that these patients have a higher prevalence of related abnormalities at earlier ages than the general population. Steps initiated in the ED prior to discharge, in collaboration with the patient’s primary- and specialty-care team, can assist in expediting appropriate outpatient management of any sequelae. If a patient does not have a cardiologist, a referral to one should always be made prior to discharge.

Dr Smith is a postgraduate year 3 resident in the department of emergency medicine at Alpert Medical School of Brown University, Providence, Rhode Island. Dr Merritt is an assistant professor and pediatric emergency medicine attending in the department of emergency medicine, Brown Alpert Medical School, Providence, Rhode Island. 

Introduction

Coarctation of the aorta comprises approximately 5% to 8% of congenital heart defects and is often associated with valvular malformations.¹ These defects are typically diagnosed early and are managed with surgical repair, balloon angioplasty, or endovascular stent placement. However, as the following case illustrates, complications can occur in this population despite early intervention.

Case

A 15-year-old male adolescent presented to the pediatric ED after repeated blood pressure (BP) checks by the school nurse revealed consistently elevated systolic and diastolic pressures. The patient’s hypertension was associated with symptoms of intermittent headache and light-headedness. His medical history was remarkable for a congenital aortic coarctation and a bicuspid aortic valve. The patient had undergone a subclavian flap repair prior to 1 month of age, followed by a balloon dilatation 1 year later for recurrent coarctation. The rest of the patient’s medical history was unremarkable, including normal renal function. He denied illicit drug or alcohol use, sexual activity, or trauma.

On evaluation, the patient’s cardiac examination revealed a regular rate and rhythm with normally split S2; there were no rubs, murmurs, or gallops on auscultation. He had normal and equal pulses of the upper and lower extremities bilaterally. The patient presented without cyanosis. He was alert and oriented with normal upper and lower extremity reflexes. The neurological examination, including cranial nerve, strength, and gait testing, was unremarkable. The gastrointestinal examination showed a soft, nondistended abdomen, with no pulsatile masses. There was no abnormal swelling of his extremities. Although the physical examination findings were unremarkable, the patient’s vital signs were concerning as BP in his right upper extremity was as high as 208/110 mm Hg, while BP in his right leg was 130/68 mm Hg.

The patient followed up with his cardiologist, who ordered cardiac magnetic resonance imaging (MRI). The MRI showed mild narrowing of the distal aortic arch with a minimal and clinically insignificant pressure gradient. Based on the MRI findings, the patient was referred to a pediatric nephrologist, who performed a 24-hour ambulatory BP evaluation. The results of this study showed the patient to have systolic hypertension at the 95th percentile for his age and height. Based on the patient’s athletic predilection, β-blockers were avoided, and he was instead started on the angiotensin-converting enzyme (ACE) inhibitor lisinopril, along with annual follow-up cardiac evaluation.

Discussion

The authors’ initial concern for this patient was the possibility of a recurrent coarctation causing a significant pressure gradient between the upper and lower extremities with associated symptoms. A review of the literature demonstrates such an occurrence is not uncommon in this patient population, especially in patients with a history of early intervention (ie, within the first year of life).²

Causes and Incidence

One of the factors believed to contribute to recurrent coarctation is insufficient growth versus retraction of the manipulated tissues over time. The rates of recurrence vary based on the initial technique used for repair. These recurrences have been found to be approximately 6% in patients who had subclavian flap repairs; 31% for those who had balloon angioplasty alone; and approximately 20% in patients who had aortic stenting.^3-5 As seen in this case, balloon angioplasty is usually performed in patients requiring revascularization. However, up to 32% of these patients will require further intervention due to subsequent recurrence.⁶

Evaluation

Although emergency physicians (EPs) have numerous diagnostic modalities available to evaluate patients with suspected aortic coarctation, as long as the patient is in no acute distress, much of the work-up can be performed on an outpatient basis—in conjunction with the primary- and subspecialty-care team. Regarding appropriate imaging modalities, echocardiography with Doppler or 3D reconstruction of MR angiogram can be useful in detecting both anatomical abnormalities as well as the associated gradient dysfunction; computed tomography can be used for assessing the anatomy.⁷ All of these modalities can also be used to evaluate late-term complications of aortic coarctation pathology, including aortic aneurysms. To help ensure good outcome, the EP should always keep the possibility of recurrence in the differential when evaluating these patients, regardless of the number of previous interventions attempted.

Hypertension

As this case illustrates, patients with a history of coarctation repair often develop high BP. Unfortunately, up to 23% of these patients will go on to have BP above the 95th percentile.⁵ Moreover, a significant number of patients in this population will also suffer from exercise-induced hypertension, even when at-rest BP is controlled with antihypertensive medications.⁸

β-blockers, angiotensin-receptor blockers, and ACE inhibitors are considered the first-line medications for hypertension in adults and adult-sized patients with this condition.⁹

Since a high proportion of patients as young as age 7 years may develop high BP postrepair,¹⁰ the EP should discuss the initiation of an antihypertensive agent with the patient’s care team prior to discharge. It is also important to keep in mind that elevated BP is present to a significant degree even in patients without recurrent obstruction. The negative sequelae associated with uncontrolled hypertension is well known, and patients with congenital anatomical anomalies are at higher risk for such negative outcomes.

Conclusion

This case illustrates a common presentation of a teenaged patient with a chronic medical condition due to a corrected congenital cardiac defect. It also demonstrates the unique and early opportunity the EP has to evaluate and provide appropriate intervention for patients with potentially life-threatening diseases.

Patients with a history of corrective vascular surgery due to congenital heart malformations are an at-risk population. Therefore, during evaluation, the EP should always keep in mind that that these patients have a higher prevalence of related abnormalities at earlier ages than the general population. Steps initiated in the ED prior to discharge, in collaboration with the patient’s primary- and specialty-care team, can assist in expediting appropriate outpatient management of any sequelae. If a patient does not have a cardiologist, a referral to one should always be made prior to discharge.

Dr Smith is a postgraduate year 3 resident in the department of emergency medicine at Alpert Medical School of Brown University, Providence, Rhode Island. Dr Merritt is an assistant professor and pediatric emergency medicine attending in the department of emergency medicine, Brown Alpert Medical School, Providence, Rhode Island. 

Introduction

Coarctation of the aorta comprises approximately 5% to 8% of congenital heart defects and is often associated with valvular malformations.¹ These defects are typically diagnosed early and are managed with surgical repair, balloon angioplasty, or endovascular stent placement. However, as the following case illustrates, complications can occur in this population despite early intervention.

Case

A 15-year-old male adolescent presented to the pediatric ED after repeated blood pressure (BP) checks by the school nurse revealed consistently elevated systolic and diastolic pressures. The patient’s hypertension was associated with symptoms of intermittent headache and light-headedness. His medical history was remarkable for a congenital aortic coarctation and a bicuspid aortic valve. The patient had undergone a subclavian flap repair prior to 1 month of age, followed by a balloon dilatation 1 year later for recurrent coarctation. The rest of the patient’s medical history was unremarkable, including normal renal function. He denied illicit drug or alcohol use, sexual activity, or trauma.

On evaluation, the patient’s cardiac examination revealed a regular rate and rhythm with normally split S2; there were no rubs, murmurs, or gallops on auscultation. He had normal and equal pulses of the upper and lower extremities bilaterally. The patient presented without cyanosis. He was alert and oriented with normal upper and lower extremity reflexes. The neurological examination, including cranial nerve, strength, and gait testing, was unremarkable. The gastrointestinal examination showed a soft, nondistended abdomen, with no pulsatile masses. There was no abnormal swelling of his extremities. Although the physical examination findings were unremarkable, the patient’s vital signs were concerning as BP in his right upper extremity was as high as 208/110 mm Hg, while BP in his right leg was 130/68 mm Hg.

The patient followed up with his cardiologist, who ordered cardiac magnetic resonance imaging (MRI). The MRI showed mild narrowing of the distal aortic arch with a minimal and clinically insignificant pressure gradient. Based on the MRI findings, the patient was referred to a pediatric nephrologist, who performed a 24-hour ambulatory BP evaluation. The results of this study showed the patient to have systolic hypertension at the 95th percentile for his age and height. Based on the patient’s athletic predilection, β-blockers were avoided, and he was instead started on the angiotensin-converting enzyme (ACE) inhibitor lisinopril, along with annual follow-up cardiac evaluation.

Discussion

The authors’ initial concern for this patient was the possibility of a recurrent coarctation causing a significant pressure gradient between the upper and lower extremities with associated symptoms. A review of the literature demonstrates such an occurrence is not uncommon in this patient population, especially in patients with a history of early intervention (ie, within the first year of life).²

Causes and Incidence

One of the factors believed to contribute to recurrent coarctation is insufficient growth versus retraction of the manipulated tissues over time. The rates of recurrence vary based on the initial technique used for repair. These recurrences have been found to be approximately 6% in patients who had subclavian flap repairs; 31% for those who had balloon angioplasty alone; and approximately 20% in patients who had aortic stenting.^3-5 As seen in this case, balloon angioplasty is usually performed in patients requiring revascularization. However, up to 32% of these patients will require further intervention due to subsequent recurrence.⁶

Evaluation

Although emergency physicians (EPs) have numerous diagnostic modalities available to evaluate patients with suspected aortic coarctation, as long as the patient is in no acute distress, much of the work-up can be performed on an outpatient basis—in conjunction with the primary- and subspecialty-care team. Regarding appropriate imaging modalities, echocardiography with Doppler or 3D reconstruction of MR angiogram can be useful in detecting both anatomical abnormalities as well as the associated gradient dysfunction; computed tomography can be used for assessing the anatomy.⁷ All of these modalities can also be used to evaluate late-term complications of aortic coarctation pathology, including aortic aneurysms. To help ensure good outcome, the EP should always keep the possibility of recurrence in the differential when evaluating these patients, regardless of the number of previous interventions attempted.

Hypertension

As this case illustrates, patients with a history of coarctation repair often develop high BP. Unfortunately, up to 23% of these patients will go on to have BP above the 95th percentile.⁵ Moreover, a significant number of patients in this population will also suffer from exercise-induced hypertension, even when at-rest BP is controlled with antihypertensive medications.⁸

β-blockers, angiotensin-receptor blockers, and ACE inhibitors are considered the first-line medications for hypertension in adults and adult-sized patients with this condition.⁹

Since a high proportion of patients as young as age 7 years may develop high BP postrepair,¹⁰ the EP should discuss the initiation of an antihypertensive agent with the patient’s care team prior to discharge. It is also important to keep in mind that elevated BP is present to a significant degree even in patients without recurrent obstruction. The negative sequelae associated with uncontrolled hypertension is well known, and patients with congenital anatomical anomalies are at higher risk for such negative outcomes.

Conclusion

This case illustrates a common presentation of a teenaged patient with a chronic medical condition due to a corrected congenital cardiac defect. It also demonstrates the unique and early opportunity the EP has to evaluate and provide appropriate intervention for patients with potentially life-threatening diseases.

Patients with a history of corrective vascular surgery due to congenital heart malformations are an at-risk population. Therefore, during evaluation, the EP should always keep in mind that that these patients have a higher prevalence of related abnormalities at earlier ages than the general population. Steps initiated in the ED prior to discharge, in collaboration with the patient’s primary- and specialty-care team, can assist in expediting appropriate outpatient management of any sequelae. If a patient does not have a cardiologist, a referral to one should always be made prior to discharge.

Dr Smith is a postgraduate year 3 resident in the department of emergency medicine at Alpert Medical School of Brown University, Providence, Rhode Island. Dr Merritt is an assistant professor and pediatric emergency medicine attending in the department of emergency medicine, Brown Alpert Medical School, Providence, Rhode Island.