The Clinical Picture

A 79-year-old man presented with sudden-onset bilateral weakness in the lower and upper extremities that had started 6 hours earlier. He reported no vision disturbances or urinary incontinence. He was afebrile, with a blood pressure of 148/94 mm Hg, heart rate 98 bpm, and oxygen saturation of 95% on room air.

Physical examination revealed quadriplegia with hyperreflexia, sustained ankle clonus, and bilateral Babinski reflex, as well as spontaneous adductor and extensor spasms of the lower extremities.

Funduscopy was negative for optic neuritis. Results of a complete blood cell count and renal and liver function testing were within normal limits.

Because the patient’s presentation raised concern for cervical cord compression, urgent magnetic resonance imaging (MRI) of the cervical spine was performed, with and without contrast. It showed abnormal diffuse T2 hyperintensity beginning at the level of the medulla and extending inferiorly to level C7 (Figure 1). This led to a diagnosis of longitudinally extensive transverse myelitis (LETM).

The patient was admitted to the intensive care unit. Methylprednisolone 1 g was given intravenously once daily for 5 days, with plasma exchange every other day for 5 sessions. A workup for neoplastic, autoimmune, and infectious disease was negative, as was testing for serum aquaporin-4 antibody (ie, neuromyelitis optica immunoglobulin G antibody).

Over the course of 7 days, the patient’s motor strength improved, and he was able to walk without assistance. Steroid therapy was tapered, and he was prescribed rituximab to prevent recurrence.

LONGITUDINALLY EXTENSIVE TRANSVERSE MYELITIS

A subtype of transverse myelitis, LETM is defined by partial or complete spinal cord dysfunction due to a lesion extending 3 or more vertebrae as confirmed on MRI. The clinical presentation can include paraparesis, sensory disturbances, and gait, bladder, bowel, or sexual dysfunction.¹ Identifying the cause requires an extensive workup, as the differential diagnosis includes a wide range of conditions²:

• Autoimmune disorders such as Behçet disease, systemic lupus erythematosus, and Sjögren syndrome
• Infectious disorders such as syphilis, tuberculosis, and viral and parasitic infections
• Demyelinating disorders such as multiple sclerosis and neuromyelitis optica
• Neoplastic conditions such as intramedullary metastasis and lymphoma
• Paraneoplastic syndromes.

In our patient, the evaluation did not identify a specific underlying condition, and testing for serum aquaporin-4 antibody was negative. Therefore, the LETM was ruled an isolated idiopathic episode.

Idiopathic seronegative LETM has been associated with fewer recurrences than sero­positive LETM.³ Management consists of high-dose intravenous steroids and plasma exchange. Rituximab can be used to prevent recurrence.⁴

A 79-year-old man presented with sudden-onset bilateral weakness in the lower and upper extremities that had started 6 hours earlier. He reported no vision disturbances or urinary incontinence. He was afebrile, with a blood pressure of 148/94 mm Hg, heart rate 98 bpm, and oxygen saturation of 95% on room air.

Physical examination revealed quadriplegia with hyperreflexia, sustained ankle clonus, and bilateral Babinski reflex, as well as spontaneous adductor and extensor spasms of the lower extremities.

Funduscopy was negative for optic neuritis. Results of a complete blood cell count and renal and liver function testing were within normal limits.

Because the patient’s presentation raised concern for cervical cord compression, urgent magnetic resonance imaging (MRI) of the cervical spine was performed, with and without contrast. It showed abnormal diffuse T2 hyperintensity beginning at the level of the medulla and extending inferiorly to level C7 (Figure 1). This led to a diagnosis of longitudinally extensive transverse myelitis (LETM).

The patient was admitted to the intensive care unit. Methylprednisolone 1 g was given intravenously once daily for 5 days, with plasma exchange every other day for 5 sessions. A workup for neoplastic, autoimmune, and infectious disease was negative, as was testing for serum aquaporin-4 antibody (ie, neuromyelitis optica immunoglobulin G antibody).

Over the course of 7 days, the patient’s motor strength improved, and he was able to walk without assistance. Steroid therapy was tapered, and he was prescribed rituximab to prevent recurrence.

LONGITUDINALLY EXTENSIVE TRANSVERSE MYELITIS

A subtype of transverse myelitis, LETM is defined by partial or complete spinal cord dysfunction due to a lesion extending 3 or more vertebrae as confirmed on MRI. The clinical presentation can include paraparesis, sensory disturbances, and gait, bladder, bowel, or sexual dysfunction.¹ Identifying the cause requires an extensive workup, as the differential diagnosis includes a wide range of conditions²:

• Autoimmune disorders such as Behçet disease, systemic lupus erythematosus, and Sjögren syndrome
• Infectious disorders such as syphilis, tuberculosis, and viral and parasitic infections
• Demyelinating disorders such as multiple sclerosis and neuromyelitis optica
• Neoplastic conditions such as intramedullary metastasis and lymphoma
• Paraneoplastic syndromes.

In our patient, the evaluation did not identify a specific underlying condition, and testing for serum aquaporin-4 antibody was negative. Therefore, the LETM was ruled an isolated idiopathic episode.

Idiopathic seronegative LETM has been associated with fewer recurrences than sero­positive LETM.³ Management consists of high-dose intravenous steroids and plasma exchange. Rituximab can be used to prevent recurrence.⁴

A 79-year-old man presented with sudden-onset bilateral weakness in the lower and upper extremities that had started 6 hours earlier. He reported no vision disturbances or urinary incontinence. He was afebrile, with a blood pressure of 148/94 mm Hg, heart rate 98 bpm, and oxygen saturation of 95% on room air.

Physical examination revealed quadriplegia with hyperreflexia, sustained ankle clonus, and bilateral Babinski reflex, as well as spontaneous adductor and extensor spasms of the lower extremities.

Funduscopy was negative for optic neuritis. Results of a complete blood cell count and renal and liver function testing were within normal limits.

Because the patient’s presentation raised concern for cervical cord compression, urgent magnetic resonance imaging (MRI) of the cervical spine was performed, with and without contrast. It showed abnormal diffuse T2 hyperintensity beginning at the level of the medulla and extending inferiorly to level C7 (Figure 1). This led to a diagnosis of longitudinally extensive transverse myelitis (LETM).

The patient was admitted to the intensive care unit. Methylprednisolone 1 g was given intravenously once daily for 5 days, with plasma exchange every other day for 5 sessions. A workup for neoplastic, autoimmune, and infectious disease was negative, as was testing for serum aquaporin-4 antibody (ie, neuromyelitis optica immunoglobulin G antibody).

Over the course of 7 days, the patient’s motor strength improved, and he was able to walk without assistance. Steroid therapy was tapered, and he was prescribed rituximab to prevent recurrence.

LONGITUDINALLY EXTENSIVE TRANSVERSE MYELITIS

A subtype of transverse myelitis, LETM is defined by partial or complete spinal cord dysfunction due to a lesion extending 3 or more vertebrae as confirmed on MRI. The clinical presentation can include paraparesis, sensory disturbances, and gait, bladder, bowel, or sexual dysfunction.¹ Identifying the cause requires an extensive workup, as the differential diagnosis includes a wide range of conditions²:

• Autoimmune disorders such as Behçet disease, systemic lupus erythematosus, and Sjögren syndrome
• Infectious disorders such as syphilis, tuberculosis, and viral and parasitic infections
• Demyelinating disorders such as multiple sclerosis and neuromyelitis optica
• Neoplastic conditions such as intramedullary metastasis and lymphoma
• Paraneoplastic syndromes.

In our patient, the evaluation did not identify a specific underlying condition, and testing for serum aquaporin-4 antibody was negative. Therefore, the LETM was ruled an isolated idiopathic episode.

Idiopathic seronegative LETM has been associated with fewer recurrences than sero­positive LETM.³ Management consists of high-dose intravenous steroids and plasma exchange. Rituximab can be used to prevent recurrence.⁴

A 59-year-old woman with a history of chronic kidney disease and atonic bladder was brought to the hospital by emergency medical services. She had fallen in her home 2 days earlier and remained on the floor until neighbors eventually heard her cries and called 911. She complained of abdominal pain and distention along with emesis.

On presentation, she had tachycardia and tachypnea. The examination was notable for pronounced abdominal distention, diminished bowel sounds, and costovertebral angle tenderness.

The emergency department physician started empiric treatment for abdominal sepsis, including fluid resuscitation and broad-spectrum antibiotics. Initial imaging studies included abdominal radiography, which revealed a nonobstructive bowel gas pattern but raised suspicion of gas in the bladder (Figure 1). Arterial blood gas analysis showed lactic acidosis.

While laboratory work was being done, the patient’s tachypnea progressed to respiratory distress, and she ultimately required intubation. Vasopressors were started, as the patient was hemodynamically unstable. A Foley catheter was placed, which yielded about 1,100 mL of purulent urine.

Laboratory workup showed:

• Procalcitonin 189 ng/mL (reference range < 2.0 ng/mL)  
• White blood cell count 10.7 × 10⁹/L (4.5–10.0)
• Myoglobin 20,000 ng/mL (< 71)
• Serum creatinine 4.8 mg/dL (0.06–1.10).

Urinalysis was positive for infection; blood and urine cultures later were positive for Escherichia coli.

Computed tomography of the abdomen and pelvis showed diffuse bladder dilation with urine and gas. It also revealed gas within the bladder wall and moderate hydroureter and hydronephrosis (Figure 2).

The patient went into shock that was refractory to pressors, culminating in cardiac arrest despite resuscitative measures.

EMPHYSEMATOUS CYSTITIS, A FORM OF URINARY TRACT INFECTION

Emphysematous cystitis is a rare form of complicated urinary tract infection characterized by gas inside the bladder and in the bladder wall. While the exact mechanisms underlying gas formation are not clear, gas-producing pathogens are clearly implicated in severe infection. E coli and Klebsiella pneumoniae are the most common organisms associated with emphysematous cystitis; others include Proteus mirabilis, and Enterobacter and Streptococcus species.^1,2

More than 50% of patients with emphysematous cystitis have diabetes mellitus. Other risk factors include bladder outlet obstruction, neurogenic bladder, and female sex.³ The severity of disease ranges from asymptomatic pneumaturia (up to 7% of cases)² to fulminant emphysematous cystitis, as in our patient.

The clinical presentation of emphysematous cystitis is nonspecific and can range from minimally symptomatic urinary tract infection to acute abdomen and septic shock.⁴

Some patients present with pneumaturia (the passing of gas through the urethra with micturition). Pneumaturia arises from 3 discrete causes: urologic instrumentation, fistula between the bladder and large or small bowel, and gas-producing bacteria in the bladder (emphysematous cystitis).⁵ Pneumaturia should always raise the suspicion of emphysematous cystitis.

The diagnosis can be made with either radiographic or computed tomographic evidence of gas within the bladder and bladder wall, in the absence of both bladder fistula and history of iatrogenic pneumaturia. Emphysematous cystitis should prompt urine and blood cultures to direct antimicrobial therapy, as 50% of patients with emphysematous cystitis have concomitant bacteremia.⁶

Our patient had an elevated serum level of procalcitonin, a marker of bacterial infection. Procalcitonin is a more specific biomarker of bacterial infection than acute-phase reactants such as the erythrocyte sedimentation rate or the C-reactive protein level. Measuring procalcitonin may help physicians make the diagnosis earlier, differentiate infectious from sterile causes of severe systemic inflammation, assess the severity of systemic inflammation caused by bacterial infections, and decide whether to start or discontinue antibiotic therapy.⁷

Most cases of emphysematous cystitis can be treated with antibiotics, though early diagnosis is crucial to a favorable outcome. Delay in diagnosis may contribute to the 20% mortality rate associated with this condition.⁶    

A 59-year-old woman with a history of chronic kidney disease and atonic bladder was brought to the hospital by emergency medical services. She had fallen in her home 2 days earlier and remained on the floor until neighbors eventually heard her cries and called 911. She complained of abdominal pain and distention along with emesis.

On presentation, she had tachycardia and tachypnea. The examination was notable for pronounced abdominal distention, diminished bowel sounds, and costovertebral angle tenderness.

The emergency department physician started empiric treatment for abdominal sepsis, including fluid resuscitation and broad-spectrum antibiotics. Initial imaging studies included abdominal radiography, which revealed a nonobstructive bowel gas pattern but raised suspicion of gas in the bladder (Figure 1). Arterial blood gas analysis showed lactic acidosis.

While laboratory work was being done, the patient’s tachypnea progressed to respiratory distress, and she ultimately required intubation. Vasopressors were started, as the patient was hemodynamically unstable. A Foley catheter was placed, which yielded about 1,100 mL of purulent urine.

Laboratory workup showed:

• Procalcitonin 189 ng/mL (reference range < 2.0 ng/mL)  
• White blood cell count 10.7 × 10⁹/L (4.5–10.0)
• Myoglobin 20,000 ng/mL (< 71)
• Serum creatinine 4.8 mg/dL (0.06–1.10).

Urinalysis was positive for infection; blood and urine cultures later were positive for Escherichia coli.

Computed tomography of the abdomen and pelvis showed diffuse bladder dilation with urine and gas. It also revealed gas within the bladder wall and moderate hydroureter and hydronephrosis (Figure 2).

The patient went into shock that was refractory to pressors, culminating in cardiac arrest despite resuscitative measures.

EMPHYSEMATOUS CYSTITIS, A FORM OF URINARY TRACT INFECTION

Emphysematous cystitis is a rare form of complicated urinary tract infection characterized by gas inside the bladder and in the bladder wall. While the exact mechanisms underlying gas formation are not clear, gas-producing pathogens are clearly implicated in severe infection. E coli and Klebsiella pneumoniae are the most common organisms associated with emphysematous cystitis; others include Proteus mirabilis, and Enterobacter and Streptococcus species.^1,2

More than 50% of patients with emphysematous cystitis have diabetes mellitus. Other risk factors include bladder outlet obstruction, neurogenic bladder, and female sex.³ The severity of disease ranges from asymptomatic pneumaturia (up to 7% of cases)² to fulminant emphysematous cystitis, as in our patient.

The clinical presentation of emphysematous cystitis is nonspecific and can range from minimally symptomatic urinary tract infection to acute abdomen and septic shock.⁴

Some patients present with pneumaturia (the passing of gas through the urethra with micturition). Pneumaturia arises from 3 discrete causes: urologic instrumentation, fistula between the bladder and large or small bowel, and gas-producing bacteria in the bladder (emphysematous cystitis).⁵ Pneumaturia should always raise the suspicion of emphysematous cystitis.

The diagnosis can be made with either radiographic or computed tomographic evidence of gas within the bladder and bladder wall, in the absence of both bladder fistula and history of iatrogenic pneumaturia. Emphysematous cystitis should prompt urine and blood cultures to direct antimicrobial therapy, as 50% of patients with emphysematous cystitis have concomitant bacteremia.⁶

Our patient had an elevated serum level of procalcitonin, a marker of bacterial infection. Procalcitonin is a more specific biomarker of bacterial infection than acute-phase reactants such as the erythrocyte sedimentation rate or the C-reactive protein level. Measuring procalcitonin may help physicians make the diagnosis earlier, differentiate infectious from sterile causes of severe systemic inflammation, assess the severity of systemic inflammation caused by bacterial infections, and decide whether to start or discontinue antibiotic therapy.⁷

Most cases of emphysematous cystitis can be treated with antibiotics, though early diagnosis is crucial to a favorable outcome. Delay in diagnosis may contribute to the 20% mortality rate associated with this condition.⁶    

A 59-year-old woman with a history of chronic kidney disease and atonic bladder was brought to the hospital by emergency medical services. She had fallen in her home 2 days earlier and remained on the floor until neighbors eventually heard her cries and called 911. She complained of abdominal pain and distention along with emesis.

On presentation, she had tachycardia and tachypnea. The examination was notable for pronounced abdominal distention, diminished bowel sounds, and costovertebral angle tenderness.

The emergency department physician started empiric treatment for abdominal sepsis, including fluid resuscitation and broad-spectrum antibiotics. Initial imaging studies included abdominal radiography, which revealed a nonobstructive bowel gas pattern but raised suspicion of gas in the bladder (Figure 1). Arterial blood gas analysis showed lactic acidosis.

While laboratory work was being done, the patient’s tachypnea progressed to respiratory distress, and she ultimately required intubation. Vasopressors were started, as the patient was hemodynamically unstable. A Foley catheter was placed, which yielded about 1,100 mL of purulent urine.

Laboratory workup showed:

• Procalcitonin 189 ng/mL (reference range < 2.0 ng/mL)  
• White blood cell count 10.7 × 10⁹/L (4.5–10.0)
• Myoglobin 20,000 ng/mL (< 71)
• Serum creatinine 4.8 mg/dL (0.06–1.10).

Urinalysis was positive for infection; blood and urine cultures later were positive for Escherichia coli.

Computed tomography of the abdomen and pelvis showed diffuse bladder dilation with urine and gas. It also revealed gas within the bladder wall and moderate hydroureter and hydronephrosis (Figure 2).

The patient went into shock that was refractory to pressors, culminating in cardiac arrest despite resuscitative measures.

EMPHYSEMATOUS CYSTITIS, A FORM OF URINARY TRACT INFECTION

Emphysematous cystitis is a rare form of complicated urinary tract infection characterized by gas inside the bladder and in the bladder wall. While the exact mechanisms underlying gas formation are not clear, gas-producing pathogens are clearly implicated in severe infection. E coli and Klebsiella pneumoniae are the most common organisms associated with emphysematous cystitis; others include Proteus mirabilis, and Enterobacter and Streptococcus species.^1,2

More than 50% of patients with emphysematous cystitis have diabetes mellitus. Other risk factors include bladder outlet obstruction, neurogenic bladder, and female sex.³ The severity of disease ranges from asymptomatic pneumaturia (up to 7% of cases)² to fulminant emphysematous cystitis, as in our patient.

The clinical presentation of emphysematous cystitis is nonspecific and can range from minimally symptomatic urinary tract infection to acute abdomen and septic shock.⁴

Some patients present with pneumaturia (the passing of gas through the urethra with micturition). Pneumaturia arises from 3 discrete causes: urologic instrumentation, fistula between the bladder and large or small bowel, and gas-producing bacteria in the bladder (emphysematous cystitis).⁵ Pneumaturia should always raise the suspicion of emphysematous cystitis.

The diagnosis can be made with either radiographic or computed tomographic evidence of gas within the bladder and bladder wall, in the absence of both bladder fistula and history of iatrogenic pneumaturia. Emphysematous cystitis should prompt urine and blood cultures to direct antimicrobial therapy, as 50% of patients with emphysematous cystitis have concomitant bacteremia.⁶

Our patient had an elevated serum level of procalcitonin, a marker of bacterial infection. Procalcitonin is a more specific biomarker of bacterial infection than acute-phase reactants such as the erythrocyte sedimentation rate or the C-reactive protein level. Measuring procalcitonin may help physicians make the diagnosis earlier, differentiate infectious from sterile causes of severe systemic inflammation, assess the severity of systemic inflammation caused by bacterial infections, and decide whether to start or discontinue antibiotic therapy.⁷

Most cases of emphysematous cystitis can be treated with antibiotics, though early diagnosis is crucial to a favorable outcome. Delay in diagnosis may contribute to the 20% mortality rate associated with this condition.⁶    

A 39-year-old woman presented to the emergency department with a 2-day history of exertional dyspnea, left-sided chest pain with pleuritic characteristics, and cough without fever or chills. She had a history of severe postprandial nausea and vomiting, weight loss, and malnutrition, which had necessitated placement of a peripherally inserted central catheter in her right arm for total parenteral nutrition.

On physical examination, the patient was afebrile but tachycardic and tachypneic. Her oxygen saturation on room air by pulse oximetry was 91%, though she was not in significant distress. Breath sounds were equal bilaterally and clear, with symmetrical chest wall expansion.

Her white blood cell count was 18.5 × 10⁹/L (reference range 3.5–10.5), with 19.3% eosinophils (reference range 1%–7%); her D-dimer level was also elevated.

Computed tomography of the chest showed innumerable, diffuse micronodules in a centrilobular pattern (Figure 1).

Conditions to consider in a patient with these imaging findings in the setting of leukocytosis and eosinophilia include mycobacterial infection, hypersensitivity reaction, diffuse fungal infiltrates, and possibly metastatic disease such as thyroid carcinoma or melanoma. The patient reported having had a purified protein derivative test that was positive for tuberculosis, but she denied having had active disease.

She underwent bronchosocopy. Bronchoalveolar lavage specimen study showed an elevated eosinophil count of 17%. Acid-fast staining detected no organisms. Transbronchial biopsy study revealed foreign-body granulomas from microcrystalline cellulose microemboli deposited in the microvasculature of the patient’s lungs. Upon further questioning the patient admitted she had crushed oral tablets of prescribed opioids and injected them intravenously.

A COMPLICATION OF INJECTING ORAL TABLETS

Oral tablets typically contain talc, cellulose, cornstarch, or combinations of these substances as binding agents. When pulverized, the powder can be combined with water to form an injectable solution with higher and more rapid bioavailability.^1,2 The binders, however, are insoluble and accumulate in various tissues.

Intravenous injection of microcellulose has been shown to produce pulmonary and peripheral eosinophilia in birds. In humans, the immune response in foreign body granulomatosis can vary, and case reports have not mentioned eosinophils in the lungs or serum.^3,4

Deposition of these particles in pulmonary vessels is common and can trigger a potentially fatal reaction, presenting as acute onset of cough, chest pain, dyspnea, fever, and pulmonary hypertension. The severity of these clinical findings is relative to the degree of pulmonary hypertension created by the arteriolar involvement of these emboli.^2,5

In our patient’s lung biopsy samples, microcrystalline cellulose was clearly visible under polarized light, and it stained with Congo red (Figure 2).

Our patient’s exertional dyspnea and hypoxemia resolved during 1 week of hospitalization with conservative management and supplemental oxygen. She was referred to our pain rehabilitation clinic, where she was successfully weaned from narcotics. Her pulmonary findings on computed tomography were still present 3 years after her initial images, though less prominent.

A 39-year-old woman presented to the emergency department with a 2-day history of exertional dyspnea, left-sided chest pain with pleuritic characteristics, and cough without fever or chills. She had a history of severe postprandial nausea and vomiting, weight loss, and malnutrition, which had necessitated placement of a peripherally inserted central catheter in her right arm for total parenteral nutrition.

On physical examination, the patient was afebrile but tachycardic and tachypneic. Her oxygen saturation on room air by pulse oximetry was 91%, though she was not in significant distress. Breath sounds were equal bilaterally and clear, with symmetrical chest wall expansion.

Her white blood cell count was 18.5 × 10⁹/L (reference range 3.5–10.5), with 19.3% eosinophils (reference range 1%–7%); her D-dimer level was also elevated.

Computed tomography of the chest showed innumerable, diffuse micronodules in a centrilobular pattern (Figure 1).

Conditions to consider in a patient with these imaging findings in the setting of leukocytosis and eosinophilia include mycobacterial infection, hypersensitivity reaction, diffuse fungal infiltrates, and possibly metastatic disease such as thyroid carcinoma or melanoma. The patient reported having had a purified protein derivative test that was positive for tuberculosis, but she denied having had active disease.

She underwent bronchosocopy. Bronchoalveolar lavage specimen study showed an elevated eosinophil count of 17%. Acid-fast staining detected no organisms. Transbronchial biopsy study revealed foreign-body granulomas from microcrystalline cellulose microemboli deposited in the microvasculature of the patient’s lungs. Upon further questioning the patient admitted she had crushed oral tablets of prescribed opioids and injected them intravenously.

A COMPLICATION OF INJECTING ORAL TABLETS

Oral tablets typically contain talc, cellulose, cornstarch, or combinations of these substances as binding agents. When pulverized, the powder can be combined with water to form an injectable solution with higher and more rapid bioavailability.^1,2 The binders, however, are insoluble and accumulate in various tissues.

Intravenous injection of microcellulose has been shown to produce pulmonary and peripheral eosinophilia in birds. In humans, the immune response in foreign body granulomatosis can vary, and case reports have not mentioned eosinophils in the lungs or serum.^3,4

Deposition of these particles in pulmonary vessels is common and can trigger a potentially fatal reaction, presenting as acute onset of cough, chest pain, dyspnea, fever, and pulmonary hypertension. The severity of these clinical findings is relative to the degree of pulmonary hypertension created by the arteriolar involvement of these emboli.^2,5

In our patient’s lung biopsy samples, microcrystalline cellulose was clearly visible under polarized light, and it stained with Congo red (Figure 2).

Our patient’s exertional dyspnea and hypoxemia resolved during 1 week of hospitalization with conservative management and supplemental oxygen. She was referred to our pain rehabilitation clinic, where she was successfully weaned from narcotics. Her pulmonary findings on computed tomography were still present 3 years after her initial images, though less prominent.

A 39-year-old woman presented to the emergency department with a 2-day history of exertional dyspnea, left-sided chest pain with pleuritic characteristics, and cough without fever or chills. She had a history of severe postprandial nausea and vomiting, weight loss, and malnutrition, which had necessitated placement of a peripherally inserted central catheter in her right arm for total parenteral nutrition.

On physical examination, the patient was afebrile but tachycardic and tachypneic. Her oxygen saturation on room air by pulse oximetry was 91%, though she was not in significant distress. Breath sounds were equal bilaterally and clear, with symmetrical chest wall expansion.

Her white blood cell count was 18.5 × 10⁹/L (reference range 3.5–10.5), with 19.3% eosinophils (reference range 1%–7%); her D-dimer level was also elevated.

Computed tomography of the chest showed innumerable, diffuse micronodules in a centrilobular pattern (Figure 1).

Conditions to consider in a patient with these imaging findings in the setting of leukocytosis and eosinophilia include mycobacterial infection, hypersensitivity reaction, diffuse fungal infiltrates, and possibly metastatic disease such as thyroid carcinoma or melanoma. The patient reported having had a purified protein derivative test that was positive for tuberculosis, but she denied having had active disease.

She underwent bronchosocopy. Bronchoalveolar lavage specimen study showed an elevated eosinophil count of 17%. Acid-fast staining detected no organisms. Transbronchial biopsy study revealed foreign-body granulomas from microcrystalline cellulose microemboli deposited in the microvasculature of the patient’s lungs. Upon further questioning the patient admitted she had crushed oral tablets of prescribed opioids and injected them intravenously.

A COMPLICATION OF INJECTING ORAL TABLETS

Oral tablets typically contain talc, cellulose, cornstarch, or combinations of these substances as binding agents. When pulverized, the powder can be combined with water to form an injectable solution with higher and more rapid bioavailability.^1,2 The binders, however, are insoluble and accumulate in various tissues.

Intravenous injection of microcellulose has been shown to produce pulmonary and peripheral eosinophilia in birds. In humans, the immune response in foreign body granulomatosis can vary, and case reports have not mentioned eosinophils in the lungs or serum.^3,4

Deposition of these particles in pulmonary vessels is common and can trigger a potentially fatal reaction, presenting as acute onset of cough, chest pain, dyspnea, fever, and pulmonary hypertension. The severity of these clinical findings is relative to the degree of pulmonary hypertension created by the arteriolar involvement of these emboli.^2,5

In our patient’s lung biopsy samples, microcrystalline cellulose was clearly visible under polarized light, and it stained with Congo red (Figure 2).

Our patient’s exertional dyspnea and hypoxemia resolved during 1 week of hospitalization with conservative management and supplemental oxygen. She was referred to our pain rehabilitation clinic, where she was successfully weaned from narcotics. Her pulmonary findings on computed tomography were still present 3 years after her initial images, though less prominent.

An 82-year-old man with poorly controlled diabetes mellitus presented to our emergency department with a 1-day history of confusion and coffee-ground emesis.

Blood test results suggested diabetic ketoacidosis. Upper endoscopy showed diffuse black discoloration of the esophageal mucosa that affected the distal esophagus and stopped abruptly at the gastroesophageal junction (Figure 1), with normal gastric and duodenal mucosa.

Biopsy study revealed necrosis of the esophageal mucosa. A diagnosis of acute necrotizing esophagitis was made.

The patient received nothing by mouth, and he was treated with intravenous fluids, insulin, and a proton pump inhibitor. His symptoms resolved, and upper endoscopy repeated on hospital day 14 demonstrated improvement of the mucosal lesions and esophagitis (Figure 2).

ACUTE NECROTIZING ESOPHAGITIS

Acute necrotizing esophagitis is thought to arise from a combination of an ischemic insult to the esophagus, an impaired mucosal barrier system, and a backflow injury from chemical contents of gastric secretions.¹ The tissue hypoperfusion derives from vasculopathy, hypotension, or malnutrition. It is associated with diabetes mellitus, diabetic ketoacidosis, lactic acidosis, alcohol abuse, cirrhosis, renal insufficiency, malignancy, antibiotic use, esophageal infections, and aortic dissection.

The esophagus has a diverse blood supply. The upper esophagus is supplied by the inferior thyroid arteries, the mid-esophagus by the bronchial, proper esophageal, and intercostal arteries, and the distal esophagus by the left gastric and left inferior phrenic arteries.¹

KEY FEATURES AND DIAGNOSTIC CLUES

The necrotic changes are prominent in the distal esophagus, which is more susceptible to ischemia and mucosal injury. The characteristic endoscopic finding is a diffuse black esophagus with a sharp transition to normal mucosa at the gastroesophageal junction.

The differential diagnosis includes melanosis, pseudomelanosis, malignant melanoma, acanthosis nigricans, coal dust deposition, caustic ingestion, radiation esophagitis, and infectious esophagitis caused by cytomegalovirus, herpes simplex virus, Candida albicans, or Klebsiella pneumoniae.^2–4

TREATMENT AND OUTCOME

Avoidance of oral intake and gastric acid suppression with intravenous proton pump inhibitors are recommended to prevent additional injury of the esophageal mucosa.

The condition generally resolves with restored blood flow and treatment of any coexisting illness. However, it may be complicated by perforation (6.8%), mediastinitis (5.7%), or subsequent development of esophageal stricture (10.2%).⁵ Patients with esophageal stricture require endoscopic dilation after mucosal recovery.

The overall risk of death in acute necrotizing esophagitis is high (31.8%) and most often due to the underlying disease, such as sepsis, malignancy, cardiogenic shock, or hypovolemic shock.⁵ The mortality rate directly attributed to complications of acute necrotizing esophagitis is much lower (5.7%).⁵     

An 82-year-old man with poorly controlled diabetes mellitus presented to our emergency department with a 1-day history of confusion and coffee-ground emesis.

Blood test results suggested diabetic ketoacidosis. Upper endoscopy showed diffuse black discoloration of the esophageal mucosa that affected the distal esophagus and stopped abruptly at the gastroesophageal junction (Figure 1), with normal gastric and duodenal mucosa.

Biopsy study revealed necrosis of the esophageal mucosa. A diagnosis of acute necrotizing esophagitis was made.

The patient received nothing by mouth, and he was treated with intravenous fluids, insulin, and a proton pump inhibitor. His symptoms resolved, and upper endoscopy repeated on hospital day 14 demonstrated improvement of the mucosal lesions and esophagitis (Figure 2).

ACUTE NECROTIZING ESOPHAGITIS

Acute necrotizing esophagitis is thought to arise from a combination of an ischemic insult to the esophagus, an impaired mucosal barrier system, and a backflow injury from chemical contents of gastric secretions.¹ The tissue hypoperfusion derives from vasculopathy, hypotension, or malnutrition. It is associated with diabetes mellitus, diabetic ketoacidosis, lactic acidosis, alcohol abuse, cirrhosis, renal insufficiency, malignancy, antibiotic use, esophageal infections, and aortic dissection.

The esophagus has a diverse blood supply. The upper esophagus is supplied by the inferior thyroid arteries, the mid-esophagus by the bronchial, proper esophageal, and intercostal arteries, and the distal esophagus by the left gastric and left inferior phrenic arteries.¹

KEY FEATURES AND DIAGNOSTIC CLUES

The necrotic changes are prominent in the distal esophagus, which is more susceptible to ischemia and mucosal injury. The characteristic endoscopic finding is a diffuse black esophagus with a sharp transition to normal mucosa at the gastroesophageal junction.

The differential diagnosis includes melanosis, pseudomelanosis, malignant melanoma, acanthosis nigricans, coal dust deposition, caustic ingestion, radiation esophagitis, and infectious esophagitis caused by cytomegalovirus, herpes simplex virus, Candida albicans, or Klebsiella pneumoniae.^2–4

TREATMENT AND OUTCOME

Avoidance of oral intake and gastric acid suppression with intravenous proton pump inhibitors are recommended to prevent additional injury of the esophageal mucosa.

The condition generally resolves with restored blood flow and treatment of any coexisting illness. However, it may be complicated by perforation (6.8%), mediastinitis (5.7%), or subsequent development of esophageal stricture (10.2%).⁵ Patients with esophageal stricture require endoscopic dilation after mucosal recovery.

The overall risk of death in acute necrotizing esophagitis is high (31.8%) and most often due to the underlying disease, such as sepsis, malignancy, cardiogenic shock, or hypovolemic shock.⁵ The mortality rate directly attributed to complications of acute necrotizing esophagitis is much lower (5.7%).⁵     

An 82-year-old man with poorly controlled diabetes mellitus presented to our emergency department with a 1-day history of confusion and coffee-ground emesis.

Blood test results suggested diabetic ketoacidosis. Upper endoscopy showed diffuse black discoloration of the esophageal mucosa that affected the distal esophagus and stopped abruptly at the gastroesophageal junction (Figure 1), with normal gastric and duodenal mucosa.

Biopsy study revealed necrosis of the esophageal mucosa. A diagnosis of acute necrotizing esophagitis was made.

The patient received nothing by mouth, and he was treated with intravenous fluids, insulin, and a proton pump inhibitor. His symptoms resolved, and upper endoscopy repeated on hospital day 14 demonstrated improvement of the mucosal lesions and esophagitis (Figure 2).

ACUTE NECROTIZING ESOPHAGITIS

Acute necrotizing esophagitis is thought to arise from a combination of an ischemic insult to the esophagus, an impaired mucosal barrier system, and a backflow injury from chemical contents of gastric secretions.¹ The tissue hypoperfusion derives from vasculopathy, hypotension, or malnutrition. It is associated with diabetes mellitus, diabetic ketoacidosis, lactic acidosis, alcohol abuse, cirrhosis, renal insufficiency, malignancy, antibiotic use, esophageal infections, and aortic dissection.

The esophagus has a diverse blood supply. The upper esophagus is supplied by the inferior thyroid arteries, the mid-esophagus by the bronchial, proper esophageal, and intercostal arteries, and the distal esophagus by the left gastric and left inferior phrenic arteries.¹

KEY FEATURES AND DIAGNOSTIC CLUES

The necrotic changes are prominent in the distal esophagus, which is more susceptible to ischemia and mucosal injury. The characteristic endoscopic finding is a diffuse black esophagus with a sharp transition to normal mucosa at the gastroesophageal junction.

The differential diagnosis includes melanosis, pseudomelanosis, malignant melanoma, acanthosis nigricans, coal dust deposition, caustic ingestion, radiation esophagitis, and infectious esophagitis caused by cytomegalovirus, herpes simplex virus, Candida albicans, or Klebsiella pneumoniae.^2–4

TREATMENT AND OUTCOME

Avoidance of oral intake and gastric acid suppression with intravenous proton pump inhibitors are recommended to prevent additional injury of the esophageal mucosa.

The condition generally resolves with restored blood flow and treatment of any coexisting illness. However, it may be complicated by perforation (6.8%), mediastinitis (5.7%), or subsequent development of esophageal stricture (10.2%).⁵ Patients with esophageal stricture require endoscopic dilation after mucosal recovery.

The overall risk of death in acute necrotizing esophagitis is high (31.8%) and most often due to the underlying disease, such as sepsis, malignancy, cardiogenic shock, or hypovolemic shock.⁵ The mortality rate directly attributed to complications of acute necrotizing esophagitis is much lower (5.7%).⁵     

A 49-year-old man developed nephrotic-range proteinuria (urine protein–creatinine ratio 4.1 g/g), and primary membranous nephropathy was diagnosed by kidney biopsy. He declined therapy apart from angiotensin receptor blockade.

Five months after undergoing the biopsy, he presented to the emergency room with marked dyspnea, cough, and epigastric discomfort. His blood pressure was 160/100 mm Hg, heart rate 95 beats/minute, and oxygen saturation by pulse oximetry 97% at rest on ambient air, decreasing to 92% with ambulation.

Initial laboratory testing results were as follows:

• Sodium 135 mmol/L (reference range 136–144)
• Potassium 3.9 mmol/L (3.7–5.1)
• Chloride 104 mmol/L (97–105)
• Bicarbonate 21 mmol/L (22–30)
• Blood urea nitrogen 14 mg/dL (9–24)
• Serum creatinine 1.1 mg/dL (0.73–1.22)
• Albumin 2.1 g/dL (3.4–4.9).

Urinalysis revealed the following:

• 5 red blood cells per high-power field, compared with 1 to 2 previously
• 3+ proteinuria
• Urine protein–creatinine ratio 11 g/g
• No glucosuria.

Electrocardiography revealed normal sinus rhythm without ischemic changes. Chest radiography did not show consolidation.

Computed tomography of the chest and abdomen with intravenous contrast demonstrated a nearly occlusive thrombus in the left renal vein (Figure 1) extending to the inferior vena cava with bilateral, nearly occlusive pulmonary emboli (Figure 2).

The patient was started on systemic anticoagulation with unfractionated heparin, which was then transitioned to warfarin therapy. Immunosuppressive therapy was also started, with rituximab 1,000 mg every other week for 2 doses, and 6 months of alternating monthly oral therapy with cyclophosphamide and methylprednisolone.

At 7 months after the thrombotic event, there was no evidence of residual renal vein thrombosis on magnetic resonance venography, and at 14 months his serum creatinine level was 0.9 mg/dL, albumin 4.0 g/dL, and urine protein–creatinine ratio 0.8 g/g.

RENAL VEIN THROMBOSIS: RISK FACTORS AND CLINICAL FEATURES

Severe hypoalbuminemia in the setting of nephrotic syndrome due to membranous neph­ropathy is associated with the highest risk of venous thromboembolic events, with renal vein thrombus being the classic complication.¹ Venous thromboembolic events also occur in other nephrotic syndromes, albeit at a lower frequency.²

Venous thromboembolic events are estimated to occur in 7% to 33% of patients with membranous glomerulopathy, with albumin levels less than 2.8 g/dL considered a notable risk factor.^1,2

While often a chronic complication, acute renal vein thrombosis may present with flank pain and hematuria.³ In our patient, the dramatic increase in proteinuria and possibly the increase in hematuria suggested renal vein thrombosis. Proximal tubular dysfunction, such as glucosuria, can be seen on occasion.

DIAGNOSIS AND TREATMENT

Screening asymptomatic patients for renal vein thrombosis is not recommended, and the decision to start prophylactic anticoagulation must be individualized.⁴

Although renal venography historically was the gold standard test to diagnose renal vein thrombosis, it has been replaced by noninvasive imaging such as computed tomography and magnetic resonance venography.

While anticoagulation remains the treatment of choice, catheter-directed thrombectomy or surgical thrombectomy can be considered for some patients with acute renal vein thrombosis.⁵

A 49-year-old man developed nephrotic-range proteinuria (urine protein–creatinine ratio 4.1 g/g), and primary membranous nephropathy was diagnosed by kidney biopsy. He declined therapy apart from angiotensin receptor blockade.

Five months after undergoing the biopsy, he presented to the emergency room with marked dyspnea, cough, and epigastric discomfort. His blood pressure was 160/100 mm Hg, heart rate 95 beats/minute, and oxygen saturation by pulse oximetry 97% at rest on ambient air, decreasing to 92% with ambulation.

Initial laboratory testing results were as follows:

• Sodium 135 mmol/L (reference range 136–144)
• Potassium 3.9 mmol/L (3.7–5.1)
• Chloride 104 mmol/L (97–105)
• Bicarbonate 21 mmol/L (22–30)
• Blood urea nitrogen 14 mg/dL (9–24)
• Serum creatinine 1.1 mg/dL (0.73–1.22)
• Albumin 2.1 g/dL (3.4–4.9).

Urinalysis revealed the following:

• 5 red blood cells per high-power field, compared with 1 to 2 previously
• 3+ proteinuria
• Urine protein–creatinine ratio 11 g/g
• No glucosuria.

Electrocardiography revealed normal sinus rhythm without ischemic changes. Chest radiography did not show consolidation.

Computed tomography of the chest and abdomen with intravenous contrast demonstrated a nearly occlusive thrombus in the left renal vein (Figure 1) extending to the inferior vena cava with bilateral, nearly occlusive pulmonary emboli (Figure 2).

The patient was started on systemic anticoagulation with unfractionated heparin, which was then transitioned to warfarin therapy. Immunosuppressive therapy was also started, with rituximab 1,000 mg every other week for 2 doses, and 6 months of alternating monthly oral therapy with cyclophosphamide and methylprednisolone.

At 7 months after the thrombotic event, there was no evidence of residual renal vein thrombosis on magnetic resonance venography, and at 14 months his serum creatinine level was 0.9 mg/dL, albumin 4.0 g/dL, and urine protein–creatinine ratio 0.8 g/g.

RENAL VEIN THROMBOSIS: RISK FACTORS AND CLINICAL FEATURES

Severe hypoalbuminemia in the setting of nephrotic syndrome due to membranous neph­ropathy is associated with the highest risk of venous thromboembolic events, with renal vein thrombus being the classic complication.¹ Venous thromboembolic events also occur in other nephrotic syndromes, albeit at a lower frequency.²

Venous thromboembolic events are estimated to occur in 7% to 33% of patients with membranous glomerulopathy, with albumin levels less than 2.8 g/dL considered a notable risk factor.^1,2

While often a chronic complication, acute renal vein thrombosis may present with flank pain and hematuria.³ In our patient, the dramatic increase in proteinuria and possibly the increase in hematuria suggested renal vein thrombosis. Proximal tubular dysfunction, such as glucosuria, can be seen on occasion.

DIAGNOSIS AND TREATMENT

Screening asymptomatic patients for renal vein thrombosis is not recommended, and the decision to start prophylactic anticoagulation must be individualized.⁴

Although renal venography historically was the gold standard test to diagnose renal vein thrombosis, it has been replaced by noninvasive imaging such as computed tomography and magnetic resonance venography.

While anticoagulation remains the treatment of choice, catheter-directed thrombectomy or surgical thrombectomy can be considered for some patients with acute renal vein thrombosis.⁵

A 49-year-old man developed nephrotic-range proteinuria (urine protein–creatinine ratio 4.1 g/g), and primary membranous nephropathy was diagnosed by kidney biopsy. He declined therapy apart from angiotensin receptor blockade.

Five months after undergoing the biopsy, he presented to the emergency room with marked dyspnea, cough, and epigastric discomfort. His blood pressure was 160/100 mm Hg, heart rate 95 beats/minute, and oxygen saturation by pulse oximetry 97% at rest on ambient air, decreasing to 92% with ambulation.

Initial laboratory testing results were as follows:

• Sodium 135 mmol/L (reference range 136–144)
• Potassium 3.9 mmol/L (3.7–5.1)
• Chloride 104 mmol/L (97–105)
• Bicarbonate 21 mmol/L (22–30)
• Blood urea nitrogen 14 mg/dL (9–24)
• Serum creatinine 1.1 mg/dL (0.73–1.22)
• Albumin 2.1 g/dL (3.4–4.9).

Urinalysis revealed the following:

• 5 red blood cells per high-power field, compared with 1 to 2 previously
• 3+ proteinuria
• Urine protein–creatinine ratio 11 g/g
• No glucosuria.

Electrocardiography revealed normal sinus rhythm without ischemic changes. Chest radiography did not show consolidation.

Computed tomography of the chest and abdomen with intravenous contrast demonstrated a nearly occlusive thrombus in the left renal vein (Figure 1) extending to the inferior vena cava with bilateral, nearly occlusive pulmonary emboli (Figure 2).

The patient was started on systemic anticoagulation with unfractionated heparin, which was then transitioned to warfarin therapy. Immunosuppressive therapy was also started, with rituximab 1,000 mg every other week for 2 doses, and 6 months of alternating monthly oral therapy with cyclophosphamide and methylprednisolone.

At 7 months after the thrombotic event, there was no evidence of residual renal vein thrombosis on magnetic resonance venography, and at 14 months his serum creatinine level was 0.9 mg/dL, albumin 4.0 g/dL, and urine protein–creatinine ratio 0.8 g/g.

RENAL VEIN THROMBOSIS: RISK FACTORS AND CLINICAL FEATURES

Severe hypoalbuminemia in the setting of nephrotic syndrome due to membranous neph­ropathy is associated with the highest risk of venous thromboembolic events, with renal vein thrombus being the classic complication.¹ Venous thromboembolic events also occur in other nephrotic syndromes, albeit at a lower frequency.²

Venous thromboembolic events are estimated to occur in 7% to 33% of patients with membranous glomerulopathy, with albumin levels less than 2.8 g/dL considered a notable risk factor.^1,2

While often a chronic complication, acute renal vein thrombosis may present with flank pain and hematuria.³ In our patient, the dramatic increase in proteinuria and possibly the increase in hematuria suggested renal vein thrombosis. Proximal tubular dysfunction, such as glucosuria, can be seen on occasion.

DIAGNOSIS AND TREATMENT

Screening asymptomatic patients for renal vein thrombosis is not recommended, and the decision to start prophylactic anticoagulation must be individualized.⁴

Although renal venography historically was the gold standard test to diagnose renal vein thrombosis, it has been replaced by noninvasive imaging such as computed tomography and magnetic resonance venography.

While anticoagulation remains the treatment of choice, catheter-directed thrombectomy or surgical thrombectomy can be considered for some patients with acute renal vein thrombosis.⁵

A 63-year-old woman presented with an acute exacerbation of chronic back pain after a fall. She was taking warfarin because of a history of factor V Leiden, deep vein thrombosis, and pulmonary embolism, for which a temporary inferior vena cava (IVC) filter had been placed 8 years ago. Her physicians had subsequently tried to remove the filter, without success. Some time after that, 1 of the filter struts had been removed after it migrated through her abdominal wall.

Laboratory testing revealed a supratherapeutic international normalized ratio of 8.5.

Magnetic resonance imaging of the lumbar spine showed a para-aortic heterogeneous collection, suspected of being a retroperitoneal hematoma (Figure 1).

Computed tomographic angiography of the abdomen and pelvis subsequently revealed a contained aortic rupture, a filter strut within clotted blood adjacent to an aortic pseudoaneurysm, and additional filter struts within the second portion of the duodenum and renal pelvis (Figure 2).

The patient underwent endovascular aneurysm repair with adequate placement of a vascular graft. She was discharged on therapeutic anticoagulation, and her back pain had notably improved.

COMPLICATIONS OF IVC FILTERS

In the United States, the use of IVC filters has increased significantly over the last decade, with placement rates ranging from 12% to 17% in patients with venous thromboembolism.¹

The American Heart Association recommends filter placement for patients with venous thromboembolism for whom anticoagulation has failed or is contraindicated, patients unable to withstand pulmonary embolism, and patients who are hemodynamically unstable.² While indications vary in the guidelines released by different societies, filters are most often placed in patients who have an acute bleed, significant surgery after admission for venous thromboembolism, metastatic cancer, and severe illness.³

Complications can occur during and after insertion and during removal. They are more frequent with temporary than with permanent filters, and include filter movement and fracture as well as occlusion and penetration.^4,5

In our patient, we believe that the 3 remaining filter struts likely penetrated the wall of the IVC to the extent that they encountered adjacent structures (aorta, duodenum, kidney).

Of cases of IVC filter penetration reported to a US Food and Drug Administration database, 13.1% involved small bowel perforation, 6.5% involved aortic perforation, and 4.2% involved retroperitoneal bleeding. Symptoms such as abdominal and back pain were present in 38.3% of cases involving IVC penetration.⁵

Therefore, the differential diagnosis for patients with a history of IVC filter placement presenting with these symptoms should address filter complications, including occlusion,  incorrect placement, fracture, migration, and penetration of the filter.⁴ If complications occur, treatment options include anticoagulation, endovascular repair, and surgical intervention.

A 63-year-old woman presented with an acute exacerbation of chronic back pain after a fall. She was taking warfarin because of a history of factor V Leiden, deep vein thrombosis, and pulmonary embolism, for which a temporary inferior vena cava (IVC) filter had been placed 8 years ago. Her physicians had subsequently tried to remove the filter, without success. Some time after that, 1 of the filter struts had been removed after it migrated through her abdominal wall.

Laboratory testing revealed a supratherapeutic international normalized ratio of 8.5.

Magnetic resonance imaging of the lumbar spine showed a para-aortic heterogeneous collection, suspected of being a retroperitoneal hematoma (Figure 1).

Computed tomographic angiography of the abdomen and pelvis subsequently revealed a contained aortic rupture, a filter strut within clotted blood adjacent to an aortic pseudoaneurysm, and additional filter struts within the second portion of the duodenum and renal pelvis (Figure 2).

The patient underwent endovascular aneurysm repair with adequate placement of a vascular graft. She was discharged on therapeutic anticoagulation, and her back pain had notably improved.

COMPLICATIONS OF IVC FILTERS

In the United States, the use of IVC filters has increased significantly over the last decade, with placement rates ranging from 12% to 17% in patients with venous thromboembolism.¹

The American Heart Association recommends filter placement for patients with venous thromboembolism for whom anticoagulation has failed or is contraindicated, patients unable to withstand pulmonary embolism, and patients who are hemodynamically unstable.² While indications vary in the guidelines released by different societies, filters are most often placed in patients who have an acute bleed, significant surgery after admission for venous thromboembolism, metastatic cancer, and severe illness.³

Complications can occur during and after insertion and during removal. They are more frequent with temporary than with permanent filters, and include filter movement and fracture as well as occlusion and penetration.^4,5

In our patient, we believe that the 3 remaining filter struts likely penetrated the wall of the IVC to the extent that they encountered adjacent structures (aorta, duodenum, kidney).

Of cases of IVC filter penetration reported to a US Food and Drug Administration database, 13.1% involved small bowel perforation, 6.5% involved aortic perforation, and 4.2% involved retroperitoneal bleeding. Symptoms such as abdominal and back pain were present in 38.3% of cases involving IVC penetration.⁵

Therefore, the differential diagnosis for patients with a history of IVC filter placement presenting with these symptoms should address filter complications, including occlusion,  incorrect placement, fracture, migration, and penetration of the filter.⁴ If complications occur, treatment options include anticoagulation, endovascular repair, and surgical intervention.

A 63-year-old woman presented with an acute exacerbation of chronic back pain after a fall. She was taking warfarin because of a history of factor V Leiden, deep vein thrombosis, and pulmonary embolism, for which a temporary inferior vena cava (IVC) filter had been placed 8 years ago. Her physicians had subsequently tried to remove the filter, without success. Some time after that, 1 of the filter struts had been removed after it migrated through her abdominal wall.

Laboratory testing revealed a supratherapeutic international normalized ratio of 8.5.

Magnetic resonance imaging of the lumbar spine showed a para-aortic heterogeneous collection, suspected of being a retroperitoneal hematoma (Figure 1).

Computed tomographic angiography of the abdomen and pelvis subsequently revealed a contained aortic rupture, a filter strut within clotted blood adjacent to an aortic pseudoaneurysm, and additional filter struts within the second portion of the duodenum and renal pelvis (Figure 2).

The patient underwent endovascular aneurysm repair with adequate placement of a vascular graft. She was discharged on therapeutic anticoagulation, and her back pain had notably improved.

COMPLICATIONS OF IVC FILTERS

In the United States, the use of IVC filters has increased significantly over the last decade, with placement rates ranging from 12% to 17% in patients with venous thromboembolism.¹

The American Heart Association recommends filter placement for patients with venous thromboembolism for whom anticoagulation has failed or is contraindicated, patients unable to withstand pulmonary embolism, and patients who are hemodynamically unstable.² While indications vary in the guidelines released by different societies, filters are most often placed in patients who have an acute bleed, significant surgery after admission for venous thromboembolism, metastatic cancer, and severe illness.³

Complications can occur during and after insertion and during removal. They are more frequent with temporary than with permanent filters, and include filter movement and fracture as well as occlusion and penetration.^4,5

In our patient, we believe that the 3 remaining filter struts likely penetrated the wall of the IVC to the extent that they encountered adjacent structures (aorta, duodenum, kidney).

Of cases of IVC filter penetration reported to a US Food and Drug Administration database, 13.1% involved small bowel perforation, 6.5% involved aortic perforation, and 4.2% involved retroperitoneal bleeding. Symptoms such as abdominal and back pain were present in 38.3% of cases involving IVC penetration.⁵

Therefore, the differential diagnosis for patients with a history of IVC filter placement presenting with these symptoms should address filter complications, including occlusion,  incorrect placement, fracture, migration, and penetration of the filter.⁴ If complications occur, treatment options include anticoagulation, endovascular repair, and surgical intervention.

A 54-year-old man presented to our hospital with acute-onset left-sided weakness and right facial droop. Three days earlier he had also had migraine-like headaches, which he had never experienced before. He also reported a change in behavior during the past week, which his family had described as inappropriate laughter.

He had no history of hypertension, diabetes, or dyslipidemia. He did not smoke or drink alcohol. However, he had an extensive family history of stroke. His mother had a stroke at age 50, his brother a stroke at age 57, and his sister had been admitted for a stroke 1 month earlier at the age of 52.

On examination, he had weakness of the left arm and leg, right facial droop, and hyperactive reflexes on the left side. He had no sensory or cerebellar deficits. He had episodes of laughter during the examination.

Behavioral changes such as inappropriate laughter are nonspecific and can be associated with any subcortical frontoparietal and temporal infarction.

Computed tomography of the brain without contrast (Figure 1) showed patchy confluent areas of low attenuation in both cerebral hemispheres. Magnetic resonance imaging (MRI) showed an 8-mm focus of mild restricted diffusion in the right lateral thalamus, indicating acute infarction, and axial T2 fluid-attenuated inversion recovery images (Figure 2) showed confluent and symmetric white matter hyperintensities in the frontoparietotemporal region. The extent of these white matter changes, especially in the temporal lobe, were not typical of the microvascular disease changes seen in elderly individuals with risk factors for stroke. Microvascular disease may present the same way, but not as extensively and this was not consistent with the patient’s relatively young age. The imaging findings suggested cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).

We learned that the patient’s sister had undergone a workup showing mutations in the NOTCH3 gene and a skin biopsy study consistent with CADASIL.

Our patient was started on antiplatelet and high-intensity statin therapy. His symptoms improved, and he was discharged to an acute inpatient rehabilitation facility. He was referred to a CADASIL registry.

STROKE AND HEREDITY

CADASIL is a rare hereditary vascular disorder inherited in an autosomal dominant manner. It is the most common inherited form of small-vessel disease and results from a mutation in the NOTCH3 gene that leads to degeneration of smooth muscle in cerebral blood vessels. It can manifest as migraine with aura, vascular dementia, cognitive impairment, or ischemic stroke.

The diagnosis is based on a clinical picture that typically includes stroke at a young age (age 40 to 50) in the absence of stroke risk factors, or frequent lacunar infarction episodes that can manifest as migraine, lacunar infarct, or dementia.¹ Some patients, such as ours, may have subtle nonspecific behavioral changes such as inappropriate laughter, which may herald the development of an infarct.

Characteristic findings on MRI are white matter hyperintensities that tend to be bilateral and symmetrical in the periventricular areas. Symmetrical involvement in the temporal lobes has high sensitivity and specificity for CADASIL.² Biopsy study of the skin, muscle, or sural nerve shows small-vessel changes that include thickening of the media, granular material positive on periodic acid-Schiff staining, and narrowing of the lumen. However, the gold standard for diagnosis is confirmation of the NOTCH3 mutation on chromosome 19.^1,2

There is no known treatment for CADASIL.

A 54-year-old man presented to our hospital with acute-onset left-sided weakness and right facial droop. Three days earlier he had also had migraine-like headaches, which he had never experienced before. He also reported a change in behavior during the past week, which his family had described as inappropriate laughter.

He had no history of hypertension, diabetes, or dyslipidemia. He did not smoke or drink alcohol. However, he had an extensive family history of stroke. His mother had a stroke at age 50, his brother a stroke at age 57, and his sister had been admitted for a stroke 1 month earlier at the age of 52.

On examination, he had weakness of the left arm and leg, right facial droop, and hyperactive reflexes on the left side. He had no sensory or cerebellar deficits. He had episodes of laughter during the examination.

Behavioral changes such as inappropriate laughter are nonspecific and can be associated with any subcortical frontoparietal and temporal infarction.

Computed tomography of the brain without contrast (Figure 1) showed patchy confluent areas of low attenuation in both cerebral hemispheres. Magnetic resonance imaging (MRI) showed an 8-mm focus of mild restricted diffusion in the right lateral thalamus, indicating acute infarction, and axial T2 fluid-attenuated inversion recovery images (Figure 2) showed confluent and symmetric white matter hyperintensities in the frontoparietotemporal region. The extent of these white matter changes, especially in the temporal lobe, were not typical of the microvascular disease changes seen in elderly individuals with risk factors for stroke. Microvascular disease may present the same way, but not as extensively and this was not consistent with the patient’s relatively young age. The imaging findings suggested cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).

We learned that the patient’s sister had undergone a workup showing mutations in the NOTCH3 gene and a skin biopsy study consistent with CADASIL.

Our patient was started on antiplatelet and high-intensity statin therapy. His symptoms improved, and he was discharged to an acute inpatient rehabilitation facility. He was referred to a CADASIL registry.

STROKE AND HEREDITY

CADASIL is a rare hereditary vascular disorder inherited in an autosomal dominant manner. It is the most common inherited form of small-vessel disease and results from a mutation in the NOTCH3 gene that leads to degeneration of smooth muscle in cerebral blood vessels. It can manifest as migraine with aura, vascular dementia, cognitive impairment, or ischemic stroke.

The diagnosis is based on a clinical picture that typically includes stroke at a young age (age 40 to 50) in the absence of stroke risk factors, or frequent lacunar infarction episodes that can manifest as migraine, lacunar infarct, or dementia.¹ Some patients, such as ours, may have subtle nonspecific behavioral changes such as inappropriate laughter, which may herald the development of an infarct.

Characteristic findings on MRI are white matter hyperintensities that tend to be bilateral and symmetrical in the periventricular areas. Symmetrical involvement in the temporal lobes has high sensitivity and specificity for CADASIL.² Biopsy study of the skin, muscle, or sural nerve shows small-vessel changes that include thickening of the media, granular material positive on periodic acid-Schiff staining, and narrowing of the lumen. However, the gold standard for diagnosis is confirmation of the NOTCH3 mutation on chromosome 19.^1,2

There is no known treatment for CADASIL.

A 54-year-old man presented to our hospital with acute-onset left-sided weakness and right facial droop. Three days earlier he had also had migraine-like headaches, which he had never experienced before. He also reported a change in behavior during the past week, which his family had described as inappropriate laughter.

He had no history of hypertension, diabetes, or dyslipidemia. He did not smoke or drink alcohol. However, he had an extensive family history of stroke. His mother had a stroke at age 50, his brother a stroke at age 57, and his sister had been admitted for a stroke 1 month earlier at the age of 52.

On examination, he had weakness of the left arm and leg, right facial droop, and hyperactive reflexes on the left side. He had no sensory or cerebellar deficits. He had episodes of laughter during the examination.

Behavioral changes such as inappropriate laughter are nonspecific and can be associated with any subcortical frontoparietal and temporal infarction.

Computed tomography of the brain without contrast (Figure 1) showed patchy confluent areas of low attenuation in both cerebral hemispheres. Magnetic resonance imaging (MRI) showed an 8-mm focus of mild restricted diffusion in the right lateral thalamus, indicating acute infarction, and axial T2 fluid-attenuated inversion recovery images (Figure 2) showed confluent and symmetric white matter hyperintensities in the frontoparietotemporal region. The extent of these white matter changes, especially in the temporal lobe, were not typical of the microvascular disease changes seen in elderly individuals with risk factors for stroke. Microvascular disease may present the same way, but not as extensively and this was not consistent with the patient’s relatively young age. The imaging findings suggested cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).

We learned that the patient’s sister had undergone a workup showing mutations in the NOTCH3 gene and a skin biopsy study consistent with CADASIL.

Our patient was started on antiplatelet and high-intensity statin therapy. His symptoms improved, and he was discharged to an acute inpatient rehabilitation facility. He was referred to a CADASIL registry.

STROKE AND HEREDITY

CADASIL is a rare hereditary vascular disorder inherited in an autosomal dominant manner. It is the most common inherited form of small-vessel disease and results from a mutation in the NOTCH3 gene that leads to degeneration of smooth muscle in cerebral blood vessels. It can manifest as migraine with aura, vascular dementia, cognitive impairment, or ischemic stroke.

The diagnosis is based on a clinical picture that typically includes stroke at a young age (age 40 to 50) in the absence of stroke risk factors, or frequent lacunar infarction episodes that can manifest as migraine, lacunar infarct, or dementia.¹ Some patients, such as ours, may have subtle nonspecific behavioral changes such as inappropriate laughter, which may herald the development of an infarct.

Characteristic findings on MRI are white matter hyperintensities that tend to be bilateral and symmetrical in the periventricular areas. Symmetrical involvement in the temporal lobes has high sensitivity and specificity for CADASIL.² Biopsy study of the skin, muscle, or sural nerve shows small-vessel changes that include thickening of the media, granular material positive on periodic acid-Schiff staining, and narrowing of the lumen. However, the gold standard for diagnosis is confirmation of the NOTCH3 mutation on chromosome 19.^1,2

There is no known treatment for CADASIL.