Case Presentation:

A 42–year–old woman with Angelman syndrome, severe cognitive impairment, and seizure disorder presented with changes in mental status. She was nonverbal at baseline; her caregivers had noted less interaction, poor appetite, and 4 hours of increased tremors. She had no fevers or bowel changes. Medications included lamotrigine, phenobarbital, levetiracetam, clonazepam, diazepam, propranolol, hydroxychloroquine, nitrofurantoin, and lactulose. Physical examination revealed a blood pressure of 80/40 mm Hg that normalized with IV fluid hydration, somnolence, no tremors, and a non–distended abdomen with active bowel sounds. There was no obvious tenderness to palpation. White blood cell count was 3.4 × 10³/mL. Chest radiograph demonstrated elevation of the right hemidiaphragm, possibly from gaseous distention of bowel or free air. CT of the abdomen and pelvis with IV contrast revealed extensive pneumatosis intestinalis in the wall of the ascending colon extending to the hepatic flexure with a small amount of free gas in the right abdomen (Figure 1). There was no bowel dilatation to suggest obstruction. Serum lactic acid was normal, and stool studies including culture, Clostridium difficile toxin, and occult blood were negative. General surgery recommended conservative management with serial examinations and radiographs, bowel rest, nasogastric decompression, and empiric antimicrobial therapy with ciprofloxacin and metronidazole. She remained stable, tolerated a diet and was ultimately discharged in improved condition with baseline mental status. She did not require surgical intervention.

Discussion:

Hospitalists need to rapidly differentiate life–threatening diseases from benign conditions. Classically, pneumatosis intestinalis indicates intestinal ischemia and requires prompt surgical intervention for patient survival. However, many benign conditions can also be associated with pneumatosis including pulmonary disorders (e.g., asthma), endoscopic procedures (e.g., colonoscopy), corticosteroids, inflammatory bowel disease, and gastrointestinal motility disturbances (e.g., scleroderma). As in this case, up to 15% of patients can present with idiopathic pneumatosis. She lacked fever, leukocytosis, and other signs of systemic toxicity, all of which indicated she could safely be managed nonoperatively.

Conclusions:

Pneumatosis intestinalis is a rare radiographic finding with a broad differential diagnosis. Hospitalists must be able to identify life–threatening etiologies of pneumatosis such as bowel ischemia that require prompt surgical intervention. As this case illustrates, many causes of pneumatosis are benign and can be managed without surgery.

Figure 1Computed tomography of the abdomen and pelvis (coronal section) demonstrating extensive pneumatosis intestinalis in the wall of the ascending colon as indicated by arrowheads. L = liver.