Purpose or Case Report: This case elucidates a 20mo male with history of chronic granulomatous disease (CGD) who presented with PO intolerance, recurrent emesis, fussiness, and abdominal pain. The patient was diagnosed with CGD at age 11mo after developing upper extremity cellulitis from Serratia marescens. Just 1 month before this presentation, he underwent colonoscopy/endoscopy for persistent anorexia and fussiness after clean-out for severe constipation and was found with ulcerative pancolitis. Initiation of vendolizumab was recommended; just a few days before his first dose, he developed increased vomiting, lethargy, and PO refusal. Ten days after symptom onset (5 days after first venolizumab dose) he presented to the emergency department. Initial abdominal radiograph was largely unrevealing with clinical suspicion for constipation. However, subsequent upper GI series revealed a diffusely narrowed and elongated pyloric channel with mild delay in gastric emptying, suspicious for pyloric stenosis. Follow-up ultrasound depicted increased pyloric length with mucosal and muscularis thickening consistent with pyloric stenosis in the setting of CGD. An ensuing CT of the abdomen demonstrated segmental circumferential proximal jejunal wall thickening compatible with chronic granulomatous disease enteropathy. Pyloric stenosis, which typically presents in the first 2 months of life, is an unusual finding in a child of age 20mo. Though pulmonary infections are the most commonly affected organ system in CGD, the gastrointestinal (GI) manifestations—beyond just hepatic abscesses, which occur in 25-50% of patients—are infrequently discussed. The patient presented here exhibits predominantly GI-related effects. Granulomatous inflammation can affect any part of the GI tract and may mimic Crohn's disease via bowel wall thickening, luminal narrowing, fistulation, or mucosal cobblestone/skip lesions. Gastric antral wall thickening is estimated in 16% of CGD patients, and more commonly reported upper GI study findings in patients with CGD include delayed gastric emptying, circumferential antral narrowing, gastric fold thickening, or esophageal strictures with dysmotility. Though gastric outlet obstruction from antral or diffuse gastric wall thickening has been described, there are no other prior reports of pyloric stenosis due to CGD. From this case, we posit that CGD should be considered as a causative etiology for older infants/children with new radiological findings of pyloric stenosis.
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