S4031 Mediastinal Colitis: A Rare Complication of Colonic Interposition in a Patient With Recessive Dystrophic Epidermolysis Bullosa

Introduction: Recessive dystrophic epidermolysis bullosa (RDEB) is a rare inherited skin disorder marked by recurrent mucosal blistering and scarring. Progressive esophageal stricturing from repeated injury and fibrosis often complicates the disease, sometimes requiring esophagectomy with colonic interposition to restore the esophagogastric conduit. We report a case of mediastinal colitis (MC), an extremely rare complication of colonic interposition, in a patient with RDEB—this is the first documented case in this context. Case Description/Methods: A 75-year-old woman with RDEB, COPD, hypertension, rheumatoid arthritis, and heart failure presented with shortness of breath and substernal chest pain. Sixty years prior, she underwent esophagectomy with colon interposition due to severe esophageal strictures from RDEB and has required serial esophageal dilations, most recently 7 months earlier. In the Emergency Department, she had increased oxygen needs (4 L NC vs 2 L at home), leukocytosis, and elevated procalcitonin. Chest computed tomography angiography showed patchy right midlung infiltrates suggestive of aspiration pneumonia. Notably, there was circumferential wall thickening up to 9 mm in the distal colon proximal to the gastrocolic anastomosis, with mild pericolonic fat stranding—consistent with colitis. The colon within the anterior mediastinum was also distended with food contents. The patient received a 7-day course of zosyn, with resolution of pneumonia and improvement in chest pain and dyspnea. Advancing diet from clear liquids to solids triggered recurrent chest tightness. After antibiotics, esophagogastroduodenoscopy revealed a benign esophageal stricture above the colonic graft, which was dilated from 12 mm to 13.5 mm. She was discharged tolerating a regular diet with significant chest pain improvement. Discussion: This case represents the first reported MC complication of colonic interposition in RDEB. MC likely arises as a delayed consequence of anatomical changes post-esophageal reconstruction. Proposed mechanisms include ischemia of the interposed colon, infectious colitis, mechanical stasis, acid reflux into the graft, and diverticulitis of the graft segment. Clinically, MC presents with nonspecific symptoms such as chest pain and shortness of breath, which may mimic common RDEB complications like aspiration pneumonia. Given overlapping presentations, clinicians should maintain high suspicion for graft-related pathology to ensure timely diagnosis and management.