Oesophageal narrowing in chronic granulomatous disease

Abstract

A 14 year-old boy with X-linked chronic granulomatous disease (CGD) developed a long smooth narrowing at the mid oesophagus with severe dysphagia. Endoscopy revealed a normal mucosa and biopsy showed non-specific acute inflammatory changes. Bacterial cultures of the biopsy specimens were sterile. Combined treatment with antibiotics and steroids resulted in a transitory remission, but symptoms recurred after 2.5 months. One second course of steroids and antibiotics lead to a long-term remission. Oesophagogram was more useful than endoscopy to evaluate the severity of the stricture.