Gastrointestinal complications of chronic granulomatous disease: surgical implications

Eleven of 20 patients with chronic granulomatous disease (55%) developed serious gastrointestinal complications requiring surgical consultation or operation over a 10-year period with an average of 2.2 complications per patient. The mean age of onset of symptoms was 12 years (range 2.5 months to 25 years), and 91% of the patients were male. Admission for gastrointestinal complications constituted 18% of all admissions for these patients; the mean hospitalization time was 27.8 +/- 3.5 (SEM) days. Hepatic abscess, the most common complication, occurred in 16 instances. Perirectal abscess developed in three patients and gastric outlet obstruction developed in two patients. Other complications included appendicitis, acalculous cholecystitis, and Salmonella enteritis. Open hepatic debridement and external drainage combined with long-term intravenous antibiotics (mean 25.2 +/- 4.8 days) were curative in every case, but operative morbidity was frequent and severe. Twelve major complications accompanied open hepatic drainage in 14 cases including wound disruption, prolonged febrile course, subhepatic abscess, and recurrent hepatic abscess. Five secondary operations were required for treatment of these complications. Gastric outlet obstruction, by contrast, was successfully managed nonoperatively. Staphylococcus aureus was an etiologic agent in 66% of the cases, but many other aerobic gram-positive and gram-negative organisms were isolated. Anaerobic bacteria were unusual. Bacteremia occurred only once.