THE SURGICAL IMPLICATIONS OF CHRONIC GRANULOMATOUS-DISEASE

BACKGROUND: Chronic granulomatous disease (CGD) of childhood is a rare congenital abnormality of the phagocyte NADPH oxidase system. Affecte d neutrophils and macrophages have an ineffective respiratory burst an d cannot destroy certain phagocytized bacteria and fungi. CGD patients usually present with recurrent pyogenic and fungal infections. Catala se-positive bacteria are frequently involved, since they metabolize th e hydrogen peroxide they produce, making it unavailable for augmentati on of microbicidal activity in CGD neutrophils. Afflicted patients als o have a tendency to form granulomas, which can lead to obstruction of the gastrointestinal and genitourinary tracts. METHODS: Charts of 10 patients with CGD were reviewed for age at diagnosis, surgical procedu res, complications of these procedures, and medical treatment. RESULTS : Eight of the 10 children were male. The average age at first present ation was 18 months (range 2 days to 9.8 years), Each child developed a mean of 9.9 infections and an average of 1.4 infections per year. Al l required surgical procedures, with an average of 2.9 procedures each . Five children had operative procedures for infections that preceded the diagnosis of CGD. The procedures performed most frequently were in cision and drainage of soft-tissue abscesses (7) or perirectal abscess (3), thoracentesis (3) and bronchoscopy (3). Three children had poor wound healing following surgery. Two developed partial gastric outlet obstruction which resolved with antibiotic therapy. One developed gran ulomatous cystitis with obstruction which responded to antibiotics. CO NCLUSIONS: Since patients with undiagnosed CGD may present with surgic al problems, surgeons need, to be familiar with this condition. The di agnosis should be suspected in children who have recurrent or unusual infections or unexplained problems with wound healing.