Sodium cromoglycate in the management of chronic or recurrent enterocolitis in patients with Hirschsprung's disease

Background/Purpose: Chronic or recurring enterocolitis is a rare but perple xing complication of Hirschsprung's disease affecting especially patients w ith altered immune defense such as those with Down's syndrome. Sodium cromo glycate (SCG) is a nonabsorbable mast cell stabilizing agent that has been documented to be effective in the treatment of inflammatory bowel disease. The authors studied the effect of SCG in Hirschsprung patients with refract ory chronic or recurrent enterocolitis, Methods: Eight patients (4 with Down's syndrome, 2 with other chromosomal a berrations, 2 otherwise healthy; age range from 4 to 22 years) with chronic (5 patients) or recurrent (>6 episodes/year, 3 patients) enterocolitis rec eived 100 to 200 mg of SCG 4 times a day depending on the age of the patien t. The chronic diarrhea or recurrent bouts of enterocolitis in the patients were refractory to dietary management and enteral antibiotics. Before the treatment all patients had ileocolonoscopy, the results of which showed mac roscopic and histological chronic inflammation in all cases. No neuronal ab normalities were detected in biopsy results. None of the patients had colon ic dilatation or increased anorectal resting pressures suggesting outlet ob struction. Results: The follow-up of the patients ranges from 8 months to 26 months. T hree of the 5 patients with chronic enterocolitis responded favorably. In t hese 3 patients the median number of daily bower movements decreased from 6 to 3, and none experienced bouts of abdominal distension, Diarrhea-related soiling decreased also significantly. Two of the 3 patients with recurrent enterocolitis have remained asymptomatic, and none has required antibiotic s after the onset of SCG treatment; one patient had an episode of enterocol itis after 12 months treatment. Two patients with chronic enterocolitis did not respond to SCG. No side effects of SCG were encountered. Conclusions: This preliminary and nonrandomized study suggests that SCG is an effective treatment modality for chronic or recurrent enterocolitis in p atients with Hirschsprung's disease. Because SCG is not absorbed from the i ntestinal tract there are no systemic side effects. Copyright (C) 2001 by W .B. Saunders Company.