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 perplexing complication of Hirschsprung's disease affecting especially patients with altered immune defense such as those with Down's syndrome. Sodium cromoglycate (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 refractory chronic or recurrent enterocolitis. METHODS Eight patients (4 with Down's syndrome, 2 with other chromosomal aberrations, 2 otherwise healthy; age range from 4 to 22 years) with chronic (5 patients) or recurrent (>6 episodes/year, 3 patients) enterocolitis received 100 to 200 mg of SCG 4 times a day depending on the age of the patient. 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 macroscopic and histological chronic inflammation in all cases. No neuronal abnormalities were detected in biopsy results. None of the patients had colonic dilatation or increased anorectal resting pressures suggesting outlet obstruction. RESULTS The follow-up of the patients ranges from 8 months to 26 months. Three of the 5 patients with chronic enterocolitis responded favorably. In these 3 patients the median number of daily bowel 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 antibiotics after the onset of SCG treatment; one patient had an episode of enterocolitis 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 patients with Hirschsprung's disease. Because SCG is not absorbed From the intestinal tract there are no systemic side effects.