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

https://doi.org/10.1053/jpsu.2001.24732Get rights and content

Abstract

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. J Pediatr Surg 36:1032-1035. Copyright © 2001 by W.B. Saunders Company.

Section snippets

Materials and methods

During 1997 and 1998, 8 consecutive patients, with symptoms of chronic or frequently relapsing enterocolitis, were enrolled to the sodium cromoglycate trial. The age range of the patients was from 4 to 22 years. All patients had undergone repair of Hirschsprung's disease during the first year of life. The original reconstruction was Duhamel operation in 2 patients and transanal endorectal coloanal anastomosis11 in 6 patients.

Five of the patients had symptoms of chronic enterocolitis. Three of

Results

At endoscopy the macroscopic findings were consistent with mild to moderate colitis predominantly in the left colon. None of the patients had megacolon. The histology results findings showed chronic inflammation in all cases, especially in the distal biopsies. In histology there were no specific findings suggesting other forms of chronic colonic inflammatory disease such as ulcerative colitis or Crohn's disease. Biopsy results from distal ileum were normal in all patients. Biopsy specimens for

Discussion

The prevalence of chronic or frequently recurring enterocolitis among patients with surgically repaired Hirschsprung's disease is unknown. It is clear that this problem affects more commonly patients with defective immune systems such as those with Down's syndrome. In the current series 4 of the 8 patients had Down's syndrome and 2 more had other chromosomal aberrations. Postoperative enterocolitis also has been attributed to bowel outlet obstruction.6 In the current series none of the patients

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*

Address reprint requests to R.J. Rintala, MD, PhD, Professor of Paediatric Surgery, Children's Hospital, University, P.O. Box 281, FIN-00029 HYKS, Finland.

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