Hirschsprung's disease
ICD-10 Q43.1 · ICD-11 LB16.1

Hirschsprung's Disease: Management When Bowel Decompression Requires Surgical Intervention

Clinical scenario

In some presentations of Hirschsprung's disease, rectal irrigations are insufficient to adequately decompress the bowel, or the patient develops complications — including enterocolitis unresponsive to non-operative treatment, or bowel perforation. In these situations, surgical intervention is indicated.

Treatment approach

Management in this setting centres on a surgical diversion procedure to relieve obstruction and address the complication. The specific approach — and the recommended level of intervention — depends on the clinical picture.

Full regimen details, surgical considerations, and decision criteria available in the complete protocol →
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References

A stoma is indicated if rectal irrigations do not sufficiently decompress the bowel, or there are complications such as enterocolitis unresponsive to non-operative treatment, or bowel perforation.

The safest empiric level is an ileostomy.

DOI: 10.1186/s13023-020-01362-3
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