From: ERNICA guidelines for the management of rectosigmoid Hirschsprung’s disease
Patients should receive saline rectal irrigations 1–3 times per day to decompress the bowel until the definitive pull-through operation • An additional colonic wash-out may be given for pre-operative bowel preparation • See below, if there is an inadequate response to rectal irrigations | Level of evidence III Strength of recommendation: Strong, for Level of agreement: 100% |
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 • In pneumoperitoneum, also an ileostomy provided it is proximal to the site of perforation • A representative circumferential ‘doughnut’ biopsy taken from the site of stoma formation is informative regarding the ganglionic status of the bowel at that level | Level of evidence III Strength of recommendation: Strong, for Level of agreement: 100% |
When possible, a pre-operative contrast enema is recommended to guide on the likely level of aganglianosis • A colonic caliber change suggests a histological transition zone at this level. • Proximal to the rectosigmoid junction, colonic caliber changes are less accurate in predicting the disease level, and the possibility of long-segment HSCR should be considered • Contrast studies are complementary tools during the pre-operative workup. They do not replace the need for histological assessment to confirm the diagnosis. | Level of evidence III Strength of recommendation: Conditional, for Level of agreement: 100% |
At pull-through surgery, one dose of broad-spectrum intravenous antibiotics should be given preoperatively. • The choice of antibiotics is determined by local regimens and regional resistance profiles, but should include coverage of both aerobic and anaerobic bacteria • No additional benefit has been shown for giving more than one pre-operative dose, but antibiotics may be continued for 24–48 h post-operatively | Level of evidence II-III Strength of recommendation: Strong, for Level of agreement: 100% |