Variability of the transition zone length in Hirschsprung disease

Surgical management of Hirschsprung disease (HD) involves fully excising the transition zone (TZ). The literature suggests that resection of ≥5 cm of ganglionic bowel proximal to the aganglionic segment is sufficient. Our primary aim was to evaluate the lengths of the TZ in a cohort of consecutive patients with HD. We reviewed the impact this had on the need for revision surgery. We hypothesized that the TZ can be highly variable and may lead to a TZ pull-through when the proximal donut is not reviewed intraoperatively.

A retrospective review was conducted for all patients undergoing primary pull-through surgery between January 2012 and September 2018. Data was collected on demographics, need for staged surgery, and complications following surgery.

Forty-eight patients were eligible for inclusion. 11/48 (23%) patients presented late (>6 months). 27/48 (56%) patients needed a stoma prior to definitive surgery. The median age at pull-through was 6 months (1-84 months). The median TZ length was 1.7 cm (0.3-22.9 cm). 11/48 (23%) had a TZ >5 cm. 36/48 (75%) patients did not have intraoperative review of the donut resulting in three TZ pull-throughs.

We would advocate circumferential intraoperative frozen section review of the proximal donut to minimize the risk of a TZ pull-through.

Level III.