Single-stage transanal endorectal pull-through for Hirschsprung's disease: A retrospective study of surgico-pathological correlations and technical considerations

Abstract

Background: Transanal endorectal pull-through (TEPT) is a well-established operation for the management of rectosigmoid and short-segment Hirschsprung's disease (HD). A well-performed contrast enema (CE) is an essential road map for the surgeon when attempting the TEPT. We looked at the surgicopathological–radiological correlations and technical considerations of this procedure and discussed our experience over a decade. TEPT essentially relies on radiological mapping of the disease correlating with intraoperative and pathological findings. Some investigators opine that the radiological correlation of the transition zone (rTZ) with the pathological transition zone (pTZ) is accurate whereas others disagree. We review our experience in managing HD patients with TEPT, in terms of pre-operative workup and operative and pathological considerations. Materials and Methods: A retrospective study was done in a single unit, from January 2010 to January 2020. Forty-seven patients who met the inclusion criteria such as short-segment HD and uncomplicated HD underwent CE before surgery. The patients included in the review underwent a primary TEPT. For each patient, we identified the radiological transition zone (rTZ) on the contrast enema, the gross transition zone (gTZ) on intraoperatively visual examination, the frozen section transition zone (fTZ) on intraoperative analysis of the frozen section specimens (fTZ), and the pathological transition zone (pTZ) on permanent paraffin blocks of the specimens. We determined the strength of correlation of the rTZ, the gTZ and the fTZ with reference to the pTZ. Results: Forty-seven patients underwent single-stage transanal pull-through. There were 40 males. There were 8 neonates, 20 infants and 19 children (>12 months of age). The mean age at diagnosis was 8.7 days (3–20 days) for the neonates, 5.11 months (2–12 months) for the infants and 4.3 years (1.5–9 years) for children. In our study, 85% correlation was noted between the rTZ and the pTZ (τb = 0.362, P = 0.006). There was an excellent correlation between the intraoperative gTZ, fTZ and the pTZ in all patients except one (97.8%, τb = 0.942, P < 0.001). Conclusions: The TEPT is a feasible option to manage carefully selected patients with rectosigmoid HD. The presence of robust support from pathology and a properly carried out CE are essential parameters to be considered before successfully undertaking these operations. The functional outcomes of TEPT are comparable with other procedures for HD with the added advantage of it being a scar-less and a stoma-less approach.

Keywords

• contrast enema
• frozen section
• hirschsprung's disease
• transanal endorectal pull-through
• transition zone