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Table 4 Recommendations for operative management of rectosigmoid HSCR

From: ERNICA guidelines for the management of rectosigmoid Hirschsprung’s disease

Centres should perform the type of pull-through in which they have the most experience, including management of post-operative complications and follow-up
• Transanal endorectal pull-through (ERP), including laparoscopy-assisted ERP, and Duhamel pull-through represent the most commonly performed operations. Currently, there is no evidence for overall superiority of one method over another in terms of surgical complications or long-term bowel function.
Level of evidence III
Strength of recommendation: Conditional, for Level of agreement: 100%
The pull-through operation should be performed when the patient is stable and growing well, and the bowel has been sufficiently decompressed
• Elective pull-through within 2–3 months after diagnosis is usual
• No specific advantages have been identified for performing pull-through surgery during the neonatal period
• Anaesthetic considerations, clinical and nutritional status of the patient, parental concerns and surgical risks/technical feasibility influence the timing of pull-through surgery
Level of evidence III
Strength of recommendation: Conditional, for Level of agreement: 100%
The anal canal should be preserved during pull-through surgery
• The transitional mucosa above the dentate line contains the nerve endings responsible for the reflex arc in sensation and fecal continence, including the sampling reflex.
• Transanal dissection should be commenced 0.5–2 cm proximal to the dentate line
• In endorectal pull-through, either no muscle cuff (Swenson) or a short muscle cuff (< 2-3 cm) have comparable outcomes, but long seromuscular cuffs should be avoided
Level of evidence II-III
Strength of recommendation: Strong, for Level of agreement: 100%
The colon should be transected at least 5 to 10 cm proximal to the first normal biopsy minimize the risk of a transition zone pull-through.
• If the level of disease remains intraoperatively uncertain, ‘mapping’ biopsies should be obtained from different colonic levels.
• Intraoperatively, fresh frozen sections are a valid means for determining the presence of normal ganglionated bowel but single samples may miss asymmetrical histologic extension of the transition zone.
• A circular ‘doughnut’ biopsy from the level of transection permits circumferential (4-quadrant) optimal histologic assessment
• If feasible, any abnormally dilated colon proximally should be resected to avoid a transition zone pull-through.
• At the end of the operation, the resected specimen should be sent in full (marked oral to anal) to the pathologist.
Level of evidence III
Strength of recommendation: Strong, for Level of agreement: 100%