Transanal rectal mucosectomy and partial internal anal sphincterectomy for Hirschsprung's disease

Abstract

PurposeHirschsprung-associated enterocolitis (HAEC) is a serious complication of Hirschsprung’s disease (HD), with generalized sepsis and high mortality rate. Although the surgical correction of HD is mostly successful, challenges remain in the management of children with repeated episodes of enterocolitis. The authors describe a novel modification of transanal rectal mucosectomy and partial internal anal sphincterectomy (TRM-PIAS) for HD. MethodsOne hundred twenty-seven HD children aged from 8days to 16years who successfully underwent TRM-PIAS were reviewed. TRM-PIAS was carried out circumferentially along the anorectal line. Anterior dissection was conducted between the rectal submuscosal layer and the rectal muscular sleeve. The posterior dissection was performed along the plane between internal and external anal sphincters. Normal colon was pulled through and anastomosed to anal mucosa. Aganglionic segment, rectal mucosa, part of internal anal sphincter and posterior rectal muscular cuff were removed. Twenty-five age-matched children without defecation dysfunction were used as the control group in the study of anal resting pressure. ResultsPatients were followed up for 6–12years (median: 8.2years). The median age at last follow-up was 12.2years (7.2–20.1years). The incidence of enterocolitis decreased from 33.9% (43/127) preoperatively to 1.6% (2/127) postoperatively (P<0.01). The incidence of constipation decreased from 100% (127/127) preoperatively to 2.4% (3/127) postoperatively (P<0.01). Soiling rate on postoperative 1month was 32.3%. It gradually decreased to 1.6% 6months later. Anorectal manometries showed that mean anal resting pressure was significantly reduced from 37.9±12.5mm Hg preoperatively to 20.2±6.4mm Hg on postoperative 1month and 24.8±9.9mm Hg on postoperative 6months, which were similar to age-matched normal controls (27.9±9.6mm Hg, P>0.05). ConclusionsTRM-PIAS is effective in treatment of HD. It is associated with low postoperative HD-associated enterocolitis.