EXTENDED MYECTOMY-MYOTOMY - A THERAPEUTIC ALTERNATIVE FOR TOTAL INTESTINAL AGANGLIONOSIS

Objective That total intestinal aganglionosis (extended Hirschsprung's disease) is uniformly incompatible with life as reported in 1985, is challenged by this series of patients treated over the last 7 years wi th an alternative therapy, extended myectomy-myotomy of the small bowe l. Summary Background Data A total of 16 neonates worldwide presented with intestinal obstruction secondary to total (extending to the stoma ch) or near total (to 40 cm of jejunum) intestinal aganglionosis confi rmed at one or more leveling operations. Methods A patient questionnai re was answered by the surgeon of all 16 patients. Results The sex dis tribution was eight boys and eight girls. The definitive operation inc luded extending an antimesenteric myectomy-myotomy from the ganglionic -aganglionic transition zone for variable lengths, the operative desig n being to create sufficient small bowel length to support life (40-cm minimum, total small bowel maximum). The myectomized bowel was termin ated as an end-stoma or as an isolated jejuno-ileal segment. Ten of 16 patients have survived (62.5%) whose length of ganglionated bowel var ies from 0 to 40 cm (mean, 12.4 cm; median, 6.0 cm). Six patients have died from 1 to 33 months after operation (mean, 9.5 months; median, 5 .0 months) of gut-induced infection (n = 5) and respiratory failure (n = 1); their ganglionated bowel length was similar to survivors (range , 0-26 cm; mean, 9.2 cm; median, 8.0 cm). Of the 16 patients, 15 have received enteral nutrients through the myectomized bowel. Of ten survi vors, strikingly two are totally gut nourished (2 cm, 7-cm length of g anglionated bowel), six receive from 1/5 to 4/5 of total calories ente rally, and one receives minimal enteral feeding. Conclusions From thes e patients we have learned that (1) extended myectomy-myotomy relieves the obstruction of extended Hirschsprung's disease; (2) aganglionic b owel after extended myectomy-myotomy acts as a passive conduit for pro ximally propulsed nutrients; and (3) aganglionic bowel after extended myectomy-myotomy undergoes adaptive change and is capable of absorbing life-supporting nutrients. These data demonstrate extended myectomy-m yotomy to be a therapeutic option for otherwise fatal extended Hirschs prung's disease, either as a potentially definitive therapy or as a pu tative bridge to intestinal transplantation.