SURGICAL TREATMENT OF TOTAL COLONIC AGANGLIOSIS IN CHILDREN

Abstract

Introduction. Surgical treatment of total colonic aganglionosis in children after colectomy requires complex radical reconstructive plastic surgery. There are wide ranges of views for the selection of optimal approach for the correction of such anomalies. The main purpose of surgical intervention is to eliminate the consequences of colectomy by restoring the integrity of intestinal tract and normalize the disorders of water-electrolyte balance, improve normobiosis of intestine, increase the possibility of formation and accumulation of feces, slowing the passage of chyme in the digestive tract and correction of other metabolic disorders.The aim is to develop optimal approaches for surgical treatment of total colonic aganglionosis in children.Materials and methods. From 1980 to 2020 we have analyzed the surgical treatment of 41 children with total colonic aganglionosis using optimal reconstructive surgery for the formation of a functional intestinal reservoir. Isolated total colonic aganglionosis was found in 36 (87.80%) of 41 patients, while aganglionosis of total colon and a fragment of the small intestine in 5 (12.20%) of them.Results. The imposition of a protective ileostomy was performed as the first stage of surgical treatment in all the patients. 8-14 months later these children went through radical surgical intervention - colectomy with reconstructive operation for the formation of functionally advantageous small intestinal reservoir for restoring the integrity of the intestinal tract, with the reservoirs such as "J" - reservoir (n=1); ileotransplant with lateral ileo-ileoanastomosis (n=28); ileotransplant with lateral ileo- colo anastomosis (n=7); ileo-rectal primary anastomosis (n=1) or entero-enteroanastomosis with intussusception valve (n=4). The protective ileostomy was closed in 3-4 months after formation of reservoir.There were no complications in the postoperative period. The first 3 months the frequency of stool was 10-15 times, and after 1 year - 2-4 times a day. All patients survived. Functional treatment results are good. Intestinal function is satisfactory. Radiographs showed formed rectum with sufficient volume of reservoir and colonization.Conclusions. Surgical treatment of total colonic aganglionosis in children requires a three-stage approach: the imposition of a protective small intestinal stoma (1st stage); radical operation - colectomy with reconstructive-plastic operation for the formation of functionally advantageous reservoir as variant of "neorectum" (2nd stage) and closure of the ileostomy after adaptation of the formed small intestinal reservoir (3rd stage). Interoperative period (between ileostomy and radical surgery) lasting 8-14 months provides the restoration of important structural and homeostatic changes in children at the stages of surgical correction of the total colonic aganglionosis. Restoration of integrity of intestinal tract with the formation of functionally advantageous reservoir "side-by-side" with ileo-colon graft or ileo-ileo graft – is the best option for reconstructive surgery after total colectomy in children with aganglionosis. The formation of a functionally beneficial reservoir in children prevents water-electrolyte imbalance, improves normobiosis, formation and accumulation of feces, slows down the passage in the digestive tract and prevents the development of other metabolic disorders.No conflict of interest was declared by the authors.