Protective small intestinal stoma in surgical correction of the total form of intestinal agangliosis in children

Abstract

Introduction. Agangliosis of the colon is a congenital malformation of the lower intestinal tract, in which for there is absence of intramurable nerve plexus, which leads to persistent intestinal dysfunction. Total form of intestinal agangliosis is a rare anomaly that occurs up to 1 in every 50,000 newborn. The question of the reliability of protected small intestinal stomas, the alignment of levels, type and methods of their formation for the surgical correction of this anomaly is still discussable. Aim: to develop the optimal approaches for thr requirement of of protective small intestinal stomas for the surgical correction of the total form of intestinal agangliosis in children. Materials and methods. From 1980 to 2020, the experience of surgical treatment of 41 children with a total form of intestinal agangliosis was analyzed. Isolated affection with agangliosis of the colon alone were present in 36 (87.80%) of 41 patients, and affection of the entire colon and a fragment of the small intestine were diagnosed in another 5 (12.20%) children. In all patients, the first stage of surgery was – the formation of a protective small intestinal stoma. Results. All small intestinal stomas in our patients were imposed within 1–4 days of life as per emergency indications. In the staged treatment of children with total agangliosis, we imposed terminal single-stemmed somas in (n=10 (24.40%)), terminal double-stemmed (n=5 (12.19%)) and loop intestinal ileostomy (n=26 (63.41%)). The periods between the imposition of a protective intestinal stoma and radical surgery ranged from 8 to 14 months. Intestinal stoma closure was performed 2–4 months after radical surgery. All the children survived. The functional results of their treatment are good. Conclusions. The formation of protective small intestinal stoma as the first stage of surgical correction of total intestinal agangliosis in children as emergency surgical procedure. Loop small intestinal stoma at a distance of up to 12 cm from the level of agangliosis in total colon is the most rational type of the first stage of surgical correction of this pathology without signs of perforation in children. Closure of protective small intestinal stomas during surgical correction of the total form of intestinal agangliosis in children should be performed 2–4 months after radical surgery, provided that the formed «neorectum» and ileo-anal anastomosis are ready for inclusion in the passage. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of all participating institution. The informed consent of the patient was obtained for conducting the studies.

Keywords

• діти
• тотальний агангліоз
• лікування
• кишкові стоми
• результати