SUMMARY
Introduction: Correction of total colonic agangliosis is very difficult as there are wide range of approaches. Surgical intervention should eliminate consequences of colectomy by restoring integrity of intestinal tract and correct metabolic disorders.
Aim: to develop optimal surgical approach for treatment of TCA in children.
Materials and methods: We treated 41 children from 1980 – 2020 using optimal reconstructive surgical approach for formation of reservoir. In 36 (87.80 %) isolated total colonic agangliosis was found and in 5 (12.20 %) agangliosis of total colon and fragment of small intestine.
Results: First stage – imposition of a protective ileostomy. Second stage after 8 – 14 months – colectomy with formation of small intestinal reservoir, with reservoirs such as J-shaped reservoir (n = 1); “side-by-side” ileo-ileo graft (n = 28); “side-by-side” ileo-colon graft (n = 7); ileo-rectal anastomosis (n = 1) or entero-entero anastomosis with valve (n = 4). Third stage closure ileostomy after 3 – 4 months. Postoperative period was without complications. Frequency of stool decreased with time from 10 – 15 times to 2 – 4 times a day. All patients survived with good functional results.
Conclusions: Total colonic agangliosis requires a three-stage approach: protective ileostomy (1st stage); colectomy with formation reservoir (2nd stage) and closure of ileostomy (3rd stage). Formation of “side-by-side” with ileo-colon graft or ileo-ileo graft – was best option. Reservoir in children, normalize water-electrolyte balance, normobiosis, correction of metabolic disorders.
Key words: children, total colonic agangliosis, treatment, ileostomy, results.
Lek Obz, 2021, 70 (6): 216-220
Vasyl PRYTULA 1,2, Oleh KURTASH 3
1 Bogomolets National Medical University, Kyiv, Ukraine
2 National Children’s Specialized Hospital “OKHMATDYT”, Kyiv, Ukraine
3 Ivano-Frankivsk National Medical University, Ivano-Frankivsk, Ukraine
Cite:
PRYTULA V., KURTASH O.: Total colonic agangliosis in children. Lek Obz, 2021, 70 (6): 216-220
Úplná aganglióza kolonu u detí
Citácia:
PRYTULA V., KURTASH O.: Úplná aganglióza kolonu u detí. Lek Obz, 70, 2021, č. 6, s. 216-220
