A successful treatment of total colonic aganglionosis in a child

Abstract

Introduction. The earliest mention of total colonic aganglionosis, as the form of Hirschsprung’s disease, is dated 1926. According to various sources, total colonic aganglionosis is met approximately in 1 per 500 000 live births. Currently, the curative strategy in total colonic aganglionosis is strongly personalized. There are various surgical techniques for managing this severe congenital colon anomaly. Recent achievements in pediatric coloproctology have minimized the mortality rate in children with intestinal neuronal dysplasia and total colonic aganglionosis. However, despite of the evident progress in coloproctology, questions about the volume of reconstructive surgery in total colonic aganglionosis, application of “reservoir” surgical techniques so as to promote child’s further development and socialization are still unsolved.
Material and methods. The patient was successfully treated with a reservoir technique which included an ileo-transverse anastomosis by Martin-Kimur with the formed conduit pulled to the anterior abdominal wall. Then, a radical intervention was made which consisted of combined abdominoperineal transanal endorectal pull through (TEPT) of the ileo-transverse conduit/reservoir, of the resection of rudimentary colon and a coloanal anastomosis by Boley.
Conclusion. The obtained small intestine-colon reservoir in total aganglionosis allows to preserve motor function due to peristaltic movements of the small intestine wall and to prevent intestinal obstruction, thereby facilitating the physiological function of the intestine. In addition, the colon wall in the reservoir saves the area of absorbing surface and minimizes water-electrolyte disorders.

Keywords

total colonic aganglionosis / Hirschsprung’s disease / reservoir surgical techniques

References

[1] 1. Moore S.W. Total colonic aganglionosis and Hirschsprung’s disease: a review. Pediatr. Surg. 2015; 31(1): 1–9.

[2] 2. Морозов Д.А., Пименова Е.С. Диагностика и лечение болезни Гиршпрунга у детей в Российской Федерации, подготовка проекта федеральных клинических рекомендаций. Российский вестник детской хирургии, анестезиологии и реаниматологии. 2018; 8(1): 6–12.

[3] 3. Ziegler M.M., Ross A.J. and Bishop H.C. Total Intestinal Aganglionosis: A New Technique for Prolonged Survival. Journal of Pediatric Surgery. 1987; 22(1): 82–3.

[4] 4. Чубарова А.И., Костомарова Е.А., Мокрушина О.Г., Шумихин В.С. и соавт. Опыт диагностики и лечения пациентов с тотальным и субтотальным аганглиозом кишечника. Российский вестник детской хирургии, анестезиологии и реаниматологии. 2016; 6 (2): 26–35.

[5] 5. Шелыгин Ю.А. Клинические рекомендации. Колопроктология. М.: ГЭОТАР-Медиа. 2015: 50–51.

[6] 6. Ruttenstock E., Puri P. A meta-analysis of clinical outcome in patients with total intestinal aganglionosis. Pediatr. Surg. 2009; 25: 833–9.