Colonic Stoma Creation as the First Stage of Surgical Correction of Total Colonic Aganglionosis in Children
Abstract. The use of a staged approach in surgical treatment of total colonic aganglionosis is controversial. The creation of a protective colonic stoma as the first stage of surgical correction of total colonic aganglionosis rather than one-stage correction has become an alternative in treatment of such patients. This approach contributes to better survival, reduces the incidence of ileoanal anastomotic failure and improves functional outcomes.
The objective of the research was to study and evaluate the need for colonic stoma creation as the first stage of surgical correction of total colonic aganglionosis in children.
Materials and Methods. The analysis of surgical treatment of 41 children with total colonic aganglionosis over the period 1980-2020 was conducted. Protective colonic stoma was created in all the patients, namely 36 children with isolated colonic aganglionosis and 5 children with the involvement of the entire colon and a segment of the small bowel.
Results. Twenty-four (58.54%) patients were diagnosed and underwent the first stage of treatment in National Specialized Children’s Hospital “Okhmatdyt”. There were 17 (41.46%) patients who were transferred from other clinics after being diagnosed with a pathology and undergoing colonic stoma creation. During staged treatment of children with total colonic aganglionosis, a single-barrel ileostomy (n=10, 24.40%), a double-barrel ileostomy (n=5, 12.19%), or a loop colonic ileostomy (n=26, 63.41%) were created. The periods between creating the protective colonic stoma and performing radical surgery ranged from 4 to 14 months. The colonic stomas were closed 2-4 months after radical surgery. The third stage of surgical correction of total colonic aganglionosis in children was carried out after the state of the neorectum formed and ileoanal anastomosis were assessed for readiness of inclusion in the passage. There were no complications after protective stoma closure. Within the first 3 months, the frequency of bowel movement ranged from 10 to 15 times a day, and, in a year, it was 2-4 times a day. All the children survived. Functional outcomes of treatment were found to be good. Bowel function was satisfactory. The X-ray images demonstrated the rectal reservoir of sufficient size with pronounced colonization.
Conclusions. Surgical correction of total colonic aganglionosis in children involves the three-stage approach consisting in the creation of the protective small bowel stoma (the first stage), radical surgery, namely colectomy with reconstructive plastic formation of functionally advantageous reservoir version of the neorectum (the second stage) and ileostomy closure after adaptation of the small bowel reservoir (the third stage). The creation of the protective small bowel stoma as the first stage of surgical correction of total colonic aganglionosis in children is emergency surgery. The creation of the loop small bowel stoma at 12 cm above aganglionosis level is the most rational type of the first stage of correcting total colonic aganglionosis in children.
Bischoff A, Levitt MA, Peña A. Total colonic aganglionosis: a surgical challenge. How to avoid complications? Pediatr Surg Int. 2011;27(10):1047-1052. https://doi.org/10.1007/s00383-011-2960-y
Bodnar OB, Dzham OP, Prytula VP et al. Khronichnyi kolostaz u ditei (khirurhichnyi pohliad na problemu). Chernivtsi: BDM; c2016. 199p.
Burkardt DD, Graham Jr JM, Short SS et al. Advances in Hirschsprung disease genetics and treatment strategies: an update for the primary care pediatrician. Clin Pediatr (Phila). 2014;53(1):71-81. https://doi.org/10.1177/0009922813500846
Chhabra S, Kenny SE. Hirschsprung's disease. Surgery (Oxford). 2016;34(12):628-632. https://doi.org/10.1016/j.mpsur.2016.10.002
Chun-Hui P, Ya-Jun C, Wen-Bo P et al. STROBE-anastomotic leakage after pull-through procedure for Hirschsprung disease. Medicine (Baltimore) 2018; 97(46): e13140. https://doi.org/10.1097/MD.0000000000013140
Cobellis G, Noviello C, Cruccetti A et al. Staged laparoscopic-assisted endorectal pull-through for long segment Hirschprung's disease and total colonic aganglionosis. Minerva Pediatr. 2011;63(3):163-167.
Ekenze SO, Ngaikedi C, Obasi AA. Problems and Outcome of Hirschsprung's Disease Presenting after 1 Year of Age in a Developing Country. World J Surg. 2011;35(1):22-26. https://doi.org/10.1007/s00268-010-0828-2
Fernández Ibieta M, Sánchez Morote JM, Martínez Castaño I et al. Quality of life and long term results in Hirschsprung's disease. Cir Pediatr. 2014;27(3):117-124. [published in Spanish]
Khazdouz M, Sezavar M, Imani B et al. Clinical outcome and bowel function after surgical treatment in Hirschsprung's disease. Afr J Paediatr Surg. 2015;12(2):143-147. https://doi.org/10.4103/0189-6725.160403
Kyrklund K, Koivusalo A, Rintala RJ et al. Evaluation of bowel function and fecal continence in 594 Finnish individuals aged 4 to 26 years. Dis Colon Rectum. 2012;55(6):671-676. https://doi.org/10.1097/DCR.0b013e31824c77e4
Langer JC. Hirschsprung's disease. Curr Opin Pediatr. 2013;25(3):368-374. https://doi.org/10.1097/MOP.0b013e328360c2a0
Laughlin DM, Friedmacher F, Puri P. Total colonic aganglionosis: a systematic review and meta-analysis of long-term clinical outcome. Pediatr Surg Int. 2012;28(8):773-779. https://doi.org/10.1007/s00383-012-3117-3
Levitt MA, Dickie B, Peña A. The Hirschsprungs patient who is soiling after what was considered a "successful" pull-through. Semin Pediatr Surg. 2012;21(4):344-353. https://doi.org/10.1053/j.sempedsurg.2012.07.009
Meinds RJ, Eggink MC, Heineman E et al. Dyssynergic defecation may play an important role in postoperative Hirschsprung's disease patients with severe persistent constipation: analysis of a case series. J Pediatr Surg. 2014;49(10):1488-1492. https://doi.org/10.1016/j.jpedsurg.2014.05.001
Prytula V, Levytskyi A, Silchenko M et al. Surgical approaches for long type of Hirschsprung's disease in children. Standardy Medyczne - Problemy Chirurgii Dziciecej. 2019;9(1):104.
Tran VQ, Mahler T, Dassonville M et al. Long-Term Outcomes and Quality of Life in Patients after Soave Pull-Through Operation for Hirschsprung's Disease: An Observational Retrospective Study. Eur J Pediatr Surg. 2018;28(5):445-454. https://doi.org/10.1055/s-0037-1604115
Urla C, Lieber J, Obermayr F et al. Surgical treatment of children with total colonic aganglionosis: functional and metabolic long-term outcome. BMC Surg. 2018;18(1):58. https://doi.org/10.1186/s12893-018-0383-6
Zakaria OM, El Labban GM, Shams ME. Fecal incontinence after single-stage Soave's pull-through: abdominal versus transanal endorectal pull-through. Ann Pediatr Surg. 2012;8:5-8. https://doi.org/10.1097/01.XPS.0000407759.30719.57
Copyright (c) 2020 Oleh Kurtash
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