Protective small intestinal stoma in surgical correction of the total form of intestinal agangliosis in children
DOI:
https://doi.org/10.15574/PS.2020.67.59Keywords:
children, total agangliosis, treatment, intestinal stoma, resultsAbstract
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.
References
Amerstorfer EE, Fasching G, Till H, Huber-Zeyringer A, Hollwarth ME. (2015). Long-term results of total colonic agangliosis patients treated by preservation of the aganglionic right hemicolon and the ileo-cecal valve. Pediatr Surg Int. 31(8):773–80. https://doi.org/10.1007/s00383-015-3743-7; PMid:26160361
Bodnar OB, Dzham OP, Prytula VP, Vatamanesku LI, Bodnar HB. (2016). Khronichnyi kolostaz u ditei (khirurhichnyi pohliad na problemu). Chernivtsi: BDM.
Burkardt DD, Graham Jr JM, Short SS, Frykman PK. (2014). Advances in Hirschsprung disease genetics and treatment strategies: an update for the primary care pediatrician. Clin Pediatr (Phila). 53(01): 71-81. https://doi.org/10.1177/0009922813500846; PMid:24002048
Chhabra S, Kenny SE. (2016). Hirschsprung’s disease. Surgery (Oxford). 34(12): 628–632. https://doi.org/10.1016/j.mpsur.2016.10.002
Chun-Hui P, Ya-Jun C, Wen-Bo P, Ting-Chong Zh et al. (2018). STROBE-anastomotic leakage after pull-through procedure for Hirschsprung disease. Medicine. 97:46 e13140): 1–5. https://doi.org/10.1097/MD.0000000000013140; PMid:30431584 PMCid:PMC6257430
Fernandez Ibieta M, Sanchez Morote JM, Martinez Castano I et al. (2014). Quality of life and long term results in Hirschsprung’s disease [in Spanish]. Cir Pediatr. 27(03): 117-124.
Khazdouz M, Sezavar M, Imani B, Akhavan H et al. (2015). Clinical outcome and bowel function after surgical treatment in Hirschsprung’s disease. African Journal of Paediatric Surgery. 12(2): 143-147. https://doi.org/10.4103/0189-6725.160403; PMid:26168755 PMCid:PMC4955413
Langer JC. (2013). Hirschsprung’s disease. Curr Opin Pediatr. 25(03): 368-374. https://doi.org/10.1097/MOP.0b013e328360c2a0; PMid:23615177
Laughlin, DM, Friedmacher F, Puri P. (2012). Total colonic aganglionosis: a systematic review and meta-analysis of long-term clinical outcome. Pediatric Surgery International. 28(8): 773–779. https://doi.org/10.1007/s00383-012-3117-3; PMid:22842648
Levitt MA, Dickie B, Pena A. (2012). The Hirschsprungs patient who is soiling after what was considered a «successful» pullthrough. Seminars in Pediatric Surgery. 21(4): 344–353. https://doi.org/10.1053/j.sempedsurg.2012.07.009; PMid:22985840
Meinds RJ, Eggink MC, Heineman E, Broens PM. (2014). 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. 49(10): 1488-1492. https://doi.org/10.1016/j.jpedsurg.2014.05.001; PMid:25280652
Moore SW. (2015). Total colonic aganglionosis and Hirschsprung’s disease: a review. Pediatr Surg Int. 31 (1): 1–9. https://doi.org/10.1007/s00383-014-3634-3; PMid:25367097
Prytula V, Levytskyi A, Silchenko M, Kurtash O, Hussaini F, Kuzyk A, Petryk S. (2019) Surgical approaches for long type of Hirschsprung’s disease in children. Standardy Medyczne – Problemy Chirurgii Dziciecej. 9 (1):104.
Tran VQ, Mahler T, Dassonville M, Truong DQ, Robert A, Goyens P, Steyaert H. (2018) 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. 28 (5): 445–454. https://doi.org/10.1055/s-0037-1604115; PMid:28738437
Urla C, Lieber J, Obermayr F, Busch A, Schweizer R, Warmann SW, Fuchs J. (2018). Surgical treatment of children with total colonic aganglionosis: functional and metabolic long-term outcome. BMC Surgery. 18(1). https://doi.org/10.1186/s12893-018-0383-6; PMid:30111320 PMCid:PMC6094876
Yeh YT, Tsai HL, Chen CY, Wang JB, Chin TW, Wei CF et al. (2014). Surgical outcomes of total colonic aganglionosis in children: a 26-year experience in a single institute. J Chin Med Assoc. 77(10): 519–23. https://doi.org/10.1016/j.jcma.2014.05.012
Downloads
Published
Issue
Section
License
The policy of the Journal “PAEDIATRIC SURGERY. UKRAINE” is compatible with the vast majority of funders' of open access and self-archiving policies. The journal provides immediate open access route being convinced that everyone – not only scientists - can benefit from research results, and publishes articles exclusively under open access distribution, with a Creative Commons Attribution-Noncommercial 4.0 international license(СС BY-NC).
Authors transfer the copyright to the Journal “PAEDIATRIC SURGERY.UKRAINE” when the manuscript is accepted for publication. Authors declare that this manuscript has not been published nor is under simultaneous consideration for publication elsewhere. After publication, the articles become freely available on-line to the public.
Readers have the right to use, distribute, and reproduce articles in any medium, provided the articles and the journal are properly cited.
The use of published materials for commercial purposes is strongly prohibited.