Intestinal stomas in Hirschsprung’s disease: one clinic experience
- Authors: Stepanova N.M.1,2, Novozhilov V.A.1,2,3, Zvonkov D.A.2, Khamzina D.A.4, Rasputin A.A.2, Cheremnov V.S.1,2, Baradieva P.G.1,2, Semenyta A.A.2, Tapkhanakov F.N.1
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Affiliations:
- Irkutsk State Medical University
- Ivano-Matreninsk Children’s City Clinical Hospital
- Irkutsk State Medical Academy of Postgraduate Education ― branch of the Federal State Budgetary Educational Institution of Further Professional Education «Russian Medical Academy of Continuous Professional Education»
- Pediatric Clinic Fantasy
- Issue: Vol 29, No 4 (2025)
- Pages: 242-249
- Section: Original Study Articles
- Submitted: 10.12.2024
- Accepted: 25.07.2025
- Published: 26.08.2025
- URL: https://jps-nmp.ru/jour/article/view/845
- DOI: https://doi.org/10.17816/ps845
- EDN: https://elibrary.ru/BDQENI
- ID: 845
Cite item
Abstract
BACKGROUND: Over its long history, Hirschsprung’s disease surgery has undergone significant changes: traumatic trait of the applied techniques decreased due to the introduction of laparoscopy, surgical stages at the perineum and pelvis became less aggressive due to the rational use of various electrosurgical equipment, the desire to eliminate the disease in one-stage intervention arose. However, for a number of objective reasons, in some situations, disease treating requires step-by-step correction through preliminary ostoma surgery and a series of subsequent surgical interventions.
AIM: To analyze the effectiveness of preventive stomas in various parts of the intestinal tube in patients with an established diagnosis of congenital colon agangliosis.
METHODS: A single-center retrospective study of 111 medical records of children with Hirschsprung’s disease treated in the department of neonatal surgery and surgical department No 1 of the City Ivano-Matreninsk Children’s Clinical Hospital in Irkutsk was conducted from 2001 (January) to 2024 (December).
RESULTS: Of the observed series, 79/111 (71.2%) cases of stage-by-stage treatment of degenerated agangliosis using preliminary stomatology were analyzed, 32 patients were excluded from the analysis due to simultaneous surgical correction of the defect. In 44,3% (n=35), the formation of stom at the place of the child’s primary treatment. Indications for stomy: age―69 (87.3%), the extent of agangliosis―19 (24.1%), decompensated form―18 (22.8%), repeated interventions―55 (69.6%), inability to treat and prepare enemas―9 (11.4%), Hirschsprung-associated enterocolitis―16 (20.3%). Distribution in relation to intestinal segments: ileostomy―55 (69.6%), colostomy above the level of agangliosis―20 (25.3%), sigmostomy to the transit zone―4 (5.1%). According to the method of formation: double―68 (86.1%), terminal―7 (8.9%), T-shaped (Bishop-Koop)―4 (5.1%). The age of children at the time of stomy is from 2 days to 14 years old. The closure was carried out as an independent final stage after reconstruction in 96.2% of cases.
CONCLUSION: The analyzed results have demonstrated that intestinal stomas in the treatment of colon agangliosis is a forced and preventive measure. Indications for stoma formation are most often the following: defect shape, initial somatic background, severity of chronic fecal intoxication equivalents, repeated surgical interventions, concomitant pathology, inability to prepare the intestine for reconstructive interventions by conservative methods.
Full Text

About the authors
Nataliya M. Stepanova
Irkutsk State Medical University; Ivano-Matreninsk Children’s City Clinical Hospital
Email: dm.stepanova@mail.ru
ORCID iD: 0000-0001-5821-7059
SPIN-code: 7825-8561
MD, Cand. Sci. (Medicine), Assistant Professor
Россия, 1 Krasnogo Vosstaniya st, Irkutsk, 664003; IrkutskVladimir A. Novozhilov
Irkutsk State Medical University; Ivano-Matreninsk Children’s City Clinical Hospital; Irkutsk State Medical Academy of Postgraduate Education ― branch of the Federal State Budgetary Educational Institution of Further Professional Education «Russian Medical Academy of Continuous Professional Education»
Author for correspondence.
Email: novozilov@mail.ru
ORCID iD: 0000-0002-9309-6691
SPIN-code: 5633-5491
MD, Dr. Sci. (Medicine), Professor
Россия, Irkutsk; Irkutsk; IrkutskDenis A. Zvonkov
Ivano-Matreninsk Children’s City Clinical Hospital
Email: denis.zvonkov@mail.ru
ORCID iD: 0000-0002-7167-2520
SPIN-code: 6620-6758
Россия, Irkutsk
Daria A. Khamzina
Pediatric Clinic Fantasy
Email: darahamzina533@gmail.com
ORCID iD: 0000-0002-8929-4492
Россия, Moscow
Andrey A. Rasputin
Ivano-Matreninsk Children’s City Clinical Hospital
Email: arasputin@mail.ru
ORCID iD: 0000-0002-5690-790X
SPIN-code: 8223-9093
Россия, Irkutsk
Vladislav S. Cheremnov
Irkutsk State Medical University; Ivano-Matreninsk Children’s City Clinical Hospital
Email: chervl@mail.ru
ORCID iD: 0000-0001-6135-4054
SPIN-code: 5461-8921
Россия, Irkutsk; Irkutsk
Polina G. Baradieva
Irkutsk State Medical University; Ivano-Matreninsk Children’s City Clinical Hospital
Email: polnoch_12@mail.ru
ORCID iD: 0000-0002-5463-6763
SPIN-code: 6231-3100
Россия, Irkutsk; Irkutsk
Anna A. Semenyta
Ivano-Matreninsk Children’s City Clinical Hospital
Email: ankadoc@mail.ru
ORCID iD: 0009-0004-6377-3593
SPIN-code: 4351-0580
Россия, Irkutsk
Fedor N. Tapkhanakov
Irkutsk State Medical University
Email: tapkhanakov@mail.ru
ORCID iD: 0000-0002-7658-0414
SPIN-code: 1780-5279
Россия, Irkutsk
References
- Khamraev AG, Akmollaev DS, Khamraev UA, Ergashev BB. The De La Torre technique as a method of choice for distal forms of hirschsprung disease in children. Tavricheskiy mediko-biologicheskiy vestnik. 2018;21(4):87–90. EDN: XVXQXY
- De La Torre-Mondragon L, Ortega-Salgado JA. Transanal endorectal pull through for Hirschsprung’s disease. J Pediatr Surg. 1998;33(8):1283–1286. doi: 10.1016/s0022-3468(98)90169-5
- Bond GJ. Intestinal transplant for hirschsprung’s disease: stoma for life or not? Gastroenterol Clin North Am. 2024;53(2):289–297. doi: 10.1016/j.gtc.2024.03.002
- Georgeson K, Munsterer O, Kozlov YuA. Hirschsprung disease: laparoscopic endorectal pull-through. Russian Journal of Pediatric Surgery. 2016;20(6):309–314. doi: 10.18821/1560-9510-2016-20-6-309-314 EDN: XRFZBX
- Georgeson KE, Robertson DJ. Laparoscopic assisted approaches for the definitive surgery for Hirschsprung’s disease. Semin Pediatr Surg. 2004;13(4):256–262. doi: 10.1053/j.sempedsurg.2004.10.013
- Svarich VG, Kirgizov IV. Our experience with the treatment of Hirschsprung disease in children. Russian Journal of Pediatric Surgery. 2016;20(5):264–268. doi: 10.18821/1560-9510-2016-20-5-264-268 EDN: WXFMLL
- Lin Z, Liu M, Yan L, et al. Outcome of Santulli enterostomy in patients with immaturity of ganglia: single institutional experience from a case series. BMC Surg. 2022;22(1):400. doi: 10.1186/s12893-022-01849-9 EDN: XJAPJQ
- Amiel J, Sproat-Emison E, Garcia-Barcelo M, et al. Hirschsprung disease, associated syndromes and genetics: a review. J Med Genet. 2008;45(1):1–14. doi: 10.1136/jmg.2007.053959
- Morozov DA, Pimenova ES. Diagnostics and treatment of Hirschsprung’s disease in children from the Russian Federation (preparaing the project of the Federal Clinical Recommendations). Russian journal of pediatric surgery, anesthesia and intensive care. 2018;8(1):6–12. doi: 10.30946/2219-4061-2018-8-1-6-12 EDN: XRKIRV
- Akselrov MA, Razin MP. Prevention of complications by improving indications and methods for the formation of artificial intestinal fistulas in children. Medical newsletter of Vyatka. 2017;(4):4–8. (In Russ.) EDN: YLYIXY
- Yakubov EA, Khurramov FM, Norov MM, Radjabova ShG. Choice of surgical tactics in Hirschsprung’s disease in children. Vestnik nauki i obrazovania. 2020;(9-2):70–73. EDN: UUIGNQ
- Averin VI, Akselrov MA, Degtyarev YuG, et al. Intestinal stoma’s in children. Moscow: GEOTAR-Media; 2020. 112 p. (In Russ.) doi: 10.33029/9704- 5794-8-2020-DCP-1-112 EDN: RHKXFW
- Strykovsky AE, Tarakanov VA, Starchenko VM, et al. Emergency colostomia in treatment of children with illness Hirschsprungs. Kuban scientific medical bulletin. 2010;(9):137–139. EDN: NYDXUV
- Damkjær M, Tan J, Morris JK, et al. Children with Hirschsprung’s disease have high morbidity in the first 5 years of life. Birth Defects Res. 2024;116(5):e2338. doi: 10.1002/bdr2.2338
- Wong MC, Avanzini S, Mosconi M, et al. Enterostomy-related complications in Hirschsprung’s disease in a single cohort. Minerva Pediatr (Torino). 2023;75(5):711–718. doi: 10.23736/S0026-4946.19.05593-2
- Roy C, Jaffray B. Pull through for Hirschsprung disease without planned rectal decompression is safe. J Pediatr Surg. 2023;58(2):231–235. doi: 10.1016/j.jpedsurg.2022.10.027
- Thompson DS, Davidson JR, Ford KE, et al. Transitional care in patients with hirschsprung disease: those left behind. Dis Colon Rectum. 2024;67(7):977–984. doi: 10.1097/DCR.0000000000003208 EDN: FHBBEB
- Liu Z, Zhang Y, Sun D, et al. Bowel perforation in neonates with Hirschsprung disease: a case series and literature review. Pediatr Surg Int. 2024;41(1):15. doi: 10.1007/s00383-024-05907-6 EDN: TABWTO
- Stamp LA, Lei E, Liew JJ, et al. Surgical method to prevent early death of neonatal rat pups with Hirschsprung disease, thus permitting development of long-term therapeutic approaches. Biol Methods Protoc. 2022;7(1):bpac004. doi: 10.1093/biomethods/bpac004 EDN: QFLCYD
- Srinivas S, Ahmad H, Knaus ME, et al. Laparoscopic-assisted colonic derotation in patients with Hirschsprung disease. J Pediatr Surg. 2024;59(10):161600. doi: 10.1016/j.jpedsurg.2024.06.009
- Zornoza M, Muñoz L, Ruiz A, De la Torre L. Surgical maneuvers for long-segment Hirschsprung pull-through in unique patients. Pediatr Surg Int. 2024;40(1):180. doi: 10.1007/s00383-024-05767-0
- Lin Y, Wu D, Shen Y, et al. Comparison of outcomes between two surgical techniques for patients with intestinal neuronal dysplasia. J Immunol Res. 2022;2022:9165651. doi: 10.1155/2022/9165651 Retraction in: J Immunol Res. 2023;2023:9891463. doi: 10.1155/2023/9891463
- Alsulimani S, Haoues N, Aljuhani AM, et al. Sigmoid volvulus as a clinical manifestation of acquired colonic Hypoganglionosis: a case report. Cureus. 2023;15(1):e33950. doi: 10.7759/cureus.33950
- Thobani H, Tahan D, Shah AA, et al. Age and weight stratified outcomes of single stage endorectal pull-through procedures for Hirschsprung’s disease in children: analysis of NSQIP-P data. J Pediatr Surg. 2025;60(6):162168. doi: 10.1016/j.jpedsurg.2025.162168
- Hegde SG, Raju RS, Shubha AM, et al. Total colonic aganglionosis: management and long-term outcomes at a referral centre. Pediatr Surg Int. 2024;41(1):2. doi: 10.1007/s00383-024-05882-y
- Bhandarkar K, De Coppi P, Cross K, et al. Long-term functional outcomes and multidisciplinary management after ileorectal duhamel pull-through for total colonic aganglionosis-20-year experience in a tertiary surgical center. Eur J Pediatr Surg. 2024;34(5):423–429. doi: 10.1055/a-2181-2065
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