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Experimental and Clinical Gastroenterology

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Permanent stoma in the outcome of treatment of Hirschsprung's disease in children - analysis of results and literature review

https://doi.org/10.31146/1682-8658-ecg-234-2-93-100

Abstract

Introduction. Permanent stoma (PS) is one of the most unfavorable outcomes of Hirschsprung's disease (HD) treatment, permanently depriving the patient of the opportunity to realize the physiological needs for defecation. The article analyzes own and literature data on the causes of PS. The purpose of the study. To analyze the results of treatment of patients with HD who underwent a treatment method in the form of a permanent intestinal stoma. Materials and methods. The study included 367 patients with HD who received treatment during the period from 2016 to 2024 was carried out. Most of the children underwent radical surgery in different clinics using the Soave-Georgeson method. Among them, PS occurred in 8 cases. The reason for the permanent stoma was long-term recurrent complications: abscesses (n=7), fistulas of the perineum (n=5), fecal incontinence (n=6) and rectal stenosis (n=2). 3 patients had syndromic forms of HD, total agangliosis (TA) was diagnosed in 5 cases. Results. The analysis confirmed that permanent stoma formation in Hirschsprung’s disease (HD) is a necessary measure driven by severe complications such as anastomotic failure, recurrent abscesses, fistulas, and fecal incontinence. Key risk factors for permanent stoma (PS) include total aganglionosis (TA) and delayed specialized surgical intervention. The statistically significant association between TA and PS (p=0.002) and between delayed treatment and stoma risk (p=0.023) underscores the importance of early diagnosis and timely radical surgery. Patients with syndromic forms of HD require particular attention due to their increased risk of adverse outcomes. Conclusion. The study highlights the need for individualized surgical approaches, improved anastomotic techniques, and dynamic postoperative monitoring to minimize PS risk. Reducing the frequency of stoma formation may be achievable through optimized timing of surgical interventions, especially in total forms of HD, and proactive prevention of postoperative complications.

About the Authors

V. V. Kholostova
Children’s City Clinical Hospital named after N.F. Filatov, Moscow Health Department; N.I. Pirogov Russian National Research Medical University
Russian Federation


A. N. Smirnov
Children’s City Clinical Hospital named after N.F. Filatov, Moscow Health Department; N.I. Pirogov Russian National Research Medical University
Russian Federation


A. M. Akhmadjonov
Republican specialized scientific practical medical center of paediatrics
Russian Federation


A. A. Dekhkonboev
Republican specialized scientific practical medical center of paediatrics
Russian Federation


A. G. Mannanov
Children’s City Clinical Hospital named after N.F. Filatov, Moscow Health Department
Russian Federation


N. A. Al-Mashat
Children’s City Clinical Hospital named after N.F. Filatov, Moscow Health Department; N.I. Pirogov Russian National Research Medical University
Russian Federation


P. M. Yarustovskiy
Children’s City Clinical Hospital named after N.F. Filatov, Moscow Health Department; N.I. Pirogov Russian National Research Medical University
Russian Federation


R. R. Baiazitov
The National Medical Research Center of Children’s Health
Russian Federation


V. V. Sytkov
Russian University of Medicine
Russian Federation


A. I. Khavkin
Research Clinical Institute of Childhood, Ministry of Health of the Moscow Region; Belgorod State Research University. Ministry of Science and Higher Education of the Russian Federation
Russian Federation


References

1. Ghose S.I., Squire B.R., Stringer M.D., Batcup G., Crabbe D.C. Hirschsprung’s disease: problems with transition-zone pull-through. J Pediatr Surg. 2000 Dec;35(12):1805-9. doi: 10.1053/jpsu.2000.19263.

2. Menezes M., Corbally M., Puri P. Long-term results of bowel function after treatment for Hirschsprung’s disease: a 29-year review. Pediatr Surg Int. 2006 Dec;22(12):987-90. doi: 10.1007/s00383-006-1783-8.

3. Catto-Smith A.G., Trajanovska M., Taylor R.G. Long-term continence after surgery for Hirschsprung’s disease. J Gastroenterol Hepatol. 2007 Dec;22(12):2273-82. doi: 10.1111/j.1440-1746.2006.04750.x.

4. Neuvonen M.I., Kyrklund K., Lindahl H.G., Koivusalo A.I., Rintala R.J., Pakarinen M.P. A population-based, complete follow-up of 146 consecutive patients after transanal mucosectomy for Hirschsprung disease. J Pediatr Surg. 2015 Oct;50(10):1653-8. doi: 10.1016/j.jpedsurg.2015.02.006.

5. Taghavi K., Goddard L., Evans S.M. et al. Contemporary management of Hirschsprung disease in New Zealand. ANZ J Surg. 2020 Jun;90(6):1037-1040. doi: 10.1111/ans.15923.

6. Laughlin D.M., Friedmacher F., Puri P. Total colonic aganglionosis: a systematic review and meta-analysis of long-term clinical outcome. Pediatr Surg Int. 2012 Aug;28(8):773-9. doi: 10.1007/s00383-012-3117-3.

7. Tsuji H., Spitz L., Kiely E.M., Drake D.P., Pierro A. Management and long-term follow-up of infants with total colonic aganglionosis. J Pediatr Surg. 1999 Jan;34(1):158-61; discussion 162. doi: 10.1016/s0022-3468(99)90248-8.

8. Bischoff A., Levitt M.A., Peña A. Total colonic aganglionosis: a surgical challenge. How to avoid complications? Pediatr Surg Int. 2011 Oct;27(10):1047-52. doi: 10.1007/s00383-011-2960-y.

9. Stenström P., Brautigam M., Borg H., Graneli C., Lilja H.E., Wester T. Patient-reported Swedish nationwide outcomes of children and adolescents with total colonic aganglionosis. J Pediatr Surg. 2017 Aug;52(8):1302-1307. doi: 10.1016/j.jpedsurg.2016.11.033.

10. Bhandarkar K., De Coppi P., Cross K., Blackburn S., Curry J. 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 Oct;34(5):423-429. doi: 10.1055/a-2181-2065.

11. Davidson J.R., Kyrklund K., Eaton S. et al. Outcomes in Hirschsprung’s disease with coexisting learning disability. Eur J Pediatr. 2021 Dec;180(12):3499-3507. doi: 10.1007/s00431-021-04129-5.

12. Hasegawa, Radley, Fatah, Keighley. Long-term results of colorectal resection for slow transit constipation. Colorectal Dis. 1999 May;1(3):141-5. doi: 10.1046/j.1463-1318.1999.00025.x.

13. Peña A., Elicevik M., Levitt M.A. Reoperations in Hirschsprung disease. J Pediatr Surg. 2007 Jun;42(6):1008-13; discussion 1013-4. doi: 10.1016/j.jpedsurg.2007.01.035.

14. Roorda D., Verkuijl S.J., Derikx J.P.M. et al. Did Age at Surgery Influence Outcome in Patients With Hirschsprung Disease? A Nationwide Cohort Study in the Netherlands. J Pediatr Gastroenterol Nutr. 2022 Oct 1;75(4):431-437. doi: 10.1097/MPG.0000000000003550.

15. Tan Y.W., Chacon C.S., Geoghegan N., Saxena A., Clarke S., Haddad M., Choudhry M. Late Diagnosis of Hirschsprung’s Disease: Definition and Implication on Core Outcomes. Eur J Pediatr Surg. 2022 Dec;32(6):512-520. doi: 10.1055/s-0042-1744147.

16. Gustafson E., Larsson T., Danielson J. Controlled outcome of Hirschsprung’s disease beyond adolescence: a single center experience. Pediatr Surg Int. 2019 Feb;35(2):181-185. doi: 10.1007/s00383-018-4391-5.


Review

For citations:


Kholostova V.V., Smirnov A.N., Akhmadjonov A.M., Dekhkonboev A.A., Mannanov A.G., Al-Mashat N.A., Yarustovskiy P.M., Baiazitov R.R., Sytkov V.V., Khavkin A.I. Permanent stoma in the outcome of treatment of Hirschsprung's disease in children - analysis of results and literature review. Experimental and Clinical Gastroenterology. 2025;(2):93-100. (In Russ.) https://doi.org/10.31146/1682-8658-ecg-234-2-93-100

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