Children's medicine of the North-West

Colon perforation in child with Hirschsprung-associated enterocolitis in the long term after surgical correction of Hirschsprung disease

  • Akmaljon M. Akhmadjonov Republican Specialized Scientific and Practical Medical Center of Pediatrics, 3 Talant ave., Tashkent 100179, Republic of Uzbekistan
  • Avazjon A. Dekhkonboev Republican Specialized Scientific and Practical Medical Center of Pediatrics, 3 Talant ave., Tashkent 100179, Republic of Uzbekistan
  • Victoria V. Kholostova Children’s City Clinical Hospital named after N.F. Filatov of the Moscow Department of Health, 15 Sadovaya Kudrinskaya str., Moscow 123001, Russian Federation
  • Alexey N. Smirnov Children’s City Clinical Hospital named after N.F. Filatov of the Moscow Department of Health, 15 Sadovaya Kudrinskaya str., Moscow 123001, Russian Federation
  • Artur G. Mannanov Russian National Research Medical University named after N.I. Pirogov, 1 Ostrovityanov str., Moscow 117513, Russian Federation
  • Namir A. Al-Mashat Children’s City Clinical Hospital named after N.F. Filatov of the Moscow Department of Health, 15 Sadovaya Kudrinskaya str., Moscow 123001, Russian Federation
  • Pavel M. Yarustovskiy Children’s City Clinical Hospital named after N.F. Filatov of the Moscow Department of Health, 15 Sadovaya Kudrinskaya str., Moscow 123001, Russian Federation
  • Valentin V. Sytkov Russian University of Medicine, 4 Dolgorukovskaya str., Moscow 127006, Russian Federation
  • Dmitry A. Severinov Kursk State Medical University, 3 K. Marks str., Kursk 305041, Russian Federation
  • Anatoly I. Khavkin Belgorod State National Research University, 85 Pobedy str., Belgorod 308015, Russian Federation
Keywords:
болезнь Гиршпрунга перитонит перфорация дети лапароскопия энтеростома энтероколит Hirschsprung’s disease peritonitis perforation children laparoscopy enterostoma enterocolitis

Abstract

Hirschsprung-associated enterocolitis (HAEC) is a relatively rare complication of Hirschsprung’s disease (HD). Most fatalities in patients with HD are associated with acute HAEC. However, colonic perforation as a manifestation of severe HAEC is extremely rare. Cases of colonic perforation in neonates with decompensated HD (before receiving primary radical treatment) have been described in the literature. Therefore, the specific causes of HAEC development after surgery remain poorly understood. This article describes a clinical case of colonic perforation associated with HAEC in the postoperative period in a child with a low-lying transition zone. The child's medical history, surgical treatment options, and the postoperative course are also described.

References

Глушкова В.А., Подкаменев А.В., Габрусская Т.В. Болезнь Крона у пациентов с болезнью Гиршпрунга и Гиршпрунг-ассоциированным энтероколитом. Обзор литературы. Российский вестник детской хирургии, анестезиологии и реаниматологии. 2024;14(1):97–106. DOI: 10.17816/psaic1780.

Wolfson S., Whitfield Van Buren K. Very early onset of inflammatory bowel disease in a patient with long-seg­ment Hirschsprung’s disease. ACG Case Rep J. 2020;3(7):e00353. DOI: 10.14309/crj.0000000000000353.

Ахмаджонов А.М., Дехконбоев А.А., Алиев М.М. и др. Особенности течения болезни Гиршпрун­га у пациентов с синдромом Дауна и другими генетическими аномалиями. Children’s Medi­cine of the North-West. 2024;3(12):163–172. DOI: 10.56871/CmN-W.2024.27.68.020.

Zhu T., Zhang G., Meng X. et al. Enterocolitis is a risk factor for bowel perforation in Neonates with Hirschsprung’s disease: a retrospective multicenter study. Front Pediatr. 2022;7(10):807607. DOI: 10.3389/fped.2022.807607.

Степанова Н.М., Новожилов В.А., Звонков Д.А. и др. Успешное лечение тотального аганглиоза толстой кишки у ребенка. Детская хирургия. 2021;6(25):411–417. DOI: 10.55308/1560-9510-20 21-25-6-411-417.

Roorda D., Oosterlaan J., van Heurn E., Derikx J.P.M. Risk factors for enterocolitis in patients with Hirschsprung disease: A retrospective observational study. J Pediatr Surg. 2021; 56(10):1791–1798. DOI: 10.1016/j.jpedsurg.2021.04.020.

Li S., Zhang Y., Li K. et al. Update on the pathogenesis of the Hirschsprung-associated enterocolitis. Int J Mol Sci. 2023;24(5):4602. DOI: 10.3390/ijms24054602.

Elhalaby E.A., Coran A.G., Blane C.E. et al. Enterocolitis associated with Hirschsprung’s disease: a clinical-radiological characterization based on 168 patients. J Pediatr Surg. 1995;30(1):76–83. DOI: 10.1016/0022-3468(95)90615-0.

Beltman L., Labib H., Oosterlaan J. et al. Risk factors for complications in patients with Hirschsprung disease while awaiting surgery: Beware of bowel perforation. J Pediatr Surg. 2022;57(11):561–568. DOI: 10.1016/j.jpedsurg.2022.02.022.

Halleran D.R., Ahmad H., Maloof E. et al. Does Hir­schsprung-associated enterocolitis differ in children with and without Down syndrome? J Surg Res. 2020;245:564–568. DOI: 10.1016/j.jss.2019.06.086.

Le-Nguyen A., Righini-Grunder F., Piché N. et al. Factors influencing the incidence of Hirschsprung-associated enterocolitis (HAEC). J Pediatr Surg. 2019;54(5):959–963. DOI: 10.1016/j.jpedsurg.2019.01.026.

Кесаева Т.В., Караваева С.А., Котин А.Н., Каган А.В. Особенности течения послеоперационного периода у детей с болезнью Гиршпрунга после эндоректальных вмешательств. Детская хирургия. 2022;26(1):5–9. DOI: 10.55308/1560-9510-2022-26-1-5-9.

Lewit R.A., Kuruvilla K.P., Fu M., Gosain A. Current understanding of Hirschsprung-associated enterocolitis: pathogenesis, diagnosis and treatment. Semin Pediatr Surg. 2022;31(2):151162. DOI: 10.1016/j.sempedsurg.2022.151162.

Wall N., Kastenberg Z., Zobell S. et al. Use of an enterocolitis triage and treatment protocol in children with Hirschsprung disease reduces hospital admissions. J Pediatr Surg. 2020;55(11):2371–2374. DOI: 10.1016/j.jpedsurg.2020.05.004.

Keck S., Galati-Fournier V., Kym U. et al. Lack of mucosal cholinergic innervation is associated with increased risk of enterocolitis in Hirschsprung’s disease. Cell Mol Gastroenterol Hepatol. 2021;12(2):507–545. DOI: 10.1016/j.jcmgh.2021.03.004.

How to Cite
Colon perforation in child with Hirschsprung-associated enterocolitis in the long term after surgical correction of Hirschsprung disease. (2025). Children’s Medicine of the North-West, 13(3), 200-211. https://doi.org/10.56871/CmN-W.2025.19.87.019
Issue
Vol. 13 No. 3 (2025)
Section
Practical notes
Published
2025-11-22

How to Cite

Colon perforation in child with Hirschsprung-associated enterocolitis in the long term after surgical correction of Hirschsprung disease. (2025). Children’s Medicine of the North-West, 13(3), 200-211. https://doi.org/10.56871/CmN-W.2025.19.87.019