Algorithm for Surgical Treatment of Children with Hirschsprung’s Disease
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Keywords

Hirschsprung’s disease
children
surgical treatment
colostomy

Abstract

Hirschsprung’s disease requires surgical treatment. Depending on the form of aganglionosis and severity of pathology, open or minimally invasive, one-stage or multi-stage radical surgery may be used. The objective of the research was to develop an algorithm for surgical treatment of children with Hirschsprung’s disease. Materials and methods. The analysis of surgical treatment of 1,129 children with different forms of Hirschsprung’s disease aged from birth to 18 years over the period 1980-2018 was conducted. All the patients underwent both traditional and minimally invasive radical surgery. Results and discussion. The patients were divided into three groups; each group was characterized by modernization of surgical methods, as well as intensification of diagnostics for early detection of such patients. Radical surgery without a protective colostomy was used in 773 (68.46%) cases, while in 356 (31.53%) cases, a protective colostomy was created. Due to the intensification of diagnostics that markedly contributed to early detection of patients with various forms of Hirschsprung’s disease, during each study period, there was observed a dramatic decrease in the number of stoma patients with a significant increase in the number of children diagnosed with Hirschsprung’s disease: from 68.54%  (1980-1992) to 37.93% (1993-2010) and 12.00% (2011-2018). Conclusions. Timely diagnostics contributes to early detection of children with different forms of Hirschsprung’s disease. In uncomplicated forms of Hirschsprung’s disease, it is reasonable to perform one-stage radical surgery, namely the resection of the aganglionic segment and the affected colon segment followed by colorectal anastomosis. In severe Hirschsprung’s disease, two-stage surgery should be used: creation of a protective colostomy (the first stage) and radical surgery (the second stage). In some cases, in colectomy, three-stage surgical approach is needed, namely the creation of a protective colostomy (the first stage), radical surgery (the second stage) and protective colostomy closure (the third stage).
https://doi.org/10.21802/gmj.2019.3.7
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References

Kryvchenia DYu, Prytula VP, Silchenko MI et al. Rezultaty likuvannia ditey z khvoroboiu Hirshprunha. Prohnozy ta shliakhy pokrashchennia. Paediatric Surgery. 2008;4:51-54.

Prytula VP, Silchenko MI, Hussaini SF et al. Transanal endorectal pull-through and resection of colon, as modern approach to the treatment of Hirschprung's disease in chidren. Archive of Clinical Medicine. 2014;2:93-94. [published in Ukrainian]

Burkardt DD, Graham JM Jr, 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. DOI: https://doi.org/10.1177/0009922813500846 [PMid:24002048]

Clermidi P, Podevin G, Crétolle C et al. The challenge of measuring quality of life in children with Hirschsprung's disease or anorectal malformation. J Pediatr Surg. 2013;48(10):2118-27. DOI: https://doi.org/10.1016/j.jpedsurg.2013.03.071 [PMid:24094967]

Gosemann JH, Friedmacher F, Ure B et al. Open versus transanal pull-through for Hirschsprung disease: a systematic review of long-term outcome. Eur J Pediatr Surg. 2013;23(2):94-102. DOI: https://doi.org/10.1055/s-0033-1343085 [PMid:23572464]

Han-Geurts IJ, Hendrix VC, de Blaauw I et al. Outcome after anal intrasphincteric Botox injection in children with surgically treated Hirschsprung disease. J Pediatr Gastroenterol Nutr. 2014;59(5):604-607. DOI: https://doi.org/10.1097/MPG.0000000000000483 [PMid:25000353]

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. DOI: https://doi.org/10.4103/0189-6725.160403 [PMid:26168755 PMCid:PMC4955413]

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. DOI: https://doi.org/10.1097/DCR.0b013e31824c77e4 [PMid:22595847]

Langer JC. Hirschsprung disease. Curr Opin Pediatr. 2013;25(3):368-374. DOI: https://doi.org/10.1097/MOP.0b013e328360c2a0 [PMid:23615177]

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. DOI: https://doi.org/10.1016/j.jpedsurg.2014.05.001 [PMid:25280652]

Neuvonen MI, Kyrklund K, Rintala RJ et al. Bowel function and quality of life after transanal endorectal pull-through for Hirschsprung disease: controlled outcomes up to adulthood. Ann Surg. 2017;265(3):622-629. DOI: https://doi.org/10.1097/SLA.0000000000001695 [PMid:28169931]

Prytula VP, Levytskyi AF, Silchenko MI et al. Laparoscopic-assisted transanal endorectal pull-through of colon for treatment of Hirschsprung's disease in children. Standardy Medyczne - Problemy Chirurgii Dziciecej. 2016;6(1):109.

Rajindrajith S, Devanarayana NM, Benninga MA. Review article: faecal incontinence in children: epidemiology, pathophysiology, clinical evaluation and management. Aliment Pharmacol Ther. 2013;37(1):37-48. DOI: https://doi.org/10.1111/apt.12103 [PMid:23106105]

Rintala RJ, Pakarinen MP. Long-term outcomes of Hirschsprung's disease. Semin Pediatr Surg. 2012;21(4):336-343. DOI: https://doi.org/10.1053/j.sempedsurg.2012.07.008 [PMid:22985839]

Sheng Q, Lv Zh, Xiao X. Re-operation for Hirschsprung's disease: experience in 24 patients from China. Pediatr Surg Int. 2012;28(5):501-506. DOI: https://doi.org/10.1007/s00383-012-3062-1 [PMid:22358253]

Stensrud KJ, Emblem R, Bjørnland K. Late diagnosis of Hirschsprung disease - patient characteristics and results. J Pediatr Surg. 2012;47(10):1874-1879. DOI: https://doi.org/10.1016/j.jpedsurg.2012.04.022 [PMid:23084200]

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. DOI: https://doi.org/10.1055/s-0037-1604115 [PMid:28738437]

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