Clinical manifestations of Hirschsprung’s disease in children aged 1 to 3 years
DOI:
https://doi.org/10.15574/SP.2024.137.68Keywords:
Hirschsprung’s disease, children, enterocolitis, anemia, hypotrophy, associated malformationsAbstract
Clinical manifestations of Hirschsprung's disease (HD) in children aged 1 to 3 years depend on many factors, namely the extent of colonic aganglionosis (CA), the age of patients, the presence of associated congenital malformations and severe complications.
Purpose - to study the features of the clinical manifestations of HD in children aged 1 to 3 years for the early detection and verification of this pathology.
Materials and methods. We conducted a retrospective analysis of the clinical manifestations of HD in 268 children aged 1 to 3 years: 74 (27.62%) children with the rectal form of HD, 187 (69.77%) with rectosigmoid form and 7 (2.61%) with subtotal form. Associated malformations were found in 50 (18.66%). To study the clinical course, we considered the anamnesis, physical examination, data of general clinical laboratory indicators of blood, urine and feces, electrocardiography, neurosonography, echocardiography, ultrasound and X-ray dianostics.
Results. Hypotrophy was found in 54 (20.15%) children: the first degree in 9 (3.36%) children, the second degree in 34 (12.69%) and the third degree in 11 (4.1%) patients. Laboratory tests revealed mild anemia in 4 (1.49%), moderate anemia in 11 (4.1%), and severe anemia in 31 (11.57%) children. The HD course was complicated by enterocolitis in 25 (33.78%) of 74 patients with rectal, in 86 (46.52%) of 187 children with rectosigmoidal and in 7 (100%) of 7 children with subtotal CA. Toxic megacolon was diagnosed in 6 (3.21%) patients.
Conclusions. Associated malformations and late diagnosis are the main causes of severe complications of HD in children aged 1 to 3 years: enterocolitis (44.03%), toxic megacolon (4.85%), hypotrophy (20.15%) and anemia (17.16%).
The research was carried out in accordance with the principles of the Declaration of Helsinki. The research protocol was approved by the Local Ethics Committee of all institutions mentioned in the work. Informed consent of the children’s parents was obtained for the research.
No conflict of interests was declared by the authors.
References
Avansino JR, Levitt MA. (2017). Hirschsprung disease. In Fundamentals of pediatric surgery, 2, Mattei P. et al (eds.). Springer International Publishing, Cham: 513-524. https://doi.org/10.1007/978-3-319-27443-0_62
Bandré E, Kaboré RA, Ouedraogo I, Soré O, Tapsoba T, Bambara C et al. (2010). Hirschsprung's disease: Management problem in a developing country. Afr J Paediatr Surg. 7: 166-168. https://doi.org/10.4103/0189-6725.70418; PMid:20859022
Chumpitazi B, Nurko S. (2008). Pediatric Gastrointestinal Motility Disorders: Challenges and a Clinical Update. Gastroenterol Hepatol (NY). 4: 140-148.
Ekenze SO, Ngaikedi C, Obasi AA. (2011). Problems and outcome of Hirschsprung's disease presenting after 1 year of age in a developing country. World J Surg. 35: 22-26. https://doi.org/10.1007/s00268-010-0828-2; PMid:20976451
Frykman PK, Short SS. (2012). Hirschsprung-Associated Enterocolitis: Prevention and Therapy. Semin Pediatr Surg. November. 21 (4): 328-335. https://doi.org/10.1053/j.sempedsurg.2012.07.007; PMid:22985838 PMCid:PMC3462485
Garg SR, Sathe PA, Taware AC, Surve KM. (2016). Fatal Toxic Megacolon in a Child of Hirschsprung Disease. J Clin Diagn Res. 10 (12): ED03-ED05. Published online 2016 Dec 1. https://doi.org/10.7860/JCDR/2016/21075.9083; PMid:28208866 PMCid:PMC5296439
Gershon EM, Rodriguez L, Arbizu RA. (2023). Hirschsprung's disease associated enterocolitis: A comprehensive review. World J Clin Pediatr. 12 (3): 68-76. https://doi.org/10.5409/wjcp.v12.i3.68; PMid:37342453 PMCid:PMC10278080
Kapur RP, Smith C, Ambartsumyan L. (2020). Postoperative pullthrough obstruction in Hirschsprung disease: etiologies and diagnosis. Pediatr Dev Pathol. 23: 40-59. https://doi.org/10.1177/1093526619890735; PMid:31752599
Kawaguchi AL, Guner YS, Sømme S, Quesenberry AC, Arthur LG, Sola JE et al. (2021). Management and outcomes for long-segment Hirschsprung disease: A systematic review from the APSA Outcomes and Evidence Based Practice Committee. J Pediatr Surg. 56(9): 1513-1523. https://doi.org/10.1016/j.jpedsurg.2021.03.046; PMid:33993978 PMCid:PMC8552809
Kyrklund K, Sloots CEJ, de Blaauw I, Bjørnland K, Rolle U, Cavalieri D et al. (2020). ERNICA guidelines for the management of rectosigmoid Hirschsprung's disease. Orphanet J Rare Dis. 15 (1): 164. https://doi.org/10.1186/s13023-020-01362-3; PMid:32586397 PMCid:PMC7318734
Lampus H. (2023). Overview of Hirschsprung Disease: A Narrative Literature Review. Scientific Journal of Pediatrics. 1(1): 14-16. https://doi.org/10.59345/sjped.v1i1.14
Lotfollahzadeh S, Taherian M, Anand S. (2022). Hirschsprung Disease. In: StatPearls. Treasure Island (FL): StatPearls Publishing; Jan. URL: https://www.ncbi.nlm.nih.gov/books/NBK562142/.
Maxime C, Mellul K, Stirnemann J, Khen-Dunlop N, Lapillonne A, Kermorvant-Duchemin E. (2023). First-year growth trajectory and early nutritional requirements for optimal growth in infants with congenital diaphragmatic hernia: a retrospective cohort study. Arch Dis Child Fetal Neonatal Ed. 109: 2. https://doi.org/10.1136/archdischild-2023-325713; PMid:37666658
Neuvonen MI, Kyrklund K, Rintala RJ, Pakarinen MP. (2017). Bowel function and quality of life after trans anal endorectal pull-through for Hirschsprung disease: controlled outcomes up to adulthood. Ann Surg. 265 (3): 622-629. https://doi.org/10.1097/SLA.0000000000001695; PMid:28169931
Prytula V, Kurtash O. (2022). Long-term outcomes of mini-invasive methods of surgical treatment of Hirschprung's disease in children. Lekarsky obzor. 71 (3): 111-115.
Prytula VP, Kurtash OO, Hussaini SF, Rusak PS. (2023). Comparison of clinical results of Transanal Endorectal Pull-Through of the colon with and without laparoscopic assistance in children with Hirschsprung's disease Paediatric Surgery (Ukraine). 2 (79): 71-77. https://doi.org/10.15574/PS.2023.79.71
Reategui CO, Spears CA, Allred GA. (2021). Adults Hirschsprung's disease, a call for awareness. A case report and review of the literature. Int J Surg Case Rep. 79: 496-502. https://doi.org/10.1016/j.ijscr.2021.01.090; PMid:33757270 PMCid:PMC7889450
Rennie JM. (2012). Textbook of Neonatology. 5 edition. Churchill Livingstone Elsevier: 12. ISBN 978-0-7020-3479-4.
Rybalchenko VF. (2012). Toxic megacolon, dolichomegacolon in children. Paediatric Surgery. Ukraine. 4: 5-13. URL: http://nbuv.gov.ua/UJRN/Khdv_2012_4_3.
Sharma S, Gupta DK. (2012). Hirschsprung's disease presenting beyond infancy: Surgical options and postoperative outcome. Pediatr Surg Int. 28: 5-8. https://doi.org/10.1007/s00383-011-3002-5; PMid:22033769
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
Van Hasselt Tim et al. (2023). 204 Prematurity and paediatric intensive care admissions: cohort study using a novel large-scale data linkage. Archives of Disease in Childhood. 108: 2. URL: https://adc.bmj.com/content/108/Suppl_2/A141. https://doi.org/10.1136/archdischild-2023-rcpch.232
Videholm S et al. (2021). Perinatal factors and hospitalisations for severe childhood infections: a population-based cohort study in Sweden. BMJ Open. 11: e054083. https://doi.org/10.1136/bmjopen-2021-054083; PMid:34620672 PMCid:PMC8499334
Yan Z, Poroyko V, Gu S, Zhang Z, Pan L, Wang J. (2014). Characterization of the intestinal microbiome of Hirschsprung's disease with and without enterocolitis. Biochem Biophys Res Commun. 445: 269-274. https://doi.org/10.1016/j.bbrc.2014.01.104; PMid:24525127
Zhu Yao et al. (2023). The impact of early empirical antibiotics treatment on clinical outcome of very preterm infants: a nationwide multicentre study in China. Italian Journal of Pediatrics. 49: 14. https://ijponline.biomedcentral.com/articles/10.1186/s13052-023-01414-x.
Downloads
Published
Issue
Section
License
Copyright (c) 2024 Modern pediatrics. Ukraine
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
The policy of the Journal “MODERN PEDIATRICS. 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 “MODERN PEDIATRICS. 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.
