Year 2016 / Volume 108 / Number 11
Case Report
Hirschsprung disease with debut in adult age as acute intestinal obstruction: case report

742-746

DOI: 10.17235/reed.2016.3841/2015

José Antonio López Ruiz, Luis Tallón Aguilar, Laura Sánchez Moreno, José López Pérez, Felipe Pareja Ciuró, Fernando Oliva Mompeán, F. Javier Padillo Ruiz,

Abstract
Hirschsprung's disease is characterized by absence of ganglion cells in submucosal and myenteric plexus of distal bowel. Most cases become manifest during the neonatal period, but in rare instances, this disease is initially diagnosed in adult age. It usually presents as severe constipation with colonic dilatation proximal to the aganglionic segment. The treatment is surgical, removing the aganglionic segment and restoring continuity of digestive tract. The disease rarely presents as an acute intestinal obstruction. We report a case not previously diagnosed, which presented as a massive colonic dilatation with a maximum diameter of 44 cm, with imminent risk of drilling that forced to perform an emergency surgery. We include a review of existing literature.
Share Button
New comment
Comments
No comments for this article
References
1. Bakari AA, Gali BM, Ibrahim AG et al. Congenital aganglionic megacolon in Nigerian adults: Two case reports and review of the literatura. Niger J Clin Pract 2011; 14:249-52. doi: 10.4103/1119-3077.84032.
2. Chen F, Winston J, Jain S et al. Hirschsprung´s disease in a young adult: report of a case and review of the literature. Annals of Diagnostic Pathology 2006; 10:347-351.doi: 10.1016/j.anndiagpath.2006.03.017
3. Miyamoto M, Egami K, Maeda S et al. Hirschsprung´s disease in adults: report of a case and review of the literature. J Nippon Med Sch 2005; 72:113-20. http://doi.org/10.1272/jnms.72.113
4. Fu CG, Muto T, Masaki T, et al. Zonal adult Hirschsprung´s disease. Gut 1996; 39: 765-7. http://dx.doi.org/10.1136/gut.39.5.765
5. Martucciello G, Ceccherin I, Lerone M, et al. Pathogenesis of Hirschsprung´s disease. J Pediatr Surg 2000; 35:1017-25. http://dx.doi.org/10.1053/jpsu.2000.7763
6. Arshad A, Powell C, Tighe MP. Hirschsprung´s disease. BMJ 2012;345:e5521. doi: 10.1136/bmj.e5521
7. Faucheron JL, Poncet D, Voirin D, et al. Maladie de Hirschsprung chez l´adulte. En: EMC.Techniques chirurgicales appareil digestif. Paris: Elsevier Masson SAS;2009. p. 40-602.
8. Vorobyoy GI, Achkasov SI, Biryukov OM. Clinical features, diagnostics and treatment of Hirschsprung´s disease in adults. Colorectal disease 2010;12:1242-8. doi: 10.1111/j.1463-1318.2009.02031.x.
Related articles

Letter

An uncommon cause of intestinal obstruction: Vanek’s tumor

DOI: 10.17235/reed.2023.9914/2023

Letter

Intestinal obstruction due to pneumatosis intestinalis

DOI: 10.17235/reed.2023.9608/2023

Digestive Diseases Image

Submucosal infiltrate of Anisakis larvae. A rare cause of intestinal obstruction

DOI: 10.17235/reed.2023.9582/2023

Digestive Diseases Image

Iatrogenic Small Intestinal Obstruction

DOI: 10.17235/reed.2022.9225/2022

Digestive Diseases Image

Abdominal cocoon sign: an unusual cause of intestinal obstruction

DOI: 10.17235/reed.2021.8057/2021

Letter

Adenocarcinoma of the small intestine in Crohn's disease

DOI: 10.17235/reed.2021.8050/2021

Letter

Intestinal obstruction caused by Anisakis

DOI: 10.17235/reed.2020.7409/2020

Original

Megacolon in inflammatory bowel disease: response to infliximab

DOI: 10.17235/reed.2020.6394/2019

Digestive Diseases Image

An unusual enterolith ileus

Case Report

Jejunal pseudodiverticulosis. Three cases report

DOI: 10.17235/reed.2017.4403/2016

Letter to the Editor

Intestinal endometriosis. Our experience

DOI: 10.17235/reed.2016.4292/2016

Letter to the Editor

Acute colonic complications in a patient with Chagas disease

DOI: 10.17235/reed.2016.4193/2016

Citation tools
López Ruiz J, Tallón Aguilar L, Sánchez Moreno L, López Pérez J, Pareja Ciuró F, Oliva Mompeán F, et all. Hirschsprung disease with debut in adult age as acute intestinal obstruction: case report. 3841/2015


Download to a citation manager

Download the citation for this article by clicking on one of the following citation managers:

Metrics
This article has received 962 visits.
This article has been downloaded 198 times.

Statistics from Dimensions


Statistics from Plum Analytics

Publication history

Received: 10/05/2015

Accepted: 04/09/2015

Online First: 11/02/2016

Published: 02/11/2016

Article revision time: 109 days

Article Online First time: 277 days

Article editing time: 542 days


Share
This article has been rated by 1 readers.
Reader rating:
Valora este artículo:




Asociación Española de Ecografía Digestiva Sociedad Española de Endoscopia Digestiva Sociedad Española de Patología Digestiva
The Spanish Journal of Gastroenterology is the official organ of the Sociedad Española de Patología Digestiva, the Sociedad Española de Endoscopia Digestiva and the Asociación Española de Ecografía Digestiva
Cookie policy Privacy Policy Legal Notice © Copyright 2023 y Creative Commons. The Spanish Journal of Gastroenterology