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.
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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.
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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


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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


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