A case report of a pediatric patient with Hirschsprung’s disease
DOI:
https://doi.org/10.18203/2349-3291.ijcp20230741Keywords:
Congenital megacolon, Chronic constipation, Rectosigmoid, ColostomyAbstract
The objective is to describe an uncommon case of Hirschsprung's disease (HD), also called congenital megacolon. The lack of neuronal ganglion cells from the submucosal and muscular nerve plexuses throughout a segment of varied length characterizes HD. Approx 94% of HD cases are diagnosed below the age of 5 years. A 3 years old male patient was admitted to the male surgery ward under a pediatric surgeon with the chief complaints of chronic constipation since birth with a history of delayed passage of meconium. The patient was diagnosed with a case of HD at the age of 1 year for which he was operated on colostomy was performed with multiple biopsies from narrowed rectosigmoid, from the transitional zone, and from colostomy stoma which confirmed the decision to make colostomy at the ganglionated segment of the colon based on the clinical picture during surgery. Biopsy report of narrowed rectosigmoid segment showed the absence of ganglion cells, while colostomy stoma and dilated proximal colon showed the presence of ganglion cells. The patient underwent a surgical procedure named pull-through surgery for HD, a martin's modification of the Duhamel operation.
References
Lesser PB, El-Nahas AM, Lukl P, Andrews P, Schuler JG, Filtzer HS. Adult-onset Hirschsprung's disease. JAMA. 1979;242(8):747-8.
Ramos JA, Stallings G. Hirschsprung disease of the adult mimicked by chronic constipation or vice versa? J Med Cases. 2012;3:277-9.
Gustafson E, Larsson T, Danielson J. Controlled outcome of Hirschsprung’s disease beyond adolescence: a single center experience. Pediatr Surg Int. 2019;35:181-5.
Faucheron JL, Poncet D, Voirin D, Moreno W, Stathopoulos L. Maladie de Hirschsprung chez l’adulte. EMC. Techniques chirurgicales appareil digestif. Paris: Elsevier Masson SAS. 2009:40-602.
Jarry J, Faucheron JL. Laparoscopic rectosigmoid resection with transanal colonic pull-through and delayed coloanal anastomosis: a new approach to adult Hirschsprung disease. Dis Colon Rectum. 2011;54(10):1313-9.
Doodnath R, Puri P. A systematic review and meta-analysis of Hirschsprung’s disease presenting after childhood. Pediatr Surg Int. 2010;26:1107-10.
Qiu JF, Shi YJ, Hu L, Fang L, Wang HF, Zhang MC. Adult Hirschsprung’s disease: report of four cases. Int J Clin Experimental Pathol. 2013;6(8):1624.
Miyamoto M, Egami K, Maeda S, Ohkawa K, Tanaka N, Uchida E et al. Hirschsprung's disease in adults: report of a case and review of the literature. J Nippon Med School. 2005;72(2):113-20.
Kleinhaus S, Boley SJ, Sheran M, Sieber WK. Hirschsprung's disease a survey of the members of the surgical section of the American academy of pediatrics. J Pediatr Surg. 1979;14(5):588-97.
De Lorijn F, Boeckxstaens GE, Benninga MA. Symptomatology, pathophysiology, diagnostic work-up, and treatment of Hirschsprung disease in infancy and childhood. Curr Gastroenterol Rep. 2007;9(3):245-53.
Gfroerer S, Rolle U. Pediatric intestinal motility disorders. World J Gastroenterol. 2015;21(33):9683.
de Lorijn F, Kremer LC, Reitsma JB, Benninga MA. Diagnostic tests in Hirschsprung disease: a systematic review. J Pediatr Gastroenterol Nutrit. 2006;42(5):496-505.
Staff MC. Hirschprung’s disease. Mayo Foundation for Medical Education and Research (MFMER). 2021.
Martins MR, Santos CH, Falcão GR. Diagnóstico tardio da doenc¸ a de Hirschsprung. J Coloproctol. 2015;35:178-81.
Kim HJ, Kim AY, Lee CW, Yu CS, Kim JS, Kim PN et al. Hirschsprung disease and hypoganglionosis in adults: radiologic findings and differentiation. Radiology. 2008;247(2):428-34.
Tjaden NE, Trainor PA. The developmental etiology and pathogenesis of Hirschsprung disease. Translational Res. 2013;162(1):1-5.