Abstract
Abstract:Hirschsprung disease (HD) is characterized by aganglionosis, which mainly occurs in the rectum and distal sigmoid colon. Typical HD is seldom diagnosed in adulthood, and segmental involvement is very rare. A 37-year-old man suffered from refractory constipation for 20 years. He could only defecate once a week and frequently needed an enema for defecation. A barium enema showed an annular stenotic segment of the rectosigmoid colon of 8 cm in length, which started 10 cm above anal circulation and showed dilated colon above the stenotic segment. The distal rectum was normal. The narrowed segment of the rectum was resected. At one-year follow up, the patient had normal defecation without laxatives.
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References
References1. Ikeda K, Goto S. Diagnosis and treatment of Hirschsprung’s disease in Japan. An analysis of 1628 patients. Ann Surg 1984;199:400–405.IkedaK]]GotoSDiagnosis and treatment of Hirschsprung’s disease in Japan. An analysis of 1628 patients.Ann Surg1984199400-4052. Fairgrieve J. Hirschsprung’s disease in the adult. Br J Surg 1963;50:506–514.FairgrieveJHirschsprung’s disease in the adult.Br J Surg196350506-5143. Ikeda K, Goto S. Total colonic aganglionosis with or without small bowel involvement: an analysis of 137 patients. J Pediatr Surg 1986;21:319–322.IkedaK]]GotoSTotal colonic aganglionosis with or without small bowel involvement: an analysis of 137 patients.J Pediatr Surg198621319-3224. Venugopal S, Mancer K, Shandling B. The validity of rectal biopsy in relation to morphology and distribution of ganglion cells. J Pediatr Surg 1981;16:433–437.VenugopalS]]MancerK]]ShandlingBThe validity of rectal biopsy in relation to morphology and distribution of ganglion cells.J Pediatr Surg198116433-4375. Tiffin MD, Chandler LR, Faber HK. Localized absence of the ganglion cells of the myenteric plexus in congenital megacolon. Am J Dis Child 1940;59:1071–1082.TiffinMD]]ChandlerLR]]FaberHKLocalized absence of the ganglion cells of the myenteric plexus in congenital megacolon.Am J Dis Child1940591071-10826. Swenson O, Rheinlander HF, Diamond I. Hirschsprung’s disease: a new concept of the etiology: operative results in 34 patients. N Engl J Med 1949;241:551–556.SwensonO]]RheinlanderHF]]DiamondIHirschsprung’s disease: a new concept of the etiology: operative results in 34 patients.N Engl J Med1949241551-5567. Sprinz H, Cohen A, Heaton LD. Hirschsprung’s disease with skip area. Ann Surg 1961;153:143–148.SprinzH]]CohenA]]HeatonLDHirschsprung’s disease with skip area.Ann Surg1961153143-1488. Kadair RG, Sims JE, Critchfield CF. Zonal colonic hypoganglionosis. JAMA 1977;238:1838–1840.KadairRG]]SimsJE]]CritchfieldCFZonal colonic hypoganglionosis.JAMA19772381838-18409. de-Chadarevian JP, Slim M, Akel S. Double zonal aganglionosis in long segment Hirschsprung’s disease with a “skip area” in transverse colon. J Pediatr Surg 1982;17:195–197.de-ChadarevianJP]]SlimM]]AkelSDouble zonal aganglionosis in long segment Hirschsprung’s disease with a “skip area” in transverse colon.J Pediatr Surg198217195-19710. McCready RA, Beart RW Jr. Adult Hirschsprung’s disease: results of surgical treatment at Mayo Clinic. Dis Colon Rectum 1980;23:401–407.McCreadyRA]]BeartRWJrAdult Hirschsprung’s disease: results of surgical treatment at Mayo Clinic.Dis Colon Rectum198023401-40711. Moriya H, Nakazaki H, Yokoyama S, et al. A case of segmental aganglionosis localized descending colon. Nippon Shokakibyo Gakkai Zasshi 1996;93:39–44.MoriyaH]]NakazakiH]]YokoyamaS&etal;A case of segmental aganglionosis localized descending colon.Nippon Shokakibyo Gakkai Zasshi19969339-4412. Barnes PR, Lennard-Jones JE, Hawley PR, et al. Hirschsprung’s disease and idiopathic megacolon in adults and adolescents. Gut 1986;27:534–541.BarnesPR]]Lennard-JonesJE]]HawleyPR&etal;Hirschsprung’s disease and idiopathic megacolon in adults and adolescents.Gut198627534-54113. Tomita R, Ikeda T, Fujisaki S, et al. Hirschsprung’s disease and its allied disorders in adults’ histological and clinical studies. HepatoGastroenterology 2003;50:1050–1053.TomitaR]]IkedaT]]FujisakiS&etal;Hirschsprung’s disease and its allied disorders in adults’ histological and clinical studies.HepatoGastroenterology2003501050-105314. Okamoto E, Ueda T. Embryogenesis of intramural ganglia of the gut and its relation to Hirschsprung’s disease. J Pediatr Surg 1967;2:437–443.OkamotoE]]UedaTEmbryogenesis of intramural ganglia of the gut and its relation to Hirschsprung’s disease.J Pediatr Surg19672437-44315. Skinner MA. Hirschsprung’s disease. Curr Probl Surg 1996;33:389–460.SkinnerMAHirschsprung’s disease.Curr Probl Surg199633389-46016. Puri P, Ohshiro K, Wester T. Hirschsprung’s disease: a search for etiology. Semin Pediatr Surg 1998;7:140–147.PuriP]]OhshiroK]]WesterTHirschsprung’s disease: a search for etiology.Semin Pediatr Surg19987140-147
