Isolated adult hypoganglionosis resulting in toxic megacolon: A case report

zole hipoganglionozis, intestinal inervasyon defektlerinin nadir sebeplerinden biridir. Lamina propriada düșük asetilkolinesteraz aktivitesi ve myenterik ganglion sayısında azalma ile karakterizedir. zole hipoganglionozis, ciddi kabızlık ve pseudo-obstrüksiyon kliniği ile klasik Hirschsprung hastalığına benzer. Biz bu yazıda toksik megakolon ile bașvuran izole hipoganglionozisi olan bir yetișkin

Toksik megakolona yol açan izole yetișkin hipoganglionozis: Bir olgu sunumu

Isolated hypoganglionosis is a rare form of intestinal innervation defects. It is characterized by reduced number of myenteric ganglia and low acetyl cholinesterase activity in the lamina propria. Clinically, it resembles classical Hirschsprung’s disease with se- vere constipation or even pseudo-obstruction. Here in, we re- port a case of isolated hypoganglionosis presenting with toxic megacolon.

Kaynakça

Montedonico S, Caceres P, Munoz N, et al. Histochemical staining for intestinal dysganglionosis: over 30 years experience with more than 1, 500 biopsies. Pediatr Surg Int 2011;27:479–86.

Qadir I, Salick MM, Barakzai A, et al. Isolated adult hypoganglionosis presenting as sigmoid volvulus: a case report. J Med Case Rep 2011;5:445.

Dingemann J, Puri P. Isolated hypoganglionosis: systematic review of a rare intestinal innervation defect. Pediatr Surg Int 2010;26:1111–5.

Puri P, Gosemann JH. Variants of Hirschsprung’s disease. Semin Pediatr Surg 2012;21:310–8.

Zhang HY, Feng JX, Huang L, et al. Diagnosis and surgical treatment of isolated hypoganglionosis. World J Pediatr 2008;4:295–300.

Do MY, Myung SJ, Park HJ, et al. Novel classification and pathogenetic analysis of hypoganglionosis and adult-onset Hirschsprung’s disease. Dig Dis Sci 2011;56:1818–27.

Ito T, Kimura T, Yagami T, et al. Megacolon in an adult case of hypoganglionosis, a pseudo-hirschsprung’s disease: an autopsy study. Intern Med 2008;47:421–5.

Kim HJ, Kim AY, Lee CW, et al. Hirschsprung disease and hypoganglionosis in adults: radiologic findings and differentiation. Radiology 2008;247:428–34.

Li NP, Wu RL, Zhou S. Investigation on the pathological morphology of intestinal neuronal dysplasia. Chin J Pediatr Surg 2000;21:221–2.

Zhu XM, Gu ZC, Wang XD. The morphometric study of myenteric plexus of colon in children with hypoganglionosis. Chin J Pediatr Surg 2005;26:536–9.

Meier-Ruge WA, Brunner LA, Engert J, et al. A correlative morphometric and clinical investigation of hypoganglionosis of the colon in children. Eur J Pediatr Surg 1999;9:67–74.

Taguchi T, Masumoto K, Ieiri S, et al. New classification of hypoganglionosis: congenital and acquired hypoganglionosis. J Pediatr Surg 2006;41:2046–51.

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