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2007, Volume 23, Number 2, Page(s) 121-124     
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Megacystis-microcolon-intestinal hypoperistalsis syndrome
Özgür EKİNCİ1, Hasan Aktuğ ŞİMŞEK2, Nuri YİĞİT2, Aptullah HAHOLU2, Hüseyin BALOĞLU2
1Gazi Üniversitesi Tıp Fakültesi Patoloji Anabilim Dalı, ANKARA
2Gülhane Askeri Tıp Akademisi Haydarpaşa Eğitim Hastanesi Patoloji Servisi, ANKARA
Keywords: Megacystis, malrotation, syndrome, autopsy

Megacystis-microcolon-intestinal hypoperistalsis syndrome is a rare, congenital, autosomal recessive syndrome characterized by excessive dilatation of the urinary bladder without true outlet obstruction, hypoperistaltism and malrotation of intestines, and a very poor prognosis. It was proposed that neuropathic and myopathic disorders underlie the functional obstruction and dilatation of the bladder while the syndrome exhibits heterogenous histopathological features. Our case was a male fetus diagnosed by autopsy after intrauterine death, with typical findings of the syndrome. A discussion of the gross features in autopsy and histopathological-pathophysiological correlation is presented.

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