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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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