Multiple thromboembolic events in fetofetal transfusion syndrome in triplets contributing to the understanding of pathogenesis of hydranencephaly in combination with polymicrogyria

详细信息    查看全文

• 作者：Michel Mittelbronn ; Rudi Beschorner ; Jens Schittenhelm ; David Capper ; Benjamin Goeppert ; Richard Meyermann ; Matthias Meyer-Wittkopf ; Susanne Mackensen-Haen
• 关键词：Fetofetal transfusion syndrome ; Hydranencephaly ; Thromboembolism ; Polymicrogyria ; Triplets
• 刊名：Human Pathology
• 出版年：2006
• 出版时间：November 2006
• 年：2006
• 卷：37
• 期：11
• 页码：1503-1507
• 全文大小：2190 K

文摘

Over the last 180 years, several theories concerning the origin of hydranencephaly have been proposed with an emphasis on infectious, aplastic, and vascular etiologies. In this report, we present a case of triplets with fetofetal transfusion syndrome of which 2 fetuses (1 and 2) developed almost similar hydranencephaly, whereas the third exhibited the features of a fetus papyraceus (3). In the monochorial triamniotic placenta, multiple arteriovenous anastomoses were detected, representing a probable route for the transmission of thrombi originating from fetus 3 causing visceral lesions in fetus 2. Hydranencephaly was histologically characterized by necrosis, macrophage invasion, and endothelial proliferation. In addition, polymicrogyria was seen in fetuses 1 and 2. The combination of multiple visceral thromboembolic events and the death of fetus 3 approximately in the 11th week of gestation suggested a vascular thrombotic pathogenesis of hydranencephaly. Polymicrogyria can be considered as postmigratory laminar necrosis. Our findings contribute to the pathogenetic understanding of combined hydranencephaly and polymicrogyria.