title: Multiple thromboembolic events in fetofetal transfusion syndrome in triplets contributing to the understanding of pathogenesis of hydranencephaly in combination with polymicrogyria.
abstract: 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.
authors.affiliation: Institute of Brain Research, University of Tuebingen, Tuebingen, Germany. michel-guy-andr.mittelbronn@med.uni-tuebingen.de
authors.forename: Michel
authors.initials: M
authors.lastname: Mittelbronn
authors.forename: Rudi
authors.initials: R
authors.lastname: Beschorner
authors.forename: Jens
authors.initials: J
authors.lastname: Schittenhelm
authors.forename: David
authors.initials: D
authors.lastname: Capper
authors.forename: Benjamin
authors.initials: B
authors.lastname: Goeppert
authors.forename: Richard
authors.initials: R
authors.lastname: Meyermann
authors.forename: Matthias
authors.initials: M
authors.lastname: Meyer-Wittkopf
authors.forename: Susanne
authors.initials: S
authors.lastname: Mackensen-Haen
mesh_terms: D000015:Abnormalities, Multiple / Q000196:embryology; D000028:Abortion, Induced; D002540:Cerebral Cortex / Q000002:abnormalities; D005260:Female; D005330:Fetofetal Transfusion / Q000473:pathology*; D006801:Humans; D006832:Hydranencephaly / Q000196:embryology*; D011247:Pregnancy; D013923:Thromboembolism / Q000196:embryology / Q000473:pathology*; D014310:Triplets