TY - JOUR
T1 - Multiple thromboembolic events in fetofetal transfusion syndrome in triplets contributing to the understanding of pathogenesis of hydranencephaly in combination with polymicrogyria
AU - Mittelbronn, Michel
AU - Beschorner, Rudi
AU - Schittenhelm, Jens
AU - Capper, David
AU - Goeppert, Benjamin
AU - Meyermann, Richard
AU - Meyer-Wittkopf, Matthias
AU - Mackensen-Haen, Susanne
PY - 2006/11
Y1 - 2006/11
N2 - 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.
AB - 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.
KW - Fetofetal transfusion syndrome
KW - Hydranencephaly
KW - Polymicrogyria
KW - Thromboembolism
KW - Triplets
UR - http://www.scopus.com/inward/record.url?scp=33750036725&partnerID=8YFLogxK
U2 - 10.1016/j.humpath.2006.07.005
DO - 10.1016/j.humpath.2006.07.005
M3 - Article
C2 - 16997352
AN - SCOPUS:33750036725
SN - 0046-8177
VL - 37
SP - 1503
EP - 1507
JO - Human Pathology
JF - Human Pathology
IS - 11
ER -