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

Michel Mittelbronn*, Rudi Beschorner, Jens Schittenhelm, David Capper, Benjamin Goeppert, Richard Meyermann, Matthias Meyer-Wittkopf, Susanne Mackensen-Haen

*Corresponding author for this work

Research output: Contribution to journalArticleResearchpeer-review

11 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)1503-1507
Number of pages5
JournalHuman Pathology
Volume37
Issue number11
DOIs
Publication statusPublished - Nov 2006
Externally publishedYes

Keywords

  • Fetofetal transfusion syndrome
  • Hydranencephaly
  • Polymicrogyria
  • Thromboembolism
  • Triplets

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