Affiliation:
1. L.A. Vorohobov City Clinical Hospital No. 67
2. Lomonosov Moscow State University
Abstract
Hydranencephaly is a rare abnormality of the central nervous system (CNS) of the fetus, in which the cerebral hemispheres are absent and replaced with cerebrospinal fluid, while the structures of the posterior fossa, thalami and falx cerebri remains normal. The ischemic, hemorrhagic complications, infection of the fetus, the toxic effects of carbon monoxide, butane and genetic abnormalities are potential causes of a hydranencephaly development. The article presents the modern data on the possible causes of the hydranencephaly development, and clinical case of prenatal ultrasound diagnosis of hydranencephaly at 28–29 weeks of gestation, highlighting a specific ultrasound signs of this pathology. The management strategy and genetic testing recommendations are described. The difficulties of differential diagnosis and the key signs of similar disorders are discussed, as well as recommendation for future pregnancy planning.
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