Pathology
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Fetopathology and developmental pathology of the embryo and fetus
Marta Ježová, Josef Feit et al.
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Hydranencephaly
Macroscopic appearance

The major part of fetal cerebrum supplied by internal carotis arteries are destroyed. Hemispheres are reduced to a thin membranous sac filled with abundant cerebrospinal fluid (bubble brain). There is variable preservation of inferior frontal, temporal, occipital lobes (depends on the collateral vascular supply) basal ganglia and diencephalon. The brain stem and cerebellum in the posterior fossa are always intact. Falx cerebri is absent or hypoplastic.

Clinical signs
  • normal head circumference at birth, normal face
  • seizures, deafness, blindness, hyperreflexia, poor body temperature control, respiratory problems, spastic quadruparesis
  • the condition is uniformly fatal, affected infants die in the first year of life
  • differential diagnosis: extreme hydrocephalus, holoprosencephaly and porencephaly

Hydranencephaly (73307)

Pictures

Hydranencephaly (basket brain), plod; mistaken for holoprosencephaly of lobar type at prenatal examination: Hydranencephaly, Macro, autopsy (73306) Hydranencephaly, Macro, autopsy (73307) Hydranencephaly, Macro, autopsy (73308)