Leeds Fetal Medicine Unit Website

Hydranencephaly

Definition	Complete destrruction of cerebral hemispheres (normal cerebellum and brainstem)
Incidence	1:4000
Diagnosis	Ultrasound
Gestation at diagnosis	11 weeks onwards
Known cause, chromosomal or genetic association	Intracerebral haemorrhage, infection (Herpes), autosomally recessive
Further investigations	Infection screen, Karyotype only if family history, MRI to distinguish similar brain abnormalities
Differential Diaganosis	Holoprosencepaly, Hydrocephalus
Prognosis	Very poor 85% mortality within a year, severe delay
Recurrance risk	Low (1%) if not related to familial condition
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