Hydranencephaly
Overview
Hydranencephaly is a rare condition in which the brain's cerebral hemispheres are absent and replaced by sacs filled with cerebrospinal fluid. (
Symptoms
* Normal head size (at birth but enlarges with age) * Normal reflexes * Seizures * Hydrocephalus * Visual impairment
Causes
Hydranencephaly is an extreme form of porencephaly, which is characterized by a cyst or cavity in the cerebral hemispheres, and may be caused by vascular insult or injuries, infections, or traumatic disorders after the 12th week of pregnancy.
Diagnosis
may be delayed for several months because the infant's early behavior appears to be relatively normal. Transillumination, an examination in which light is passed through body tissues, usually confirms the diagnosis. Preliminary diagnosis may be made in utero via standard ultrasound, and can be confirmed with a level II or higher ultrasound.
Prognosis
There is no standard treatment for hydranencephaly. Treatment is symptomatic and supportive. Hydrocephalus may be treated with a shunt. The prognosis for children with Hydranencephaly is generally quite poor. Death usually occurs in the first year of life.
Treatment
* Symptomatic and supportive * Shunt - for hydrocephalus