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Hydranencephaly



Hydranencephaly Causes


Hydranencephaly is considered to be an extreme form of porencephaly (a rare disorder marked by a cyst or cavity in the cerebral hemispheres) and may be caused by vascular infections or traumatic disorders after the 12th week of pregnancy.


Hydranencephaly Definition


Hydranencephaly is a rare condition in which the brain's cerebral hemispheres are missing and replaced by sacs filled with cerebrospinal fluid.


Hydranencephaly Diagnosis


Diagnosis may be delayed for several months due to the early behavior appearing to be relatively normal. Some infants may have additional abnormalities at birth including myoclonus (spasm or twitching of a muscle or group of muscles), seizures, and respiratory problems


Hydranencephaly Symptoms and Signs


An infant with hydranencephaly may appear normal at birth, and the infant's head size and spontaneous reflexes (such as sucking, swallowing, crying, and moving the arms and legs) may all seem normal. After a few weeks, however, the infant usually becomes irritable and has increased muscle tone. After a few months of life, seizures and hydrocephalus (excessive accumulation of cerebrospinal fluid in the brain) may arise. Other symptoms may include visual impairment, deafness, lack of growth, blindness, spastic quadriparesis (paralysis), and intellectual deficits.


Hydranencephaly Treatment


There is no known treatment for hydranencephaly. Treatment of hydranencephaly is supportive and symptomatic. Hydrocephalus may be treated with a shunt, which is a surgically implanted tube that diverts fluid from one pathway to another.


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