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hydrancephaly
An extreme form of porencephaly.
Etiology:
- may be caused by vascular infections or traumatic disorders after the 12th week of pregnancy
Epidemiology:
- rare
Pathology:
1) cerebral hemispheres are absent & replaced by sacs filled CSF
Clinical manifestations:
1) infant may appear normal at birth
a) head size may be normal
b) spontaneous reflexes may all seem normal
2) in a few weeks
a) the infant usually becomes irritable
b) muscle tone increased
3) in a few months
a) seizures
b) hydrocephalus
4) other signs/symptoms may include
- visual impairment, growth retardation, deafness, blindness, spastic quadriparesis or paralysis, intellectual deficits
5) diagnosis may be delayed for several months because early behavior appears to be relatively normal
6) seizures
7) myoclonus
8) respiratory problems
Management:
1) no definitive treatment
2) treatment is symptomatic & supportive
3) hydrocephalus require ventriculoperitoneal shunt
4) prognosis is generally poor
a) many die before age 1
b) in rare cases, children may survive for several years
General
porencephaly
References
- NINDS Hydrancephaly Information Page
https://www.ninds.nih.gov/Disorders/All-Disorders/Hydranencephaly-Information-Page