A congenital condition where the greater portions of the cerebral hemispheres and CORPUS STRIATUM are replaced by CSF and glial tissue. The meninges and the skull are well formed, which is consistent with earlier normal embryogenesis of the telencephalon. Bilateral occlusions of the internal carotid arteries in utero is a potential mechanism. Clinical features include intact brainstem reflexes without evidence of higher cortical activity. (Menkes, Textbook of Child Neurology, 5th ed, p307)
ID
http://purl.bioontology.org/ontology/MESH/D006832
altLabel
Hemihydranencephalies
Hydranencephaly with Proliferative Vasculopathy
Cerebral Hemispheres, Absence, Congenital
Hydranencephalies
Congenital Absence of Cerebral Hemispheres
Hemihydranencephaly
Absence of Cerebral Hemispheres, Congenital
AQL
BL CF CI CL CO DG DH DI DT EC EH EM EN EP ET GE HI IM ME MI MO NU PA PC PP PS PX RH RT SU TH UR VE VI
cui
C0431383
C0020225
C0751210
DC
1
definition
A congenital condition where the greater portions of the cerebral hemispheres and CORPUS STRIATUM are replaced by CSF and glial tissue. The meninges and the skull are well formed, which is consistent with earlier normal embryogenesis of the telencephalon. Bilateral occlusions of the internal carotid arteries in utero is a potential mechanism. Clinical features include intact brainstem reflexes without evidence of higher cortical activity. (Menkes, Textbook of Child Neurology, 5th ed, p307)
DX
19910101
HN
91(64); was see under ANENCEPHALY 1979-90; was see under ANENCEPHALUS 1967-78
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