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Descriptor in English: Hydranencephaly
Descriptor in Spanish: Hidranencefalia
Descriptor in Portuguese: Hidranencefalia
Descriptor in French: Hydranencéphalie
Entry term(s): Absence of Cerebral Hemispheres, Congenital
Cerebral Hemispheres, Absence, Congenital
Congenital Absence of Cerebral Hemispheres
Hemihydranencephalies
Hemihydranencephaly
Hydranencephalies
Hydranencephaly with Proliferative Vasculopathy
Tree number(s): C10.500.450
C16.131.666.450
Scope note: 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)
Allowable Qualifiers: BL blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
History Note: 91(64); was see under ANENCEPHALY 1979-90; was see under ANENCEPHALUS 1967-78
DeCS UI: 6984
Descriptor UI: D006832
Date Established: 1991/01/01
Date of Entry: 1999/01/01
Revision Date: 2005/07/30
Hydranencephaly - Preferred
Concept UI M0010681
Scope note 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)
Preferred term Hydranencephaly
Entry term(s) Absence of Cerebral Hemispheres, Congenital
Cerebral Hemispheres, Absence, Congenital
Congenital Absence of Cerebral Hemispheres
Hydranencephalies
Hemihydranencephaly - Narrower
Concept UI M0336863
Preferred term Hemihydranencephaly
Entry term(s) Hemihydranencephalies
Hydranencephaly with Proliferative Vasculopathy - Narrower
Concept UI M0336864
Preferred term Hydranencephaly with Proliferative Vasculopathy



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