Archives of Medicine and Health Sciences

: 2020  |  Volume : 8  |  Issue : 1  |  Page : 163--164

Hemihydrencephaly in adult

Suhail Rafiq, Musaib Ahmad Dar 
 Department of Radiodiagnosis, GMC, Srinagar, Jammu and Kashmir, India

Correspondence Address:
Dr. Musaib Ahmad Dar
Department of Radiodiagnosis, GMC, Srinagar, Jammu and Kashmir

How to cite this article:
Rafiq S, Dar MA. Hemihydrencephaly in adult.Arch Med Health Sci 2020;8:163-164

How to cite this URL:
Rafiq S, Dar MA. Hemihydrencephaly in adult. Arch Med Health Sci [serial online] 2020 [cited 2022 Sep 30 ];8:163-164
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Hemihydrancephaly (HHE) is a very rare anomaly characterized by complete/near complete absence of one cerebral hemisphere. HHE is much rarer than hydranencephaly, which has been reported with a prevalence of 0.2% at autopsy.[1] The affected hemisphere is transformed into cerebrospinal fluid (CSF) containing membranous sac containing minimal glioneuronal tissue along with some blood vessels.[2] It occurs due to vascular occlusion of unilateral internal cerebral artery [3] prior to third trimester of pregnancy occurring after neural migration and before synaptogenesis.[4] It tends to favor left side more than the right.

A 27-year-old male presents to emergency with seizures. He has history of right sided hemiplegia, right lower limb hyperflexia and gaze abnormalities from childhood. He suffers from mild mental retardation. He has right homonymous hemianopia on visual field examination. He was able to walk with assistance. Language was appropriate. He was stabilized in emergency with intravenous diazepam and was referred to radiology department for computerized tomography (CT) of Brain. Routine laboratory investigations were normal. Noncontrast CT of brain revealed evidence of whole of left cerebral hemisphere replaced by fluid filled sac having attenuation similar to CSF with preservation of thin mantle of cortex in occipital region [Figure 1] and [Figure 2]. Although HHE usually spares basal ganglia but in our case basal ganglia were also affected. Falx was present and right cerebral hemisphere had normal morphology and attenuation. There was evidence of mild ventriculomegaly. Posterior fossa and brain stem were normal.{Figure 1}{Figure 2}

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