|Year : 2020 | Volume
| Issue : 1 | Page : 163-164
Hemihydrencephaly in adult
Suhail Rafiq, Musaib Ahmad Dar
Department of Radiodiagnosis, GMC, Srinagar, Jammu and Kashmir, India
|Date of Submission||14-Apr-2019|
|Date of Decision||21-Apr-2019|
|Date of Acceptance||29-Apr-2019|
|Date of Web Publication||20-Jun-2020|
Dr. Musaib Ahmad Dar
Department of Radiodiagnosis, GMC, Srinagar, Jammu and Kashmir
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Rafiq S, Dar MA. Hemihydrencephaly in adult. Arch Med Health Sci 2020;8:163-4
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. The affected hemisphere is transformed into cerebrospinal fluid (CSF) containing membranous sac containing minimal glioneuronal tissue along with some blood vessels. It occurs due to vascular occlusion of unilateral internal cerebral artery  prior to third trimester of pregnancy occurring after neural migration and before synaptogenesis. 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: Axial non contrast computerized tomography revealing replacement of left cerebral hemisphere by cerebrospinal fluid attenuation fluid with preservation of minimal cortical mantle in occipital region. There is evidence of slight displacement of falx towards right side with dilated right lateral ventricle|
Click here to view
|Figure 2: Axial non contrast computerized tomography at level of basal ganglia revealing involvement of basal ganglia along with left cerebral hemisphere in a case of hemihydrancephaly|
Click here to view
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Dähnert W. Brain Disorders: Radiology Review Manual. New York: Lippincott Williams & Wilkins; 2003. p. 298-99.
Mori K: Anomalies of the central nervous system. Neuroradiology and neurosurgery. New York: Thieme-Stratton; 1985. p. 39-42.
Myers RE. Cerebral ischemia in the developing primate fetus. Biomed Biochim Acta 1989;48:S137-42.
Eyre JA, Miller S, Clowry GJ, Conway EA, Watts C. Functional corticospinal projections are established prenatally in the human foetus permitting involvement in the development of spinal motor centres. Brain 2000;123(Pt 1):51-64.
[Figure 1], [Figure 2]