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Expanding Ventricular Diverticulum Overlying the Cerebral Hemisphere through an Open-Lip Schizencephalic Cleft: A
Nobuya Murakami1, Ai Kurogi1, Tadahisa Shono2
1Department of Neurosurgery, Fukuoka Children's Hospital, Fukuoka, Fukuoka, Japan.
Insights
Open-lip schizencephaly can cause expanding cerebrospinal fluid (CSF) cavities leading to intracranial hypertension. Shunting these cavities proved effective in managing this rare condition.
Area of Science:
- Neuroscience
- Pediatric Neurology
- Neurosurgery
Background:
- Open-lip schizencephaly involves trans-cerebral clefts filled with cerebrospinal fluid (CSF), potentially causing hydrocephalus and intracranial hypertension.
- Detailed neuroimaging and surgical outcomes for this condition are infrequently reported.
Observation:
- Two cases of open-lip schizencephaly presented with expanding CSF-filled cavities causing intracranial hypertension.
- Advanced MRI revealed thin membranes bordering the cavity, connected to the lateral ventricle but not the subarachnoid space.
Findings:
- Neuroendoscopy confirmed the cavity's connection to the lateral ventricle.
- Endoscopic fenestration was ineffective, but shunting the CSF-filled cavity or lateral ventricle reduced cavity size.
Implications:
- The expanding cavity may represent a ventricular diverticulum through the schizencephalic cleft.
- Shunting is an effective treatment for such cavities in open-lip schizencephaly.
- Detailed MRI is crucial for evaluating associated CSF retention disorders.
Introduction:
Open-lip-type schizencephaly is characterized by trans-cerebral clefts filled with cerebrospinal fluid (CSF) between the subarachnoid space at the hemisphere surface and the lateral ventricles. Disorders related to CSF retention, including hydrocephalus and arachnoid cysts, have reportedly been associated with open-lip schizencephaly and have induced intracranial hypertension in some cases. However, detailed neuroimaging and surgical treatment findings have rarely been described.
Case Presentation:
We report 2 cases of open-lip schizencephaly with an expanding CSF-filled cavity overlying the ipsilateral cerebral hemisphere that manifested as signs of intracranial hypertension. Detailed three-dimensional heavily T2-weighted imaging revealed thin borders between the CSF-filled cavity and the subarachnoid space, but no separating structures between the cavity and the lateral ventricle, suggesting that the cavity was directly connected to the lateral ventricle through the schizencephalic cleft but not to the subarachnoid space. Neuroendoscopic observation in case 1 confirmed this finding. Endoscopic fenestration of the cavity to the prepontine cistern was ineffective in case 1. Shunting between the lateral ventricle (case 1) or CSF-filled cavity (case 2) and the peritoneal cavity slightly decreased the size of the CSF-filled cavity.
Discussion:
We speculate that the thin borders along the margin of the CSF-filled cavity are membranes that previously covered the schizencephalic cleft and are now pushed peripherally. In addition, we believe that the cavity is a ventricular diverticulum protruding through the cleft and that shunting operation is effective against such expanding cavity. Detailed magnetic resonance imaging can be useful for evaluating patients with schizencephaly associated with CSF retention disorders.
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