Schizencephaly A case report

Julita, Fitratul Ilahi, M. Hidayat

Department of Ophthalmology, Faculty of Medicine
Andalas University / Dr. M. Djamil Hospital
Padang
Abstract

Introduction
Schizencephaly is an uncommon congenital cerebral malformation that involves the
cerebral mantle and consists of a cleft that extends through the entire cerebral hemisphere
from the lateral ventricle to cerebral cortex. The condition is present at birth and present
early in life. Patient with schizencephaly presenting with first onset seizure.

Objective
To report a case of a young girl with brain CT scan shown schizencephaly

Method
A 8 month old female baby consulted from pediatric neurology
sub division at Dr. M. Djamil Hospital with clinical history of
nystagmus since 4 month before. The head was enlarged since
birth with diameter 48 Cm (> 2 SD Nellhaus standar). No fever
and no seizure. This patient was shown delay in psychomotor
development. Visual aquity no light perception both eyes, and
funduscopy shown pale papil and blurred edges. CT scan of the
head shown schizencephaly with bilateral open cleft and aplasia
of septum pellucidum.

Result
The patient was admitted in pediatric neurology with roborantia therapy and observation.
From ophthalmology examination we found secondary papil atrophy of both eyes.

Conclusion
Prognosis for individuals with schizencephaly varies depending on the size of the clefts and
the neurological deficit degree. Generally, treatment for individuals with schizencephaly
usually consists of physical therapy, treatment for
seizures and, in a few cases if there was complication of
hydrocephalus, a shunt is required.
Key Work: Schizencephaly, septum pellucidum, papil atrophy
References:
1.Silveira JOF et al Schizencephaly: Case Report. American Medical Journal 3 ,
228-230, 2012
2. Avcu S at al. Bilateral giant open lip schizencephaly with associated
cerebral anomalies: a case report. Cases journal. Aprilo, 2009
3. Al-Alawi et al.Sporadic neonatal schizencephaly associated with
brain calcification.Annals of Tropical Paediatrics, 3438 2001
4. Pradhan M. et al. Case of Schizencephaly: a case report. Kathmandu University Medical Journal , 2003