MyPACS.net: Radiology Teaching Files > Case 70655

never visited SCHIZENCEPHALY
Contributed by: Faculty and residents Children's Hospital, Radiologist, Children's Health System, Birmingham, Alabama., USA.
Patient: 1 year 1 month old male
History: 13 month old with developmental delay, right sided weakness, and seizures.
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Findings: A unilateral full thickness cleft throught the left hemisphere with grey matter that lines the cleft. Abnormality involves the frontal lobe. The septum pellucidum is not completely formed and the cleft extends into the left ventricle.
Diagnosis: Unilateral left Schizencephaly (open lip)
Discussion: Schizencephaly (aka agenetic porencephaly) results from genetic and aquired causes. In utero transmantle injury during the middle portion of second trimester or can be familial (associated with EMX2 homeobox gene which is expressed in germinal matrix). Divided into unilateral(60%) and bilateral(40%). Then subdivided into fused lips and separated lips. closed lip schizencepahly will have grey matter which apposses one another obliterating the CSF space, however,in open lip schizencephaly the lips are separate and CSF fills the space from the subarachnoid space to the lateral ventricle. Patients usually present with developmental delay, seizures, and hemiparesis. The more involved the brain the worse the symptoms. Therefore the least severe is the fused lip unilateral schizencephaly and the worse would be a large bilateral open lip schizencephaly. Optic nerve hypoplasia is seen in 1/3 of patients along with a high incidence of septum pellucidum dysplasia (septo- optic displasia). the location is frontal/ parietal in 60-65% of cases. clefts are open bilaterally in 60% and closed bilaterally in 20% and only on one side in 20%. If unilateral they have open lips in 65% of cases and closed lips in 35%. " Mirror image", dysplastic cortex can be seen on the contralateral side with a unilateral schizencephaly, and therefore the oppposite hemisphere should be evaluated closely. the calvarium will often be expanded over the defect secondary to the CSF pulsations.
References: Pediatric Neuroimaging, third edition. A. James Barkovich, M.D.copyright 2000 Lippincott Williams & Wilkins
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Case Number: 70655Last Updated: 10-02-2003
Anatomy: Cranium and Contents   Pathology: Congenital
Modality: MRExam Date: 04-18-2003Access Level: Readable by all users

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