Schizencephaly

Last edited and medically reviewed by Alaric Steinmetz on 07.08.2022

Synonyms

schizencephaly, Schizenzephalie

ICD-10

Q04.6

Schizencephaly is a rare congenital cortical migration anomaly of the brain characterized by a cleft extending from the ependymal lining of the cerebral ventricles to the pia mater of the brain surface. Schizencephaly may occur unilaterally or bilaterally, and the septum pellucidum is absent in 80-90% of patients¹

Epidemiology

The worldwide incidence of schizencephaly is reported in the literature as 1.5 : 1,000,000 live births and 1 : 1,650 among children with epilepsy².

Symptoms

Schizencephaly may present with varying degrees of clinical severity, ranging from epileptic seizures to hemiparesis³.

Classification

Schizencephaly can be classified as either open-lip or closed-lip⁴.

Closed-lip form

The closed-lip form of schizencephaly is characterized by a very thin cleft, and its inner or outer end may be lined by ependyma or pia mater, respectively. A dimple may be seen in the lateral ventricle directly beneath the cortical cleft, which may resemble an enlarged sulcus⁵.

Open-lip form

There is a cleft communicating with the ventricular system. Severe forms may mimic hydranencephaly⁶.

Imaging

Schizencephaly is visible on CT and MRI imaging.

Schizencephaly CT — Axial head CT image of a patient with schizencephaly (open-lip type). Figure from Wikimedia Commons under the CC BY-SA 3.0 license.

References

Handbook of Neurosurgery. Greenberg M, ed. 9th Edition. New York: Thieme; 2019. ↩ ↩ ↩ ↩ ↩ ↩ ↩ ↩ ↩ ↩
Ugboma, Enighe W., and C. E. Agi. "Schizencephaly: A case report and review of literature." Nigerian Postgraduate Medical Journal 23.1 (2016): 38. ↩ ↩