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Schizencephaly

2007; Elsevier BV; Linguagem: Inglês

10.1016/s0072-9752(07)87015-1

2212-4152

Tiziana Granata, Giorgio Battaglia,

Cerebrospinal fluid and hydrocephalus

Schizencephalies are brain malformations characterized by full thickness holes or clefts in the cerebral mantle. The clefts span the cerebral hemispheres unilaterally or bilaterally, extending from the external pial surface to the lateral ventricles and they are lined throughout by heterotopic gray matter. The schizencephalic clefts are divided into closed-lipped (type 1) and open-lipped (type 2). The cleft is defined as closed when the walls are in contact with each other and the cerebrospinal fluid space within the cleft is obliterated. Conversely, in open-lipped schizencephaly, the walls are separated and the cleft is filled with cerebrospinal fluid from the lateral ventricle to the subarachnoid space. In unilateral schizencephalies, closed-lipped and open-lipped clefts are observed equally often. In bilateral cases, the clefts are more frequently open-lipped and usually symmetrical. Both unilateral and bilateral schizencephalies are frequently associated with other brain abnormalities, thus, suggesting that in most cases the clefts are part of a widespread developmental disorder.