Veerapaneni Poornachand, Veerapaneni Karthika Durga, Yadala Sisira
University of Arkansas Medical Sciences
University of Arkansas for Medical Sciences
Schizencephaly is a rare congenital neuronal migration disorder characterized by the presence of a full-thickness cleft, lined with heterotopic gray matter and filled with cerebrospinal fluid (CSF), which connects the pial surface of the cerebral hemisphere with the ependymal surface of the lateral ventricle. Schizencephaly was first described by Wilmarth in 1887. The term was coined from the Greek word "schizen" 'to divide' and introduced by Yakovlev and Wadsworth in 1946, based on their work on cadavers, that classified schizencephaly into two types. These are: Type I (closed-lip): Cleft is fused, preventing CSF passage. Type II (open-lip): A cleft is present, which permits CSF to pass between the ventricular cavity and subarachnoid space. Schizencephaly can be either unilateral or bilateral and has a prevalence of 1.48 per 100,000 live births. Recent literature classifies schizencephaly into three types, as the full-thickness cleft containing CSF is not mandatory for the definition. Type 1 (trans-mantle): No CSF-containing cleft on magnetic resonance imaging (MRI), but contains a trans-mantle column of abnormal gray matter. Type 2 (closed-lip): Presence of cleft containing CSF, but the lining lips of abnormal gray matter are abutting and opposed to each other. Type 3 (open-lip): Presence of cleft containing CSF. The lining lips of abnormal gray matter are not abutting each other.
脑裂畸形是一种罕见的先天性神经元迁移障碍，其特征是存在一个全层裂沟，裂沟内衬异位灰质并充满脑脊液（CSF），该裂沟将大脑半球的软膜表面与侧脑室的室管膜表面相连。脑裂畸形于1887年由威尔玛思首次描述。该术语由希腊词“schizen”（意为“分裂”）衍生而来，并于1946年由雅科夫列夫和沃兹沃思引入，他们基于对尸体的研究将脑裂畸形分为两种类型。这两种类型分别是：I型（闭唇型）：裂沟融合，阻止脑脊液通过。II型（开唇型）：存在裂沟，允许脑脊液在脑室腔和蛛网膜下腔之间通过。脑裂畸形可以是单侧的或双侧的，每10万例活产中的患病率为1.48。最近的文献将脑裂畸形分为三种类型，因为对于定义来说，含有脑脊液的全层裂沟并非必需。1型（穿通型）：磁共振成像（MRI）上无含脑脊液的裂沟，但含有异常灰质的穿通柱。2型（闭唇型）：存在含脑脊液的裂沟，但异常灰质的内衬唇相互邻接并相对。3型（开唇型）：存在含脑脊液的裂沟。异常灰质的内衬唇不相互邻接。
