Atretic Cephalocele: Contribution of Magnetic Resonance Imaging in Preoperative Diagnosis

Abstract

We describe a case of parietal cephalocele. MRI and MR venography were useful for accurate anatomical depiction. We speculate on developmental pathogenesis in the context of the literature available.

Completely Intraosseous Atretic Meningocele

“…Most reported lesions are located in the parietal and occipital areas [3,4,5,6,7,8,9,10,11]. Although they tend to have a benign appearance, their recognition may be of some significance as they may be associated with developmental delay and intracranial malformations such as agenesis of the corpus callosum or cerebellar vermis, intracranial cysts, hydrocephalus, and abnormal venous drainage [3, 4, 7, 10].…”

Atretic Parietal Cephalocele

“…Atretic encephaloceles are generally viewed as benign malformations; however, they may be frequently associated with other complex cerebral, ocular or muscular anomalies and a high prevalence of mental retardation [1] . Parietal cephaloceles have a poor prognosis.…”

“…Atretic cephaloceles are very rare congenital herniations of intracranial structures through a skull defect with a poor prognosis [1,2] . They are considered to be types of cranium bifidum and defined as skin-covered subscalp lesions that contain meninges and glial/neural elements.…”

Intradiploic meningoencephalocele

“…Usually they are associated with numerous significant brain anomalies such as callosal agenesis, Chiari II and Dandy-Walker malformations, and Walker-Warburg syndrome [3,4,5,6]. A high percentage of cephaloceles belong to the group of atretic cephaloceles that present as small hairless midline masses that communicate through a calvarial defect with the intracranial cavity [2,5,7,8]. They are also frequently associated with accompanying cerebral anomalies [2].…”