Atretic cephalocele: the tip of the iceberg

Abstract

Atretic cephalocele appears as an unimportant and benign lesion. This malformation consists of meningeal and vestigial tissues (arachnoid, glial, or central nervous system rests). The authors report the findings in 16 cases (seven parietal and nine occipital) of rudimentary cephaloceles. Twelve patients presented with associated brain abnormalities detected by either computerized tomography (CT) or magnetic resonance imaging (MR). Nine lesions also exhibited an anomalous vascular component demonstrated by CT or MR imaging or at surgery. The existence of this tiny malformation in five cases was the main diagnostic clue to a severe complex of cerebral anomalies, namely cerebro-oculomuscular (Walker-Warburg) syndrome. An occipital location of the atretic cephalocele was associated with the worst prognosis, with only two children developing normally. However, a parietal location carried a better prognosis, which is contrary to the outcome reported in the current literature. The authors classify atretic cephaloceles into two types based on histological examination of the surgical specimens, and suggest that these types represent different stages in the development of this malformation. It is concluded that, in the evaluation of the atretic cephalocele, the neurosurgeon is obliged to proceed to a detailed neuroradiological study of the patient and that the prognosis does not depend on the existence of the cephalocele itself, but rather on associated "occult" brain anomalies.

Congenital Brain and Spinal Cord Malformations and Their Associated Cutaneous Markers

“…Despite its innocuous appearance, the parietal encephalocele may be associated with other congenital brain malformations and significant cognitive deficits in some cases. 38,39 Parietal encephaloceles must be distinguished from CAC, a disorder of full-thickness skin of uncertain etiology that most commonly involves the scalp (Fig 15); 20% to 30% have skull aplasia as well. 40 In the midline, CAC may extend to involve the dura.…”

Recurrence of atretic parietal cephalocele in adult: a case report and review of literature

“…Martinez-Lage et al [32,33,34] classified AC into two types: Type 1 consisting of arachnoid tissue with clusters of anomalous blood vessels limited to the stalk of the lesion, Type 2 consisting of meningeal tissue intermingled with dermal and fibrous tissue, as well as clusters of anomalous blood vessels, extending as a net, and ectopic neural or glial elements. In according to classification of Martinez-Lage et al [32,33,34], our case can be evaluated as type 1. Because, it is clear that localized soft tissue mass under the scalp ( Figure1,2 and 3).…”

“…; occipital type, if the defect lies between foramen magnum and the lambda; parietal type, if the defect lies between the lambda and the bregma; lateral type, if the defect lies along the coronal or lambdoid sutures as far inferiorly as the anterolateral and posterolateral fontanelles; interfrontal type, if the defect lies between the bregma and the nasal bones; temporal, if the defect lies along the superior surface of the petrous pyramid; fronto-ethmoidal type (synonym: sincipital), if the defect lies between the nasal bones and the ethmoid bone; spheno-orbital type, if the ostium for the herniation involves the optic foramen, the superior orbital fissure, or a defect in the orbital wall; sphenomaxillary type, if the ostium for the herniation extends through the superior orbital fissure and the inferior orbital fissure into the pterygopalatine fossa; nasopharyngeal type, if the defect lies within the ethmoid, sphenoid, or basioccipital bones [48]. Martinez-Lage et al [32,33,34] classified AC into two types: Type 1 consisting of arachnoid tissue with clusters of anomalous blood vessels limited to the stalk of the lesion, Type 2 consisting of meningeal tissue intermingled with dermal and fibrous tissue, as well as clusters of anomalous blood vessels, extending as a net, and ectopic neural or glial elements. In according to classification of Martinez-Lage et al [32,33,34], our case can be evaluated as type 1.…”

“…Pathological classification of cephaloceles is known as meningocele (leptomeninges and cerebro spinal fluid-CSF), meningoencephalocele (leptomeninges, CSF and brain), meningoencephalocystocele (leptomeninges, CSF, brain and ventricles), atretic cephalocele (small nodule of fibrous fatty tissue), gliocele (CSF lined by glial tissue) [7,39]. Atretic parietal cephaloceles are associated with anomalies of Chiari II and III malformations, corpus callosum agenesis and Dandy-Walker malformation [32,38].…”

Recurrent Atretic Parietal Cephalocele in Adult and Radiolodical Findings

“…Atretic parietal cephaloceles are extremely rare and occult forms of congenital herniations that involve the meninges, remnants of glial cells or central nervous system (CNS) structures, such as intracranial venous sinuses [1]. They are detected as subscalp lesions that are covered by skin [2].…”