Atretic cephalocele: the tip of the iceberg

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✓ 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.

Article Information

Address reprint requests to: Juan F. Martinez-Lage, M.D., Regional Service of Neurosurgery, University Hospital Virgen de Arrixaca, El Palmar, E-30120 Murcia, Spain.

© AANS, except where prohibited by US copyright law.

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Figures

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    Schematic representation of the two types of atretic cephaloceles. a = arachnoid tissue: b = bone: d = dermis; n = neuroglial foci; p = brain parenchyma; v = vessels.

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    Left: Photomicrograph of a specimen from the dome of a Type 1 atretic cephalocele showing atrophic squamous epithelium and the underlying rich capillary network that intermingles with fibrous hyaline tissue. H & E, × 68. Right: Photomicrograph of the stalk of a Type 2 atretic cephalocele. Note the dilated net of dysplastic vessels, foci of neuroglial elements, and clusters of meningeal cells. H & E, × 170.

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    Photograph of a child with an occipital atretic cephalocele showing an alopecic lesion surrounded by a hairy collar.

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    Contrast-enhanced computerized tomography scan in a patient with Walker-Warburg syndrome. Note marked hydrocephalus, the occipital cephalocele, and the bright enhancement of the lesion.

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    Computerized tomography scan showing an interhemispheric occipital arachnoid cyst, a frequent finding in parietal rudimentary cephaloceles.

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    Magnetic resonance image of a child with a parietal atretic cephalocele demonstrating the relationship of the lesion with the bone defect and the underlying sagittal sinus.

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