Translated title of the contribution: Cephaloceles

B. Bernardi, C. Fonda

Research output: Contribution to journalArticlepeer-review


Cephaloceles are congenital or acquired defect in the cranium and dura with extracranial herniation of any intracranial structure. The simple midline or paired paramedian skull defect(s) without prolapse of intracranial tissues is defined cranium bifidum occultum. Congenital cephaloceles can be subdivided into four groups following the nature of the tissue protruding in the herniated sac: - Meningoceles, in which the herniating structures are meninges and cerebrospinal fluid (CSF). - Meningoenchephaloceles, in which the protruding structeres are leptomeninges, CSF, and brain tissue. When a part of the ventricular system is present into the herniated sac the lesion is defined hydroencephalomeningocele. - Glioceles, in which the herniated sac consist of a cystic CST collection lined by neuroglial tissue. - Atretic cephaloceles are formes fruste of cephaloceles localized into the parietal or occipital bone. The lesion is small and flat. The congenital cranial dysraphisms are less common than spinal dysraphism. The incidence of cephaloceles ranges from 0.3 to 4 per 10.000 births. In the Western hemisphere 80 to 90 per cent of all encephaloceles are located in the occipital area in contrast to a prevalence of the frontoetmoidal (sincipital) location in a large portion of the Eastern hemisphere population. Occipital cephaloceles are more common in females, whereas parietal and sincipital locations show male predominance. Cephaloceles are classified according to site of intracranial defect into four major groups: occipital cephaloceles, cranial vault cephaloceles, frontoethmoidal (sincipital) cephaloceles, and cephaloceles involving the skull base. Ultrasonographically the cephaloceles with an important extracranial extension can be detected in utero since the first trimester of fetal life. Plain film followed by tomography is able to detect location and extension of bony decect. Computerized Tomography (CT) clearly shows the bony defect margin at the same time of the soft tissue component of cephalocele. The multiplanar rappresentation is often necessary to a complete recognition of the anomaly. The 3D images of CT scans are of great value to evaluate the skeletal anomalies. MRI is the best modality to identify the presence of brain tissue in the cephalocele and to define the malformative involvement of intracranial structures. MR angiography is needed to evaluate the vascular structures planning surgery.

Translated title of the contributionCephaloceles
Original languageItalian
Pages (from-to)171-186
Number of pages16
JournalRivista di Neuroradiologia
Issue number2
Publication statusPublished - 1994

ASJC Scopus subject areas

  • Clinical Neurology
  • Radiology Nuclear Medicine and imaging
  • Radiological and Ultrasound Technology


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