title: Occipital cephalocele with neural crest remnants? Radiological and pathological findings in a newborn boy.
abstract: OBJECTIVE
  A cephalocele is a congenital anomaly involving the herniation of intracranial tissue from a skull defect. The sac containing the central nervous system (CNS) with the ventricle system is called the encephalocystocele. An atretic cephalocele is thought to be an abortive form of cephalocele, and the essential nature is still controversial.
  
  
  METHODS
  Here, we report the case of a newborn boy with an occipital cephalocele containing a small cystic component which was composed of ependymal cells and the immature CNS tissue. A newborn boy was admitted to our hospital because of an occipital mass, which was about 2.5 cm in diameter, located at the posterior midline, and covered with alopetic skin without CSF leakage. He had a cleft palate. Magnetic resonance imaging (MRI) clearly showed an occipital cephalocele with a tiny cystic component connecting to the subarachnoid space. MRI also showed mild hydrocephalus, hypoplasia of the corpus callosum and tentorium cerebelli, dropping down of the bilateral occipital lobes and vermicular agenesis. We performed the extirpation of the subscalp module under general anesthesia and histologically examined the resected mass. On immunohistopathological examination, most part of the subscalp module was fibrous tissue with numerous vessels and meningeal origin cells. In a small part of the innermost layer, we found a small island consisting of CNS tissue and a tiny cyst lined with a single layer of ependymal cells.
  
  
  CONCLUSIONS
  Based on radiological and immunohistopathological findings, we speculate that the cystic component at the base of the nodule seems to correspond to neural crest remnants but not to true herniation of the brain and cerebral ventricles.
authors.affiliation: Department of Neurosurgery, University of Fukui, 23-3, Matsuokashimoaizuki, Eiheiji-cho, Yoshida-gun, Fukui, 910-1193, Japan. ari@u-fukui.ac.jp.
authors.forename: Hidetaka
authors.initials: H
authors.lastname: Arishima
authors.affiliation: Department of Neurosurgery, University of Fukui, 23-3, Matsuokashimoaizuki, Eiheiji-cho, Yoshida-gun, Fukui, 910-1193, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Neishi
authors.affiliation: Department of Neurosurgery, University of Fukui, 23-3, Matsuokashimoaizuki, Eiheiji-cho, Yoshida-gun, Fukui, 910-1193, Japan.
authors.forename: Ken-Ichiro
authors.initials: K
authors.lastname: Kikuta
mesh_terms: D004677:Encephalocele / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery*; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D006801:Humans; D021621:Imaging, Three-Dimensional; D007231:Infant, Newborn; D008297:Male; D018396:Mucin-1 / Q000378:metabolism; D009432:Neural Crest / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D009778:Occipital Lobe / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D014057:Tomography, X-Ray Computed
chemical_list: D005904:Glial Fibrillary Acidic Protein; D018396:Mucin-1
keywords: Cephalocele; Encephalocystocele; Magnetic resonance imaging; Neural crest