Child's Nervous System

, Volume 32, Issue 6, pp 1141–1144 | Cite as

Occipital cephalocele with neural crest remnants? Radiological and pathological findings in a newborn boy

  • Hidetaka ArishimaEmail author
  • Hiroyuki Neishi
  • Ken-ichiro Kikuta
Case Report



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.

Case report

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.


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.


Cephalocele Encephalocystocele Magnetic resonance imaging Neural crest 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


  1. 1.
    Naidich TP, Altman NR, Braffman BH, McLone DG, Zimmerman RA (1992) Cephaloceles and related malformations. AJNR Am J Neuroradiol 13:655–690PubMedGoogle Scholar
  2. 2.
    James CCM, Lassman LP (1972) Spinal dysraphisms: spina bifida occulta. Butterworths, London, pp. 89–97Google Scholar
  3. 3.
    Commens C, Rogers M, Kan A (1989) Heterotropic brain tissue presenting as bald cysts with a collar of hypertrophic hair. The ’hair collar’ sign. Arch Dermatol 125:1253–1256CrossRefPubMedGoogle Scholar
  4. 4.
    Drapkin AJ (1990) Rudimentary cephalocele or neural crest remnant? Neurosurgery 26:667–674CrossRefPubMedGoogle Scholar
  5. 5.
    Inoue Y, Hakuba A, Fujitani K, Fukuda T, Nemoto Y, Umekawa T, et al. (1983) Occult cranium bifidum. Radiological and Surgical Findings Neuroradiology 25:217–223PubMedGoogle Scholar
  6. 6.
    Martinez-Lage JF, Sola J, Casas C, Poza M, Almagro MJ, Girona DG (1992) Atretic cephalocele: the tip of the iceberg. J Neurosurg 77:230–235CrossRefPubMedGoogle Scholar
  7. 7.
    Yokota A, Kajiwara H, Kohchi M, Fuwa I, Wada H (1988) Parietal cephalocele: clinical importance of its atretic form and associated malformations. J Neurosurg 69:545–551CrossRefPubMedGoogle Scholar
  8. 8.
    McLone DG, Bondareff W (1975) Developmental morphology of the subarachnoid space and contiguous structures in the mouse. Am J Anat 142:273–293CrossRefPubMedGoogle Scholar
  9. 9.
    Yamazaki T, Enomoto T, Iguchi M, Nose T (2001) Atretic cephalocele—report of two cases with special reference to embryology. Childs Nerv Syst 17:674–678CrossRefPubMedGoogle Scholar
  10. 10.
    Gulati K, Phadke RV, Kumar R, Gupta RK (2000) Atretic cephalocele: contribution of magnetic resonance imaging in preoperative diagnosis. Pediatr Neurosurg 33:208–210CrossRefPubMedGoogle Scholar
  11. 11.
    Saatci I, Yelgec S, Aydin K, Akalan N (1998) An atretic parietal cephalocele associated with multiple intracranial and eye anomalies. Neuroradiology 40:812–815CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  • Hidetaka Arishima
    • 1
    Email author
  • Hiroyuki Neishi
    • 1
  • Ken-ichiro Kikuta
    • 1
  1. 1.Department of NeurosurgeryUniversity of FukuiYoshida-gunJapan

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