Dermoid cyst in the posterior fossa accompanied by Klippel-Feil syndrome

Childs Nerv Syst. 2001 Jan;17(1-2):97-100. doi: 10.1007/pl00013726.


Objects: Dermoid cysts accompanied by Klippel-Feil syndrome are uncommon, and the coincidence of these two rare diseases suggests an interesting idea about the pathogenesis of a dermoid cyst, which the authors consider with reference to an actual case. A posterior fossa dermoid cyst with dermal sinus and Klippel-Feil syndrome are reported in the same patient. A 23-year-old woman was admitted to hospital with progressive headaches.

Methods: Cervical radiography demonstrated C4-5 vertebral fusion, and magnetic resonance imaging revealed a large cystic mass in the midline of the posterior fossa. The cystic lesion was totally removed along with the accompanying dermal sinus. It was histopathologically diagnosed as a dermoid cyst.

Conclusion: The rare coincidence of a dermoid cyst and Klippel-Feil syndrome suggests the possibility that a disturbance in the mesoderm before the fourth week of gestation might play an important part in the causation of dermoid cyst.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Cranial Fossa, Posterior / pathology*
  • Cranial Fossa, Posterior / surgery
  • Dermoid Cyst / complications*
  • Dermoid Cyst / diagnosis*
  • Dermoid Cyst / surgery
  • Female
  • Humans
  • Klippel-Feil Syndrome / complications*
  • Magnetic Resonance Imaging