Spinal Neurenteric Cyst in Association with Klippel-Feil Syndrome: Case Report and Literature Review

World Neurosurg. 2015 Aug;84(2):592.e9-14. doi: 10.1016/j.wneu.2015.03.015. Epub 2015 Mar 17.


Background: Spinal neurenteric cysts, also known as endodermal or enterogenous cysts, are rare epithelium-lined structures of presumed endodermal origin. Congenital vertebral anomalies are frequently seen in patients with neurenteric cysts, most typically anterior spina bifida, butterfly vertebrae, and hemivertebrae. However, few series of intraspinal neurenteric cysts accompanied by Klippel-Feil syndrome have been reported previously.

Case description: Our purpose is to present the clinical, radiological, and histological results of a 29-year-old patient with a spinal neurenteric cyst associated with Klippel-Feil syndrome and to review previous reported cases of neurenteric cysts associated with Klippel-Feil syndrome. In our patient, cervical radiography demonstrated C5-T1 vertebral fusion and magnetic resonance imaging revealed a large intradural cystic mass. The cystic lesion was removed successfully, and it was histopathologically diagnosed as a neurenteric cyst.

Conclusion: Neurenteric cysts should always be considered in the differential diagnosis of an intraspinal cystic mass seen in the setting of vertebral anomalies.

Keywords: Congenital cyst; Endodermal cyst; Enterogenous cyst; Klippel-Feil anomaly; Neurenteric cyst; Spinal cyst.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Cervical Vertebrae / pathology*
  • Cervical Vertebrae / surgery*
  • Diagnosis, Differential
  • Follow-Up Studies
  • Humans
  • Klippel-Feil Syndrome / diagnosis*
  • Klippel-Feil Syndrome / pathology
  • Klippel-Feil Syndrome / surgery*
  • Magnetic Resonance Imaging
  • Male
  • Neural Tube Defects / diagnosis*
  • Neural Tube Defects / pathology
  • Neural Tube Defects / surgery*
  • Thoracic Vertebrae / pathology*
  • Thoracic Vertebrae / surgery*