Iniencephaly: neuroradiological and surgical features. Case report and review of the literature

J Neurosurg. 1998 Aug;89(2):317-20. doi: 10.3171/jns.1998.89.2.0317.


Iniencephaly is a rare congenital anomaly characterized by spina bifida of the cervical vertebrae, fixed retroflexion of the head on the cervical spine, and occipital bone defect. There are only five reports of surviving patients with iniencephaly. The authors report the case of a newborn who presented with iniencephaly and an encephalocele that were surgically treated in our service. Neurological examination of the patient yielded normal results except for a moderate psychomotor retardation. The neuroradiological and surgical findings of the case suggested that the trigger of the anomaly was the occipital bone defect and rachischisis of the posterior vertebral arches.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Brain / pathology
  • Cerebral Angiography
  • Cervical Vertebrae / abnormalities*
  • Cervical Vertebrae / diagnostic imaging
  • Cervical Vertebrae / surgery
  • Child Development
  • Encephalocele / complications*
  • Encephalocele / diagnostic imaging
  • Encephalocele / surgery
  • Follow-Up Studies
  • Head / abnormalities*
  • Head / diagnostic imaging
  • Head / surgery
  • Humans
  • Infant, Newborn
  • Magnetic Resonance Imaging
  • Male
  • Occipital Bone / abnormalities*
  • Occipital Bone / diagnostic imaging
  • Occipital Bone / surgery
  • Posture
  • Psychomotor Performance
  • Spina Bifida Cystica / complications*
  • Spina Bifida Cystica / diagnostic imaging
  • Spina Bifida Cystica / surgery
  • Tomography, X-Ray Computed