Author/Authors :
Bakhti, Souad Pediatric Neurosurgery Division - Mustapha Pacha University Hospital, Algiers, Algeria , Tighilt, Nabila Pediatric Neurosurgery Division - Mustapha Pacha University Hospital, Algiers, Algeria , Khoudir, Wahiba Pediatric Neurosurgery Division - Mustapha Pacha University Hospital, Algiers, Algeria , Djennas, Mohamed Mustapha Pacha University Hospital, Algiers, Algeria
Abstract :
Background & Importance: Iniencephaly is a rare cranio-cervical deformity. It belongs to neural tube defects. This disorder is
characterized by a retroflexion of head, a very short neck, varying degrees of incomplete posterior closure of vertebrae and cervical and
thoracic spinal synostosis. This malformation is associated with central nervous system and systemic malformations. Most of patients are
dead born and others die after few hours of life. There are only few long-term survivors reported in literature.
Case Presentation: A seven-year-old girl with iniencephaly signs who has been managed conservatively and still alive after six years of
follow-up.
Conclusion: Iniencephaly is a rare complexe disorder with a dismal prognosis but not uniformly fatal.
