Iniencephaly with Long Survival: A Rare Condition

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.