Iniencephaly with Long Survival: A Rare Condition

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.

A rare case of fetal neural tube defect; Iniencephaly clausus

“…Iniencephaly is always lethal in the neonatal period; however, a few case reports showing increased longevity (13). Prenatal diagnosis is possible with careful and early ultra-sonographic examination.…”