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IrJNS 2016, 2(3): 19-21 Back to browse issues page
Iniencephaly with Long Survival: A Rare Condition
Souad Bakhti 1, Nabila Tighilt , Wahiba Khoudir , Mohamed Djennas
1- Professor, Pediatric Neurosurgery Division, Mustapha Pacha University Hospital
Abstract:   (1018 Views)

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.

Keywords: Iniencephaly, Neural Tube Defect, Spine
Full Text [PDF 1154 kb]   (343 Downloads) |   |   Full Text (HTML)  (175 Views)  
Type of Study: Case report | Subject: Gamma Knife Radiosurgery
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DOI: 10.18869/acadpub.irjns.2.3.19

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Bakhti S, Tighilt N, Khoudir W, Djennas M. Iniencephaly with Long Survival: A Rare Condition. IrJNS. 2016; 2 (3) :19-21
URL: http://irjns.org/article-1-64-en.html
Volume 2, Issue 3 (12-2016) Back to browse issues page
مجله جراحی مغز و اعصاب ایران Iranian Journal of Neurosurgery
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