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Deformity correction and long-term survival in an infant with iniencephaly. Case report
J J Gartman1, T E Melin, W T Lawrence
1Division of Neurological Surgery, University of North Carolina, Chapel Hill, North Carolina.
Journal of Neurosurgery
|July 1, 1991
Insights
Iniencephaly, a severe congenital defect, is not always fatal. Neurosurgical interventions can significantly improve cervical deformities in affected infants.
Area of Science:
- Medical Case Study
- Pediatric Neurosurgery
- Congenital Malformations
Background:
- Iniencephaly is a rare and severe congenital disorder characterized by the absence of the upper cervical and thoracic spine.
- Historically, iniencephaly has been associated with a very high mortality rate, often leading to stillbirth or early death.
Abstract:
The authors describe the case of a male infant who was diagnosed prenatally as having iniencephaly. Since birth, the child has grown, thrived, and undergone two successful operations to correct his cervical deformity. This case demonstrates that the iniencephaly defect is not uniformly fatal and that neurosurgical intervention may offer significant improvement in the cervical deformity.