Open AccessIniencephaly: Case Report
Author(s) -
Hernando Raphael Alvis-Miranda,
Doris Bula-Anichiarico,
Willem Guillermo Calderón-Miranda,
Luís Rafael Moscote-Salazar
Publication year - 2015
Publication title -
journal of pediatric neurosciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.247
H-Index - 18
eISSN - 1998-3948
pISSN - 1817-1745
DOI - 10.4103/1817-1745.159211
Subject(s) - medicine , foramen magnum , vertebral column , neural tube , thoracic vertebrae , spinal cord , anatomy , cervical vertebrae , spinal column , neural tube defect , skull , lumbosacral joint , thorax (insect anatomy) , surgery , lumbar vertebrae , lumbar , embryo , psychiatry , biology , microbiology and biotechnology
The iniencephaly involves a variable defect in the occipital bone, resulting in a large foramen magnum, partial or total absence of the cervical and thoracic vertebrae, accompanied by incomplete closure of arcs and/or vertebral bodies, significant shortening of the spinal column and hyperextension of the malformed cervicothoracic spine; the individual's face is deviated upward, the mandibular skin is directly continuous with anterior thorax due to the absence of neck. Its incidence is about 1:1000-1:2000 births, so this is a pretty rare neural tube defect. We present a case of iniencephaly in association with cardiovascular, spinal cord, and intracranial malformations that ended demonstrating the low survival of patients affected with this condition.