Abstract:
Iniencephaly is a rare neural tube defect characterized by extreme retroflexion of the head with the absence
of neck due to spinal deformities. The important features that help us to diagnose a case of iniencephaly
are occipital bone deficit leading to enlarged foramen magnum, fusion of malformed cervical and thoracic
vertebrae, and upward turned face with chin continuous with chest because of the absence of neck. The
differential diagnoses include anencephaly with spinal retroflexion, Klippel–Fiel syndrome, nuchal tumors
such as teratoma, goiter, and lymphangioma and Jarcho–Levin syndrome. Previously many case reports on
radiological features of iniencephaly are published, but there are very few articles on necropsy findings and
differential diagnosis. In the present case we have discussed in detail the necropsy findings of iniencephaly
clausus with special reference to differential diagnosis.
