A Rare Case Report of Iniencephaly with Anencephaly and its Rare Associated Congenital Malformation

• Main Authors: Tanima Dwivedi, Ranjit Kangle
• Format: Article
• Language: English
• Published: JCDR Research and Publications Private Limited 2018-01-01
• Series: Journal of Clinical and Diagnostic Research
• Subjects: asplenia; congenital diaphragmatic hernia; neural tube defect; occipital cervical spina bifida
• Online Access: https://jcdr.net/articles/PDF/11084/31633_240118_31633_CE(RA1)_F(T)_PF1(PB_BT_SU_AP)_PFA(AnG)_PN(AP).pdf

Iniencephaly is an extremely rare neural tube defect with incidence range from 0.1 to 10 in 10,000. It is diagnosed by a triad of fixed retroflexion of the head, variable degrees of cervical lordosis and dysraphism, and an occipital bone defect involving the foramen magnum. It is usually reported as an isolated anomaly and it rarely occurs along with anencephaly. We report here such a rare case of Iniencephaly in association with anencephaly and multiple rare congenital malformations such as asplenia, craniospinal rachischisis, occipital cervical spina bifida and congenital diaphragmatic hernia. It was rare type of congenital diaphragmatic hernia in which intestine coils were lying in front of vertebrae. These anomalies are rare in themselves.