*3.7.1. Pregnancy management*

Iniencephaly is a rare, lethal developmental anomaly. Associated malformations occur in up to 84% of cases and include hydrocephaly, microcephaly, ventricular atresia, holoprosencephaly, polymicrogyria, agenesis of the cerebellar vermis, occipital encephalocele, diaphragmatic hernia, thoracic cage deformities, urinary tract anomalies, cleft lip and palate, omphalocele, and polyhydramnios [20]. The sonographic diagnosis has been made as early as 12.5–13 postmenstrual weeks. Detailed sonography or MRI should be performed to verify the diagnosis and to search for associated anomalies. If the iniencephaly is diagnosed at less than 22–24 weeks of gestation, the termination of the pregnancy can be offered to the parents.
