Discordant exencephaly in case of a twin delivery: a case report

Neural tube defect occurs as a result of failure of spontaneous closure of the neural tube between the third and fourth weeks of foetal life. Exencephaly is a rare malformation of the neural tube characterized by a large amount of protruding brain tissue in the absence of the calvarium.

The authors report a 29-year-old female, non-compliant to iron, calcium and folic acid tablets due to nauseating and itchy sensation after intake for 2 weeks, was admitted in ward Obstetrics ward in view of twin pregnancy. After proper counselling, she was advised for caesarean section, which revealed gross malformation in the form of cleft lip, cleft palate and exposed brain tissue covered by thin layer of membrane with incompletely formed cranial vault and multiple-haematoma and ulcerations in the exposed brain tissue suggestive of Exencephaly. The deformed baby survived for 2 days after birth while the other baby was grossly healthy.

Exencephaly is said to be the embryological precursor anomaly of anencephaly. Exencephaly is a type of cranial malformation that characteristically involves a large disorganized mass of brain tissue. The flat bones of calvaria are absent and the brain mass is left uncovered. This condition is incompatible with life.

Each and every pregnant lady must be advised to undergo ultrasonography in every trimester, especially second trimester scan (anomaly scan) to diagnose any gross congenital malformations. Each pregnant lady is suggested to take the necessary vitamins (like folic acid) to avoid any Neural tube defects.