Abstract

Monoamniotic twin pregnancy discordant for anencephaly (MATDA) is a rare occurrence with only nine prior reported cases. Selective termination has been advocated in managing this kind of twins. We report the case, including expectant management with serial amniocentesis and a good outcome. The case was a 22-year-old primigravida woman diagnosed with MATDA at 24 weeks. Ultrasound findings included anencephaly, cervical rachischisis, a single placenta, absent membrane, severe polyhydramnios. Cordo- and placentocentesis showed a normal karyotype for the twins. Serial amnioreductions were performed. Amniotic fluid samples were submitted to the shake (Clements) test for fetal lung maturity assessment. The woman was managed expectantly until 35 weeks when a preterm labor began and Cesarean section was performed. The surviving male infant weighed 2100 g. and had a deformed right auricle and atresia of the auditory canal. He was discharged home in good condition one month later.

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