A Monoamniotic Pregnancy Discordant for Limb Body Wall Complex and Complicated by Acute TTTS

We are reporting a case of monoamniotic twin pregnancy discordant for Limb Body Wall Complex (LBWC), complicated by acute twinto-twin transfusion syndrome. The twins were delivered by an emergency cesarean section at 29+6 weeks’ gestation because of persistent fetal tachycardia of the unaffected twin. The phenotipically normal twin was anemic at birth (donor twin), while postmortem examination revealed a condition of overloaded fetal circulation of the LBWC twin (recipient twin). This is, to our knowledge, the first case of acute TTTS in a monoamniotic pregnancy reported in Literature. In monoamniotic pregnancies discordant for LBWC careful fetal monitoring should be granted, as the presence of the malformation masks the classic signs of twin-to-twin transfusion syndrome.