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

Pace Carlotta

doi:10.19080/ajpn.2016.01.555553

Abstract

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.

Highlights

Limb-body wall complex (LBWC) is a rare and lethal fetal malformation (1/7500 pregnancies, 1/14000 births [1]), characterized by a large abdominal wall defect with rudimental or absent umbilical cord and abdominal placental attachment
We are reporting a case of monoamniotic twin pregnancy discordant for LBWC, complicated by acute twin-to-twin transfusion syndrome (TTTS)
The diagnosis of TTTS, according to Quintero’s staging system [13], is based on the presence of an amniotic membrane separating the twins. In monoamniotic pregnancies this staging system cannot be applied and the diagnosis is established on the observation of polyhydramnios in the receiver, non-visualization of the bladder of the donor fetus, discordant biometry and abnormal Doppler velocimetry. In this specific case the presence of LBWC prevented a correct evaluation of fetal biometry and the visualization of the bladder in the affected twin

Summary

Case Report

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 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.

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