Acardiac amorphous twin with prune belly sequence in the co‐twin

Abstract

We describe a twin with acardia acephalus or "Twin Reversed Arterial Perfusion Sequence" and prune belly sequence in the co-twin. In a former quite similar case a prune belly appearance of the co-twin of an acardiac fetus was found to be secondary to the ascites caused by cardiac failure. In the present case, we are dealing with the prune belly sequence as a separate condition, given the fact there were no signs of ascites or cardiac failure. We also found associated anomalies: agenesis of the left ureter and kidney, dysplastic right kidney and anal atresia. Urinary tract obstruction has never been described in the co-twin of an acardiac amorphous fetus.

An Acephalus Acardius Amorphous Fetus in a Monochorionic Pregnancy With Sex Discrepancy

“…The mortality rate is around 50% to 75% in the pump twin, mostly due to heart failure, and 100% in the perfused one (Czichos et al, 2005;Fries et al, 1992;Malinowski & Szwalski, 2004;Moore et al, 1990;Rajesh et al, 2004;Torres Borrego et al, 2000). In the case here reported we observed gross dysmorphic features in the malformed twin and extensive vascular anastomoses in the fused placenta, suggesting that the low pressure and hypoxigenated blood which nourished it could explain the extremely severe dismorphic sequence as suggested in previous reports (Buntinx et al, 1991;Cardwell, 1988;Coulam & Wright, 2000;Goh et al, 1994;Kosno-Kruszewska et al, 2003;Malhotra et al, 2004;Moore et al, 1987;Platt et al, 1983;Rajesh et al, 2004;Robie et al, 1989;Sergi et al, 2000;Sharma et al, 1993;Torres Borrego et al, 2000).…”

Acardia: Epidemiologic findings and literature review from the International Clearinghouse for Birth Defects Surveillance and Research

“…The co‐twin has been reported to have an increased risk of structural malformations, as high as 10% according to a recent review [Phelan and Hall, 2006]. Several structural malformations have been reported, including anencephaly [Pavone et al, 1985], prune belly sequence with dysplastic kidney [Buntinx et al, 1991], gastroschisis [Habbal et al, 2005], small intestinal atresia [Wong et al, 2007], skeletal malformations of limbs and ribs [Chen et al, 2007], and malformations consistent with the VATER association [Moore et al, 1987], among others. In one triplet, two acardiac twins were reported [Ventura et al, 2011].…”

Twin reversed arterial perfusion syndrome in a monochorionic monoamniotic twin pregnancy

“…However, Klinefelter syndrome and Prune Belly sequence have been reported in co-twin of acardiac fetuses. 10,11 The optimal management of TRAP syndrome is controversial ranging from conservative management to elective termination or surgical intervention such as occlusion of umbilical cord of acardiac fetus with laser coagulation, bipolar cord coagulation and radiofrequency ablation (RFA) and rarely fetoscopic cord ligation. [12][13][14][15] Treatment is focused on improving the outcome of pump fetus.…”