Amniotic band syndrome and limb body wall complex in Europe 1980–2019

Abstract

Amniotic band syndrome (ABS) and limb body wall complex (LBWC) have an overlapping phenotype of multiple congenital anomalies and their etiology is unknown. We aimed to determine the prevalence of ABS and LBWC in Europe from 1980 to 2019 and to describe the spectrum of congenital anomalies. In addition, we investigated maternal age and multiple birth as possible risk factors for the occurrence of ABS and LBWC. We used data from the European surveillance of congenital anomalies (EUROCAT) network including data from 30 registries over 1980-2019. We included all pregnancy outcomes, including live births, stillbirths, and terminations of pregnancy for fetal anomalies. ABS and LBWC cases were extracted from the central EUROCAT database using coding information responses from the registries. In total, 866 ABS cases and 451 LBWC cases were included in this study. The mean prevalence was 0.53/10,000 births for ABS and 0.34/10,000 births for LBWC during the 40 years. Prevalence of both ABS and LBWC was lower in the 1980s and higher in the United Kingdom. Limb anomalies and neural tube defects were commonly seen † Deceased October 2022.

Surveillance of multiple congenital anomalies; searching for new associations

“…The classification of significant associations as known associations or sequences was based on published literature. Ten anomaly pairs were part of the limb body wall complex [ 14 , 15 ], OEIS complex [ 16 , 17 ] or VACTERL association [ 18 , 19 ]. The association of neural tube defects and omphalocele was documented previously and explains three of the anomaly pairs found in this study [ 9 , 20 ].…”