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

Amniotic band syndrome (ABS) and limb body wall complex (LBWC) have an overlap-ping phenotype of multiple congenital anomalies and their etiology is unknown. Weaimed to determine the prevalence of ABS and LBWC in Europe from 1980 to 2019and to describe the spectrum of congenital anomalies. In addition, we investigatedmaternal age and multiple birth as possible risk factors for the occurrence of ABS andLBWC. We used data from the European surveillance of congenital anomalies(EUROCAT) network including data from 30 registries over 1980-2019. We includedall pregnancy outcomes, including live births, stillbirths, and terminations of preg-nancy for fetal anomalies. ABS and LBWC cases were extracted from the centralEUROCAT database using coding information responses from the registries. In total,866 ABS cases and 451 LBWC cases were included in this study. The mean preva-lence was 0.53/10,000 births for ABS and 0.34/10,000 births for LBWC during the40 years. Prevalence of both ABS and LBWC was lower in the 1980s and higher inthe United Kingdom. Limb anomalies and neural tube defects were commonly seenin ABS, whereas in LBWC abdominal and thoracic wall defects and limb anomalieswere most prevalent. Twinning was confirmed as a risk factor for both ABS andLBWC. This study includes the largest cohort of ABS and LBWC cases ever reportedover a large time period using standardized EUROCAT data. Prevalence, clinical char-acteristics, and the phenotypic spectrum are described, and twinning is confirmed asa risk factor.