Extrauterine twin–twin transfusion syndrome in conjoined twins: prevalence and clinical implications

Abstract

Purpose: Conjoined twins present a rare and unique clinical phenomenon known as extrauterine twin–twin transfusion syndrome (EUTTTS), which is underrecognized and underreported in the literature. The present study aimed to conduct a comprehensive analysis of EUTTTS and highlight its various clinical manifestations to help physicians effectively identify and manage this condition. Methods: A retrospective review was conducted on 10 sets of conjoined twins managed by a single medical team over a 3-year period. The impact of cross-circulation, management approaches, and clinical outcomes were evaluated. Results: Seven of the sets (70%) exhibited features of EUTTTS. Four of these sets (57%) had nutritional imbalances, and three (43%) had medication crossover, which included fluid administration. Both craniopagus sets (29%) had hemodynamic instability. These clinical manifestations posed significant diagnostic challenges and management complexities. Furthermore, donor twins had a higher risk of morbidity and mortality as compared to recipients. Conclusion: EUTTTS is a prevalent condition among conjoined twins, and it manifests as a spectrum of clinical presentations independent of the type of conjoined twins. Awareness of these presentations is essential for physicians managing conjoined twins to ensure timely diagnosis and optimal management strategies.