Mert Ozan Bahtiyar, Manuela Tavares de Sousa, Silvia Arévalo, Asma Khalil, Femke Slaghekke, Anne Sophie Weingertner, Mark D. Kilby, Lisanne S.A. Tollenaar, Greg Ryan, Enrico Lopriore, Philipp Klaritsch, C. Colmant, Liesbeth Lewi, Glenn Gardener, Eleonor Tiblad, Kirill V. Kostyukov, Ramesha Papanna, Dick Oepkes, Mariano Lanna, Elisa Bevilacqua, Institut Català de la Salut, [Tollenaar LSA, Slaghekke F] Division of Fetal Therapy, Department of Obstetrics. [Lewi L] Leiden University Medical Center, Leiden, the Netherlands. Department of Obstetrics and Gynecology, University Hospitals Leuven, Leuven, Belgium. [Colmant C] Department of Obstetrics and Maternal-Fetal Medicine, Hôpital Necker-Enfants Malades, AP-HP, Paris, France. [Lanna M] Fetal Therapy Unit 'U. Nicolini,' Vittore Buzzi Children’s Hospital, University of Milan, Milan, Italy. [Weingertner AS] Department of Obstetrics and Gynecology, Strasbourg University Hospital, Strasbourg Cedex, France. [Arévalo S] Unitat de Medicina Maternal-Fetal, Servei d’Obstetrícia, Vall d’Hebron Hospital Universitari, Barcelona, Spain, and Vall d'Hebron Barcelona Hospital Campus
Background: Twin anemia polycythemia sequence is a chronic form of unbalanced fetofetal transfusion through minuscule placental anastomoses in monochorionic twins, leading to anemia in the donor and polycythemia in the recipient. Owing to the low incidence of twin anemia polycythemia sequence, data on diagnosis, management, and outcome are limited. Objective: This study aimed to investigate the diagnosis, management, and outcome in a large international cohort of spontaneous twin anemia polycythemia sequence. Study Design: Data from the international twin anemia polycythemia sequence registry, retrospectively collected between 2014 and 2019, were used for this study. A total of 17 fetal therapy centers contributed to the data collection. The primary outcomes were perinatal mortality and severe neonatal morbidity. Secondary outcomes included a risk factor analysis for perinatal mortality and severe neonatal morbidity. Results: A total of 249 cases of spontaneous twin anemia polycythemia sequence were included in this study, 219 (88%) of which were diagnosed antenatally and 30 (12%) postnatally. Twin anemia polycythemia sequence was diagnosed antenatally at a median gestational age of 23.7 weeks (interquartile range, 9.7–28.8; range, 15.1–35.3). Antenatal management included laser surgery in 39% (86 of 219), expectant management in 23% (51 of 219), delivery in 16% (34 of 219), intrauterine transfusion (with partial exchange transfusion) in 12% (26 of 219), selective feticide in 8% (18 of 219), and termination of pregnancy in 1% (3 of 219) of cases. Perinatal mortality rate was 15% (72 of 493) for the total group, 22% (54 of 243) for donors, and 7% (18 of 242) for recipients (P, SCOPUS: ar.j, info:eu-repo/semantics/published