What—and Why—the Pathologist Should Know about Twin-to-Twin Transfusion Syndrome

Abstract

Approximately 20% of all twin pregnancies are monochorionic. Between 9% and 15% of all monochorionic twin gestations are complicated by severe chronic twin-to-twin transfusion syndrome (TTTS), characterized by a gradual shift of blood volume from the donor twin to the recipient twin through placental vascular connections [ 1 – 3 ]. The prognosis of severe, untreated chronic TTTS diagnosed in midtrimester fetuses is extremely poor, with mortality rates exceeding 70% [ 4 ]. Since publication of the results of the Eurofoetus trial in 2004, laser photocoagulation of the intertwin anastomoses has become accepted as the optimal first-line therapy for severe TTTS diagnosed before 26 weeks of gestation. While laser treatment of vascular communications was initially limited to selected fetal treatment centers, its increasingly widespread use has resulted in the exposure of more pathologists, even in less specialized institutions, to laser-treated placentas. Furthermore, the surge in laser coagulation has revived the general medical, scientific, and public interest in the placental and choriovascular findings in monochorionic twin placentas. The pathologist's understanding of the pathophysiology of TTTS and of TTTS-associated placental pathology, including the findings related to laser ablation of the anastomoses, can be of great benefit to the involved obstetric/neonatal/surgical team and, ultimately, to the patients. In this review, we summarize the current knowledge of the placental contributions to TTTS and other complications of monochorionic twinning and describe the strengths and limitations of placental examination in these settings. It is our expectation that overviews such as this may serve as a template to generate consensus guidelines for standardized and evidence-based pathologic evaluation of monochorionic twin placentas.