Transfusion syndromes in monochorionic multiplets: An overview

Abstract

The west Africa sub-region, notably Nigeria, records the highest twin and triplet birth rates globally. Therefore, the time has come for attention to be focused on an area of gemellology, feto-fetal transfusion syndromes in multiplets, in order to attend to what the authors consider a yet-to-be-explored major significant contributor to the overall unacceptably high fetal, perinatal and neonatal losses in the region. This review examines the genetics, embryology and pathophysiology of twinning in general to provide the background to the spectrum of clinical presentations of feto-fetal transfusion syndromes. Twin-to-twin transfusion syndromes (TTTS) are unique prenatal complications of monochorionic multiplets and manifest as twin oligohydramnios polyhydramnios sequence (TOPS), twin anaemia polyhydramnios sequence (TAPS) and twin reversed arterial perfusion syndrome (TRAPS). These grave complications are associated with fetal malformations and early miscarriages as well as fetal weight and haemoglobin discordances, discordant haemodynamic changes in addition to intrauterine deaths, perinatal asphyxia, cerebral palsy and brain damage. Most importantly, the management of TTTS requires highly skilled interventions, expensive equipment, rare expertise and costly treatment options that are currently not available in Nigeria and other developing countries. Moreover, these management options are unavailable in Nigeria because considerable attention of the health system is directed at the burden of high levels of maternal, perinatal, and childhood morbidity and mortality. Regardless of these overwhelming obstetric and paediatric challenges, there is still urgent need to develop feto-maternal medicine units in the country to focus attention on the management of TTTS because of high twinning rate and attending fetal, perinatal and neonatal wastages. Furthermore, Nigeria is now witnessing an increased incidence of twin births from the rapidly developing assisted reproductive therapy centres in the country. All these provide justification for devoting attention to this unique area of perinatal care that will, on balance, be robustly cost effective. Hence, this review of transfusion syndromes in monochorionic multiplets aims to sensitize health workers and researchers in Nigeria, particularly perinatologists and feto-maternal physicians, neonatal paediatricians, as well as policy makers and other stakeholders, on the need to focus attention on the problem.