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Objectives To review the experience of the University of Toronto Perinatal Complex, Ontario, Canada concerning antenatally diagnosed monoamniotic twin pregnancies; and to compare our results with cases reported in the literature with respect to antenatal surveillance and perinatal outcome. Methods A retrospective chart review of all twin gestations from 1993 to April 2000 was performed. A systematic review of the literature, 1966 to April 2000, of perinatal outcome in monoamniotic twin pregnancies was undertaken. Setting All monoamniotic twin gestations at the University of Toronto. Results Case-series: 25 prenatally diagnosed monoamniotic twin pregnancies were identified. Seven pregnancies were affected by fetal anomalies. One fetus died at 29 weeks. Neonatal complications occurred below 33 weeks of gestational age and were related to immaturity. Systematic review of the literature: 49 studies met our …