Background: Detection of a single yolk sac on early first trimester ultrasound was previously thought to be a reliable diagnostic feature of monochorionic monoamniotic (MCMA) twin pregnancies. Objectives: To determine the frequency of two yolk sacs in MCMA twin pregnancies and the association of yolk sac number with pregnancy outcomes. Methods: A retrospective cohort analysis of MCMA twins managed at a tertiary obstetric centre from January 2003 until February 2017. All MCMA twin pregnancies were diagnosed on tertiary centre ultrasound and, where possible, placental histopathology postnatally. All MCMA twin pregnancies, including conjoined twins, with available first trimester ultrasounds from 5 to 11 weeks’ gestation were included in the analysis. MCMA pregnancies without available first trimester ultrasounds and triplet pregnancies which included a MCMA pair were excluded from the study. Results: Sixty-seven MCMA cases were identified over 14 years. Thirty-eight cases were included in the analysis. There was one yolk sac identified in 26 cases (68%) and two yolk sacs in 12 cases (32%). Two yolk sacs were associated with a higher proportion of male fetuses (33%, 4 out of 12, vs. 8%, 2 out of 26; p = 0.01). There were no other significant differences between one and two yolk sacs for maternal or neonatal outcomes. Conclusions: Two yolk sacs are present in up to a third of all MCMA twin pregnancies, dispelling the original concept that a single yolk sac is diagnostic of MCMA pregnancies. Yolk sac number should not be used to determine amnionicity. The presence of two yolk sacs on first trimester ultrasound is associated with an increased rate of male fetuses. The number of yolk sacs has no other significant impact on perinatal outcomes.

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