Management of pyopagus conjoined twins

Abstract

Introduction: Approximately 18% of conjoined twins (1 in 200,000 live births) are joined at the sacrum ("pyopagus"). As the joined structures are not life-threatening, there is generally a good prognosis, with time for investigation and planning of operative separation.

Case report: This paper reports on the management of pyopagus twin girls, the first in the UK delivered at 36 weeks by Caesarean section. The diagnosis had been made by ultrasound at 12 weeks' and confirmed by MRI scan at 26 weeks' gestation. Each twin had wasting and weakness below one knee, but no deficit in the other leg. The perineum had two urethras but only one anus. One infant had a colostomy in the immediate neonatal period. With appropriate further imaging, surgery was planned in two stages. First, a balloon expander was inserted in the bridging area. At 3 months of age a team comprising Plastic, Paediatric and Neurosurgeons undertook the definitive separation.

Discussion: Details of the multidisciplinary planning, operative separation and follow-up of these two children will be discussed.