Separation of omphalopagus conjoined twins with ruptured omphalocele, complex biliary sharing and a choledochal cyst

Abstract

Separating conjoined twins requires a multidisciplinary team to manage all the phases of care. These phases include initial resuscitation, managing the often discordant physiology between each twin prior to separation, planning for and carrying out the separation operation, and postoperative management. Each phase has unique challenges. The decision on timing of separation is critical in achieving success and is influenced by the stability of each infant, the confidence and experience of the team and full preoperative understanding of the shared anatomy to the extent possible. We successfully separated omphalopagus conjoined twins complicated by complex sharing of the biliary tree and the presence of an infected choledochal cyst. Preoperative optimization, MRI and MRCP, intraoperative US along with an experienced pediatric surgical team proved critical to successful separation and survival of each twin.