Successful separation of pygopagus conjoined twins: A case report

Abstract

Introduction: Twins conjoined at the sacrum present a complex anatomy that requires the highest levels of medical and surgical care. We present the successful management and separation of pygopagus babies in a tertiary care center. Case presentation: The conjoined female twins were diagnosed by routine prenatal ultrasonography (US) and magnetic resonance (MR) imaging and were delivered uneventfully at 37 weeks of gestation. Postnatal imaging included US of the brain and urinary system, contrast enema, intravenous (IV) contrast-enhanced computed tomography (CT), and MR of the pelvis, which confirmed the sacral and pelvic-perineal organ fusion as well as brain abnormalities in twin B. For the separation, appropriate allocation of the intrapelvic muscles was guided by preoperative and intraoperative sphincter muscle mapping. Baby A received a repaired sphincter with a natural anus, while a functional neo-anus was created for the other baby. Mild superficial wound dehiscence occurred in both twins, which resolved conservatively in twin B and by temporary colostomy diversion in twin A A. Twin B underwent placement of a gastrostomy and a tracheostomy and remains hospitalized. Conclusion: Careful preoperative planning can lead to the successful separation of pygopagus conjoined twins, even in places with limited resources. © 2023 The Authors