A case report of conjoined twin(PYGOPAGUS) separation

Abstract

We report successful separation of PYGOPAGUS twins. Meticulous planning and multidisciplinary approach are the core aspects in the management of Pygopagus conjoined twins. Early elective separation versus delayed separation of the conjoined twins improves the survival from 40% to 80% . Preoperative assessment of sickle cell disease, cross-circulation and organ sharing between twins proved valuable for planning sedation and analgesia and allowing detailed scripting of the complex anesthetic induction sequence, airway management, complex neurological monitoring and post operative intensive care related issues. Fusion of lower back, spinal cord , nerve roots , and rectal and genitourinary tract was a major surgical challenge related to potentially adverse neurological outcomes, bladder /bowel dysfunction and intact long term survival. Post operative critical care issues such as pain , prone ventilation, sickle cell sequalae, cerebrospinal fluid leak and meningitis, wound infection and colostomy care, nutrition, and other potential complications are discussed in detail in this report. We emphasize the role of pediatric intensive care, preoperative planning with multidisciplinary team, and staged surgical intervention and a good postoperative care in the successful management of complicated variants.