A Successful Separation of Total Vertical Craneopagus Conjoined Twins in Costa Rica

Abstract

Conjoined twins that are attached at the head are classified as craneopagus twins and they represent an extremely rare congenital malfor - mation. The broad phenotypic variation within this group requires an extensive workup to determine the internal shared anatomy, since a separation surgery may require dividing not only skin and calvarium, but also dura mater, venous circulation and even brain cortex. We present such a case in a 2-year-old male pair of type I total vertical craneopagus twins who were surgically separated successfully at the National Children’s Hospital of Costa Rica. Given the highly complex shared venous morphology, this single surgery required the division of a circumferential dural sinus, a common torcula herophili and abundant secondary venous drainage. To achieve this, novel techniques were implemented to optimize surgical time and blood loss as well as attain an equitable distribution of structures. Despite the high morbidity and mortality associated with this type of surgery, both twins now live with their parents one year later.