[Anesthesic management during total organic separation of pygopagus conjoined twins]

Conjoined twins are among the most rare and challenging congenital malformations. The incidence varies from 1:30 000 to 1:200 000 live births. Pygopagus twins account for about 10 to 18% of all conjoined twins.

We report the case of male conjoined pygopagus twins. They shared medullary canal, anal canal and sacrococcygeal region, without any degree of cross circulation. At the moment of the surgery, twin 1 had congenital heart disease without medical treatment. Twin 2 presented craniofacial malformations. The total organic separation was performed electively, by being driven by two teams of anesthesiologists with balanced general anesthesia, intravenous and sequential induction intubation. Non-invasive and invasive monitoring was used for both twins. During the transanesthetic period, twin 1 presented multiple hypercyanotic episodes associated to hypovolemia and hypotension. Twin 2 remained stable. During the final separation acute bleeding and hemodynamic instability showed up, requiring the use of inotropes, vasopressors and blood components. The twins were sent to intensive care where they remained under surveillance for three days. They were discharged a month and a half after the surgical event.

Each type of siamese has specific characteristics depending on the type of union; comprehensive knowledge of the morphology and physiopathology of the twins is essential for optimal anesthetic management.