A unique approach in the management of external vena iliaca hypoplasia in a patient with Klippel-Trenaunay Syndrome: case report

Klippel-Trenaunay syndrome (KTS) is a rare and complex malformation. It shows the typical triad of vascular malformation, bone and soft tissue hypertrophy and venous varicosis. The system manifestations are observed in an individually variable pattern. However, isolated congenital hypoplasia of the venous system rarely occurs. For this reason, early detection plays an important role in further treatment planning. This makes it possible to avoid a severe course of disease or late sequelae by timely treatment. Isolated congenital hypoplasia is extremely rare and predisposes to pelvic or leg vein thrombosis, especially in young adults. With this disease, surgical reconstruction is rarely performed. We describe a very rare case of a 60-year-old woman with KTS who received a venous crossover bypass (Palma procedure) 28 years ago using left graft saphenous vein with hypoplasia of the right external iliac vein. The clinical presentation, diagnostic process, and approach to management along with a literature review on the operative management are presented in this case report.