Four Rare Pediatric Cases of Klippel–Trenaunay Syndrome with Scoliosis, Bilateral Nevus of Ota, Acroangiodermatitis, and Lymphangioma Circumscriptum

Klippel–Trenaunay syndrome (KTS) is a rare phakomatosis or neurocutaneous syndrome characterized by a triad of cutaneous and visceral hemangiomas, venous varicosities, and soft-tissue or bone hypertrophy. The exact etiology remains unclear, but multiple genetic mutations have been associated with KTS. Recently, the PIK3CA gene has been implicated in its pathogenesis. Here, we report four unique cases of KTS with scoliosis in one case, bilateral nevus of Ota in the second, acroangiodermatitis and scoliosis in the third, and lymphangioma circumscriptum in the fourth.