Klippel-Trenaunay Syndrome of the Extremities- A Report of Two Cases

Abstract

Klippel-Trenaunay Syndrome (KTS) is a rare disorder, with an incidence of 1 in 100,000 people worldwide. The hallmark features of this syndrome include venous malformations, capillary malformations, and soft tissue hypertrophy. There is no gender or racial predilection and it manifests in childhood. It usually involves one limb, predominantly the lower limb. But, in rare incidences, upper limb involvement is also reported. The common presentation of KTS is pain with spontaneous cutaneous haemorrhage, soft tissue enlargement, venous thrombosis, leg length discrepancy, cortical thickening, macular patches and phleboliths. The present case report described two patients (both females) with KTS, who visited the hospital. One of them was an adult female, presented with left lower limb swelling. Computed Tomography (CT) showed, multiple dilated lower limb veins, draining into the internal iliac and deep femoral veins. Following this, the patient had undergone one cycle of Ultrasound Guided Sclerotherapy (UGS) and was advised to follow-up. The patient underwent subsequent two cycles of UGS at an interval of three months. Another patient was of the paediatric age group, presented with swelling and pain in the left lower limb. Magnetic Resonance Imaging (MRI) revealed, multiple veno-capillary malformations with a Persistent Sciatic Vein (PSV). Liposuction of the bilateral gluteal region was performed with the application of topical Neosporin and was advised to follow-up. After five months, Ultrasonography (USG) of the bilateral gluteal region was done, which was normal with no obvious residual disease.