BILATERAL KLIPPEL TRENAUNAY SYNDROME: A RARE CASE REPORT

Introduction: Klippel Trenaunay is a Neurocutaneous syndrome Mesodermal abnormality syndrome characterized by clinical trial of (1) portwine stain due to capillary malformation, (2) bone and soft tissue hypertrophy; and (3) varicose veins.

Presentation of Case: 57 years old male presented to our hospital in outpatient department with history of asymptomatic port wine stain over right ear, right upper extremity, right side of thorax anteriorly and posteriorly, over both sclera and both legs present since birth with history of varicose veins over left leg and hypertrophy of limbs. We report a rare case of bilateral multi-extremity Klippel Trenaunay syndrome.

Discussion: Klippel Trenaunay Syndrome is a sporadic non-heriditary rare mesodermal abnormality that usually affects a single lower limb. It is characterized by a triad of varicose veins, port wine stain, bony and soft tissue hypertrophy of the affected limb. Klippel and Trenaunay described the association of the three findings in 1900. In minority of patients an upper extremity is affected. Infrequently patients have involvement of both limbs on one side and rarely all 4 extremities will be affected.

Conclusion: Patients with Klippel-Trenaunay syndrome (KTS) should be monitored at least annually and more often if clinically indicated. Stable disease can be followed clinically. KTS is not always a static disease process. Our patient had all the three features of the syndrome and the involvement was bilateral involving multiple extremities, which is rarely seen.