ABSTRACT
Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by capillary malformation (CM), venous malformation (VM) with or without lymphatic malformation, and limb length and/or circumference discrepancy. In addition to the physical manifestations, many patients with KTS experience acute, subacute, or chronic pain and are burdened with psychiatric and psychosocial issues. Pain has been reported to occur in 38%–88% of patients with KTS. Etiologies include chronic venous insufficiency, cellulitis, lymphangitis, thrombophlebitis, deep vein thrombosis, intraosseous or calcified vascular malformations, phleboliths, arthritis, and neuropathic pain. Psychiatric conditions were reported in 23% of patients with the most common diagnoses being depression and anxiety. Patients experiencing pain are more likely to have a psychiatric diagnosis. Patients with KTS should be screened routinely for symptoms of pain and psychiatric and psychosocial burden. Multidisciplinary clinics are of help, and the patient should be referred to psychiatrists, psychologists, and support groups if needed.
