Evaluation and Management of Pain in Patients with Klippel-Trenaunay Syndrome: A Review

Abstract

Klippel-Trenaunay syndrome (KTS) is a rare disorder that consists of a triad of capillary vascular malformation, venous malformations and/or varicose veins, and soft tissue and/or bony hypertrophy. Pain is a real and debilitating problem in these patients. We have observed 9 common causes of pain in KTS: (1) chronic venous insufficiency, (2) cellulitis, (3) superficial thrombophlebitis, (4) deep vein thrombosis, (5) calcification of vascular malformations, (6) growing pains, (7) intraosseous vascular malformation, (8) arthritis, and (9) neuropathic pain. The management of pain in patients with KTS depends on its cause. These patients are best evaluated initially in a center with an experienced multidisciplinary team that includes a primary health care provider, surgeons, and ancillary staff. The ongoing care of a patient with KTS often depends on a local provider who is more readily accessible to the patient but may not have the expertise of a large center to manage the complications of KTS. The purpose of this communication is to review the common causes of pain in these patients to provide local health care providers and patients and their families with appropriate management strategies.

Klippel-Trenaunay Syndrome: a case report with brief review of literature

“…Anticoagulant therapy is indicated in acute thrombosis and prophylactically prior to surgical procedures. 19 Surgery is reserved selectively for symptomatic patients with KTS those are not candidate for less invasive treatment. Vascular interventions must be preceded by careful evaluation of the extent of malformations and patency of the deep system.…”

Klippel Trenaunay Sendromu ile birlikte görülen popliteal ven aplazisi: Nadir Bir Olgunun Doppler US, BT Anjiografi ve MR Anjiografi Bulguları

“…Bu kardinal bulgulardan bir ya da ikisine sahip olanlar ise KTS'nin inkomplet formu olarak sınıflanır (2,4). Venöz, kapiller ve kenfatik malformasyonlar sıklıkla bir alt ekstremiteyi, daha çok sağ alt ekstremiteyi tutar (3, 5,6 (14). Aplastik derin venöz sistem varlığı semptomatik venlerin cerrahi tedavisi ya da perkutan skleroterapi için kesin kontraendikasyon olduğundan dolayı her ne kadar nadir de görülse tedavi öncesi araştırılmalıdır (2,3,4).…”

Klippel Trenaunay Syndrome, Inverse Klippel Trenaunay Syndrome: Hypertrophy of Lower Limbs and Atrophy of the Upper Limbs and Facial Muscles: Case Report and Literature Review

“…On the other hand, In some subsets of patients only 2 of the 3 classic findings are present [21]. The clinical presentation of KTS is variable [22]. It ranges from port wine stains and few varicose veins causing cosmetic deformity to severe disability associated with massive limb overgrowths, chronic pain syndrome, skin infections and arthritis [23].…”

“…Anticoagulant therapy is indicated in acute thrombosis and prophylactically prior to surgical procedures [22]. Intraoperative use of tourniquet will decrease bleeding and selective use of an inferior vena cava filter will prevent pulmonary embolism [3].…”