Diverse manifestations and management options in Klippel-Trenaunay syndrome: A single centre 10-year experience

Abstract

Klippel-Trenaunay syndrome is a well-known conglomeration of capillary malformations, bony or soft tissue hypertrophy, and abnormal deep or superficial veins. Although it generally presents with grossly enlarged limbs, it can present with more serious features like haematuria, haematochezia, and seizures. This retrospective study included patients admitted with the diagnosis of Klippel-Trenaunay syndrome in this institute from 2001-2010. The patients' demographic data, clinical features, associated findings, and treatments given were tabulated. A total of 19 patients were included in the study. Two patients presented with haematocezia and had to undergo bowel resection. Five presented with bleeding and ulceration. Debulking surgery was done in three of them. Patients also presented with abdominal distension, jaundice, seizures, and haematuria. Although the common presentation of varicose veins was treated with sclerotherapy, the treatment was tailored to each patient. Klippel-Trenaunay syndrome is a multifaceted disorder which can manifest in a number of different ways. These features may be missed by an unwary plastic surgeon treating them only for the limb hypertrophy.

Internal venous anomalies in patients with a genital venous malformation

“…3,5,6 History and physical examination are usually sufficient for diagnosis. 3 Because patients with Klippel-Trenaunay syndrome have cutaneous VMs and internal vascular anomalies, 7,8 we posited that patients with a focal genital VM may also have internal findings.…”

A Rare Case of Primary Upper Extremity Varicose Veins

“…Congenital arteriovenous fistulas are associated with cutaneous hemangiomas and may be diffused or localized. 6,7 In the secondary type of varicose veins, there will be underlying precipitating causes. Secondary varicose veins can be due to venous outflow obstruction as a result of deep vein thrombosis or arteriovenous fistulae.…”

Wound-Healing Problems Associated with Combined Vascular Malformations in Klippel–Trenaunay Syndrome

“…Wound complications in KTS include skin ulcers and/or bleeding from the cutaneous vascular le-sions, 21,22,[41][42][43]76 and wound healing problems after surgery have also been reported. 31,[66][67][68][69] Various options for local treatment of skin ulcers in KTS patients have been reported, including negative pressure wound therapy, 77 low-level laser therapy, 78 and topical application of recombinant human platelet-derived growth factor-BB.…”