by Jose Almeida, MD

What type of venous disease did your patient have?
Klippel-Trenaunay syndrome is the well-worn eponym for capillary-lymphatic-venous malformation (CLVM), which is associated with soft tissue/skeletal hypertrophy, usually of one or more limbs and sometimes the trunk. CLVM is thought to be sporadic; it is obvious at birth. The capillary malformations (CM) are multiple, sometimes contiguous, and disposed in a characteristic geographic pattern over the lateral side of the extremity, buttock, or thorax. The CM component is macular in a newborn, but in time, stained areas become studded with hemolymphatic vesicles. Anomalous lateral veins become increasingly prominent because of incompetent valves and deep venous anomalies. Lymphatic hypoplasia is present in more than 50% of patients, and there can be lymphedema or isolated lymphatic macrocysts. Limb hypertrophy also is obvious at birth; axial overgrowth can be progressive in childhood. Pelvic involvement commonly is seen with CLVM in the lower extremity, although usually asymptomatic. Hematuria, hematochezia, constipation, bladder outlet obstruction, and recurrent infection with gut flora can occur, however. Thrombophlebitis occurs in 20% to 45% of patients with CLVM, and pulmonary embolism has been reported in 4% to 25% of patients.

What was your patient's history with the disease?
The patient came to me when she was age 16 - she is currently 21 years old. She has had this condition since birth, and had many of the usual complications of the disease prior to seeing me. She had recurrent lower extremity ulceration, bleeding, and superficial thrombophlebitis. Other physicians she saw had nothing to offer. She was referred to me for endovenous laser ablation therapy.

What treatments did you use?
The disease is incurable. Her disease would be considered severe. The cosmetic issue-was the least of her problems. The best I hoped for was control of her bleeding and healing of her ulcers. I chose minimally invasive treatment over traditional surgery. Initial treatment focused on control of her large straight incompetent superficial veins with endovenous laser ablation. Currently, she wears elastic compression hose daily and comes every 3 months for ultrasound guided sclerotherapy to control her varicosities. She has done well.

What were the results of your treatments?
Luckily she has tolerated all procedures well with no complications. Her ulcers recur about once per year and we use non-elastic compression until she heals. The cautionary note here is to ensure a patent, functioning deep venous system prior to treatment. This often requires venography to better understand the often complex anatomy. Deep venous atresia would preclude superficial vein ablation.

We have successfully treated other Klippel-Trenaunay patients with less severe disease- and the only pre operative imaging required was duplex ultrasound. The results in these cases have been excellent.

What type of prevention do you recommend for this disease?
This disease is congenital and cannot be prevented. Females afflicted with this disease should be counseled on having children – the pregnancy will exacerbate the condition. My patient gave birth to a normal child at age 19, and we controlled her legs during the pregnancy with tight compression. All patients need lifelong follow-up.

A board certified vascular surgeon, Jose Almeida, MD, FACS, RVT, is medical director and founder of the Miami Vein Center in Florida. He is the author of numerous published abstracts, articles and textbook chapters on vascular surgery. He is also a frequent speaker, panelist and workshop director at medical conventions throughout the U.S.