Abstract
The aim of this study is to report the results of intensive therapy of lymphedema associated with Klippel-Trenaunay syndrome. A 24-year-old female patient reported that her family had observed edema in her right leg and port wine stains from birth. For ten years, they consulted with different specialists in the region but the prognosis did not change and no specific treatment was found. In 2014, at the age of 24, with massive lymphedema, a leg ulcer, and recurrent infections, she started treatment at the Clínica Godoy in São José do Rio Preto. She was evaluated by clinical history, physical examination, water displacement volumetry, and bioimpedance. Intensive therapy (8 hours daily) was proposed using Manual Lymphatic Therapy (Godoy & Godoy), Cervical Stimulation Therapy, Mechanical Lymphatic Therapy, a grosgrain stocking adjusted several times a day, and the use of Unna boot in the region of the ulcer. The volume of edema was reduced by about 44% within the first week with further reductions in the following weeks and healing of the ulcer. Subsequently, it was possible to control and maintain the reduction in swelling with less intense treatment. It is possible to reduce and maintain the treatment results of lymphedema associated with Klippel-Trenaunay syndrome.
Highlights
Klippel-Trenaunay syndrome is congenital angiodysplasia characterized by venous and lymphatic malformations, hypertrophy of the bone, and soft tissues, which, in most cases, affect just one extremity [1]
Her parents consulted with a specialist who arrived at a diagnosis of Klippel-Trenaunay syndrome and told the parents that the prognosis was bad with no prospect of normal motor development
We report on a case of elephantiasis associated with Klippel-Trenaunay syndrome in a 24-year-old patient with an untreated ulcer and recurrent erysipelas in which an intensive approach was used to treat the lymphedema
Summary
Klippel-Trenaunay syndrome is congenital angiodysplasia characterized by venous and lymphatic malformations, hypertrophy of the bone, and soft tissues, which, in most cases, affect just one extremity [1]. Lymphedema is a chronic medical condition in which there is an accumulation of water, salts, electrolytes, high molecular weight proteins, and other elements in the interstitial space resulting in dynamic or mechanical changes of the lymphatic system. This condition causes a reduction in the functional and immunological capacity and morphological changes of the system and weight gain [4]. The aim of this case study is to report on the evolution of intensive treatment in lymphedema associated with KlippelTrenaunay syndrome
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