Clinical Experience of the Klippel-Trenaunay Syndrome

Abstract

BackgroundThe Klippel-Trenaunay syndrome (KTS) is characterized by three clinical features, namely cutaneous capillary malformations, venous malformations, and soft tissue and/or bony hypertrophy of the extremities. The varied manifestations are attributed to the unpredictable clinical nature and prognosis of the syndrome. To elucidate the clinical characteristics of this disease, we reviewed a relatively large number of KTS patients who presented to our vascular anomalies center.MethodsWe conducted a retrospective study with 19 patients who were diagnosed with KTS and treated in our vascular anomalies clinic between 2003 and 2014, and examined their demographic characteristics, their clinical features, and the treatments administered.ResultsThe sex distribution was balanced, with 9 (47%) males and 10 (53%) females. The mean follow-up period was 4.1 years (range, 7 months-9 years). Most of the patients received conservative treatments such as medication or physiotherapy. Compression therapies such as wearing of elastic garments/bandages were also administered, and surgical interventions were considered only when the patients became excessively symptomatic. Other treatments included laser therapy and sclerotherapy, and all the treatments were adjusted according to each case, tailored to the conditions of the individual patients.ConclusionsKTS is an extremely rare, multifactorial disorder that induces widely varied symptoms. Because of this unique feature, plastic surgeons, when not careful, tend to attach a one-sided importance to typical symptoms such as limb hypertrophy or capillary malformation and thus overlook other symptoms and clinical features. KTS can be suspected in all infants who show capillary malformations or limb hypertrophy and require a multi-disciplinary approach for comprehensive management.

Chronic foot ulcer caused by Parkes Weber syndrome

“…Most of these patients are already diagnosed in childhood or puberty (8,9). In contrast, in our patient's case, the disease did not present itself until the age of 62 years.…”

Medical treatment of a female patient with complicated Klippel–Trenaunay syndrome

“…5 It is thought to occur sporadically, with an incidence rate of approximately 1 per 100,000 live births, and carries no predilection for a particular sex or race. 7 Because no AVM was noted in our case, KTS was easily distinguished from ParkereWeber Syndrome, in which an enlarged extremity occurs due to being related to an underlying AVM.…”

Manifestações musculoesqueléticas na síndrome de Klippel-Trenaunay

“…1 Na maioria dos casos tanto os hemangiomas como as veias varicosas podem estar presentes ao nascimento, e geralmente ficam mais proeminentes até a adolescência. 2 Alterações linfáticas são observadas em 70% dos pacientes. As anormalidades venosas caracterizam-se por: agenesia, hipoplasia, atresia, incompetência valvular e oclusão do sistema venoso profundo secundária à fibrose.…”