Abstract
The article presents the Klippel-Trenaunay syndrome. The diagnosis was set upon birth based on typical skin angiomatous nevi and hypertrophy of soft tissue and bones. Later on, the patient also developed the third element of the syndrome, i.e. varicose veins. The patient has a rare form of the disease, with hypertrophy of soft tissue in the left forearm and soft tissue and bones of his right leg. The static type macrodactylia is present on both hands and feet. The paper gives clinical description of this rare, nonhereditary disease. The need of continuous multidisciplinary monitoring of such patients is emphasized, since this syndrome should be distinguished from Parkes Weber syndrome which has essentially different prognosis.
Highlights
What are the classical associations in Klippel-Trenaunay syndrome (KTS)? 2
The disease has a classic triad of capillary malformations, vascular anomalies, and hypertrophy of bony and soft tissues
Low-flow arteriovenous malformations (AVMs) are seen in KTS and have relatively low morbidity, whereas high-flow AVMs are more appropriately assigned as Parkes-Weber syndrome
Summary
1. What are the classical associations in Klippel-Trenaunay syndrome (KTS)? Klippel-Trenaunay syndrome is a rare disease, with estimates of approximately 1 per 30,000 live births.[1] The disease has a classic triad of capillary malformations, vascular anomalies (classically varicosities such as the persistent embryonic lateral vein of Servelle), and hypertrophy of bony and soft tissues. Patients with Parkes-Weber syndrome have similar presentations as those with KTS and can often be indistinguishable on physical examination.
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