The condition should be considered a sporadic event. APWS was first described in a German publication by Fegeler in 1949, hence the term Fegeler syndrome. The etiology and pathogenesis of KTS are not well understood and remain obscure. Port wine stains (PWS, aka nevus flammeus, nevus simplex, salmon patch) are cutaneous capillary malformations that are characteristically congenital (CPWS) but may be acquired (APWS). Measure the length and girth of the affected extremity during the initial examination and at every subsequent visit. 5 When examining a newborn with an extensive vascular malformation involving a limb, check for asymmetric growth, which is often present at birth. However, hypotrophy of the body part can occur. 4 The growth affects either bone or soft tissue or both and can encompass an entire limb or be isolated to a single digit. 3 Limb hypertrophy is usually evident, although subtle, at birth and is progressive, possibly because of increased blood flow. 2 Varicosities may not be apparent until the infant is ambulatory. The vascular nevus is present at birth, is widespread, and usually involves the trunk and one extremity. A vascular nevus, varicosities, and ipsilateral hypertrophy are all part of Klippel-Trenaunay syndrome (KTS), first described by Klippel and Trenaunay 1 in 1900. Pulses and blood pressures were normal in all extremities. The infant had no respiratory distress and no heart murmur. Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. The baby had been delivered vaginally at term. Measurement of the mid thigh circumference and mid calf circumference of both legs also demonstrated hypertrophy of the left limb. He was also noted to have subtle hypertrophy of the left lower extremity: his left foot was about 0.5 cm wider and longer than the right ( B), and his left leg was 0.75 cm longer than the right. This newborn was noted to have an impressive nevus flammeus on the trunk and left lower extremity ( A).
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