Vascular congenital malformations and the associated consequences commonly present with skin abnormalities including varicosities, leg ulcers and soft tissue hypertrophy.1 This point is illustrated here.
We present the case of a 67-year-old male patient with a 2 year history of recalcitrant leg ulcer overlying an extensive vascular malformation and varicosities. Clinical examination revealed asymmetric hypertrophy of the upper and lower extremities, an extensive geographic port wine stain which severely affected the face, trunk, both legs, arms, as well as prominent venous varicosities of both legs and truncal abdominal veins (fig 1). Upper and lower limb measurements on the right side were larger than those on the left side (fig 2). The chronic wound was diagnosed as ulceration due to venous stasis and lymphoedema (fig 3). Magnetic resonance imaging scans of both legs confirmed the presence of a venous–lymphatic vascular malformation involving the whole subcutis. These features suggest Klippel–Trenauny syndrome (KTS).
Figure 1.
Clinical examination revealed asymmetric hypertrophy of the upper and lower extremities, an extensive geographic port wine stain which severely affected the patient’s face, trunk, both legs, arms, as well as prominent venous varicosities of both legs and trunk abdominal vein.
Figure 2.
Right hands and feet showed striking palmoplantar hypertrophy, and macrodactyly. Right arm is 2 cm longer than left, and right palm size is 4 cm larger than left. Width and length of the right leg are both 1 cm longer than the left, and the right foot is 1 cm longer than the left.
Figure 3.
The chronic wound was diagnosed as venous stasis ulceration.
Evaluation and management of KTS should be individualised and depends on the age of the patient and any additional signs or symptoms.2 Non-surgical treatment in patients with KTS includes compression therapy (such as with elastic support) of the limb (particularly to try to heal leg ulceration), antibiotics when skin infection is present, possibly diuretics for oedema of lower limbs, and anticoagulants if deep vein thrombosis occurs.3
Footnotes
Competing interests: none.
Patient consent: Patient/guardian consent was obtained for publication
REFERENCES
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