Diagnosis and treatment of Parkes Weber syndrome: a review of 10 consecutive patients.

ABSTRACT

BACKGROUND: We sought to retrospectively analyze the clinical presentation, diagnosis, treatment, and outcomes of patients with Parkes Weber syndrome (PWS) who were treated at a single institution. METHODS: A retrospective review was conducted of medical records of all patients with PWS treated at La Paz Children's Hospital between 1994 and 2010. RESULTS: Ten patients (median age, 14.8 years [range, 2-52 years]) were identified, including 7 women and 3 men. Six patients presented with lower limb hypertrophy and capillary malformation at birth, and both the right and left lower extremities were equally involved. Severe tricuspid insufficiency was observed in 1 patient. The median dysmetria between both lower extremities was 2.19 cm. Four patients are being treated successfully with compression garment therapy. Three patients underwent resection of multiple arteriovenous nidus. Three patients had palliative embolizations. One patient required above the knee amputation secondary to ischemia and chronic severe pain. CONCLUSIONS: The early recognition of PWS is required to establish the most appropriate treatment and prevent short-term morbidity and unnecessary invasive diagnostic tests. Treatment should be individualized according to the age and clinical features of each patient. Although initial conservative management is recommended, surgery continues to play an important role in order to improve the quality of life in these patients.