EXTENSIVE PURE VENOUS MALFORMATIONS IN THE UPPER OR LOWER-LIMB - A REVIEW OF 27 CASES

Background:: Extensive pure venous malformations (VMs) involving the e ntire lower or up per Limb and adjacent trunk form a particular group of rare vascular malformations. Objective: Our purpose was to review 2 7 cases of extensive limb VMs and describe their characteristics and m anagement. Methods: Cases of extensive Limb VMs were investigated, tre ated, and observed for a mean of 7 years. Results: Eleven cases involv ed the upper limbs and 16 involved the lower limbs. All involved skin and muscle, In 81% of cases in the lower limb there was also knee join t involvement that created severe functional impairment. Ultrasonograp hy with Doppler (duplex scans), computed tomographic scans, and magnet ic resonance imaging were helpful noninvasive diagnostic procedures in these patients, whereas arteriography and phlebography were less info rmative. Muscle involvement was present in 100% of patients and bone a bnormalities in 63%. Leg length was either normal or there was slight limb undergrowth, except in three patients who had minor overgrowth of the affected Limb. Coagulation profiles demonstrated localized intrav ascular coagulation in 88% of patients. The majority of patients had c onservative management (elastic stockings). In a few, percutaneous scl erotherapy or partial excision of skin and muscle VMs was beneficial. Knee joint involvement required synovectomy and VM excision during chi ldhood in 7 of 16 patients. Conclusion: Extensive limb VMs are charact erized by diffuse involvement of the skin, muscle, and joints, and by a specific localized intravascular coagulopathy with general consequen ces. This group of vascular malformations should be separated from the Klippel-Trenaunay and Parkes Weber syndromes.