title: Capillary-venous malformation in the lower limb.
abstract: Regional capillary malformation of a lower extremity is associated with the overgrowth of bone or soft tissue in several disorders, most commonly Klippel-Trenaunay syndrome and Parkes Weber syndrome. We have observed a subset of patients with a capillary malformation of the leg, minor growth disturbance, and prominent veins. The objective of the current study is to describe a series of patients with regional capillary malformation of the lower extremity in association with phlebectasia. This is a retrospective series of 17 patients diagnosed with capillary-venous malformation of the lower extremity. We excluded patients with clinical or radiographic evidence of lymphatic or arteriovenous malformation. Age, presentation, associated features, radiographic findings, and management were documented. In most patients the capillary malformation covered a large area without sharply demarcated borders. Four patients had one or more discrete, well-defined capillary stains involving less than 5% of the total surface area of the affected lower limb. Prominent veins were most common in the popliteal fossa and on the knee and dorsal foot. Approximately two-thirds of patients had a leg length discrepancy, with the affected leg being longer (n = 6) or shorter (n = 4); in many the affected leg was also slightly larger (n = 8) or smaller (n = 4) in girth. Radiographic imaging showed dilatation of superficial (n = 16), muscular (n = 9), and deep veins (n = 6). We characterize a subset of patients with regional capillary-venous malformation of the lower extremity with prominent veins and minor hypotrophy/hypertrophy that differs from Klippel-Trenaunay syndrome (capillary-lymphatic-venous malformation) but belongs at the minor end of the spectrum of vascular disorders with overgrowth.
authors.affiliation: Dermatology Program, Boston Children's Hospital, Boston, Massachusetts.
authors.forename: Lily C
authors.initials: LC
authors.lastname: Uihlein
authors.forename: Marilyn G
authors.initials: MG
authors.lastname: Liang
authors.forename: Steven J
authors.initials: SJ
authors.lastname: Fishman
authors.forename: Ahmad I
authors.initials: AI
authors.lastname: Alomari
authors.forename: John B
authors.initials: JB
authors.lastname: Mulliken
mesh_terms: D000293:Adolescent; D000328:Adult; D002196:Capillaries / Q000002:abnormalities* / Q000254:growth & development; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007715:Klippel-Trenaunay-Weber Syndrome / Q000145:classification / Q000150:complications / Q000473:pathology*; D007866:Leg / Q000098:blood supply / Q000000981:diagnostic imaging / Q000254:growth & development; D007870:Leg Length Inequality / Q000000981:diagnostic imaging / Q000209:etiology; D008297:Male; D011152:Popliteal Vein / Q000002:abnormalities* / Q000254:growth & development; D011859:Radiography; D012189:Retrospective Studies; D012501:Saphenous Vein / Q000002:abnormalities* / Q000254:growth & development; D054079:Vascular Malformations / Q000145:classification / Q000150:complications / Q000473:pathology*