Differential diagnosis in congenital vascular-bone syndromes.
Congenital vascular-bone syndrome is an alteration in limb growth caused by congenital vascular malformations in childhood. A precise study of the anatomic and hemodynamic nature of the underlying defect is necessary, not only for diagnosis, but also for therapy. The use of old eponyms for diagnosis, such as "Klippel-Trenaunay," "Parkes-Weber," and "Servelle-Martorell" should be abandoned because they are meaningless and misleading. An anatomic/pathological categorization, according to the simple "Hamburg classification," is more practical for clinical purposes. Seven different types of vascular defects, all associated with a-v fistulas, are related with limb overgrowth. Four different types of vascular malformations may produce limb shortening. Complete diagnostic study should allow classifying the malformation into one of the groups listed and illustrated above. Precise diagnosis is useful because interventional therapy can correct limb length discrepancy if performed before epiphyseal closure.
Mattassi
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Center for Diagnosis and Treatment of Vascular Malformations, Hospital Santa Corona, Garbagnate Milanese, Italy.
D000293:Adolescent; D000328:Adult; D001164:Arteriovenous Fistula / Q000150:complications; D001165:Arteriovenous Malformations / Q000150:complications / Q000175:diagnosis*; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007866:Leg / Q000098:blood supply*; D007870:Leg Length Inequality / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D008297:Male; D008875:Middle Aged