Differential diagnosis in congenital vascular-bone syndromes. 1993

R Mattassi
Center for Diagnosis and Treatment of Vascular Malformations, Hospital Santa Corona, Garbagnate Milanese, Italy.

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.

UI MeSH Term Description Entries
D007866 Leg The inferior part of the lower extremity between the KNEE and the ANKLE. Legs
D007870 Leg Length Inequality A condition in which one of a pair of legs fails to grow as long as the other, which could result from injury or surgery. Inequalities, Leg Length,Inequality, Leg Length,Leg Length Inequalities,Length Inequalities, Leg,Length Inequality, Leg
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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