Angio-osteohypertrophic syndrome

Description

Angio-osteohypertrophic (AOH) syndrome is a congenital vascular bone syndrome characterised by the presence of vascular malformations in a limb resulting in limb overgrowth. Depending on whether the malformations are slow flow venous or fast flow arteriovenous the syndrome may be divided into two subtypes, Klippel-Trénaunay and Parkes-Weber syndromes respectively. Some cases of the latter are associated with mutations in the RASA1 gene.

Index terms

Angio-osteohypertrophic syndrome
Haemangiectatic hypertrophy
Klippel-Trénaunay syndrome

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Klippel-Trenaunay-Weber syndrome
Parkes-Weber syndrome

Exclusions from above levels

http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

LD26.6Y – Other specified congenital vascular bone syndromes
LD26.6Z – Congenital vascular bone syndromes, unspecified

Foundation entity

Angio-osteohypertrophic syndrome – http://id.who.int/icd/entity/1561120378

Part of: LD26.6: Congenital vascular bone syndromes › LD26: Syndromes with limb anomalies as a major feature › 20: Developmental anomalies