ICD-11 classes
20 Developmental anomalies
Multiple developmental anomalies or syndromes
LD26 — Syndromes with limb anomalies as a major feature
LD26.6 — Congenital vascular bone syndromes
LD26.60 — Angio-osteohypertrophic syndrome

ICD-11 LD26.60 — Angio-osteohypertrophic syndrome

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.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

• Complex or combined developmental vascular malformations involving the skin #16131
• Complex or combined developmental vascular malformations involving the skin #16591
• Congenital vascular bone syndromes #18082
• Complex or combined developmental vascular malformations involving the skin #23185
• Syndromes with vascular anomalies as a major feature #23221