ICD-11 classes
20 Developmental anomalies
Structural developmental anomalies primarily affecting one...
Structural developmental anomalies of the skin
Developmental anomalies of cutaneous vasculature
LC52 — Complex or combined developmental vascular malformations...
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
- Syndromes with vascular anomalies as a major feature #23221
- Congenital vascular bone syndromes #23318