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angio-osteohypotrofiesyndroom (aandoening)
angio-osteohypotrofiesyndroom
flebectatische osteohypoplastische angiodysplasie
Servelle-Martorell-syndroom
syndroom van Servelle-Martorell
Angioosteohypotrophic syndrome
Phlebectatic osteohypoplastic angiodysplasia
Angio-osteohypotrophic syndrome
Servelle Martorell syndrome
A rare congenital vascular anomaly syndrome characterized by venous or on occasion arterial malformations that lead to soft tissue hypertrophy and bone hypoplasia. An affected limb is generally shortened, highly deformed, painful and edematous with associated bone and muscle hypotrophy. Single parts or multiple small parts of limbs are typically affected but more extensive involvement including complete extremity shoulder girdle and axilla have been reported.
Id765750001
StatusPrimitive
Associated morphologymorfologische afwijking
Finding sitestructuur van bloedvat
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
Associated morphologyhypoplasie
Finding sitebotstructuur
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetQ87.5
TermOverige congenitale gestoorde-ontwikkelingssyndromen met overige skeletveranderingen
SNOMED CT to Orphanet simple map75508
SNOMED CT to ICD-10 extended map
TargetQ87.5
RuleTRUE
AdviceALWAYS Q87.5 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified