congenitale pseudoartrose van extremiteit (aandoening)
congenitale pseudoartrose van extremiteit
Congenital pseudoarthrosis of limb
A rare genetic non-syndromic limb malformation with characteristics of delayed union or non-union of a long bone, resulting in formation of a false joint, with abnormal mobility and angulation at the pseudoarthrosis site, which manifests with progressive anterolateral forearm or leg bowing, limb shortening, and non-healing fractures. Typical histopathological findings include fibromatosis-like proliferation in the soft tissues with cystic or dysplastic lesions. Neurofibromatosis and osteofibrous dysplasia are frequently associated.
referentieset met complexe 'mapping' naar ICD-10
AdviceALWAYS Q74.8
CorrelationSNOMED CT source code to target map code correlation not specified