|||
congenitaal primair lymfoedeem van Gordon (aandoening)
congenitaal primair lymfoedeem van Gordon
VEGFC-gerelateerd congenitaal primair lymfoedeem
Congenital primary lymphedema of Gordon
VEGFC-related congenital primary lymphedema
A rare primary lymphedema characterized by bilateral, painless lower limb swelling present at birth. Prominent veins around the ankles and on the dorsa of the feet, dysplastic and upslanting toenails due to edema of the nailbed, and subtle dysmorphic facial features (such as high forehead, hypertelorism, depressed nasal bridge, mild bilateral ear dysplasia, and short neck) have also been described. The degree of lymphatic impairment is milder than in the otherwise clinically similar Milroy disease, as evidenced by slightly less severe lymphedema and significantly more uptake of tracers on lymphoscintigraphy.
Id1222669009
StatusPrimitive
Associated morphologylymfoedeem
Finding sitestructuur van extremiteit
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
SNOMED CT to Orphanet simple map569821
SNOMED CT to ICD-10 extended map
TargetI89.0
RuleTRUE
AdviceALWAYS I89.0 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified