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posterieure amorfe corneadystrofie (aandoening)
posterieure amorfe corneadystrofie
Posterior amorphous corneal dystrophy
Posterior amorphous stromal dystrophy
A very rare form of stromal corneal dystrophy with characteristics of irregular amorphous sheet-like opacities in the posterior corneal stroma and in the Descemet membrane along with mildly impaired vision. Prevalence of this form of corneal dystrophy is not known. To date cases have been reported primarily in the USA. Patients usually develop corneal abnormalities in infancy or childhood. The condition is non-progressive or slowly progressive. Unlike other corneal dystrophies, non-corneal manifestations have been observed and include abnormalities of the iris including iridocorneal adhesions, corectopia, and pseudopolycoria. An autosomal dominant pattern of inheritance has been reported.
Id719296002
StatusPrimitive
Associated morphologydystrofie
Finding sitestructuur van substantia propria corneae
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetH18.5
TermHereditaire corneadystrofie├źn
SNOMED CT to Orphanet simple map98971
SNOMED CT to ICD-10 extended map
TargetH18.5
RuleTRUE
AdviceALWAYS H18.5
CorrelationSNOMED CT source code to target map code correlation not specified