| 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. |
| | Id | 719296002 | | Status | Primitive |
| DHD Diagnosis thesaurus reference set |
| RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set | | Target | H18.5 | | Term | Hereditaire corneadystrofieën |
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| SNOMED CT to Orphanet simple map | 98971 |
| SNOMED CT to ICD-10 extended map | | Target | H18.5 | | Rule | TRUE | | Advice | ALWAYS H18.5 | | Correlation | SNOMED CT source code to target map code correlation not specified |
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