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Martinique crinkled retinal pigment epitheliopathy (disorder)
Martinique crinkled retinal pigment epitheliopathy
MCRPE - Martinique crinkled retinal pigment epitheliopathy
A rare genetic retinal disease with characteristics of dried-out soil pattern of the fundus due to diffuse deep white lines in the macula, to the level of the retinal pigment epithelium, which is slightly elevated and rippled. Macular exudation may be associated and Bruch's membrane may be affected too. Occasionally, peripheral nummular pigmentary changes may be observed, associated with blindness. The lesions enlarge with time, with a preferential macular extension and confluence. Complications may include polypoidal choroidal vasculopathy, choroidal neovascularization or atrophic fibrous macular scarring that can lead to reduced visual acuity over time.
Id1187639002
StatusPrimitive
Associated morphologydystrofie
Finding sitestructuur van macula lutea
SNOMED CT to Orphanet simple map466718
SNOMED CT to ICD-10 extended map
TargetH35.5
RuleTRUE
AdviceALWAYS H35.5 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified