9B70

ICD-11 MMS 2026-01

Inherited retinal dystrophies

Index terms

  • Inherited retinal dystrophies
  • hereditary retinal dystrophies
  • Amaurosis - hypertrichosis
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  • Autosomal dominant late-onset retinal degeneration
  • Bothnia retinal dystrophy
  • Västerbotten retinal dystrophy
  • Familial flecked retinopathy
  • Familial drusen
  • Kandori fleck retina
  • Retinitis punctata albescens
  • Helicoid peripapillary chorioretinal degeneration
  • Atrophia areata
  • Sveinsson chorioretinal atrophy
  • Leber congenital amaurosis
  • Leber amaurosis
  • MORM syndrome
  • Intellectual deficit - truncal obesity - retinal dystrophy - micropenis
  • Mental retardation - truncal obesity - retinal dystrophy - micropenis
  • Progressive bifocal chorioretinal atrophy
  • Stationary retinal dystrophies
  • Oguchi disease
  • Fundus albipunctatus
  • Achromatopsia, complete
  • Achromatopsia, incomplete
  • Blue cone monochromatism
  • Oligocone trichromacy
  • X-linked cone dysfunction syndrome with myopia
  • Bornholm eye disease
  • Progressive retinal dystrophies
  • Cone rod dystrophy
  • Cone-rod type amaurosis congenita – congenital hypertrichosis
  • Progressive cone dystrophy
  • Goldmann-Favre syndrome
  • Retinal dystrophies with systemic associations
  • Senior-Loken syndrome
  • McKusick Kaufman syndrome
  • Macular dystrophies
  • Stargardt disease
  • FFM - [fundus flavimaculatus]
  • Fundus flavimaculatus
  • Best disease
  • Vitelliform macular dystrophy
  • Pattern Dystrophies of the RPE
  • Vitreoretinal dystrophy
  • Autosomal dominant vitreoretinochoroidopathy
  • Snowflake vitreoretinal degeneration
  • Pigmentary retinal dystrophy
  • Retinitis pigmentosa and intellectual deficit due to Xp11.3 deletion
  • Retinitis pigmentosa associated with systemic disease
  • Retinitis pigmentosa not associated with systemic disease
  • Dystrophies primarily involving the retinal pigment epithelium
  • Adult-onset foveomacular vitelliform dystrophy
  • Butterfly-shaped pigment dystrophy
  • Fundus pulverulentus
  • Multifocal pattern dystrophy simulating fundus flavimaculatus
  • Reticular dystrophy of the retinal pigment epithelium
  • Adult vitelliform macula dystrophy
  • adult vitelliform macula dystrophy, NOS, atypical
  • Albipunctate retinal dystrophy
  • Lauber disease
  • Behr disease
  • Congenital or hereditary macular degeneration
  • macular heredodegeneration
  • Familial juvenile macular degeneration syndrome
  • Congenital retinal blindness
  • CRB - [congenital retinal blindness]
  • Hereditary retinal degeneration
  • hereditary cerebroretinal degeneration
  • peripheral hereditary retinal degeneration
  • Juvenile hereditary retinal degeneration
  • Malattia Leventinese
  • Pigmentary degeneration of retina
  • RP - [retinitis pigmentosa]
  • pigmentary retinal degeneration
  • pigmentary retinopathy
  • primary pigmentary degeneration of retina
  • retinal depigmentation
  • retinal pigment degeneration
  • retinopathia pigmentosa without sectoral
  • Tapetoretinal degeneration
  • Tapetoretinal dystrophy
  • Vitelliform retinal dystrophy
  • vitelliform dystrophy

Inclusions

  • Leber congenital amaurosis
  • Stargardt disease
  • Vitreoretinal dystrophy

Exclusions from above levels

  • 01 – Certain infectious or parasitic diseases
  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth and the puerperium
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  • 19 – Certain conditions originating in the perinatal period
  • 22 – Injury, poisoning or certain other consequences of external causes
  • 8A21.0 – Posterior cortical atrophy

Foundation children elsewhere

  • 5C52.03 – Sjögren-Larsson syndrome
  • LD24.B1 – Asphyxiating thoracic dystrophy
  • LD2H.4 – Usher syndrome

Sibling codes

  • 9B71 – Retinopathy
  • 9B72 – Inflammatory diseases of the retina
  • 9B73 – Retinal detachments or breaks
  • 9B74 – Retinal vascular occlusions
  • 9B75 – Macular disorders
  • 9B76 – Degenerative high myopia
  • 9B77 – Eales disease
  • 9B78 – Certain specified retinal disorders
  • 9B7Y – Other specified disorders of the retina
  • 9B7Z – Disorders of the retina, unspecified

Foundation entity

Inherited retinal dystrophies – http://id.who.int/icd/entity/1060480722

Allowed postcoordination axes

Part of: 09: Diseases of the visual system