H47.22
BillableHereditary optic atrophy
Hereditary optic atrophy
Coding Notes
Inclusion Terms
Alternative clinical terms for this condition
- Leber's optic atrophy
Excludes 2
Conditions not included here, but the patient may have both
- •certain conditions originating in the perinatal periodP04-P96
- •certain infectious and parasitic diseasesA00-B99
- •complications of pregnancy, childbirth and the puerperiumO9A)O00
- •congenital malformations, deformations, and chromosomal abnormalitiesQ00-Q99
- •diabetes mellitus related eye conditions, , ,E09.3, E10.3, E11.3, E13.3
- •endocrine, nutritional and metabolic diseasesE00-E88
- •injury (trauma) of eye and orbitS05
- •injury, poisoning and certain other consequences of external causesS00-T88
- •neoplasmsC00-D49
- •symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classifiedR00-R94
- •syphilis related eye disorders, , ,A50.01, A50.3, A51.43, A52.71
Related Codes(4)
Also Known As / Clinical Terms(325)
SNOMED CT
- Autosomal dominant optic atrophy2065009
- Dominant hereditary optic atrophy2065009
- Pelger-Huet cell15111002
- Pelger-Huët cell15111002
- Hereditary optic atrophy26360005
- Genetic anomaly of leucocyte47986005
- Genetic anomaly of leukocyte47986005
- Genetic leucocyte disorder47986005
- Genetic leukocyte disorder47986005
- LHON - Leber hereditary optic neuropathy58610003
- LHON - Leber's hereditary optic neuropathy58610003
- Leber hereditary optic neuropathy58610003
- Leber optic atrophy58610003
- Leber's optic atrophy58610003
- Axonal neuropathy60703000
- DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy, deafness) syndrome70694009
- DIDMOAD syndrome70694009
- Marquardt-Loriaux syndrome70694009
- Wolfram syndrome70694009
- Pelger Huet anomaly85559002
- Pelger-Huet anomaly85559002
- Pelger-Huët anomaly85559002
- Optic nerve finding106152006
- Second cranial nerve finding106152006
- False anodontia109440005
- HMSN VI128203003
- Hereditary motor and sensory neuropathy type VI128203003
- Hereditary motor and sensory neuropathy with optic atrophy128203003
- Hereditary motor-sensory neuropathy with optic atrophy128203003
- Hereditary motor-sensory neuropathy, type VI128203003
- Hereditary sensory and motor neuropathy, type VI128203003
- Tooth absent234948008
- Tooth missing234948008
- Generalised dystonia425492002
- Generalized dystonia425492002
- ADOA (autosomal dominant optic atrophy) plus715374003
- Autosomal dominant optic atrophy plus syndrome715374003
- Autosomal dominant optic atrophy Kjer type717336005
- Autosomal dominant optic atrophy classic form717336005
- Kjer optic atrophy717336005
- Optic atrophy type 1717336005
- Autosomal dominant optic atrophy and peripheral neuropathy syndrome717975006
- Leber plus disease719430008
- Autosomal dominant optic atrophy and cataract719517009
- Autosomal dominant optic atrophy type 3719517009
- CAPOS (cerebellar ataxia, areflexia, pes cavus, optic atrophy, sensorineural hearing loss) syndrome720634003
- Cerebellar ataxia, areflexia, pes cavus, optic atrophy, sensorineural hearing loss syndrome720634003
- Early-onset X-linked optic atrophy721200000
- Non-Leber type optic atrophy with early-onset721200000
- Optic atrophy type 2721200000
- GAPO (growth retardation, alopecia, pseudoanodontia, optic atrophy) syndrome721843003
- GAPO syndrome721843003
- Growth retardation, alopecia, pseudoanodontia, optic atrophy syndrome721843003
- Severe X-linked intellectual disability Gustavson type722213009
- Congenital atrophy of optic nerve722990003
- Congenital optic atrophy722990003
- SPOAN (spastic paraplegia, optic atrophy, neuropathy) syndrome725139005
- Spastic paraplegia, optic atrophy, neuropathy syndrome725139005
- CAMOS (cerebellar ataxia, mental retardation, optic atrophy, skin abnormalities) syndrome726031001
- CAMOS syndrome726031001
- Cerebellar ataxia, intellectual disability, optic atrophy, skin abnormalities syndrome726031001
- SCAR5 - spinocerebellar ataxia autosomal recessive 5726031001
- BBSOAS - Bosch Boonstra Schaaf optic atrophy syndrome770723007
- Bosch Boonstra Schaaf optic atrophy syndrome770723007
- Optic atrophy, intellectual disability syndrome770723007
- Autosomal recessive optic atrophy OPA7 (optic atrophy type 7)783065004
- Autosomal recessive optic atrophy OPA7 type783065004
- Autosomal recessive optic atrophy type 7783065004
- Autosomal recessive spastic ataxia type 4784347002
- Autosomal recessive spastic ataxia, optic atrophy, dysarthria syndrome784347002
- SPAX 4 - autosomal recessive spastic ataxia type 4784347002
- SPOAN and SPOAN-related disorder789674008
- Spastic paraplegia, optic atrophy, neuropathy and spastic paraplegia, optic atrophy neuropathy-related disorder789674008
- Childhood-onset autosomal dominant optic atrophy838307002
- Autosomal recessive optic atrophy type 6838345001
- Diffuse atrophy of cerebral structure1144427006
- Diffuse atrophy of cerebrum1144427006
- Global brain atrophy1144430004
- Autosomal recessive bilateral optic atrophy1156823006
- Absence of teeth1162783002
- Early-onset progressive diffuse brain atrophy, microcephaly, muscle weakness, optic atrophy syndrome1172593006
- Autosomal recessive isolated optic atrophy1197151003
- Autosomal recessive non-syndromic optic atrophy1197151003
- Auditory neuropathy, optic atrophy syndrome1222649004
- Optic atrophy, ataxia, peripheral neuropathy, global developmental delay syndrome1222655009
- X-linked optic atrophy1231634003
- Autosomal recessive childhood-onset dystonia DYT29 type1236805005
- Childhood-onset generalised dystonia, optic atrophy syndrome1236805005
- Childhood-onset generalized dystonia, optic atrophy syndrome1236805005
- DYT29 - dystonia 291236805005
- MEPAN syndrome1236805005
- Mitochondrial enoyl coenzyme A reductase protein-associated neurodegeneration syndrome1236805005
- SOPH (short stature, optic nerve atrophy, Pelger-Huët anomaly) syndrome1237618009
- Short stature, optic nerve atrophy, Pelger-Huët anomaly syndrome1237618009
- ANOAC (axonal neuropathy, optic atrophy, cognitive deficit) syndrome1260129000
- Axonal neuropathy, optic atrophy, cognitive deficit syndrome1260129000
- Infantile-onset axonal motor and sensory neuropathy, optic atrophy, neurodegenerative syndrome1260129000
- WFS1 - Wolfram syndrome-11285518004
- Wolfram syndrome type 11285518004
- Wolfram syndrome type I1285518004
- WFS2 - Wolfram syndrome-21285519007
- Wolfram syndrome type 21285519007
- Wolfram syndrome type II1285519007
- Bilateral optic atrophy of eyes15632891000119109
- Optic atrophy of bilateral eyes15632891000119109
- Optic atrophy of both eyes15632891000119109
- Bilateral primary optic atrophy344271000119107
- Primary optic atrophy of bilateral eyes344271000119107
- Primary optic atrophy of both eyes344271000119107
- Hereditary bilateral optic atrophy15699081000119101
- Hereditary left optic atrophy15699161000119109
- Hereditary right optic atrophy15699121000119104
- Left primary optic atrophy338501000119109
- Primary optic atrophy of left eye338501000119109
- Primary optic atrophy of right eye332891000119102
- Right primary optic atrophy332891000119102
UMLS
- Atrophies, Hereditary OpticC0029125
- Atrophy, Hereditary OpticC0029125
- Hereditary Optic AtrophiesC0029125
- Hereditary Optic AtrophyC0029125
- Hereditary optic atrophyC0029125
- Hereditary optic atrophy (disorder)C0029125
- Optic Atrophies, HereditaryC0029125
- Optic Atrophy, HereditaryC0029125
- atrophy hereditary opticC0029125
- hereditary optic atrophiesC0029125
- Disease, Leber'sC0917796
- Diseases, Leber'sC0917796
- Hereditary Optic NeuroretinopathiesC0917796
- Hereditary Optic NeuroretinopathyC0917796
- Hereditary optic neuroretinopathyC0917796
- LEBER HEREDITARY OPTIC NEUROPATHYC0917796
- LEBER OPTIC ATROPHYC0917796
- LHONC0917796
- LHON - Leber hereditary optic neuropathyC0917796
- LHON - Leber's hereditary optic neuropathyC0917796
- Leber DiseaseC0917796
- Leber Hereditary Optic AtrophyC0917796
- Leber Hereditary Optic NeuropathyC0917796
- Leber Optic AtrophyC0917796
- Leber Optic Atrophy and DystoniaC0917796
- Leber Optic NeuropathyC0917796
- Leber hereditary optic atrophyC0917796
- Leber hereditary optic neuropathyC0917796
- Leber optic atrophyC0917796
- Leber optic atrophy featuresC0917796
- Leber optic degenerationC0917796
- Leber's DiseaseC0917796
- Leber's DiseasesC0917796
- Leber's Hereditary Optic AtrophyC0917796
- Leber's Hereditary Optic NeuropathyC0917796
- Leber's Optic AtrophyC0917796
- Leber's Optic NeuropathyC0917796
- Leber's diseaseC0917796
- Leber's hereditary optic atrophy neuropathyC0917796
- Leber's hereditary optic neuropathyC0917796
- Leber's optic atrophyC0917796
- Leber's optic atrophy (disorder)C0917796
- Leber's optic neuropathyC0917796
- Lebers DiseaseC0917796
- Lebers Optic NeuropathyC0917796
- Neuropathy, Leber's OpticC0917796
- Neuroretinopathies, Hereditary OpticC0917796
- Neuroretinopathy, Hereditary OpticC0917796
- Optic Atrophy, Hereditary, LeberC0917796
- Optic Atrophy, LeberC0917796
- Optic Atrophy, Leber TypeC0917796
- Optic Atrophy, Leber, HereditaryC0917796
- Optic Neuropathy, Leber'sC0917796
- Optic Neuroretinopathies, HereditaryC0917796
- Optic Neuroretinopathy, HereditaryC0917796
- leber optic atrophyC0917796
- leber's optic atrophyC0917796
Clinical Terms
- Pelger-Huet cell
- Axonal neuropathy
- Leber Optic Atrophy
- Optic Atrophy, Leber, Hereditary
- Leber hereditary optic atrophy
- Kjer optic atrophy
- Leber optic atrophy features
- GAPO syndrome
- Genetic leukocyte disorder
- Diseases, Leber's
- Wolfram syndrome type I
- Optic Atrophy, Leber
- Global brain atrophy
- Autosomal recessive non-syndromic optic atrophy
- Autosomal recessive bilateral optic atrophy
- Autosomal recessive spastic ataxia type 4
- Autosomal dominant optic atrophy
- GAPO (growth retardation, alopecia, pseudoanodontia, optic atrophy) syndrome
- DYT29 - dystonia 29
- Mitochondrial enoyl coenzyme A reductase protein-associated neurodegeneration syndrome
- Primary optic atrophy of both eyes
- Leber's Hereditary Optic Atrophy
- Growth retardation, alopecia, pseudoanodontia, optic atrophy syndrome
- Atrophy, Hereditary Optic
- Marquardt-Loriaux syndrome
- Leber's hereditary optic atrophy neuropathy
- Bilateral primary optic atrophy
- Spastic paraplegia, optic atrophy, neuropathy syndrome
- Congenital atrophy of optic nerve
- atrophy hereditary optic
- Autosomal recessive optic atrophy OPA7 type
- WFS1 - Wolfram syndrome-1
- Leber's disease
- Optic Atrophies, Hereditary
- ANOAC (axonal neuropathy, optic atrophy, cognitive deficit) syndrome
- MEPAN syndrome
- Left primary optic atrophy
- Leber Disease
- SOPH (short stature, optic nerve atrophy, Pelger-Huët anomaly) syndrome
- Cerebellar ataxia, intellectual disability, optic atrophy, skin abnormalities syndrome
- Second cranial nerve finding
- Leber Optic Atrophy and Dystonia
- Neuropathy, Leber's Optic
- Pelger-Huet anomaly
- Optic Neuropathy, Leber's
- Diffuse atrophy of cerebrum
- Hereditary motor-sensory neuropathy, type VI
- Dominant hereditary optic atrophy
- Neuroretinopathies, Hereditary Optic
- Bosch Boonstra Schaaf optic atrophy syndrome
- Leber's hereditary optic neuropathy
- Bilateral optic atrophy of eyes
- Short stature, optic nerve atrophy, Pelger-Huët anomaly syndrome
- Diffuse atrophy of cerebral structure
- Genetic anomaly of leukocyte
- Genetic anomaly of leucocyte
- Childhood-onset generalized dystonia, optic atrophy syndrome
- Genetic leucocyte disorder
- Hereditary left optic atrophy
- Optic atrophy type 1
- Optic atrophy of both eyes
- Pelger-Huët cell
- CAMOS (cerebellar ataxia, mental retardation, optic atrophy, skin abnormalities) syndrome
- Leber optic degeneration
- Leber's Diseases
- Primary optic atrophy of left eye
- Optic Atrophy, Leber Type
- False anodontia
- Autosomal recessive spastic ataxia, optic atrophy, dysarthria syndrome
- LHON - Leber hereditary optic neuropathy
- SPOAN (spastic paraplegia, optic atrophy, neuropathy) syndrome
- Pelger-Huët anomaly
- Optic atrophy, intellectual disability syndrome
- Autosomal recessive optic atrophy type 6
- DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy, deafness) syndrome
- CAPOS (cerebellar ataxia, areflexia, pes cavus, optic atrophy, sensorineural hearing loss) syndrome
- Hereditary sensory and motor neuropathy, type VI
- Autosomal dominant optic atrophy plus syndrome
- Hereditary optic atrophy (disorder)
- SPAX 4 - autosomal recessive spastic ataxia type 4
- Hereditary Optic Atrophies
- Wolfram syndrome type 1
- Lebers Optic Neuropathy
- Autosomal recessive childhood-onset dystonia DYT29 type
- WFS2 - Wolfram syndrome-2
- Optic atrophy of bilateral eyes
- Wolfram syndrome type 2
- leber's optic atrophy
- LHON
- LHON - Leber's hereditary optic neuropathy
- Tooth missing
- Wolfram syndrome
- Leber's Optic Neuropathy
- Childhood-onset autosomal dominant optic atrophy
- Right primary optic atrophy
- Absence of teeth
- Auditory neuropathy, optic atrophy syndrome
- Optic atrophy, ataxia, peripheral neuropathy, global developmental delay syndrome
- Hereditary right optic atrophy
- Wolfram syndrome type II
- HMSN VI
- Leber hereditary optic neuropathy
- Lebers Disease
- Hereditary motor-sensory neuropathy with optic atrophy
- Severe X-linked intellectual disability Gustavson type
- Optic Atrophy, Hereditary
- X-linked optic atrophy
- Primary optic atrophy of right eye
- Autosomal dominant optic atrophy and cataract
- Hereditary optic neuroretinopathy
- Autosomal dominant optic atrophy classic form
- Optic Neuroretinopathies, Hereditary
- Childhood-onset generalised dystonia, optic atrophy syndrome
- Disease, Leber's
- BBSOAS - Bosch Boonstra Schaaf optic atrophy syndrome
- ADOA (autosomal dominant optic atrophy) plus
- SCAR5 - spinocerebellar ataxia autosomal recessive 5
- Autosomal dominant optic atrophy type 3
- Generalised dystonia
- Congenital optic atrophy
- Spastic paraplegia, optic atrophy, neuropathy and spastic paraplegia, optic atrophy neuropathy-related disorder
- Cerebellar ataxia, areflexia, pes cavus, optic atrophy, sensorineural hearing loss syndrome
- Hereditary motor and sensory neuropathy with optic atrophy
- Primary optic atrophy of bilateral eyes
- Optic atrophy type 2
- Neuroretinopathy, Hereditary Optic
- Non-Leber type optic atrophy with early-onset
- Leber plus disease
- Autosomal recessive optic atrophy type 7
- Autosomal recessive isolated optic atrophy
- Hereditary motor and sensory neuropathy type VI
- SPOAN and SPOAN-related disorder
- Early-onset X-linked optic atrophy
- Generalized dystonia
- CAMOS syndrome
- Leber's optic atrophy (disorder)
- Autosomal dominant optic atrophy and peripheral neuropathy syndrome
- Atrophies, Hereditary Optic
- Autosomal recessive optic atrophy OPA7 (optic atrophy type 7)
- Axonal neuropathy, optic atrophy, cognitive deficit syndrome
- DIDMOAD syndrome
- Hereditary Optic Neuroretinopathies
- Infantile-onset axonal motor and sensory neuropathy, optic atrophy, neurodegenerative syndrome
- Leber Optic Neuropathy
- Optic Atrophy, Hereditary, Leber
- Early-onset progressive diffuse brain atrophy, microcephaly, muscle weakness, optic atrophy syndrome
- Autosomal dominant optic atrophy Kjer type
- Optic nerve finding
- Pelger Huet anomaly
- Hereditary bilateral optic atrophy
- Tooth absent
- Optic Neuroretinopathy, Hereditary
Frequently Asked Questions
What is the ICD-10 code for hereditary optic atrophy?
The ICD-10-CM code for hereditary optic atrophy is H47.22. The full clinical description is "Hereditary optic atrophy". H47.22 is a billable/specific code that can be used on insurance claims and medical billing.
What does ICD-10 code H47.22 mean?
ICD-10-CM code H47.22 represents “Hereditary optic atrophy”. It is classified under Chapter 7: Diseases of the Eye and Adnexa and is a billable/specific code that can be used on a claim.
Is H47.22 a billable code?
Yes, H47.22 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.
What chapter is H47.22 in?
H47.22 is in Chapter 7: Diseases of the Eye and Adnexa (codes H00-H59).
What SNOMED CT codes does H47.22 map to?
H47.22 maps to 52 SNOMED CT concepts: 715374003, 1260129000, 1162783002, 1222649004, 2065009, and 47 more. SNOMED CT is a clinical terminology used in electronic health records.
What are the UMLS CUIs for H47.22?
H47.22 is linked to 2 UMLS Concept Unique Identifiers: C0029125, C0917796. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.
How does H47.22 relate to ICF functioning codes?
ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like hereditary optic atrophy affect a person's functioning — body functions, activities, participation, and environmental factors. AutoICD provides ICF Core Sets for 12+ conditions and can map clinical text to ICF categories automatically. Browse the ICF directory to explore functioning codes.
What is the ICD-11 equivalent of H47.22?
There is no direct ICD-11 mapping available for H47.22 in the WHO crosswalk tables. This may mean the concept is classified differently in ICD-11. Use the ICD-10 to ICD-11 converter to search for related codes.
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Includes SNOMED Clinical Terms® (SNOMED CT®) used by permission of SNOMED International. Includes content from the UMLS Metathesaurus, courtesy of the U.S. National Library of Medicine.