2024 ICD-10-CM Diagnosis Code H47.22

Hereditary optic atrophy

ICD-10-CM Code:
H47.22
ICD-10 Code for:
Hereditary optic atrophy
Is Billable?
Yes - Valid for Submission
Chronic Condition Indicator: [1]
Chronic
Code Navigator:

Code Classification

  • Diseases of the eye and adnexa
    (H00–H59)
    • Disorders of optic nerve and visual pathways
      (H46-H47)
      • Other disorders of optic [2nd] nerve and visual pathways
        (H47)

H47.22 is a billable diagnosis code used to specify a medical diagnosis of hereditary optic atrophy. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2023 through September 30, 2024.

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Absence of teeth
  • Auditory neuropathy, optic atrophy syndrome
  • Autosomal dominant optic atrophy and cataract
  • Autosomal dominant optic atrophy and peripheral neuropathy syndrome
  • Autosomal dominant optic atrophy classic form
  • Autosomal dominant optic atrophy plus syndrome
  • Autosomal recessive bilateral optic atrophy
  • Autosomal recessive isolated optic atrophy
  • Autosomal recessive optic atrophy type 6
  • Autosomal recessive optic atrophy type 7
  • Autosomal recessive spastic ataxia, optic atrophy, dysarthria syndrome
  • Axonal neuropathy
  • Bilateral optic atrophy of eyes
  • Bilateral optic atrophy of eyes
  • Bilateral optic atrophy of eyes
  • Bilateral primary optic atrophy
  • Bilateral primary optic atrophy
  • CAMOS syndrome
  • Cerebellar ataxia, areflexia, pes cavus, optic atrophy, sensorineural hearing loss syndrome
  • Childhood-onset autosomal dominant optic atrophy
  • Congenital atrophy of optic nerve
  • Congenital atrophy of optic nerve
  • Congenital atrophy of optic nerve
  • Congenital atrophy of optic nerve
  • Congenital atrophy of optic nerve
  • Dominant hereditary optic atrophy
  • Dominant hereditary optic atrophy
  • Dominant hereditary optic atrophy
  • Dominant hereditary optic atrophy
  • Early-onset progressive diffuse brain atrophy, microcephaly, muscle weakness, optic atrophy syndrome
  • Early-onset X-linked optic atrophy
  • False anodontia
  • GAPO syndrome
  • Generalized dystonia
  • Genetic anomaly of leukocyte
  • Hereditary bilateral optic atrophy
  • Hereditary left optic atrophy
  • Hereditary motor and sensory neuropathy with optic atrophy
  • Hereditary optic atrophy
  • Hereditary right optic atrophy
  • Infantile-onset axonal motor and sensory neuropathy, optic atrophy, neurodegenerative syndrome
  • Leber plus disease
  • Leber's optic atrophy
  • Maternally inherited mitochondrial deoxyribonucleic acid disease
  • Maternally inherited mitochondrial deoxyribonucleic acid disease
  • MEPAN syndrome
  • Optic atrophy, ataxia, peripheral neuropathy, global developmental delay syndrome
  • Optic atrophy, intellectual disability syndrome
  • Pelger-Huët anomaly
  • Pelger-Huët cell
  • Second cranial nerve finding
  • Second cranial nerve finding
  • Second cranial nerve finding
  • Second cranial nerve finding
  • Severe X-linked intellectual disability Gustavson type
  • Short stature, optic nerve atrophy, Pelger-Huët anomaly syndrome
  • Spastic paraplegia, optic atrophy, neuropathy syndrome
  • SPOAN and SPOAN-related disorder
  • SPOAN and SPOAN-related disorder
  • Tooth absent
  • X-linked optic atrophy

Clinical Classification

Clinical Information

  • Hereditary Optic Atrophy

    a family of inherited disorders characterized by progressive loss of vision secondary to death of the retinal ganglion cell axons that comprise the optic nerve.
  • Leber Hereditary Optic Atrophy

    a hereditary disorder caused by mitochondrial mutations, resulting in the degeneration of the retinal ganglion cells and optic atrophy. it is characterized by an acute or subacute loss of central vision. it may initially affect one eye only, but eventually the central loss of vision becomes bilateral.
  • Acute Motor and Sensory Axonal Neuropathy|Acute Motor And Sensory Axonal Neuropathy|Acute Motor-Sensory Axonal Neuropathy|Acute Motor-Sensory Axonal Neuropathy

    a subtype of guillain-barre syndrome that targets sensory motor axons, and is characterized by acute onset of quadriparesis, distal sensory loss, areflexia, and respiratory insufficiency.
  • Acute Motor Axonal Neuropathy|AMAN

    a subtype of guillain-barre syndrome that targets motor axons, and is characterized by symmetric limb weakness, diffuse areflexia, facial and oropharyngeal muscle weakness, and respiratory insufficiency.
  • Axonal Neuropathy

    any nerve disorder affecting the axon of a nerve.
  • GAN wt Allele|GAN1|Giant Axonal Neuropathy (Gigaxonin) Gene|Gigaxonin wt Allele|KLHL16

    human gan wild-type allele is located in the vicinity of 16q24.1 and is approximately 65 kb in length. this allele, which encodes gigaxonin protein, is involved in both ubiquitination and neurofilament structure. mutation of the gene is associated with giant axonal neuropathy.
  • Giant Axonal Neuropathy

    a rare inherited disorder affecting the neurofilaments. it is caused by mutations in the gan gene. it is characterized by the presence of abnormally large nerve cell axons. signs and symptoms include difficulty walking, sensory disturbances, lack of motor coordination and abnormal reflexes in the limbs.
  • Spinocerebellar Ataxia, Autosomal Recessive, with Axonal Neuropathy 2|AOA2|Ataxia with Oculomotor Apraxia Type 2|SCAN2

    an autosomal recessive condition caused by mutation(s) in the setx gene, encoding probable helicase senataxin. it is characterized by juvenile onset progressive cerebellar ataxia, axonal sensorimotor peripheral neuropathy, and increased concentrations of serum alpha-fetoprotein. oculomotor apraxia is common, but is not always present.

Tabular List of Diseases and Injuries

The following annotation back-references are applicable to this diagnosis code. The Tabular List of Diseases and Injuries is a list of ICD-10-CM codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more.


Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
  • Leber's optic atrophy

Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

Convert H47.22 to ICD-9-CM

  • ICD-9-CM Code: 377.16 - Hereditary optic atrophy

Patient Education


Optic Nerve Disorders

The optic nerve is a bundle of more than 1 million nerve fibers that carry visual messages. You have one connecting the back of each eye (your retina) to your brain. Damage to an optic nerve can cause vision loss. The type of vision loss and how severe it is depends on where the damage occurs. It may affect one or both eyes.

There are many different types of optic nerve disorders, including:

  • Glaucoma is a group of diseases that are the leading cause of blindness in the United States. Glaucoma usually happens when the fluid pressure inside the eyes slowly rises and damages the optic nerve.
  • Optic neuritis is an inflammation of the optic nerve. Causes include infections and immune-related illnesses such as multiple sclerosis. Sometimes the cause is unknown.
  • Optic nerve atrophy is damage to the optic nerve. Causes include poor blood flow to the eye, disease, trauma, or exposure to toxic substances.
  • Optic nerve head drusen are pockets of protein and calcium salts that build up in the optic nerve over time

Contact your health care provider if you are having vision problems. Tests for optic nerve disorders may include eye exams, ophthalmoscopy (an examination of the back of your eye), and imaging tests. Treatment depends on which disorder that you have. With some optic nerve disorders, you may get your vision back. With others, there is no treatment, or treatment may only prevent further vision loss.


[Learn More in MedlinePlus]

Autosomal dominant optic atrophy and cataract

Autosomal dominant optic atrophy and cataract is an eye disorder that is characterized by impaired vision. Most affected individuals have decreased sharpness of vision (visual acuity) from birth, while others begin to experience vision problems in early childhood or later. In affected individuals, both eyes are usually affected equally. However, the severity of the vision loss varies widely, even among affected members of the same family, ranging from nearly normal vision to complete blindness.

Several abnormalities contribute to impaired vision in people with autosomal dominant optic atrophy and cataract. In the early stages of the condition, affected individuals experience a progressive loss of certain cells within the retina, which is a specialized light-sensitive tissue that lines the back of the eye. The loss of these cells (known as retinal ganglion cells) is followed by the degeneration (atrophy) of the nerves that relay visual information from the eyes to the brain (optic nerves), which contributes to vision loss. Atrophy of these nerves causes an abnormally pale appearance (pallor) of the optic nerves, which can be seen only during an eye examination. Most people with this disorder also have clouding of the lenses of the eyes (cataracts). This eye abnormality can develop anytime but typically appears in childhood. Other common eye problems in autosomal dominant optic atrophy and cataract include involuntary movements of the eyes (nystagmus), or problems with color vision (color vision deficiency) that make it difficult or impossible to distinguish between shades of blue and green.

Some people with autosomal dominant optic atrophy and cataract develop disturbances in the function of other nerves (neuropathy) besides the optic nerves. These disturbances can lead to problems with balance and coordination (cerebellar ataxia), an unsteady style of walking (gait), prickling or tingling sensations (paresthesias) in the arms and legs, progressive muscle stiffness (spasticity), or rhythmic shaking (tremors). In some cases, affected individuals have hearing loss caused by abnormalities of the inner ear (sensorineural deafness).


[Learn More in MedlinePlus]

Leber hereditary optic neuropathy

Leber hereditary optic neuropathy (LHON) is an inherited form of vision loss. Although this condition usually begins in a person's teens or twenties, rare cases may appear in early childhood or later in adulthood. For unknown reasons, males are affected much more often than females.

Blurring and clouding of vision are usually the first symptoms of LHON. These vision problems may begin in one eye or simultaneously in both eyes; if vision loss starts in one eye, the other eye is usually affected within several weeks or months. Over time, vision in both eyes worsens with a severe loss of sharpness (visual acuity) and color vision. This condition mainly affects central vision, which is needed for detailed tasks such as reading, driving, and recognizing faces. Vision loss results from the death of cells in the nerve that relays visual information from the eyes to the brain (the optic nerve). Although central vision gradually improves in a small percentage of cases, in most cases the vision loss is profound and permanent.

Vision loss is typically the only symptom of LHON; however, some families with additional signs and symptoms have been reported. In these individuals, the condition is described as "LHON plus." In addition to vision loss, the features of LHON plus can include movement disorders, tremors, and abnormalities of the electrical signals that control the heartbeat (cardiac conduction defects). Some affected individuals develop features similar to multiple sclerosis, which is a chronic disorder characterized by muscle weakness, poor coordination, numbness, and a variety of other health problems.


[Learn More in MedlinePlus]

Optic atrophy type 1

Optic atrophy type 1 is a condition that often causes slowly worsening vision, usually beginning in childhood. People with optic atrophy type 1 typically experience a narrowing of their field of vision (tunnel vision). Affected individuals gradually lose their sight as their field of vision becomes smaller. Both eyes are usually affected equally, but the severity of the vision loss varies widely, even among affected members of the same family, ranging from nearly normal vision to complete blindness.

In addition to vision loss, people with optic atrophy type 1 frequently have problems with color vision (color vision deficiency) that make it difficult or impossible to distinguish between shades of blue and green.

In the early stages of the condition, individuals with optic atrophy type 1 experience a progressive loss of certain cells within the retina, which is a specialized light-sensitive tissue that lines the back of the eye. The loss of these cells (known as retinal ganglion cells) is followed by the degeneration (atrophy) of the nerves that relay visual information from the eye to the brain (optic nerves), which results in further vision loss. Atrophy causes these nerves to have an abnormally pale appearance (pallor), which can be seen during an eye examination.


[Learn More in MedlinePlus]

Code History

  • FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
  • FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
  • FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.