ICD-10-CM Code H47.22

Hereditary optic atrophy

Version 2021 Billable Code

Valid for Submission

H47.22 is a billable code used to specify a medical diagnosis of hereditary optic atrophy. The code is valid for the fiscal year 2021 for the submission of HIPAA-covered transactions. The ICD-10-CM code H47.22 might also be used to specify conditions or terms like 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 optic atrophy type 7, autosomal recessive spastic ataxia, optic atrophy, dysarthria syndrome, etc

ICD-10:H47.22
Short Description:Hereditary optic atrophy
Long Description:Hereditary optic atrophy

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code H47.22:

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

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code H47.22 are found in the index:


Synonyms

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

  • 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 optic atrophy type 7
  • Autosomal recessive spastic ataxia, optic atrophy, dysarthria syndrome
  • Bilateral optic atrophy of eyes
  • CAMOS syndrome
  • Cerebellar ataxia, areflexia, pes cavus, optic atrophy, sensorineural hearing loss syndrome
  • Chronic paraplegia
  • Dominant hereditary optic atrophy
  • Early-onset X-linked optic atrophy
  • GAPO syndrome
  • Hereditary bilateral optic atrophy
  • Hereditary left optic atrophy
  • Hereditary motor and sensory neuropathy with optic atrophy
  • Hereditary optic atrophy
  • Hereditary right optic atrophy
  • Leber plus disease
  • Leber's optic atrophy
  • Optic atrophy of left eye
  • Optic atrophy of left eye
  • Optic atrophy of right eye
  • Optic atrophy of right eye
  • Optic atrophy, intellectual disability syndrome
  • Second cranial nerve finding
  • Spastic paraplegia, optic atrophy, neuropathy syndrome
  • SPOAN and SPOAN-related disorder

Clinical Information

  • OPTIC ATROPHIES HEREDITARY-. hereditary conditions that feature progressive visual loss in association with optic atrophy. relatively common forms include autosomal dominant optic atrophy optic atrophy autosomal dominant and leber hereditary optic atrophy optic atrophy hereditary leber.

Convert H47.22 to ICD-9

  • 377.16 - Hereditary optic atrophy

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)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021

Information for Patients


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.

  • Optic glioma (Medical Encyclopedia)
  • Optic nerve atrophy (Medical Encyclopedia)
  • Optic neuritis (Medical Encyclopedia)

[Learn More]

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]

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]

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]