BILATERAL OPTIC ATROPHY - INFANTILE

Infantile Bilateral Optic Atrophy; Bilateral Optic Neuropathy; Idiopathic Bilateral Optic Atrophy; Congenital Optic Atrophy; Pediatric Bilateral Optic Atrophy

Bilateral Optic Atrophy (BOA) is a condition that affects the optic nerve, which carries impulses from the eye to the brain. Characteristics of BOA involve deficits in central vision, difficulties distinguishing contrast, loss of visual acuity, and changes in the color and the structure of the optic disc. BOA occurs in both eyes from time of birth and may cause rhythmic, involuntary eye movements (nystagmus). This disorder may be caused by a number of diseases and conditions, such as tumors of the visual pathways, hypoxia before or shortly after birth, birth trauma, hydrocephalus, heredity, and degenerative diseases. Many children with BOA may have other neurological problems such as seizures, developmental delays or motor problems.

Diagnostic testing:

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  Neuroimaging;

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  Electroretinogram; or

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  VER testing.

Physical findings:

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  Diminished visual acuity;

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  Inability to fixate and follow;

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  Abnormal pupil reaction to light; and

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  Evidence of nystagmus.

ICD-9: 377.10

BOA symptoms may occur suddenly or process gradually depending on the cause. Visual acuity may range from nearly normal to blindness.

There is no cure for BOA. Treatment is symptomatic and based on the primary cause of this disorder (i.e. tumors may be surgically removed).

Suggested MER for Evaluation:

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  Clinical history and examination that describes the diagnostic features and physical findings;

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  Evidence measuring best corrected visual acuity or the extent of visual fields;

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  If the child is unable to use the Snellen methodology or other comparable testing, documentation of fixation and visual following behavior; and

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  Reports of anatomical findings or results of neuroimaging, electroretinogram, or VER testing.

Suggested Listings for Evaluation:

REMARKS

Listing level severity must be documented.

Listing level severity must be documented.

Listing level severity must be documented.