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H47.211

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ICD-10-CM H47.211: Primary Optic Atrophy, Right Eye

Quick reference

ElementValue
ICD-10-CM codeH47.211
Official descriptorPrimary optic atrophy, right eye
Parent categoryH47.21 - Primary optic atrophy; H47.2 - Optic atrophy; H47 - Other disorders of optic nerve and visual pathways
ICD-10-CM chapterH00-H59 (Diseases of the eye and adnexa) → H46-H47 (Disorders of optic nerve and visual pathways)
LateralityRight eye (OD)
Billable✓ Yes (laterality-specific terminal code)
Clinical definitionDegeneration and shrinkage of optic nerve axons without preceding optic disc swelling, resulting in permanent vision loss
PathophysiologyLoss of retinal ganglion cell (RGC) axons with orderly degeneration and gliosis; “primary” = no antecedent disc edema
HCC statusNot a CMS-HCC code (symptom/manifestation; underlying cause may be HCC-relevant)
Chronic conditionYes (irreversible optic nerve damage; permanent visual deficit)
PrognosisPoor; optic atrophy represents end-stage irreversible damage to optic nerve

Short description

H47.211 codes primary optic atrophy of the right eye - permanent degeneration and loss of retinal ganglion cell axons in the right optic nerve without preceding optic disc swelling, resulting in pallor of the optic disc, irreversible vision loss, and visual field defects.

The term “primary” distinguishes this from secondary optic atrophy (which follows disc edema/papilledema) and glaucomatous optic atrophy (which has distinct cupping morphology).

Full description (clinical context)

Pathophysiology of primary optic atrophy

Optic atrophy is a pathological term referring to optic nerve shrinkage caused by degeneration of retinal ganglion cell (RGC) axons.

Primary optic atrophy characteristics:

  • Occurs without any preceding swelling of the optic nerve head (no prior disc edema or papilledema).
  • Caused by lesions in the anterior visual system extending from the RGCs to the lateral geniculate body (LGB).
  • Axons degenerate in an orderly manner with subsequent laying down of columns of glial cells (gliosis).
  • Results in a pale, well-demarcated optic disc with sharp margins and visible lamina cribrosa.
  • Retinal blood vessels remain normal caliber (distinguishes from secondary atrophy where vessels may be sheathed/attenuated).

Contrasted with other optic nerve pathology:

  • Secondary optic atrophy: Follows chronic disc edema/papilledema; disc margins blurred with glial tissue overgrowth; vessels may be sheathed.
  • Glaucomatous optic atrophy: Progressive cupping with neuroretinal rim thinning but remaining rim has fine blood supply; distinct from primary atrophy pattern.
  • Consecutive optic atrophy: Due to retinal/choroidal disease (e.g., retinitis pigmentosa, chorioretinitis).

Common etiologies of primary optic atrophy

Compressive lesions (mass effect on optic nerve):

  • Pituitary tumors (adenoma, craniopharyngioma) compressing optic chiasm.
  • Meningiomas of optic nerve sheath or sphenoid wing.
  • Gliomas of optic nerve (especially in neurofibromatosis type 1).
  • Aneurysms (carotid-ophthalmic, anterior communicating artery).
  • Orbital masses (thyroid eye disease, lymphoma, metastases).

Hereditary optic neuropathies:

  • Dominant optic atrophy (Kjer’s optic neuropathy): Autosomal dominant; OPA1 gene mutation (chromosome 3q28-qter); slowly progressive bilateral vision loss starting in first decade; temporal disc pallor; centrocecal scotomas; mitochondrial dysfunction.
  • Leber’s hereditary optic neuropathy (LHON): Mitochondrial DNA mutations (most common: 11778, 14484, 3460); predominantly young males; acute/subacute bilateral sequential vision loss; eventual optic atrophy with poor prognosis (VA typically ≤20/200).
  • Other hereditary: OPA2-OPA6 variants (X-linked, autosomal dominant, autosomal recessive).

Ischemic optic neuropathies (after acute phase):

  • Anterior ischemic optic neuropathy (AION): Acute painless vision loss with disc swelling initially; evolves to optic atrophy over weeks/months.
    • Non-arteritic AION (NAION): Most common optic neuropathy in patients >50 years; associated with diabetes, hypertension, sleep apnea, cardiovascular disease; typically altitudinal visual field defect.
    • Arteritic AION (AAION): Giant cell arteritis (temporal arteritis); older patients (>70 years); severe vision loss; requires urgent high-dose steroids to prevent fellow eye involvement.
  • Posterior ischemic optic neuropathy (PION): Acute vision loss without initial disc edema; optic atrophy develops subsequently; associated with surgical blood loss, cardiac arrest, severe anemia.

Traumatic optic neuropathy:

  • Direct injury to optic nerve from orbital/skull fractures.
  • Indirect injury from deceleration forces (motor vehicle accidents, falls).
  • Immediate or delayed vision loss.

Toxic/nutritional optic neuropathies:

  • Toxic: Methanol, ethambutol, isoniazid, linezolid, chloramphenicol, digitalis, chloroquine, amiodarone, tobacco-alcohol amblyopia, arsenic, bromine, lead.
  • Nutritional: Vitamin B12 deficiency, folate deficiency, thiamine deficiency (beriberi), malnutrition.
  • Typically bilateral, painless, progressive central vision loss.

Inflammatory optic neuropathies (sequelae):

  • Optic neuritis: Demyelinating inflammation (multiple sclerosis, neuromyelitis optica spectrum disorder); acute painful vision loss with disc swelling or retrobulbar neuritis; may result in residual optic atrophy with permanent deficits.
  • Chronic relapsing inflammatory optic neuropathy (CRION).
  • Sarcoidosis, tuberculosis, syphilis.

Glaucoma (advanced):

  • While glaucoma causes “glaucomatous optic atrophy” (H47.23x), advanced cases may resemble primary optic atrophy.
  • Differentiation: glaucoma has progressive cupping with thinned but vascularized rim; primary atrophy has pallor without typical cupping pattern.

Radiation optic neuropathy:

  • Develops 3 months to 8+ years after radiation therapy to brain/orbit (typically ~1.5 years post-treatment).
  • Risk significantly increased with doses >50 Gy.
  • Irreversible severe vision loss; may be associated with radiation retinopathy.

Other causes:

  • Increased intracranial pressure.
  • Sudden blood flow reduction (e.g., under high stress).
  • Nerve compression (ischemia).

Clinical presentation & exam findings

Symptoms:

  • Progressive vision loss (severity depends on extent of axonal loss).
  • Central vision loss (if maculopapillary bundle affected).
  • Peripheral visual field defects (scotomas, arcuate defects, altitudinal defects, hemianopic patterns depending on lesion location).
  • Color vision defects (especially red-green desaturation).
  • Contrast sensitivity loss.
  • Painless (unless associated with compressive lesion or inflammatory process).

Exam findings (primary optic atrophy):

  • Optic disc appearance:
    • Pallor (pale, white/gray disc color indicating axonal loss and gliosis).
    • Well-demarcated disc margins (sharp, distinct borders - distinguishes from secondary atrophy).
    • Visible lamina cribrosa (sieve-like perforations visible through pale disc).
    • Normal or slightly reduced cup (unlike glaucomatous cupping).
    • Normal caliber retinal vessels (not sheathed or attenuated - distinguishes from secondary atrophy).
  • Relative afferent pupillary defect (RAPD) if unilateral or asymmetric; Marcus Gunn pupil.
  • Reduced visual acuity (ranges from mild to no light perception depending on severity).
  • Visual field defects correlating with pattern of nerve damage.
  • Color vision deficiency (Ishihara, Farnsworth D-15, Hardy-Rand-Rittler testing).

Patterns of optic disc pallor (localizing value)

Pallor patternTypical etiologyClinical correlation
Temporal pallorDominant optic atrophy, optic neuritis, chiasmal compressionAffects papillomacular bundle; central/centrocecal scotoma
Diffuse pallorLHON, NAION, AAION, severe glaucoma, traumatic optic neuropathyExtensive axonal loss; severe vision loss
Segmental pallorAION (superior or inferior sector), branch vascular occlusionAltitudinal visual field defect
Band atrophy (horizontal pallor)Chiasmal lesions (pituitary tumor)Bitemporal hemianopia

Coding specifics (coder workflow)

Code structure breakdown

Character positionValueMeaning
1stHDiseases of the eye and adnexa
2nd-3rd47Other disorders of optic nerve and visual pathways
4th.2Optic atrophy
5th1Primary subtype
6th1Right eye

When to code H47.211

Use H47.211 when:

  • Provider explicitly documents “primary optic atrophy” of right eye.
  • Optic disc shows pallor without preceding history of disc edema.
  • Vision loss and optic nerve changes are present in right eye only (or right eye being documented).
  • Documentation describes optic nerve pallor, well-demarcated disc margins, visible lamina cribrosa.
  • Axonal loss has occurred through compressive, hereditary, ischemic (post-acute), traumatic, toxic, or other non-edematous mechanisms.

Supporting documentation phrases:

  • “Primary optic atrophy OD with reduced visual acuity.”
  • “Optic disc pallor right eye consistent with primary optic atrophy secondary to [underlying cause].”
  • “Right optic nerve atrophy; pale disc with sharp margins; RAPD present.”

When NOT to code H47.211 (use other codes)

Do not use H47.211 when:

ConditionUse insteadReason
Left eye affectedH47.212Laterality mismatch
Both eyes affectedH47.213Bilateral primary optic atrophy
Laterality not specifiedH47.219Unspecified eye (avoid when laterality known)
Glaucomatous optic atrophyH47.231 (right eye)Glaucoma-related atrophy has distinct coding
Secondary optic atrophy (follows disc edema)H47.291 (right eye) - Other optic atrophyNot “primary”; preceded by swelling
Papilledema with atrophyH47.11-H47.13 (papilledema) + H47.291Code swelling primarily; atrophy as sequela
Unspecified optic atrophyH47.20When mechanism (primary vs secondary vs glaucomatous) not documented
Hereditary optic atrophy syndromesH47.22 - Hereditary optic atrophyMay use instead of or with H47.211

Sibling codes (same H47.21 family)

ICD-10-CMDescriptionUse whenBillable
H47.21Primary optic atrophy (category)Non-billable parent✗ No
H47.211Primary optic atrophy, right eyeRight eye (THIS NOTE)✓ Yes
H47.212Primary optic atrophy, left eyeLeft eye✓ Yes
H47.213Primary optic atrophy, bilateralBoth eyes✓ Yes
H47.219Primary optic atrophy, unspecified eyeAvoid when laterality documented✓ Yes
ICD-10-CMDescriptionUse when
H47.2Optic atrophy (category)Non-billable parent
H47.20Unspecified optic atrophyMechanism not documented
H47.22Hereditary optic atrophyDominant optic atrophy, LHON, OPA variants
H47.231Glaucomatous optic atrophy, right eyeGlaucoma-related right eye
H47.291Other optic atrophy, right eyeSecondary atrophy, consecutive atrophy right

HCC information (risk adjustment)

H47.211 is NOT a CMS-HCC code.

Optic atrophy is a manifestation/end-stage finding of underlying disease processes rather than a chronic systemic condition that drives risk adjustment.

However, the underlying etiology causing optic atrophy may be HCC-relevant:

  • Multiple sclerosis (G35) causing optic neuritis → atrophy may be HCC-weighted.
  • Giant cell arteritis (M31.6) causing AAION → atrophy may be HCC-weighted.
  • Diabetes mellitus (E08-E13) with vascular complications leading to NAION.
  • Pituitary tumors (D35.2, C75.1) compressing optic nerve.
  • Neuromyelitis optica spectrum disorder (G36.0) with severe optic neuritis.

Note

Best practice: Code H47.211 to document the optic nerve damage, and always code the underlying condition separately when documented (e.g., G35 for MS, M31.6 for temporal arteritis, E11.x for diabetes with complications) for complete clinical picture and potential HCC capture.

Documentation requirements (work checklist)

Essential elements for H47.211

To support accurate coding and payer scrutiny:

  1. Explicit diagnosis statement

    • “Primary optic atrophy OD” or “Primary optic atrophy right eye.”
    • Specify “primary” to distinguish from secondary or glaucomatous.

  2. Laterality clearly stated

    • “Right eye” or “OD” must be documented.

  3. Optic disc appearance described

    • Pallor (pale, white, gray disc color).
    • Well-demarcated margins (sharp borders).
    • Visible lamina cribrosa (if applicable).
    • Absence of disc edema (confirms “primary” designation).
    • Normal caliber vessels (distinguishes from secondary atrophy).

  4. Visual function documentation

    • Visual acuity OD: Best corrected VA (e.g., 20/60, 20/200, count fingers, hand motion, light perception, no light perception).
    • Relative afferent pupillary defect (RAPD): Present or absent.
    • Visual field defects: Pattern and extent (e.g., central scotoma, altitudinal defect, arcuate defect, bitemporal hemianopia).
    • Color vision deficits: Reduced/absent (Ishihara, D-15, HRR testing).

  5. Underlying etiology documented (if known)

    • Cause of optic atrophy: Compressive lesion (specify), ischemic (NAION/AAION), hereditary (dominant optic atrophy, LHON), traumatic, toxic, post-inflammatory, radiation-induced.
    • Code the underlying condition separately.

  6. Diagnostic testing performed/referenced

    • OCT optic nerve: Reduced RNFL thickness (retinal nerve fiber layer thinning confirms axonal loss).
    • Visual fields: Humphrey 24-2, 10-2, or Goldmann perimetry showing defects corresponding to atrophy.
    • VEP (visual evoked potentials): Decreased amplitude or absent P100 response; increased latency (indicates optic nerve dysfunction).
    • MRI brain/orbits: If compressive lesion, demyelinating disease, or infiltrative process suspected.
    • Lab testing: Vitamin B12, folate, ESR/CRP (temporal arteritis), genetic testing (LHON, dominant optic atrophy).

  7. Chronicity and stability

    • “Stable optic atrophy” vs “progressive vision loss” (indicates whether underlying process is active or inactive).

Common auditor red flags

  • “Optic nerve pallor” without “optic atrophy” stated → pallor alone doesn’t equal atrophy; query provider.
  • “Optic atrophy” without specifying primary vs secondary vs glaucomatous → should default to H47.20 (unspecified); query for specificity.
  • Glaucoma in problem list but coded as H47.211 → should be H47.231 (glaucomatous optic atrophy) if glaucoma is the cause.
  • History of papilledema or chronic disc edema but coded as “primary” → this is secondary optic atrophy (H47.291), not primary.
  • No documentation of disc appearance → cannot code optic atrophy without ophthalmic exam describing disc pallor.
  • Underlying cause documented but not coded separately → missed capture of potentially HCC-relevant diagnosis.

Associated CPT codes (common pairings)

E/M and comprehensive eye exam codes

CPTDescriptionContext for H47.211
99202-99205New patient office visitInitial evaluation of vision loss/optic atrophy
99212-99215Established patient visitFollow-up for known optic atrophy
92002-92004New ophthalmological servicesComprehensive eye exam for optic nerve evaluation
92012-92014Established ophthalmological servicesOngoing optic atrophy monitoring

Optic nerve diagnostic imaging (KEY TESTS)

Optical Coherence Tomography (OCT) is the PRIMARY diagnostic modality for optic atrophy.

CPTDescriptionClinical useDocumentation requirements
92133OCT posterior segment, optic nerveMost common code for optic atrophy evaluationRNFL thickness measurements; peripapillary analysis; comparison to normative database; interpretation documenting thinning/atrophy
92134OCT posterior segment, retinaRetinal layer analysis (if macular involvement suspected)Ganglion cell layer thickness; macular thinning assessment
92132OCT anterior segmentNot typically for optic atrophy (cornea/angle imaging)-
92250Fundus photographyOptic disc color documentation (pallor)Serial photos comparing disc appearance over time

OCT findings in optic atrophy:

  • Reduced RNFL thickness (retinal nerve fiber layer thinning; normal ~100 µm average; atrophy often <70 µm).
  • Thinning pattern corresponds to visual field defects (e.g., superior/inferior RNFL loss with altitudinal VF defects in AION).
  • Ganglion cell-inner plexiform layer (GCIPL) thinning (macular OCT showing reduced ganglion cell layer).

H47.211 is listed in CMS billing/coding article for posterior segment imaging as covered diagnosis. [1139]

Visual field testing

CPTDescriptionUse in optic atrophy
92081Visual field examination, limitedScreening confrontation fields
92082Visual field examination, intermediateHumphrey 24-2 or 30-2 threshold testing
92083Visual field examination, extendedComplex field testing (10-2, Goldmann) for central defects

Visual field patterns in optic atrophy:

  • Central scotoma: Dominant optic atrophy, LHON, toxic neuropathy (papillomacular bundle involvement).
  • Centrocecal scotoma: Extends from fixation to blind spot; typical of optic neuropathies.
  • Altitudinal defect: AION (superior or inferior hemianopia respecting horizontal meridian).
  • Arcuate/Bjerrum scotoma: Glaucomatous atrophy, advanced NAION.
  • Bitemporal hemianopia: Chiasmal compression (pituitary tumor); evolves to band atrophy.

Electrophysiology testing

Visual Evoked Potentials (VEP) assess functional integrity of visual pathway from retina to occipital cortex.

| CPT | Description | VEP findings in optic atrophy | |---|---|---|---| | 95930 | Visual evoked potential (VEP) checkerboard or flash testing | Decreased amplitude or absent P100 response (indicates axonal loss); increased latency suggests demyelination (optic neuritis sequelae) |

VEP utility: >

  • Most useful for anterior visual pathway dysfunction (retina → optic nerve → chiasm).
  • Sensitive for subclinical optic nerve disease (may detect abnormalities not visible on exam/MRI).
  • Optic atrophy findings: Severely attenuated or absent P100; confirms objective visual pathway damage.
  • Distinguishes demyelinating from other causes: Prolonged latency (>120 ms) suggests prior demyelination (MS, optic neuritis); normal latency with reduced amplitude suggests axonal loss (ischemic, compressive, traumatic, hereditary).
  • Pattern-shift VEP normally appears as straight line with single positive peak at 100 msec after stimulus; abnormalities in waveform seen in optic nerve pathology.
  • Peak latency sensitive measure of conduction delay in optic nerve caused by demyelination.

H47.211 is listed in payer VEP medical necessity policies as covered diagnosis.

Advanced imaging

CPTDescriptionIndication for optic atrophy workup
70450-70453CT head/orbits without/with contrastTrauma, orbital mass, bony abnormalities
70540-70543MRI brain without/with contrastCompressive lesions (pituitary tumor, meningioma), demyelinating disease (MS), infiltrative processes
70540-70543MRI orbits with fat saturationOptic nerve sheath meningioma, optic glioma, orbital inflammation

Color vision testing

CPTDescriptionOptic atrophy application
92283Color vision examination, extendedFarnsworth D-15, Hardy-Rand-Rittler (HRR) tests document red-green deficiency in optic neuropathies

Lab testing (based on suspected etiology)

TestCPTIndication
Vitamin B1282607Nutritional optic neuropathy
Folate82746Nutritional optic neuropathy
ESR85651Giant cell arteritis (AAION) workup; ESR >50 mm/hr suggestive
C-reactive protein86140Temporal arteritis; more specific than ESR
Genetic testing81401-81479LHON (mitochondrial DNA mutations: 11778, 14484, 3460), OPA1 gene (dominant optic atrophy) [1115]
ACE level82164Sarcoidosis-related optic neuropathy
Syphilis serology (RPR, FTA-ABS)86592, 86781Infectious optic neuropathy

Treatment overview (coding context)

Management depends on underlying cause

Primary optic atrophy is IRREVERSIBLE - the goal is to identify and treat the underlying cause to prevent further damage and protect the fellow eye.

By etiology:

Compressive lesions

  • Surgical decompression (transsphenoidal pituitary resection, optic nerve sheath fenestration for meningioma).
  • Radiation therapy for non-resectable tumors.
  • Goal: Decompress optic nerve before irreversible atrophy occurs; prognosis depends on duration/severity of compression.

AION (arteritic)

  • High-dose IV methylprednisolone (1000 mg daily × 3-5 days) followed by oral prednisone taper (prevents fellow eye involvement).
  • Temporal artery biopsy (within 1-2 weeks of steroid initiation).
  • Urgent treatment (delays increase risk of bilateral blindness).

AION (non-arteritic)

  • No proven treatment for NAION; proposed therapies (aspirin, hyperbaric oxygen, levodopa, optic nerve decompression) have not shown benefit.
  • Risk factor modification: Control hypertension, diabetes, hyperlipidemia, sleep apnea.
  • Aspirin may reduce risk of fellow eye involvement (controversial).

Optic neuritis sequelae

  • High-dose IV methylprednisolone during acute phase speeds visual recovery but doesn’t affect long-term outcome.
  • Disease-modifying therapy for multiple sclerosis (if present) to reduce relapses.
  • Residual optic atrophy is common after severe optic neuritis.

Hereditary optic neuropathies

  • Dominant optic atrophy: No specific treatment; low vision aids; genetic counseling.
  • LHON: Idebenone (mitochondrial antioxidant) may improve outcomes in some patients; avoid smoking/alcohol; gene therapy trials underway.

Toxic/nutritional

  • Remove offending agent (stop toxic medication).
  • Vitamin supplementation: B12, folate, thiamine (if deficient).
  • Prognosis: Variable; early cessation may prevent progression but established atrophy is irreversible.

Traumatic

  • High-dose steroids (controversial; no strong evidence but sometimes used for indirect traumatic optic neuropathy).
  • Surgical decompression (optic canal decompression) in select cases.

Supportive care & rehabilitation

Low vision services:

  • Magnification devices (CCTV, handheld magnifiers, telescopic glasses).
  • Contrast enhancement.
  • Occupational therapy for activities of daily living.
  • Vision rehabilitation specialist referral.

Driving assessment if binocular visual fields inadequate.

Protective eyewear for fellow eye (if unilateral atrophy).

Sample ICD-10 combinations (work scenarios)

Scenario 1: Primary optic atrophy OD from compressive pituitary adenoma

ICD-10-CM codes:

  • H47.211 - Primary optic atrophy, right eye
  • D35.2 - Benign neoplasm of pituitary gland
  • H53.46 - Homonymous bilateral field defects (if bitemporal hemianopia documented)

CPT:

  • 99215 - Established patient office visit, high complexity
  • 92133 - OCT optic nerve (reduced RNFL OD)
  • 92082 - Visual field examination (bitemporal defect)
  • 70553 - MRI brain with contrast (pituitary mass)

Rationale: Captures optic atrophy manifestation + underlying compressive cause + associated visual field defect. [1120]

Scenario 2: Post-NAION optic atrophy OD with diabetes

ICD-10-CM codes:

  • H47.211 - Primary optic atrophy, right eye
  • H34.23 - Anterior ischemic optic neuropathy (if in acute/subacute phase; may use if documenting history)
  • E11.36 - Type 2 diabetes mellitus with diabetic cataract (if present)
  • E11.9 - Type 2 diabetes mellitus without complications (if no specific diabetic complication coded)
  • I10 - Essential hypertension (risk factor for NAION)

CPT:

  • 92014 - Established ophthalmological service, comprehensive
  • 92133 - OCT optic nerve (superior RNFL thinning OD)
  • 92082 - Visual fields (inferior altitudinal defect OD)

Rationale: Optic atrophy is sequela of NAION; code both; include diabetes and hypertension as contributing vascular risk factors.

Scenario 3: Dominant optic atrophy (Kjer’s) bilateral, right worse than left

ICD-10-CM codes:

  • H47.22 - Hereditary optic atrophy (captures genetic etiology) [1115]
  • H47.211 - Primary optic atrophy, right eye (if documenting right side severity)
  • H47.212 - Primary optic atrophy, left eye (if documenting left side) OR
  • H47.213 - Primary optic atrophy, bilateral (if coding bilaterality once)

CPT:

  • 99204 - New patient visit, moderate-high complexity
  • 92133-50 - OCT optic nerve, bilateral (reduced RNFL both eyes)
  • 92082-50 - Visual fields, bilateral (centrocecal scotomas)
  • 81404 - Molecular pathology, Level 5 (OPA1 gene sequencing)

Rationale: Hereditary optic atrophy (H47.22) is primary diagnosis; can also code H47.21x to specify laterality/severity; genetic testing confirms diagnosis. [1115]

Scenario 4: Optic atrophy OD following optic neuritis in MS patient

ICD-10-CM codes:

  • G35 - Multiple sclerosis (primary systemic diagnosis; HCC-relevant) [1156]
  • H47.211 - Primary optic atrophy, right eye (sequela of optic neuritis)
  • H46.9 - Unspecified optic neuritis (if documenting history; use Z86.69 personal history if remote)

CPT:

  • 99214 - Established patient visit
  • 92133 - OCT optic nerve (temporal RNFL thinning OD)
  • 95930 - Visual evoked potentials (prolonged P100 latency, reduced amplitude OD)

Rationale: MS (G35) is HCC diagnosis and underlying cause; optic atrophy is sequela of demyelinating optic neuritis; VEP confirms optic nerve dysfunction with demyelinating pattern. [1104][1156]

Sample documentation (clinic note template)

Chief Complaint: Vision loss right eye / Follow-up optic atrophy OD.

HPI: [Age]-year-old [male/female] with [known/newly diagnosed] primary optic atrophy of the right eye presenting for evaluation and management. Patient reports [onset and progression: sudden vs gradual; timeframe] vision loss OD. [Associated symptoms: pain, color vision changes, visual field defects, headache]. [Precipitating events: trauma, infection, medication use]. [Prior workup: MRI, labs, prior treatments]. [Known underlying diagnosis: diabetes, MS, pituitary tumor, etc.].

Past Ocular History:

  • Primary optic atrophy, right eye (diagnosed [date]; etiology: [compressive/ischemic/hereditary/traumatic/toxic/post-inflammatory])
  • [NAION right eye [date] / Optic neuritis right eye [date] / s/p transsphenoidal pituitary surgery [date]]
  • [Other ocular history]

Past Medical History:

  • [Underlying conditions: MS, diabetes, hypertension, temporal arteritis, pituitary adenoma, etc.]

Medications:

  • [List relevant medications; note any neurotoxic drugs]

Exam:

  • Visual Acuity:
    • OD: [20/XX, count fingers, hand motion, light perception, NLP]
    • OS: [20/XX]
  • Pupils:
    • RAPD present OD (Marcus Gunn pupil; grade +1 to +4) [if unilateral or asymmetric atrophy]
    • Pupils equal, round, reactive to light (if no RAPD)
  • Color Vision:
    • OD: [Reduced/absent] - [X/Y Ishihara plates, D-15 total color confusion plot]
    • OS: [Normal - Y/Y plates]
  • Confrontation Visual Fields:
    • OD: [Central scotoma / Altitudinal defect / Full to confrontation]
    • OS: [Full / Defect noted]
  • Dilated Fundus Exam - Right Eye:
    • Optic Disc: Pale, white/gray appearance consistent with optic atrophy. Well-demarcated disc margins (sharp borders). Lamina cribrosa visible. Cup-to-disc ratio [0.X]. No disc edema or hemorrhages present. Retinal vessels normal caliber (not sheathed/attenuated).
    • Macula: [Normal / Subtle RPE changes / Atrophy]
    • Periphery: [Normal / Findings]
  • Dilated Fundus Exam - Left Eye:
    • Optic Disc: [Normal pink color, sharp margins, C/D 0.X / Findings if fellow eye affected]
    • Macula, Periphery: [Normal / Findings]

Ancillary Testing:

  • OCT Optic Nerve (CPT 92133):
    • OD: Reduced RNFL thickness - Average [XX µm] (normal ~100 µm). Superior quadrant [XX µm], inferior [XX µm], temporal [XX µm], nasal [XX µm]. Findings consistent with optic atrophy. [Pattern: diffuse thinning / superior > inferior / temporal wedge defect]
    • OS: RNFL within normal limits [or findings if bilateral]
  • Visual Fields (CPT 92082/92083):
    • OD: [Pattern: central scotoma, altitudinal defect, arcuate scotoma, diffuse depression]. MD [value] dB, PSD [value] dB, VFI [value]%.
    • OS: [Normal / Findings]
  • VEP (CPT 95930): [If performed]
    • OD: Decreased amplitude / Absent P100 response [+ prolonged latency if demyelinating cause]. Confirms optic nerve dysfunction.
    • OS: [Normal / Findings]
  • MRI Brain/Orbits: [If performed - describe findings: pituitary mass, optic nerve enhancement, demyelinating lesions, etc.]
  • Labs: [ESR/CRP if AAION suspected; B12/folate if nutritional cause; genetic testing if hereditary]

Assessment:

  • Primary optic atrophy, right eye (H47.211) secondary to [underlying cause] [1106][1120]
  • [Underlying diagnosis code]
  • [Associated visual field defect code]

Etiology: [Compressive lesion (pituitary adenoma) / Anterior ischemic optic neuropathy (NAION) / Hereditary (dominant optic atrophy, OPA1 mutation) / Post-optic neuritis (MS) / Traumatic optic neuropathy / Toxic (medication-induced) / Radiation-induced]

Prognosis: Guarded; optic atrophy represents irreversible end-stage optic nerve damage. Vision loss is permanent. [1120][1155]

Plan:

  • Treat underlying cause (if modifiable): [Neurosurgery referral for pituitary resection / High-dose IV methylprednisolone for AAION / Discontinue offending medication / Vitamin B12 supplementation / MS disease-modifying therapy]
  • Protect fellow eye: Monitor left eye closely; educate patient on symptoms requiring urgent evaluation (sudden vision loss, headache, jaw claudication).
  • Low vision referral for adaptive devices and occupational therapy (if significant vision loss).
  • Repeat OCT and visual fields in [3-6 months] to monitor for stability vs progression.
  • Genetic counseling (if hereditary optic atrophy confirmed). [1115]
  • Driving assessment if binocular visual fields inadequate for licensure.
  • Patient education provided regarding permanent nature of optic atrophy, need for ongoing monitoring, and warning signs of fellow eye involvement.

ICD-10-CM:

  • H47.211 - Primary optic atrophy, right eye
  • [Underlying cause code: D35.2, H34.23, E11.9, G35, etc.]
  • [H53.4x visual field defect code if applicable]

CPT:

  • 92014 - Established ophthalmological services, comprehensive (or appropriate E/M)
  • 92133 - OCT optic nerve
  • 92082 - Visual field examination, intermediate
  • [95930 VEP, 70553 MRI brain, etc. if performed]

Billing & compliance pearls

  • H47.211 is NOT HCC but underlying cause (MS, temporal arteritis, pituitary tumor) may be HCC-relevant - always code etiology separately. [1156]
  • “Primary” must be explicitly stated - “optic atrophy” alone defaults to H47.20 (unspecified); “optic disc pallor” alone is insufficient without “atrophy” statement. [1110][1120]
  • Glaucoma patients: If glaucoma caused the atrophy, use H47.231 (glaucomatous optic atrophy right eye), not H47.211.
  • OCT optic nerve (92133) supports diagnosis - RNFL thinning objectively confirms atrophy; include in documentation for medical necessity and audit defense. [1139]
  • Laterality matters - right eye = H47.211, left eye = H47.212, bilateral = H47.213; do NOT use H47.219 (unspecified) when laterality documented. [1110]
  • VEP (95930) useful for equivocal cases - objective confirmation of optic nerve dysfunction when exam/imaging findings are subtle or patient reliability questioned. [1154][1156]
  • H47.211 listed in payer policies as covered diagnosis for OCT (92133) and VEP (95930) medical necessity. [1139][1154]
  • Document chronicity - “stable optic atrophy” vs “progressive vision loss” affects treatment planning and frequency of follow-up visits/testing.
  • Treatment focuses on underlying cause - if primary disease treated early, progression of optic atrophy can be slowed or stopped. [1155]

Key sources (compact format)

[1105]: ECGWaves H47.211 primary optic atrophy right eye ICD-10 classification
[1106]: AAPC H47.211 official descriptor WHO classification billable code
[1109]: FindACode H47.211 ICD-10-CM hierarchy diagnosis code
[1110]: AAPC H47.21 primary optic atrophy category child codes laterality structure
[1120]: NCBI StatPearls optic atrophy pathophysiology RGC-LGB lesions orderly axon degeneration glial columns etiologies primary vs secondary differentiation well-demarcated margins normal vessel caliber
[1139]: CMS billing coding ophthalmology posterior segment imaging H47.211 covered diagnosis
[1154]: Centene vision VEP policy H47.211 covered diagnosis P100 waveform latency amplitude pattern-shift sensitivity
[1156]: Anthem VEP policy 95930 CPT H47.211 multiple sclerosis G35 visual pathway retina-occipital cortex
[722]: HCPCS system Level I CPT Level II alphanumeric codes
[788]: Eye disease chapter H47.2 Leber’s hereditary optic neuropathy H47.3 optic disc drusen
[891]: MeSH codes C11.640 optic nerve diseases C11.640.451 optic atrophy hereditary types
[1104]: Optic neuritis typical atypical MS NMO MOGAD IV methylprednisolone treatment
[1115]: Kjer’s dominant optic atrophy vision loss mitochondria dysfunction OPA1 gene chromosome 3q28-qter OPA2-OPA6 variants
[1155]: Savir-Center optic atrophy causes treatment underlying condition early intervention progression slowing traumatic injuries glaucoma ischemia tumors poisoning hereditary diseases
[965]: ICD-9 vitamin deficiencies vitamin A B12 nutritional optic neuropathy

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