2027 ICD-10-CM Diagnosis Code H49.819Kearns-Sayre syndrome, unspecified eye

ICD-10-CM Codes›H00–H59›H49-H52›H49

ICD-10-CM H49.819
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

H49.819 is a billable ICD-10-CM diagnosis code for Kearns-Sayre syndrome, unspecified eye. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 642. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 41 closely related codes. The code is flagged as an unspecified code, since codes identifying laterality exist in the same family. Coders also document this condition as Kearns-Sayre syndrome. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Neuro-ophthalmology and Strabismus.

H49.819 no longer risk-adjusts for Medicare Advantage: it mapped to HCC 23 under the retired CMS-HCC V24 model through payment year 2025 but maps to no category in the live V28 model. It still risk-adjusts in the PACE (CMS-HCC V22) category 23, ESRD (V21) category 23, ESRD (V24) category 23, and RxHCC Part D (V08) category 43 for payment year 2027.

Code Identity

ICD-10-CM Code
H49.819
Billable Status
Yes — Valid for Submission
Code Describes
Kearns-Sayre syndrome, unspecified eye
Short Description
Kearns-Sayre syndrome, unspecified eye
Same as the full description in the CMS dataset.
Parent Code
H49.81
Kearns-Sayre syndrome

Code Classification

ChapterH00–H59Diseases of the eye and adnexa
SectionH49-H52Disorders of ocular muscles, binocular movement, accommodation and refraction
CategoryH49Paralytic strabismus
This CodeH49.819Kearns-Sayre syndrome, unspecified eye

Code EditsBilling

Medicare Code Editor checks that affect claim validity for H49.819.

Unspecified codes
Unspecified codes exist in the ICD-10-CM classification for circumstances when documentation in the medical record does not provide the level of detail needed to support reporting a more specific code. However, in the inpatient setting, there should generally be very limited and rare circumstances for which the laterality (right, left, bilateral) of a condition is unable to be documented and reported. The following pages contain the list of unspecified ICD-10-CM diagnosis codes for which there is a more specific code to identify laterality (right, left, bilateral) within that code family.

Medicare Risk Adjustment (HCC)Billing

H49.819 no longer risk-adjusts for Medicare Advantage: it maps to no payment category in the live CMS-HCC V28 model, although it still risk-adjusts in the other CMS models shown below.

CMS-HCC V28 (Medicare Advantage Payment Model)
Not mapped
Dropped in V28 see all codes that no longer risk-adjust
Prior Model (CMS-HCC V24)
HCC 23
V24 retired last contributed to a Medicare Advantage risk score in payment year 2025
Other CMS Models
PACE (CMS-HCC V22): HCC 23 · ESRD (V21): HCC 23 · ESRD (V24): HCC 23
ESRD V21 weights: 0.013 dialysis, 0.234–0.359 functioning graft · ESRD V24 weights: 0.036 dialysis, 0.217–0.390 functioning graft
Part D (RxHCC)
RxHCC 43 — Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders
also risk-adjusts in the Part D prescription drug model (V08)

Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Kearns-Sayre syndrome

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to H49.819: its own notes plus those printed at H49.81, H49, block H49-H52, and Chapter 7. A note printed at a category, block or chapter applies to every code under it.

Excludes2

Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.

From H49 Paralytic strabismus applies to 29 codes
  • internal ophthalmoplegia (H52.51-)
  • internuclear ophthalmoplegia (H51.2-)
  • progressive supranuclear ophthalmoplegia (G23.1)
From Block H49-H52 Disorders of ocular muscles, binocular movement, accommodation and refraction applies to 150 codes
  • nystagmus and other irregular eye movements (H55)
From Chapter 7 (H00-H59) Diseases of the eye and adnexa applies to 2,677 codes
  • certain conditions originating in the perinatal period (P04-P96)
  • certain infectious and parasitic diseases (A00-B99)
  • complications of pregnancy, childbirth and the puerperium (O00-O9A)
  • congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99)
  • diabetes mellitus related eye conditions (E09.3-, E10.3-, E11.3-, E13.3-)
  • endocrine, nutritional and metabolic diseases (E00-E88)
  • injury (trauma) of eye and orbit (S05.-)
  • injury, poisoning and certain other consequences of external causes (S00-T88)
  • neoplasms (C00-D49)
  • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
  • syphilis related eye disorders (A50.01, A50.3-, A51.43, A52.71)

Code Also

A second code may be needed to fully describe the condition; the note gives no sequencing direction.

From H49.81 Kearns-Sayre syndrome applies to 4 codes
  • if applicable, other manifestations, such as:
  • heart block (I45.9)

Notes

General instructions on how these codes are used.

From Chapter 7 (H00-H59) Diseases of the eye and adnexa applies to 2,677 codes
  • Use an external cause code following the code for the eye condition, if applicable, to identify the cause of the eye condition

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name H49.819, its category, or a range that includes it.

Excludes1 3

These codes carry an Excludes1 note naming H49.819: they are not reported together with it, unless the two conditions are unrelated.

  • E88.4 Mitochondrial metabolism disorders
    Kearns-Sayre syndrome (H49.81) names H49.81, which includes this code
  • G71.3 Mitochondrial myopathy, not elsewhere classified
    Kearns-Sayre syndrome (H49.81) names H49.81, which includes this code
  • H49.4 Progressive external ophthalmoplegia
    Kearns-Sayre syndrome (H49.81-) names H49.81-, which includes this code

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR EYE006
Neuro-ophthalmology
Default principal diagnosis: inpatient Yes · outpatient Yes
CCSR EYE007
Strabismus
Default principal diagnosis: inpatient No · outpatient No

Clinical InformationClinical

  • Kearns-Sayre Syndrome

    a mitochondrial disorder featuring the triad of chronic progressive external ophthalmoplegia, cardiomyopathy (cardiomyopathies) with conduction block (heart block), and retinitis pigmentosa. disease onset is in the first or second decade. elevated csf protein, sensorineural deafness, seizures, and pyramidal signs may also be present. ragged-red fibers are found on muscle biopsy. (adams et al., principles of neurology, 6th ed, p984)

Patient EducationClinical

Eye Movement Disorders

When you look at an object, you're using several muscles to move both eyes to focus on it. If you have a problem with the muscles, the eyes don't work properly.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert H49.819 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
277.87 Dis mitochondrial metab
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About H49.819Overview

What is the ICD-10 code for Kearns-Sayre syndrome, unspecified eye?

The ICD-10-CM code for Kearns-Sayre syndrome, unspecified eye is H49.819 (sometimes written as H49819). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is H49.819 (Kearns-Sayre syndrome) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report Kearns-Sayre syndrome, unspecified eye on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

What MS-DRG does H49.819 group to?

When Kearns-Sayre syndrome, unspecified eye is the principal diagnosis on an inpatient stay, it groups to MS-DRG 642 (inborn and Other Disorders of Metabolism), which carries a relative weight of 1.3226. Higher weights mean higher Medicare reimbursement.

Is H49.819 a CC or MCC?

CMS lists H49.819 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 41 closely related codes in its exclusion list.

What is the ICD-9 equivalent of H49.819?

Under the General Equivalence Mappings, Kearns-Sayre syndrome, unspecified eye converts to ICD-9-CM 277.87 (dis mitochondrial metab). The mapping is approximate, so confirm the match fits the documentation.

Does H49.819 risk-adjust for Medicare Advantage payment?

Not for Medicare Advantage. H49.819 mapped to HCC 23 in the retired CMS-HCC V24 model, which last determined payment in 2025, but it maps to no category in the live V28 model; see all codes that no longer risk-adjust. It still risk-adjusts in the PACE (CMS-HCC V22) category 23 (Other Significant Endocrine and Metabolic Disorders), ESRD (V21) category 23 (Other Significant Endocrine and Metabolic Disorders), ESRD (V24) category 23 (Other Significant Endocrine and Metabolic Disorders), and RxHCC Part D (V08) category 43 (Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders).