2027 ICD-10-CM Diagnosis Code H49.813Kearns-Sayre syndrome, bilateral
ICD-10-CM Codes›H00–H59›H49-H52›H49
- Billable — Valid for Submission
- CC — Complication or Comorbidity
- Chronic Condition
H49.813 is a billable ICD-10-CM diagnosis code for Kearns-Sayre syndrome, bilateral. 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. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Neuro-ophthalmology and Strabismus.
H49.813 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
Code Classification
Medicare Risk Adjustment (HCC)Billing
H49.813 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.
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.
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to H49.813: 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.
- nystagmus and other irregular eye movements (H55)
- 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.
Notes
General instructions on how these codes are used.
- 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 H49.813, its category, or a range that includes it.
Excludes1 3
These codes carry an Excludes1 note naming H49.813: they are not reported together with it, unless the two conditions are unrelated.
- E88.4 Mitochondrial metabolism disordersKearns-Sayre syndrome (H49.81) names H49.81, which includes this code
- G71.3 Mitochondrial myopathy, not elsewhere classifiedKearns-Sayre syndrome (H49.81) names H49.81, which includes this code
- H49.4 Progressive external ophthalmoplegiaKearns-Sayre syndrome (H49.81-) names H49.81-, which includes this code
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
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 H49.813 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About H49.813Overview
What is the ICD-10 code for Kearns-Sayre syndrome, bilateral?
The ICD-10-CM code for Kearns-Sayre syndrome, bilateral is H49.813 (sometimes written as H49813). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is H49.813 (Kearns-Sayre syndrome) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report Kearns-Sayre syndrome, bilateral on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does H49.813 group to?
When Kearns-Sayre syndrome, bilateral 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 H49.813 a CC or MCC?
CMS lists H49.813 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 H49.813?
Under the General Equivalence Mappings, Kearns-Sayre syndrome, bilateral converts to ICD-9-CM 277.87 (dis mitochondrial metab). The mapping is approximate, so confirm the match fits the documentation.
Does H49.813 risk-adjust for Medicare Advantage payment?
Not for Medicare Advantage. H49.813 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).
