H49.819 Kearns-Sayre syndrome, unspecified eye

Billable ICD-10-CM code, FY2026. Acts as a Complication or Comorbidity (CC) as a secondary diagnosis.

Applies from H49 Paralytic strabismus

Excludes2 (not included here)

• internal ophthalmoplegia (H52.51-)
• internuclear ophthalmoplegia (H51.2-)
• progressive supranuclear ophthalmoplegia (G23.1)

Applies from H49.81 Kearns-Sayre syndrome

Applicable to

• Progressive external ophthalmoplegia with pigmentary retinopathy

Code also

• , if applicable, other manifestations, such as:
• heart block (I45.9)

Risk adjustment (HCC)

• CMS-HCC V22: HCC 23 — Other Significant Endocrine and Metabolic Disorders
• RxHCC V08: HCC 43 — Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders
• ESRD V24: HCC 23

MS-DRG index

Defines the logic of DRG(s) 642 in MDC 10.

Approximate ICD-9-CM (GEMs)

• 277.87 Disorders of mitochondrial metabolism (approximate)

Medicare Code Edits (MCE v43.1 (April 2026))

• Unspecified code (laterality): Unspecified codes for which a more specific code identifying laterality (right, left, bilateral) exists within the same code family.

Code annotations containing back-references to H49.819

Back-references to H49.81

• Type 1 Excludes: E88.4 Mitochondrial metabolism disorders ("Kearns-Sayre syndrome (H49.81)")
• Type 1 Excludes: G71.3 Mitochondrial myopathy, not elsewhere classified ("Kearns-Sayre syndrome (H49.81)")
• Type 1 Excludes: H49.4 Progressive external ophthalmoplegia ("Kearns-Sayre syndrome (H49.81-)")

Nearby codes

• H49.8 Other paralytic strabismus
• H49.81 Kearns-Sayre syndrome
• H49.811 Kearns-Sayre syndrome, right eye
• H49.812 Kearns-Sayre syndrome, left eye
• H49.813 Kearns-Sayre syndrome, bilateral
• H49.819 Kearns-Sayre syndrome, unspecified eye (this code)
• H49.88 Other paralytic strabismus
• H49.881 Other paralytic strabismus, right eye
• H49.882 Other paralytic strabismus, left eye
• H49.883 Other paralytic strabismus, bilateral
• H49.889 Other paralytic strabismus, unspecified eye