2027 ICD-10-CM Diagnosis Code H49.43Progressive external ophthalmoplegia, bilateral

ICD-10-CM Codes›H00–H59›H49-H52›H49

ICD-10-CM H49.43
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

H49.43 is a billable ICD-10-CM diagnosis code for progressive external ophthalmoplegia, bilateral. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 123. Coders also document this condition as bilateral progressive external ophthalmoplegia. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Neuro-ophthalmology and Strabismus.

Code Identity

ICD-10-CM Code
H49.43
Billable Status
Yes — Valid for Submission
Code Describes
Progressive external ophthalmoplegia, bilateral
Short Description
Progressive external ophthalmoplegia, bilateral
Same as the full description in the CMS dataset.
Parent Code
Progressive external ophthalmoplegia

Code Classification

ChapterH00–H59Diseases of the eye and adnexa
SectionH49-H52Disorders of ocular muscles, binocular movement, accommodation and refraction
CategoryH49Paralytic strabismus
This CodeH49.43Progressive external ophthalmoplegia, bilateral

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Bilateral progressive external ophthalmoplegia
  • External ophthalmoplegia of bilateral eyes
  • External ophthalmoplegia of left eye
  • External ophthalmoplegia of right eye
  • Left progressive external ophthalmoplegia
  • Right progressive external ophthalmoplegia

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to H49.43: its own notes plus those printed at H49.4, H49, block H49-H52, and Chapter 7. A note printed at a category, block or chapter applies to every code under it.

Excludes1

Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.

From H49.4 Progressive external ophthalmoplegia applies to 4 codes

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)

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.

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

  • Ophthalmoplegia, Chronic Progressive External

    a mitochondrial myopathy characterized by slowly progressive paralysis of the levator palpebrae, orbicularis oculi, and extraocular muscles. ragged-red fibers and atrophy are found on muscle biopsy. familial and sporadic forms may occur. disease onset is usually in the first or second decade of life, and the illness slowly progresses until usually all ocular motility is lost. (from adams et al., principles of neurology, 6th ed, p1422)

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 H49.43 to ICD-9-CMHistory

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

ICD-9-CM
378.55 External ophthalmoplegia
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 H49.43Overview

What is the ICD-10 code for progressive external ophthalmoplegia, bilateral?

The ICD-10-CM code for progressive external ophthalmoplegia, bilateral is H49.43 (sometimes written as H4943). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is H49.43 (Progressive external ophthalmoplegia) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report progressive external ophthalmoplegia, bilateral on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

What MS-DRG does H49.43 group to?

When progressive external ophthalmoplegia, bilateral is the principal diagnosis on an inpatient stay, it groups to MS-DRG 123 (neurological Eye Disorders), which carries a relative weight of 0.8218. Higher weights mean higher Medicare reimbursement.

Can H49.43 be reported with a code from H49.81?

Not as a rule. The Excludes1 note at H49.4, which applies to H49.43, lists "Kearns-Sayre syndrome (H49.81-)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).

What is the ICD-9 equivalent of H49.43?

Under the General Equivalence Mappings, progressive external ophthalmoplegia, bilateral converts to ICD-9-CM 378.55 (external ophthalmoplegia). The mapping is approximate, so confirm the match fits the documentation.