ICD-10-CM Code H49.40

Progressive external ophthalmoplegia, unspecified eye

Version 2021 Billable Code

Valid for Submission

H49.40 is a billable code used to specify a medical diagnosis of progressive external ophthalmoplegia, unspecified eye. The code is valid for the fiscal year 2021 for the submission of HIPAA-covered transactions. The ICD-10-CM code H49.40 might also be used to specify conditions or terms like adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy, autosomal dominant progressive external ophthalmoplegia, autosomal recessive progressive external ophthalmoplegia, ophthalmoplegia plus syndrome, painful ophthalmoplegia, progressive external ophthalmoplegia, etc

ICD-10:H49.40
Short Description:Progressive external ophthalmoplegia, unspecified eye
Long Description:Progressive external ophthalmoplegia, unspecified eye

Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy
  • Autosomal dominant progressive external ophthalmoplegia
  • Autosomal recessive progressive external ophthalmoplegia
  • Ophthalmoplegia plus syndrome
  • Painful ophthalmoplegia
  • Progressive external ophthalmoplegia
  • Progressive external ophthalmoplegia, myopathy, emaciation syndrome
  • Third cranial nerve finding
  • Tolosa-Hunt syndrome

Convert H49.40 to ICD-9

  • 378.55 - External ophthalmoplegia (Approximate Flag)

Code Classification

  • Diseases of the eye and adnexa (H00–H59)
    • Disorders of ocular muscles, binocular movement, accommodation and refraction (H49-H52)
      • Paralytic strabismus (H49)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021

Information for Patients


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.

There are many kinds of eye movement disorders. Two common ones are

  • Strabismus - a disorder in which the two eyes don't line up in the same direction. This results in "crossed eyes" or "walleye."
  • Nystagmus - fast, uncontrollable movements of the eyes, sometimes called "dancing eyes"

Some eye movement disorders are present at birth. Others develop over time and may be associated with other problems, such as injuries. Treatments include glasses, patches, eye muscle exercises, and surgery. There is no cure for some kinds of eye movement disorders, such as most kinds of nystagmus.

  • Cranial mononeuropathy III (Medical Encyclopedia)
  • Cranial mononeuropathy VI (Medical Encyclopedia)
  • Eye muscle repair (Medical Encyclopedia)
  • Nystagmus (Medical Encyclopedia)
  • Strabismus (Medical Encyclopedia)
  • Supranuclear ophthalmoplegia (Medical Encyclopedia)

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Progressive external ophthalmoplegia Progressive external ophthalmoplegia is a condition characterized by weakness of the eye muscles. The condition typically appears in adults between ages 18 and 40 and slowly worsens over time. The first sign of progressive external ophthalmoplegia is typically drooping eyelids (ptosis), which can affect one or both eyelids. As ptosis worsens, affected individuals may use the forehead muscles to try to lift the eyelids, or they may lift up their chin in order to see. Another characteristic feature of progressive external ophthalmoplegia is weakness or paralysis of the muscles that move the eye (ophthalmoplegia). Affected individuals have to turn their head to see in different directions, especially as the ophthalmoplegia worsens. People with progressive external ophthalmoplegia may also have general weakness of the muscles used for movement (myopathy), particularly those in the neck, arms, or legs. The weakness may be especially noticeable during exercise (exercise intolerance). Muscle weakness may also cause difficulty swallowing (dysphagia).When the muscle cells of affected individuals are stained and viewed under a microscope, these cells usually appear abnormal. These abnormal muscle cells contain an excess of cell structures called mitochondria and are known as ragged-red fibers.Although muscle weakness is the primary symptom of progressive external ophthalmoplegia, this condition can be accompanied by other signs and symptoms. In these instances, the condition is referred to as progressive external ophthalmoplegia plus (PEO+). Additional signs and symptoms can include hearing loss caused by nerve damage in the inner ear (sensorineural hearing loss), weakness and loss of sensation in the limbs due to nerve damage (neuropathy), impaired muscle coordination (ataxia), a pattern of movement abnormalities known as parkinsonism, and depression.Progressive external ophthalmoplegia is part of a spectrum of disorders with overlapping signs and symptoms. Similar disorders include ataxia neuropathy spectrum and Kearns-Sayre syndrome. Like progressive external ophthalmoplegia, the other conditions in this spectrum can involve weakness of the eye muscles. However, these conditions have many additional features not shared by most people with progressive external ophthalmoplegia.
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