Elsevier

Survey of Ophthalmology

Volume 44, Issue 3, Novemberā€“December 1999, Pages 187-213
Survey of Ophthalmology

Review
Nonthyroid Causes of Extraocular Muscle Disease

https://doi.org/10.1016/S0039-6257(99)00101-0Get rights and content

Abstract

Extraocular muscle involvement in orbital disease is most frequently seen as a feature of thyroid orbitopathy (Graves' disease). However, a wide range of other conditions may alter the size, shape, and function of these muscles, with characteristic clinical manifestations or abnormalities visible on orbital imaging. The differential diagnosis of muscle disease can be narrowed by careful analysis of clinical features and ancillary tests. Imaging facilitates recognition in many cases, but in some instances, accurate diagnosis requires biopsy. This review highlights the differential diagnoses for diseases of extraocular muscles based on the clinical and investigative records of 103 patients at our institution combined with data from the world literature. We found that the most common nonthyroid causes of muscle disease were inflammatory, vascular, and neoplastic processes (in decreasing order of frequency). Emphasis is placed on investigations that provide a logical approach to, and appropriate management of, disease of the extraocular muscles.

Section snippets

Incidence

The exact incidence of extraocular muscle disease is difficult to quantify accurately because of underreporting of many cases of disorders, such as thyroid orbitopathy and arteriovenous shunts, in which involvement of extraocular muscles is seen and expected. Imaging techniques have allowed adequate resolution of the extraocular muscles only within the past 20 years. Reports of muscle involvement before the availability of computed tomography (CT) were, in the absence of biopsy, based on

Anatomy and function

There are seven muscles in each orbit: the four recti, the inferior and superior obliques, and the levator palpebrae superioris. The recti and the superior oblique arise apically from the annulus of Zinn and insert into the anterior sclera at various distances from the corneoscleral limbus along the spiral of Tillaux. The inferior oblique arises from the orbital floor just lateral to the nasolacrimal duct; it and the tendinous portion of the superior oblique muscle pass laterally and slightly

Summary of the Diagnostic Features of Thyroid Orbitopathy

Thyroid orbitopathy is responsible for approximately 50% of orbital disorders seen at our clinic. It is thought to be an organ-specific autoimmune process177, 179 and is associated with thyroid disease in 80% to 90% of cases.7, 134 Characteristics of the disease include inflammation, edema, and secondary fibrosis, and bilateral symmetric muscle involvement is seen in 70% of cases.124 On imaging, we found that the superior muscle group is the most commonly affected,112 but the pattern of

Nonspecific Orbital Myositis

The cause of this condition is unclear, but it is considered to be a variant of the nonspecific orbital inflammatory syndrome. In our experience, myositis may occur as three different types: isolated, recurrent, and atypical (authors' work in progress).

In the isolated type, the orbits manifest the typical features of inflammation. There is a short history (days to weeks) of pain, swelling and/or diplopia, and patients frequently complain of exacerbated pain on active or passive movement of the

Clinical Presentation of Extraocular Muscle Disease

Extraocular muscle disease may present with a number of symptoms and signs, including diplopia, pain, proptosis, ptosis, lid swelling, episcleral injection, and blurring of vision. The temporal characteristics will reflect the underlying pathophysiologic process. Orbital myositis is usually subacute in onset (occurring over days to weeks) and frequently associated with pain. Vascular congestion caused by arteriovenous fistulas may be catastrophic in direct CCF, or it may have a gradual onset

Imaging techniques

Computed tomography, ultrasonography, and magnetic resonance imaging (MRI) are complementary techniques that allow demonstration of normal and pathologic orbital features and demonstrate extraocular muscle involvement.

Computed Tomography

High-resolution axial and coronal scans clearly display orbital structures and lesions in a familiar format and, therefore, are the investigation of first choice for most disorders. The low-density fat provides an excellent natural contrast for the extraocular muscles, and

Ocular and orbital disease

Orbital myositis (nonspecific) may, rarely, be accompanied by optic neuritis, and we have noted optic nerve sheath thickening and superior ophthalmic vein enlargement on CT scans. Visual loss is uncommon in our experience and in that reported by others. Specific forms of myositis may be associated with uveitis, retinal vasculitis (SLE), or dacryoadenitis (sarcoid). Scleritis may accompany myositis, but this usually implies a necrotizing disease or a vasculitis, such as Wegener's granulomatosis.

Treatment

Orbital myositis (nonspecific) may respond to nonsteroidal anti-inflammatory drugs, particularly if it affects a single muscle and it is the primary episode, but it is characteristically very sensitive to corticosteroids.103, 104 We suggest the use of nonsteroidal anti-inflammatory drugs or low-dose steroids (20 mg of prednisone) as a primary treatment with single muscle involvement or if the disease is particularly mild. On the other hand, if there is multiple muscle involvement or bilateral

Summary

Discrete extraocular muscle disease is most commonly associated with thyroid orbitopathy. In a review of the literature and in our own experience, we have identified the most common nonthyroid conditions as inflammatory, vascular, and neoplastic. The majority of cases can be diagnosed by assessing the nature and temporal progression of orbital symptoms and signs, with collaborative information from systemic history and examination, combined with orbital imaging studies. Diagnostic dilemmas

Method of Literature Search

The literature was searched by MEDLINE (up to January 1998). Broad subject searches were conducted on a variety of headings related to extraocular muscles, extraocular muscle diseases, ocular motility diseases, and orbital diseases. Citations within material obtained from the MEDLINE search were also used. General ophthalmology texts also provided information; these included Duane's Clinical Ophthalmology, Albert and Jakobiec's Principles and Practice of Ophthalmology, and Walsh and Hoyt's

Outline

I. Incidence

II. Anatomic, embryologic, physiologic, and pathophysiologic background

A. Anatomy and function

B. Embryology and physiology

C. Pathophysiology

III. Summary of the diagnostic features of thyroid orbitopathy

IV. Nonthyroid causes of extraocular muscle disease (Table 3)

A. Inflammatory disorders

1. Nonspecific orbital myositis

2. Specific myositis

a. Systemic lupus erythematosus

b. Sarcoidosis

c. Crohn's disease

d. Giant cell myocarditis

e. Linear scleroderma

f. Sclerosing inflammation

g. Other

Acknowledgements

The authors would like to thank Dr. Subrahmanyam Mallajosyula, Professor of Ophthalmology at the Regional Eye Hospital in Warangal, India, for his permission to use Figure 9. The authors would also like to thank Dr. Jeffrey Nerad, Professor of Ophthalmology at the University of Iowa for the use of the information displayed in Table 4.

The authors have no proprietary or commercial interest in any product or concept discussed in this article.

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