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Richmond Eye Associates
Eye Health and Disorders
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Introduction: Medical Disorders Causing Eye Problems
This page discusses a selection of medical disorders which are known to lead
to a variety of eye problems. Links to other pages discussing medical conditions
causing eye problems are provided as well. There are many medical disorders
where eye complications are found as part of a larger symptom complex. In some
cases, several different parts of the eye, orbit, or visual system can be
affected by the condition, which is why they are discussed on this page rather
than on a specific symptom category page.
One example is thyroid related ocular
problems, which is discussed on the
Double
Vision page. This condition not only causes double vision due to eye muscle
involvement, but also can cause dry eye problems, eyelid problems, and potential
loss of vision. Since the eye muscle problems are relatively common with thyroid
dysfunction, it is discussed on that page.
In some cases, a medical physician may request an eye examination to
determine if the eyes are being affected by the medical condition. There are
numerous conditions which potentially have eye complications, and only a few are
discussed here.
This page is divided into a group of medical disorders which
also can affect the eyes. The next section discusses other medical disorders
discussed elsewhere that have ocular complications.
Read this important information
before proceeding further:
These sections are not intended to replace the professional examination and
diagnosis by a physician, and they are presented here purely for informational purposes.
All possible diagnoses and treatment options are not covered, and the information
discussed should not be taken as a recommendation to self-diagnose and self-treat a
condition. A misdiagnosed or improperly treated eye condition can result in a permanent
loss of vision, or a permanent loss of function of the eye or visual system. In the case
of any eye problem, seek medical attention promptly. This can include emergency room
treatment, as well as treatment by a medical physician or eyecare provider.
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Conditions Discussed on this Page:
For eye anatomy explanations, go to
ANATOMY
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Cicatricial Pemphigoid (also known as ocular cicatricial pemphigoid, and
benign mucous membrane pemphigoid) is a relatively rare chronic inflammatory
disease mainly affecting mucous membranes, such as the conjunctiva and
inside of the mouth. Sometimes the throat, esophagus, and other areas are
affected as well. In 25% of cases, the skin itself is involved. Patients
most commonly affected are females under the age of 60.
When the eyes are involved (75% of the time), the condition usually
begins as a chronic conjunctivitis, followed by scarring of the conjunctiva.
One, or more commonly both, eyes can be affected. Over time, this leads to
bands of scar tissue connecting the surface of the eye to the inside of the
eyelid (symblepharon), with a loss of the space between the eyelid and the
eye (the conjuctival fornix). This can lead to inturning of the eyelid
(entropion), and inturning of eyelashes (trichiasis) which can scratch the
eye. The scarring of the conjunctiva can lead to a loss of mucous secreting
cells which help to lubricate the eye, as well as closure of tear glands
(lacrimal ducts). This leads to drying of the corneal and ocular surface,
which potentially can cause symptoms of dry eye (gritty sensation, burning,
light sensitivity, and loss of vision), as well as more severe corneal
ulceration, scarring, and neovascularization (growth of blood vessels on the
corneal surface). Blindness from these problems occurs in 25% to 33% of
patients with the disorder. Other areas of the body can be involved as well,
requiring care from dermatologists, gastroenterologists, and ENT
specialists. A rheumatologist or internist may coordinate treatment of the
patient.
The diagnosis of the disorder is usually based on clinical findings.
However, biopsy of the conjunctiva, or other involved mucous membranes or
skin, can give a definitive diagnosis 80% of the time. Immunopathologic
techniques, such as direct immunofluorescence or direct immunoelecton
microscopy can identify linear immune deposits at the level of the
epithelial basement membrane of the examined tissue. Other disorders which
can simulate ocular cicatricial pemphigoid include:
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Acne rosacea
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Drug induced pseudo-pemphigoid
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Infectious disorders, such as fungal infections
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Chemical burns
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Sarcoidosis
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Squamous cell carcinoma of the conjunctiva
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Stevens-Johnson syndrome
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Trachoma
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Epidermal bullosa
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Atopic keratoconjunctivitis
The disorder requires systemic treatment (oral medication) rather than
simply local treatment to the eye. Immunosuppressive agents, such as
steroids and dapsone (a sulfa derivative), are used, but can have side
effects. Dapsone has showed improvement in the ocular and oral condition in
up to 88% of cases. Laboratory testing is required while dapsone is used to
rule out hemolytic anemia. In more severe cases, cyclophosphamide, steroids,
and azathioprine in combination can be used.
Supportive ocular care involves dealing with complications of the
conjunctival scarring, and restoring the ocular surface lubrication.
Preservative free lubricating ointments and drops need to be used
frequently. Treatment of eyelid malpositions and inturning eyelashes can
prevent corneal scarring. In some cases, corneal transplant may be necessary
to restore corneal clarity, but the results are often disappointing even
when the underlying disease can be controlled.
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Myasthenia Gravis is an autoimmune disease of the muscles leading to
weakness and easy fatigability. Commonly (80% to 90% of cases), the disorder
presents itself with drooping upper eyelids (ptosis) and double vision. The
symptoms are typically highly variable, intermittent, and may be
asymmetrical between the two eyes. There may also be speech and swallowing
difficulties, problems with facial expression, and weakness of the muscles
of the arms and legs. The symptoms tend to improve after rest and in the
morning, and tend to be worse later in the day and after exercise. The
underlying problem is the development of antibodies to neuromuscular
receptors present in all muscles (acetylcholine receptors). There is some
relationship with the thymus gland, and there is a positive family history
in about 5% of cases. Symptoms tend to appear in the middle age years for
men and women. Some cases have occurred after bone marrow transplantation.
Patients with prominent ocular symptoms often develop a fairly severe
drooping of one or both upper eyelids, worse when tired, that may interfere
with vision. The double vision associated with myasthenia can be variable,
and does not usually fit into a typical pattern of any one specific eye
muscle being involved. Two recent tests that can be suggestive of myasthenia
are the sleep test, and the ice pack test. In the sleep test, the degree of
eyelid drooping and double vision lessens after the patient sleeps, or rests
in a quiet, darkened room, for 30 minutes. In the ice pack test, the ice is
placed over the droopy eyelid for 2 minutes. If the drooping lessens by 2
millimeters or more, myasthenia may be a cause. There are other office tests
that can be performed to aid in diagnosis (Tensilon test).
Treatment of the disorder has typically been with drugs that block the
enzyme that degrades the neurotransmitter acetylcholine at the neuromuscular
junction. Thus, the neurotransmitter will stay in the junction longer, and
is better able to stimulate the muscle to contract. However, these drugs
seem to be less effective in reducing the ocular symptoms than those related
to other parts of the body. Steroids and other immunosuppressants such as
azathioprine have been successful in reducing ocular symptoms, and in
slowing the worsening of the disease over time. These medications are not
without side effects, and need to be closely monitored by the treating
physician. In some cases, removal of the thymus gland may help the disorder.
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Sarcoidosis is an inflammatory disease of the body which commonly affects
the eyes (25% to 50% of patients). It is more frequently seen in
African-Americans at a rate of 10:1 compared to Caucasians. Females tend to
be more commonly affected. Multiple systems throughout the body can show
symptoms. Granulomas, or inflammatory nodules, develop in organs and
throughout the body. They lungs are commonly affected (90% of cases), and
pulmonary function may be reduced. Chest X-rays are commonly monitored in
sarcoidosis, since nodules in and around the lungs can be visualized. Skin
nodules can occur, as well as muscle aches, generalized fatigue, and low
grade fever.
Less commonly, neurological and central nervous system involvement can
occur, potentially affecting the visual system. An inflammatory neuropathy
of the optic nerve of one or both eyes can lead to a loss of vision, or
blind spots in the vision. Involvement of the brain itself can cause visual
loss. Paralysis of the third cranial nerve has been reported, leading to
double vision, as well as the facial nerve (seventh cranial nerve), leading
to a paralysis of the facial muscles.
The most common ocular complication of sarcoid is inflammation within the
eye, known as iritis or uveitis. In fact, sarcoidosis is one of the most
common identifiable causes of uveitis in adults. Symptoms of uveitis can
range from ocular redness, aching, and sensitivity to light, to blurred
vision and floaters in the vision.
Eyelid nodules and orbital nodules can affect ocular movement, and in
some cases, cause protrusion of the eye itself. If the tear gland (lacrimal
gland) is involved, tear production may stop, and a significant dry eye
problem can ensue. In some cases, nodules of the conjunctiva can occur.
Biopsy of conjunctival or lacrimal gland nodules can be useful for
diagnosis.
The cause of sarcoidosis is unknown. Diagnosis is by clinical findings as
well as laboratory testing (elevation of the ACE level, or angiotensin
converting enzyme level), chest X-ray, biopsy of nodules, and in some cases,
Gallium scan of the head and neck. Treatment is by anti-inflammatory agents,
most commonly steroids by mouth. Since the disorder tends to be chronically
recurrent, the amount of steroid is titrated to the level of disease
activity. In some cases, sarcoid has only a mild, self-limited course. In
more severe cases, or in those affecting the central nervous system,
stronger anti-inflammatory agents such as cyclophosphamide may be needed.
Ocular steroids, usually in the form of eyedrops, can control inflammation,
but may lead to complications of cataract and glaucoma.
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Lyme disease is a multi-system disorder caused by an immune reaction to
the spirochete Borelia burgdorferi transmitted by the Dear tick (Ixodes
dammini). There are common ocular complications during all stages of
Lyme disease. The disease is most common in the Northeast and upper Midwest
United States. It involves joint pain and dermatological and ophthalmic
findings, as well as neurologic and cardiac abnormalities. Three stages of
Lyme disease have been described, which may overlap:
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Stage 1: The earliest stage includes a flu-like illness with a typical
expanding "bull's-eye" rash. This may or may not be associated
with a known tick bite. Some patients can develop conjunctivitis during
this stage.
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Stage 2: The second stage of the disease (after weeks to months of the
disease) includes cardiac involvement (8%) and neurologic involvement
(15%). This can include meningitis and paralysis of cranial nerves.
Paralysis of the third or sixth cranial nerves affect eye movement, and
lead to double vision. Paralysis of the seventh cranial nerve causes
Bell's palsy, or drooping of one side of the face. During this stage
inflammatory ocular disorders such as iritis, retinal vasculitis,
chorioretinitis, and optic disc edema can occur. These conditions can
cause a loss of vision.
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Stage 3: The last stage of the disease, starting within 2 weeks to 2
years of the infection, include arthritis and chronic neurologic
syndromes. This includes fatigue syndromes and focal central nervous
system disorders. Ocular findings in this stage include corneal
inflammation (keratitis) and double vision.
Treatment of Lyme disease is by commonly available antibiotics. Longer
treatment is required for cases with neurological involvement, and
intravenous antibiotics are required for severe neurological involvement and
arthritis.
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There are numerous malignancies that can affect virtually any part of the
eye, eyelids, orbit, and optic nerve. Fortunately, these conditions are very
rare. Due to the wide scope of information concerning ocular and orbital
malignancies, I recommend the following site for further information on this
subject: Eye Cancer.com.
This site contains an excellent review of ocular, orbital, and eyelid
malignancies, including photographs, case histories, and treatment options.
The site is written by a prominent specialist in the field of ocular cancer.
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