Sympathetic ophthalmia
Encyclopedia
Sympathetic ophthalmia is a granulomatous uveitis
(a kind of inflammation) of both eyes following trauma to one eye. It can leave the patient completely blind. Symptoms may develop from days to several years after a penetrating eye injury. See also the reviews by Damico et al. (2005), Chu and Foster (2002), and Friedlaender et al. (2001).
It is thought that Louis Braille
, who injured his left eye as a child, lost vision in his right eye owing to SO (Kaden 1977). James Thurber
's adult blindness was also diagnosed as sympathetic ophthalmia deriving from the loss of an eye when he was six years old.http://archopht.ama-assn.org/cgi/reprint/120/5/628.pdf
are among the earliest symptoms. The disease may progress to severe iridocyclitis
with pain and photophobia
. Commonly the eye remains relatively painless while the inflammatory disease spreads through the uvea
, where characteristic focal infiltrates in the choroid named Dalén-Fuchs nodules can be seen. The retina, however, usually remains uninvolved, although perivascular cuffing of the retinal vessels with inflammatory cells may occur. Papilledema
, secondary glaucoma
, vitiligo
, and poliosis
of the eyelashes may accompany SO.
In approximately 80% of cases, the uveitis appears within 2–12 weeks after injury, and 90% occur within 1 year from the time of injury. However, isolated cases as early as 1 week (2003) or as late as 66 years after initial injury have been reported (Zaharia et al., 1984).
to melanin
-containing structures from the outer segments of the photoreceptor layer of the retina
. The immune system, which normally is not exposed to ocular antigens, is introduced to the contents of the eye following traumatic injury. Once exposed, it senses these antigens as foreign, and begins attacking them. The onset of this process can be from days to years after the inciting traumatic event.
(VKH), which is thought to have the same pathogenesis, without a history of surgery or penetrating eye injury.
Still experimental, skin tests with soluble extracts of human or bovine uveal tissue are said to elicit delayed hypersensitivity responses in these patients. Additionally, circulating antibodies to uveal antigens have been found in patients with SO and VKH, as well as those with long-standing uveitis, making this a less than specific assay for SO and VKH.
Because SO is so rarely encountered following eye injury, even when the injured eye is retained, the first choice of treatment may not be enucleation or evisceration, especially if there is a chance that the injured eye may regain some function (Gurdal et al., 2002). Additionally, with current advanced surgical techniques, many eyes once considered nonviable now have a fair prognosis.
Immunosuppressive therapy is the mainstay of treatment for SO. When initiated promptly following injury, it is effective in controlling the inflammation and improving the prognosis. Mild cases may be treated with local application of corticosteroids and pupillary dilators. More severe or progressive cases require high-dose systemic corticosteroids for months to years. Patients who become resistant to corticosteroids or develop side effects of long-term corticosteroid therapy (osteoporosis
and pathologic fractures, mental status changes, etc.), may be candidates for therapy with chlorambucil
, cyclophosphamide
, or ciclosporin
.
Uveitis
Uveitis specifically refers to inflammation of the middle layer of the eye, termed the "uvea" but in common usage may refer to any inflammatory process involving the interior of the eye....
(a kind of inflammation) of both eyes following trauma to one eye. It can leave the patient completely blind. Symptoms may develop from days to several years after a penetrating eye injury. See also the reviews by Damico et al. (2005), Chu and Foster (2002), and Friedlaender et al. (2001).
History
The descriptions of sympathetic ophthalmia can be found in Greek texts (Albert and Diaz-Rohena, 1989), modern understanding of SO derives from the works of William MacKenzie, who characterized and named the disease sympathetic ophthalmitis. At that time, oral mercury and leeches applied to the conjunctiva were the treatments of choice for SO (MacKenzie, 1954).It is thought that Louis Braille
Louis Braille
Louis Braille was the inventor of braille, a system of reading and writing used by people who are blind or visually impaired...
, who injured his left eye as a child, lost vision in his right eye owing to SO (Kaden 1977). James Thurber
James Thurber
James Grover Thurber was an American author, cartoonist and celebrated wit. Thurber was best known for his cartoons and short stories published in The New Yorker magazine.-Life:...
's adult blindness was also diagnosed as sympathetic ophthalmia deriving from the loss of an eye when he was six years old.http://archopht.ama-assn.org/cgi/reprint/120/5/628.pdf
Clinical features
Floating spots and loss of accommodationAccommodation (eye)
Accommodation is the process by which the vertebrate eye changes optical power to maintain a clear image on an object as its distance changes....
are among the earliest symptoms. The disease may progress to severe iridocyclitis
Iridocyclitis
Iridocyclitis, a type of anterior uveitis, is a condition in which the uvea of the eye is inflamed.Iridocyclitis isInflammation of the iris and the ciliary body.- Symptoms :Symptoms include:* Photophobia* Redness* Watering of the eyes* Lacrimation...
with pain and photophobia
Photophobia
Photophobia is a symptom of abnormal intolerance to visual perception of light. As a medical symptom photophobia is not a morbid fear or phobia, but an experience of discomfort or pain to the eyes due to light exposure or by presence of actual physical photosensitivity of the eyes, though the term...
. Commonly the eye remains relatively painless while the inflammatory disease spreads through the uvea
Uvea
The uvea , also called the uveal layer, uveal coat, uveal tract, or vascular tunic, is the pigmented middle of the three concentric layers that make up an eye. The name is possibly a reference to its reddish-blue or almost black colour, wrinkled appearance and grape-like size and shape when...
, where characteristic focal infiltrates in the choroid named Dalén-Fuchs nodules can be seen. The retina, however, usually remains uninvolved, although perivascular cuffing of the retinal vessels with inflammatory cells may occur. Papilledema
Papilledema
Papilledema is optic disc swelling that is caused by increased intracranial pressure. The swelling is usually bilateral and can occur over a period of hours to weeks. Unilateral presentation is extremely rare....
, secondary glaucoma
Glaucoma
Glaucoma is an eye disorder in which the optic nerve suffers damage, permanently damaging vision in the affected eye and progressing to complete blindness if untreated. It is often, but not always, associated with increased pressure of the fluid in the eye...
, vitiligo
Vitiligo
Vitiligo is a condition that causes depigmentation of sections of skin. It occurs when melanocytes, the cells responsible for skin pigmentation, die or are unable to function. The cause of vitiligo is unknown, but research suggests that it may arise from autoimmune, genetic, oxidative stress,...
, and poliosis
Poliosis
Poliosis is the decrease or absence of melanin in head hair, eyebrows, or eyelashes. It can give rise to a "Mallen Streak" that can be hereditary....
of the eyelashes may accompany SO.
In approximately 80% of cases, the uveitis appears within 2–12 weeks after injury, and 90% occur within 1 year from the time of injury. However, isolated cases as early as 1 week (2003) or as late as 66 years after initial injury have been reported (Zaharia et al., 1984).
Epidemiology
Sympathetic ophthalmia is rare, affecting 0.2% to 0.5% of non-surgical eye wounds, and less than 0.1% of surgical penetrating eye wounds. There are no gender or racial differences in incidence of SO.Pathophysiology
Sympathetic ophthalmia is currently thought to be an autoimmune inflammatory response toward ocular antigens, specifically a delayed hypersensitivityHypersensitivity
Hypersensitivity refers to undesirable reactions produced by the normal immune system, including allergies and autoimmunity. These reactions may be damaging, uncomfortable, or occasionally fatal. Hypersensitivity reactions require a pre-sensitized state of the host. The four-group classification...
to melanin
Melanin
Melanin is a pigment that is ubiquitous in nature, being found in most organisms . In animals melanin pigments are derivatives of the amino acid tyrosine. The most common form of biological melanin is eumelanin, a brown-black polymer of dihydroxyindole carboxylic acids, and their reduced forms...
-containing structures from the outer segments of the photoreceptor layer of the retina
Retina
The vertebrate retina is a light-sensitive tissue lining the inner surface of the eye. The optics of the eye create an image of the visual world on the retina, which serves much the same function as the film in a camera. Light striking the retina initiates a cascade of chemical and electrical...
. The immune system, which normally is not exposed to ocular antigens, is introduced to the contents of the eye following traumatic injury. Once exposed, it senses these antigens as foreign, and begins attacking them. The onset of this process can be from days to years after the inciting traumatic event.
Diagnosis
Diagnosis is clinical, seeking a history of eye injury. An important differential diagnosis is Vogt-Koyanagi-Harada syndromeVogt-Koyanagi-Harada syndrome
Vogt–Koyanagi–Harada syndrome is a condition seen in humans and dogs involving various melanocyte-containing organs, characterized by uveitis , poliosis , vitiligo , and meningitis, although dogs with this syndrome rarely develop meningitis...
(VKH), which is thought to have the same pathogenesis, without a history of surgery or penetrating eye injury.
Still experimental, skin tests with soluble extracts of human or bovine uveal tissue are said to elicit delayed hypersensitivity responses in these patients. Additionally, circulating antibodies to uveal antigens have been found in patients with SO and VKH, as well as those with long-standing uveitis, making this a less than specific assay for SO and VKH.
Prevention and treatment
Definitive prevention of SO requires prompt (within the first 7 to 10 days following injury) enucleation of the injured eye. Evisceration—the removal of the contents of the globe while leaving the sclera and extraocular muscles intact—is easier to perform, offers long-term orbital stability, and is more aesthetically pleasing. There is concern, however, that evisceration may lead to a higher incidence of SO compared to enucleation (reviewed by Migliori, 2002). Several retrospective studies involving over 3000 eviscerations, however, have failed to identify a single case of SO.Because SO is so rarely encountered following eye injury, even when the injured eye is retained, the first choice of treatment may not be enucleation or evisceration, especially if there is a chance that the injured eye may regain some function (Gurdal et al., 2002). Additionally, with current advanced surgical techniques, many eyes once considered nonviable now have a fair prognosis.
Immunosuppressive therapy is the mainstay of treatment for SO. When initiated promptly following injury, it is effective in controlling the inflammation and improving the prognosis. Mild cases may be treated with local application of corticosteroids and pupillary dilators. More severe or progressive cases require high-dose systemic corticosteroids for months to years. Patients who become resistant to corticosteroids or develop side effects of long-term corticosteroid therapy (osteoporosis
Osteoporosis
Osteoporosis is a disease of bones that leads to an increased risk of fracture. In osteoporosis the bone mineral density is reduced, bone microarchitecture is deteriorating, and the amount and variety of proteins in bone is altered...
and pathologic fractures, mental status changes, etc.), may be candidates for therapy with chlorambucil
Chlorambucil
Chlorambucil is a chemotherapy drug that has been mainly used in the treatment of chronic lymphocytic leukemia...
, cyclophosphamide
Cyclophosphamide
Cyclophosphamide , also known as cytophosphane, is a nitrogen mustard alkylating agent, from the oxazophorines group....
, or ciclosporin
Ciclosporin
Ciclosporin , cyclosporine , cyclosporin , or cyclosporin A is an immunosuppressant drug widely used in post-allogeneic organ transplant to reduce the activity of the immune system, and therefore the risk of organ rejection...
.