Sympathetic ophthalmia

Sympathetic ophthalmia (SO) 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).

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, who injured his left eye as a child, lost vision in his right eye due 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.

Clinical features
Floating spots and loss of accommodation 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. 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).

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 hypersensitivity 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.
Diagnosis
Diagnosis is clinical, seeking a history of eye injury. An important differential diagnosis is Vogt-
Koyanagi-Harada syndrome (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 and pathologic fractures, mental status changes, etc.), may be candidates for therapy
with chlorambucil, cyclophosphamide, or ciclosporin.

Associated conditions
 Vogt-Koyanagi-Harada syndrome
Vernal keratoconjunctivitis
Vernal keratoconjunctivitis (VKC) or Spring catarrh is a recurrent, bilateral, and self limiting
inflammation of conjunctiva, having a periodic seasonal incidence.

Etiology
VKC is thought to be an allergic disorder in which IgE mediated mechanism play a role. Such
patients often give family history of other atopic diseases such as hay fever, asthma or eczema and
their peripheral blood shows eosinophiliaand increased serum IgE levels.

Predisposing factors
 Age & sex- 4–20 years; more common in boys than girls.
 Season- More common in summer. Hence the name Spring catarrh is a misnomer. Recently
it is being labelled as Warm weather conjunctivitis.
 Climate- More prevalent in the tropics.

Pathology
 Conjunctival epithelium undergoes hyperplasia and sends downward projection into sub-
epithelial tissue.
 Adenoid layer shows marked cellular infiltration by eosinophils, lymphocytes, plasma
cells and histiocytes.
 Fibrous layer show proliferation which later undergoes hyaline changes.
 Conjunctival vessels also show proliferation, increased permeability and vasodilation.

Clinical picture
 Symptoms- VKC is characterised by marked burning and itchy sensations which may be
intolerable and accentuates when patient comes in a warm humid atmosphere. Associated
symptoms include mild photophobia, lacrimation, stringy discharge and heaviness of
eyelids.

 Signs of VKC can be described in three clinical forms.

1. Palpebral form- Usually upper tarsal conjunctiva of both the eyes is involved. Typical lesion
is characterized by the presence of hard, flat topped papillae arranged in cobble stone or
pavement stone fashion. In severe cases papillae undergo hypertrophy to produce
cauliflower like excrescences of ' giant papillae '.
2. Bulbar form- It is characterised by dusky red triangular congestion of bulbar conjunctiva in
palpebral area, gelatinous thickened accumulation of tissue around limbus and presence of
discrete whitish raised dots along the limbus(Tranta's spots).
3. Mixed form- Shows the features of both palpebral and bulbar types.
Vernal keratopathy
Corneal involvement in VKC may be primary or secondary due to extension of limbal lesions.
Vernal keratopathy includes 5 types of lesions.

1. Punctuate epithelial keratitis.

2. Ulcerative vernal keratitis.
3. Vernal corneal plaques.
4. Subepithelial scarring.
5. Pseudogerontoxon.

Treatment
 Local therapy- Topical steroids are effective. Commonly used solutions are
of fluorometholone, medrysone, betamethasone or dexamethasone. Mast cell stabilizers such
as sodium cromoglycate (2%) drops 4-5 times a day are quite effective in controlling VKC,
especially atopic ones. Azelastine eyedrops are also effective. Topical antihistamines can be
used. Acetyl cysteine (.0.5%) used topically has mucolytic properties and is useful in the
treatment of early plaque formation. Topical Cyclosporine is reserved for unresponsive
cases.
 Systemic therapy- Oral antihistamines and oral steroids for severe cases.
 Treatment of large papillae- Cryo application, surgical excision or suupratarsal application
of long acting steroids.
 General measures include use of dark goggles to prevent photophobia, cold compresses and
ice pack for soothing effects, change of place from hot to cold areas.
 Desensitization has also been tried without much rewarding results.
 Treatment of vernal keratopathy- Punctuate epithelial keratitis require no extra treatment
except that instillation of steroids should be increased. Large vernal plaque requires surgical
excision. Ulcerative vernal keratitis require surgical treatment in the form of debridement,
superficial keratectomy, excimer laser therapeutic keratectomy, as well as amniotic
membrane transplantation to enhance re-epithelialisation.
Buphthalmos
Buphthalmos (plural: buphthalmoses) is a condition which affects the eyes of newborn humans. It
is sometimes called infant glaucoma or buphthalmia (plural buphthalmias).[1] It appears to be
an autosomal recessive trait. It appears in newborns or within the first 3 months of life.[2] An
abnormally narrow angle between thecornea and iris blocks the outflow of aqueous humor,[3] which
causes increased intraocular pressure and enlarged eyeball ("bulging").

Symptoms
Patients with buphthalmos will exhibit excessive tearing, extreme sensitivity to light
(photophobia), increased intraocular pressure, and distortion of the optic disc. The eyes appear
abnormally large, often with hazy corneas.

Prognosis and treatment

Untreated buphthalmos leads to total blindness. Surgical treatment is required, to open or enlarge
the Schlemm's canal. Presently-utilized surgical procedures include goniotomy, trabeculotomy,
or trabeculectomy.