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The exact cause of IOI is unknown, but infectious and immune-mediated mechanisms have been proposed. Several studies have described cases where onset of orbital pseudotumor was seen simultaneously or several weeks after upper respiratory infections. Another study by Wirostko et al. proposes that organisms resembling Mollicutes cause orbital inflammation by destroying the cytoplasmic organelles of parasitized cells.
Orbital pseudotumor has also been observed in association with Crohn’s disease, systemic lupus erythematosus, rheumatoid arthritis, diabetes mellitus, myasthenia gravis, and ankylosing spondylitis all of which strengthen the basis of IOI being an immune-mediated disease. Response to corticosteroid treatment and immunosuppressive agents also support this idea.
Trauma has also been seen to precede some cases of orbital pseudotumor. However, one study by Mottow-Lippe, Jakobiec, and Smith suggests that the release of circulating antigens caused by local vascular permeability triggers an inflammatory cascade in the affected tissues.
Although these mechanisms have been postulated as possible causes of IOI, their exact nature and relationships to the condition still remain unclear.
IOI or orbital pseudotumor is the second most common cause of exophthalmos following Grave’s orbitopathy and the third most common orbital disorder following thyroid orbitopathy and lymphoproliferative disease accounting for 5–17.6% of orbital disorders, There is no age, sex, or race predilection, but it is most frequently seen in middle-aged individuals. Pediatric cases account for about 17% of all cases of IOI.
IgG4-related ophthalmic disease (IgG4-ROD) is the recommended term to describe orbital (eye socket) manifestations of the systemic condition IgG4-related disease, which is characterised by infiltration of lymphocytes and plasma cells and subsequent fibrosis in involved structures. It can involve one or more of the orbital structures.
Frequently involved structures include the lacrimal glands, extraocular muscles, infraorbital nerve, supraorbital nerve and eyelids. It has also been speculated that ligneous conjunctivitis may be a manifestation of IgG4-related disease (IgG4-RD).
As is the case with other manifestations of IgG4-related disease, a prompt response to steroid therapy is a characteristic feature of IgG4-ROD in most cases, unless significant fibrosis has already occurred.
Symptoms, if any, can be mild even in the presence of significant swelling or masses.
Lacrimal gland involvement may cause swelling of the upper eyelid, or proptosis if there is severe swelling. Other orbital masses or inflammation can result in visual disturbance (blurred vision, double vision, visual field impairment), restricted eye movements, pain or discomfort, numbness in the distribution of the supraorbital and/or infraorbital nerves, or proptosis.
IgG4-related ophthalmic disease has been estimated to account for approximately 25% of all cases of proptosis, eyelid swelling and other features of orbital swelling.
Most patients will fully recover from dacryoadenitis. For conditions with more serious causes, such as sarcoidosis, the prognosis is that of the underlying condition.
Risk factors of progressive and severe thyroid-associated orbitopathy are:
- Age greater than 50 years
- Rapid onset of symptoms under 3 months
- Cigarette smoking
- Diabetes
- Severe or uncontrolled hyperthyroidism
- Presence of pretibial myxedema
- High cholesterol levels (hyperlipidemia)
- Peripheral vascular disease
Sympathetic ophthalmia is rare, affecting 0.2% to 0.5% of non-surgical eye wounds, and less than 0.01% of surgical penetrating eye wounds. There are no gender or racial differences in incidence of SO.
Bacterial infections of the orbit have long been associated with a risk of catastrophic local
sequelae and intracranial spread.
The natural course of the disease, as documented by Gamble (1933), in the pre-antibiotic era,
resulted in death in 17% of patients and permanent blindness in 20%.
It is usually caused by allergies or viral infections, often inciting excessive eye rubbing. Chemosis is also included in the Chandler Classification system of orbital infections.
If chemosis has occurred due to excessive rubbing of the eye, the first aid to be given is a cold water wash for eyes.
Other causes of chemosis include:
- Superior vena cava obstruction, accompanied by facial oedema
- Hyperthyroidism, associated with exophthalmos, periorbital puffiness, lid retraction, and lid lag
- Cavernous sinus thrombosis, associated with infection of the paranasal sinuses, proptosis, periorbital oedema, retinal haemorrhages, papilledema, extraocular movement abnormalities, and trigeminal nerve sensory loss
- Carotid-cavernous fistula - classic triad of chemosis, pulsatile proptosis, and ocular bruit
- Cluster headache
- Trichinellosis
- Systemic lupus erythematosus (SLE)
- Angioedema
- Acute glaucoma
- Panophthalmitis
- Orbital cellulitis
- Gonorrheal conjunctivitis
- Dacryocystitis
- Spitting cobra venom to the eye
- High concentrations of phenacyl chloride in chemical mace spray
- Urticaria
- Trauma
- Post surgical
- Rhabdomyosarcoma of the orbit
If the cause of dacryoadenitis is a viral condition such as mumps, simple rest and warm compresses may be all that is needed. For other causes, the treatment is specific to the causative disease.
Periorbital cellulitis, also known as preseptal cellulitis (and not to be confused with orbital cellulitis, which is behind the septum), is an inflammation and infection of the eyelid and portions of skin around the eye, anterior to the orbital septum. It may be caused by breaks in the skin around the eye, and subsequent spread to the eyelid; infection of the sinuses around the nose (sinusitis); or from spread of an infection elsewhere through the blood.
Inflammatory/Infection:
- Graves' ophthalmopathy due to Graves' disease, usually causes bilateral proptosis.
- Orbital cellulitis – often with unilateral proptosis, severe redness, and moderate to severe pain, sinusitis and an elevated white blood cell count.
- Dacryoadenitis
- Erdheim-Chester Disease
- Mucormycosis
- Orbital pseudotumor – presents with acute, usually unilateral proptosis with severe pain.
- High altitude cerebral edema
- Granulomatosis with polyangiitis
Neoplastic:
- Leukemias
- Meningioma, (of sphenoid wing)
- Nasopharyngeal angiofibroma
- Hand–Schüller–Christian disease
- Hemangioma, cavernous
Cystic:
- Dermoid cyst
Vascular:
- Carotid-cavernous fistula
- Aortic insufficiency: manifests as a pulsatile pseudoproptosis, described by British cardiothoracic surgeon, Hutan Ashrafian in 2006
Others:
- Orbital fracture: apex, floor, medial wall, zygomatic
- Retrobulbar hemorrhage: trauma to the orbit can lead to bleeding behind the eye. The hemorrhage has nowhere to escape and the increased pressure pushes the eye out of the socket, leading to proptosis and can also cause blindness if not treated promptly.
- Cushing's syndrome (due to fat in the orbital cave)
- Pfeiffer syndrome
Enophthalmos is the posterior displacement of the eyeball within the orbit due to changes in the volume of the orbit (bone) relative to its contents (the eyeball and orbital fat), or loss of function of the orbitalis muscle. It should not be confused with its opposite, exophthalmos, which is the anterior displacement of the eye.
It may be a congenital anomaly, or be acquired as a result of trauma (such as in a blowout fracture of the orbit), Horner's syndrome (apparent enophthalmos due to ptosis), Marfan syndrome, Duane's syndrome, silent sinus syndrome or phthisis bulbi.
Antibiotics are aimed at gram positive bacteria. Medical attention should be sought if symptoms persist beyond 2–3 days.
Trochleitis is diagnosed based on three criteria: 1) demonstration of inflammation of superior oblique tendon/ trochlea region, 2) periorbital pain and tenderness to palpation in the area of the sore trochlea, and 3) worsening of pain on attempted vertical eye movement, particularly with adduction of the eye. It is important to identify trochleitis because it is a treatable condition and the patient can benefit much from pain relief. Treatment consists of a single injection of corticosteroids to the affected peritrochlear region. A specific "cocktail" consisting of 0.5 ml of depomedrol (80 mg/ml) and 0.5 ml of 2% lidocaine can be injected into the trochlea; immediate relief due to the effects of the local anesthetic indicates successful placement. However, great care must be taken as the injection is in the region of several arteries, veins and nerves. The needle should not be too small (so as not to penetrate tiny structures), the surgeon should draw back on the syringe (to ensure not have pierced a vessel), the lidocaine should not contain epinephrine (which could cause vasospasm), and the pressure of the injection must always be controlled. Only a limited number of injections can be made as they would otherwise lead to muscle atrophy. Diagnosis can be confirmed by response to this treatment; pain and swelling are expected to disappear in 48–72 hours. Some patients experience recurrence of trochleitis.
Complications include hearing loss, blood infection, meningitis, cavernous sinus thrombosis, and optic nerve damage (which could lead to blindness).
Dermatochalasis commonly affects the elderly, although sometimes it is congenitally acquired. The elderly version may begin to develop as early as 40 years of age, and it continues to progress with age. The congenital version may begin around 20 years of age. There is no racial predisposition towards developing dermatochalasis, and men and women are equally affected.
Orbital fractures, in general, are more prevalent in men than women. In one study in children, 81% of cases were boys (mean age 12.5 years). In another study in adults, men accounted for 72% of orbital fractures (mean age 81).
The pathology mostly affects persons of 30 to 50 years of age. Females are four times more likely to develop TAO than males. When males are affected, they tend to have a later onset and a poor prognosis. A study demonstrated that at the time of diagnosis, 90% of the patients with clinical orbitopathy were hyperthyroid according to thyroid function tests, while 3% had Hashimoto's thyroiditis, 1% were hypothyroid and 6% did not have any thyroid function tests abnormality. Of patients with Graves' hyperthyroidism, 20 to 25 percent have clinically obvious Graves' ophthalmopathy, while only 3–5% will develop severe ophthalmopathy.
Exophthalmos (also called exophthalmus, exophthalmia, proptosis, or exorbitism) is a bulging of the eye anteriorly out of the orbit. Exophthalmos can be either bilateral (as is often seen in Graves' disease) or unilateral (as is often seen in an orbital tumor). Complete or partial dislocation from the orbit is also possible from trauma or swelling of surrounding tissue resulting from trauma.
In the case of Graves' disease, the displacement of the eye is due to abnormal connective tissue deposition in the orbit and extraocular muscles which can be visualized by CT or MRI.
If left untreated, exophthalmos can cause the eyelids to fail to close during sleep leading to corneal dryness and damage. Another possible complication would be a form of redness or irritation called "Superior limbic keratoconjunctivitis", where the area above the cornea becomes inflamed as a result of increased friction when blinking. The process that is causing the displacement of the eye may also compress the optic nerve or ophthalmic artery, leading to blindness.
Affected individuals are typically 20 to 50 years old. The female to male ratio is 2:1. By definition, there is no history of either surgical or accidental ocular trauma. VKH is more common in Asians, Latinos, Middle Easterners, American Indians, and Mexican Mestizos; it is much less common in Caucasians and in blacks from sub-Saharan Africa.
VKH is associated with a variety of genetic polymorphisms that relate to immune function. For example, VKH has been associated with human leukocyte antigens (HLA) HLA-DR4 and DRB1/DQA1, copy-number variations (CNV) of complement component 4, a variant IL-23R locus and with various other non-HLA genes. HLA-DRB1*0405 in particular appears to play an important susceptibility role.
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. Additionally, with current advanced surgical techniques, many eyes once considered nonviable now have a fair prognosis.
However, only if the injured eye has completely lost its vision and has no potential for any visual recovery, prevention of SO is done by enucleation of the injured eye preferably within the first 2 weeks of injury. 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, i.e., a greater measure of movement of the prosthesis and thus a more natural appearance. There is concern, however, that evisceration may lead to a higher incidence of SO compared to enucleation. Several retrospective studies involving over 3000 eviscerations, however, have failed to identify a single case of SO.
Once SO is developed, Immunosuppressive therapy is the mainstay of treatment. 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.
Chemosis is the swelling (or edema) of the conjunctiva. It is due to the oozing of exudate from abnormally permeable capillaries. In general, chemosis is a nonspecific sign of eye irritation. The outer surface covering appears to have fluid in it. The conjunctiva becomes swollen and gelatinous in appearance. Often, the eye area swells so much that the eyes become difficult or impossible to close fully. Sometimes, it may also appear as if the eyeball has moved slightly backwards from the white part of the eye due to the fluid filled in the conjunctiva all over the eyes except the iris. The iris is not covered by this fluid and so it appears to be moved slightly inwards.
Although there is sometimes a preceding viral infection, or skin or eye trauma, the exact underlying initiator of VKH disease remains unknown. However, VKH is attributed to aberrant T-cell-mediated immune response directed against self-antigens found on melanocytes. Stimulated by interleukin 23 (IL-23), T helper 17 cells and cytokines such as interleukin 17 (IL-17) appear to target proteins in the melanocyte.
A recent study estimated that from 2002-2003 there were 27,152 injuries in the United States related to the wearing of eyeglasses. The same study concluded that sports-related injuries due to eyeglasses wear were more common in those under the age of 18 and that fall-related injuries due to eyeglasses wear were more common in those aged 65 or more. Although eyeglasses-related injuries do occur, prescription eyeglasses and non-prescription sunglasses have been found to "offer measurable protection which results in a lower incidence of severe eye injuries to those wearing [them]".
In India study conducted by Dr.Shukla, injuries are found more in n males(81%).This is true for both rural and urban population but in 0-10 age group, the difference between males and females is less.Females account for 28% injuries in this age group.However, in sedentary workers, farmers, labourers and industrial workers the male % is as high as 95%.Chemical injuries are the comments cause of bilateral injuries in the eye .