Fuchs heterochromic iridocyclitis | |
---|---|
Specialty | Ophthalmology |
Fuchs heterochromic iridocyclitis (FHI) is a chronic unilateral uveitis appearing with the triad of heterochromia, predisposition to cataract and glaucoma, and keratitic precipitates on the posterior corneal surface. Patients are often asymptomatic and the disease is often discovered through investigation of the cause of the heterochromia or cataract. Neovascularisation (growth of new abnormal vessels) is possible and any eye surgery, such as cataract surgery, can cause bleeding from the fragile vessels in the atrophic iris causing accumulation of blood in the anterior chamber of the eye, also known as hyphema.
This condition is usually unilateral, and its symptoms vary from none to mild blurring and discomfort. Signs include diffuse iris atrophy and small white keratic precipitates (deposits on the inner surface of the cornea), cells presenting in the anterior chamber as well as the anterior vitreous. Glaucoma and cataract occur frequently.
According to recent research, not a single theory is able to explain the cause fully. However current plausible theories include infection with Toxoplasma gondii , Herpes simplex virus, Rubella, neurogenic causes, and autoimmune pathology.
Diagnosis is made by an ophthalmologist or optometrist based on the clinical presentation. One indication can be the Amsler sign, which is the presence of blood (hyphema) in the aspirated vitreous fluid, in paracentesis of the anterior chamber. This is caused due to iris atrophy usually seen in FHI and exposure of the fragile iris vasculature to the vitreous fluid. The sudden change of pressure in the anterior chamber upon suction induced by the paracentesis, or during cataract surgery, causes bursting of the fragile superficial iris capillaries resulting in micro-bleeding. This is one clinical diagnostic sign of FHI slit-lamp examination shows stringy keratic precipitates
Patients usually do not require treatment due to the benign nature of the disease. In case a cataract develops, patients generally do well with cataract surgery.
First described using available patient presentations observed, by an Austrian ophthalmologist, Ernst Fuchs in 1906.
Classification | |
---|---|
External resources |
Ophthalmology is a branch of medicine and surgery that deals with the diagnosis and treatment of disorders of the eye. An ophthalmologist is a physician who specializes in ophthalmology. The credentials include a degree in medicine, followed by additional four to five years of residency training in ophthalmology. Residency training programs for ophthalmology may require a one-year internship with training in internal medicine, pediatrics, or general surgery. Additional specialty training may be sought in a particular aspect of eye pathology. Ophthalmologists are allowed to prescribe medications to treat eye diseases, implement laser therapy, and perform surgery when needed. Ophthalmologists may participate in academic research on the diagnosis and treatment for eye disorders.
Glaucoma is a group of eye diseases which result in damage to the optic nerve and cause vision loss. The most common type is open-angle glaucoma, in which the drainage angle for fluid within the eye remains open, with less common types including closed-angle glaucoma and normal-tension glaucoma. Open-angle glaucoma develops slowly over time and there is no pain. Peripheral vision may begin to decrease, followed by central vision, resulting in blindness if not treated. Closed-angle glaucoma can present gradually or suddenly. The sudden presentation may involve severe eye pain, blurred vision, mid-dilated pupil, redness of the eye, and nausea. Vision loss from glaucoma, once it has occurred, is permanent. Eyes affected by glaucoma are referred to as being glaucomatous.
Vitrectomy is a surgery to remove some or all of the vitreous humor from the eye.
Heterochromia is a variation in coloration. The term is most often used to describe color differences of the iris, but can also be applied to color variation of hair or skin. Heterochromia is determined by the production, delivery, and concentration of melanin. It may be inherited, or caused by genetic mosaicism, chimerism, disease, or injury. It occurs in humans and certain breeds of domesticated animals.
Intraocular lens (IOL) is a lens implanted in the eye as part of a treatment for cataracts or myopia. If someone has a natural lens in the eye it is known as phakic and if someone has an artificial lens in the eye it is known as pseudophakic or false lens. These are implanted during cataract surgery, after the cloudy eye's natural lens has been removed. The pseudophakic IOL provides the same light focusing function as the natural crystalline lens. The second type of IOL, more commonly known as a phakic intraocular lens (PIOL), is a lens which is placed over the existing natural lens and is used in refractive surgery to change the eye's optical power as a treatment for myopia (nearsightedness). In other words this is an alternative to Lasik.
A phakic intraocular lens (PIOL) is a special kind of intraocular lens that is implanted surgically into the eye to correct myopia (nearsightedness). It is called "phakic" because the eye's natural lens is left untouched. Intraocular lenses that are implanted into eyes after the eye's natural lens has been removed during cataract surgery are known as pseudophakic.
Uveitis is the inflammation of the uvea, the pigmented layer that lies between the inner retina and the outer fibrous layer composed of the sclera and cornea. The uvea consists of the middle layer of pigmented vascular structures of the eye and includes the iris, ciliary body, and choroid. Uveitis is an ophthalmic emergency and requires a thorough examination by an ophthalmologist or optometrist and urgent treatment to control the inflammation. It is commonly associated with other ocular disorders, such as glaucoma, retinal detachment, optic nerve damage, cataracts, and, in some cases, a permanent loss of vision.
Cataract surgery, also called lens replacement surgery, is the removal of the natural lens of the eye that has developed an opacification, which is referred to as a cataract, and its replacement with an intraocular lens. Metabolic changes of the crystalline lens fibers over time lead to the development of the cataract, causing impairment or loss of vision. Some infants are born with congenital cataracts, and certain environmental factors may also lead to cataract formation. Early symptoms may include strong glare from lights and small light sources at night, and reduced acuity at low light levels.
Sturge–Weber syndrome, sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and skin disorder. It is one of the phakomatoses and is often associated with port-wine stains of the face, glaucoma, seizures, intellectual disability, and ipsilateral leptomeningeal angioma. Sturge–Weber syndrome can be classified into three different types. Type 1 includes facial and leptomeningeal angiomas as well as the possibility of glaucoma or choroidal lesions. Normally, only one side of the brain is affected. This type is the most common. Type 2 involvement includes a facial angioma with a possibility of glaucoma developing. There is no evidence of brain involvement. Symptoms can show at any time beyond the initial diagnosis of the facial angioma. The symptoms can include glaucoma, cerebral blood flow abnormalities and headaches. More research is needed on this type of Sturge–Weber syndrome. Type 3 has leptomeningeal angioma involvement exclusively. The facial angioma is absent and glaucoma rarely occurs. This type is only diagnosed via brain scan.
Aphakia is the absence of the lens of the eye, due to surgical removal, such as in cataract surgery, a perforating wound or ulcer, or congenital anomaly. It causes a loss of accommodation, high degree of farsightedness (hyperopia), and a deep anterior chamber. Complications include detachment of the vitreous or retina, and glaucoma.
Hyphema is a condition that occurs when blood enters the front (anterior) chamber of the eye between the iris and the cornea. People usually first notice a loss of vision or decrease in vision. The eye may also appear to have a reddish tinge, or it may appear as a small pool of blood at the bottom of the iris or in the cornea. A traumatic hyphema is caused by a hit to the eye from a projected object or a blow to the eye. A hyphema can also occur spontaneously.
The anterior chamber (AC) is the aqueous humor-filled space inside the eye between the iris and the cornea's innermost surface, the endothelium. Hyphema, anterior uveitis and glaucoma are three main pathologies in this area. In hyphema, blood fills the anterior chamber as a result of a hemorrhage, most commonly after a blunt eye injury. Anterior uveitis is an inflammatory process affecting the iris and ciliary body, with resulting inflammatory signs in the anterior chamber. In glaucoma, blockage of the trabecular meshwork prevents the normal outflow of aqueous humour, resulting in increased intraocular pressure, progressive damage to the optic nerve head, and eventually blindness.
Ectopia lentis is a displacement or malposition of the eye's crystalline lens from its normal location. A partial dislocation of a lens is termed lens subluxation or subluxated lens; a complete dislocation of a lens is termed lens luxation or luxated lens.
Iris cysts are hollow cavities in the eye filled with secretion. They come in various sizes, numbers, shapes, pigments and can be free-floating, attached to the pupillary margin or within the posterior chamber. Most frequently iris cysts don't cause any issues, but they can cause problems like: “fly biting” behavior, corneal endothelial pigment, lens capsular pigmentation, altered iris movement, decreased aqueous outflow with subsequent glaucoma or block the vision when grown too big. They can be acquired or innate. Possible causes are inflammation, drug-induced, uveitis, a trauma, tumor-induced, parasitic or implantation. Most frequently iris cysts are benign and need no treatment. Sometimes iris cysts are causing problems and need to be deflated. Iris cysts can be treated with trans corneal diode laser treatment, fine-needle aspiration or surgical excision. For the treatment of iris cysts is a conservative approach favored.
Hypopyon is a medical condition involving inflammatory cells in the anterior chamber of the eye.
Intermediate uveitis is a form of uveitis localized to the vitreous and peripheral retina. Primary sites of inflammation include the vitreous of which other such entities as pars planitis, posterior cyclitis, and hyalitis are encompassed. Intermediate uveitis may either be an isolated eye disease or associated with the development of a systemic disease such as multiple sclerosis or sarcoidosis. As such, intermediate uveitis may be the first expression of a systemic condition. Infectious causes of intermediate uveitis include Epstein-Barr virus infection, Lyme disease, HTLV-1 virus infection, cat scratch disease, and hepatitis C.
Juvenile xanthogranuloma is a form of histiocytosis, classified as "non-Langerhans cell histiocytosis", or more specifically, "type 2".
Amsler sign also known as Amsler–Verrey sign is the name of the diagnostic finding seen in people with Fuchs heterochromic iridocyclitis (FHI). It is described as presence of blood (hyphema) in the aspirated vitreous fluid, in paracentesis of the anterior chamber, and is caused due to iris atrophy usually seen in FHI and exposure of the fragile iris vasculature to the vitreous fluid. The sudden change of pressure in the anterior chamber upon suction induced by the paracentesis, or during a cataract surgery, causes bursting of the fragile superficial iris capillaries resulting in micro-bleeding. This is one clinical diagnostic sign of FHI.
Herpes zoster ophthalmicus (HZO), also known as ophthalmic zoster, is shingles involving the eye. Symptoms generally include a rash of the forehead with swelling of the eyelid. There may also be eye pain, eye redness, and light sensitivity. Before the rash appears tingling may occur in the forehead along with a fever. Complications may include vision loss, increased pressure within the eye, and chronic pain.
Uveitis–Glaucoma–Hyphaema (UGH) syndrome also known as Ellingson syndrome is a complication of cataract surgery, caused by intraocular lens subluxation or dislocation. The chafing of mispositioned intraocular lens over iris, ciliary body or iridocorneal angle cause elevated intraocular pressure (IOP) anterior uveitis and hyphema. It is most commonly caused by anterior chamber IOLs and sulcus IOLs but, the condition can be seen with any type of IOL, including posterior chamber lenses and cosmetic iris implants.