Fuchs heterochromic uveitis fhu in its classic presentation is a unilateral, chronic, low grade, often asymptomatic anterior uveitis. Fhi often affects young adults and most often involves a single eye. Fuchs heterochromic iridocyclitis fhi is a frequently overlooked cause of anterior uveitis. Research article fuchs heterochromic iridocyclitis in an. Improper diagnosis may lead to unnecessary therapy. Fuchs heterochromic iridocyclitis a condition characterized by a low grade, asymptomatic uveitis in which the iris in the affected eye becomes hypochromic and has a washedout, somewhat moth eaten appearance. Patients are generally asymptomatic until they develop cataract or vitreous opacities. It is believed that the heterochromic iridocyclitis of fuchs is a relatively rare form of anterior uveitis, ranging from 1. Fhi is a frequently overlooked cause of anterior uveitis. Corneal astigmatism in unilateral fuchs heterochromic iridocyclitis.
Fuchs heterochromic iridocyclitis is a chronic but relatively mild form of anterior uveitis associated with cataract and glaucoma. Uveitis is swelling and irritation of the middle layer of the eye. A retrospective analysis was performed on 158 patients with fhi. The heterochromia can be very subtle, especially in patients with lighter colored irides. The majority of cases are unilateral and present with characteristic lowgrade, asymptomatic iridocyclitis, iris heterochromia, and diffuse stellate keratic percipitates. This article describes specific etiopathologies and general strategies for managing these conditions. Fuchs heterochromic iridocyclitis is an ocular condition characterized by the presence of keratic precipitates, iris heterochromia and iris atrophy. To investigate an epidemiologic association between fuchs heterochromic iridocyclitis fhi and the rubella vaccination program in the united states. Symptoms most often floaters as well as blurred vision and visual deterioration is pushing patients to consult. Ernst fuchs was the rst in to report both the clinical and pathologic features of a consistent number of patients with a chronic low grade anterior chamber in ammation, heterochromia, and cataract. Fuchs heterochromic iridocyclitis unilateral uveitis, heterochromia, iris stromal atrophy, small white stellate kps scattered diffusely over endothelium synechiae almost never forms but glaucoma and cataracts often occur associations with toxoplamosis, hsv, and cmv infections 33. While the incidence of all forms of uveitis is approximately 0.
To study epidemiology, clinical findings and visual prognosis of patients with fuchs heterochromic iridocyclitis fhi. Dark brown irides may demonstrate heterochromia poorly. Rubeosis in fuchs heterochromic iridocyclitis jama. Online survey fuchs heterochromic iridocyclitis and. Iridocyclitis uveitis types, causes, symptoms, treatment. Common immunogenic and inflammatory pathways have been proposed to explain the higher incidence of this uveitic phenomenon in patients with retinal dystrophies without definitive answers. The etiology is unknown, but herpes simplex, ocular toxoplasmosis and rubella infection have been implicated in the pathogenesis of the disease. This form of uveitis is misdiagnosed more than any other in the entire field of uveitis. The condition was first coined by ernst fuchs in the early twentieth century who investigated the pathological. Associations wit h ocular toxoplasmosis, herpes simplex virus, and cmv infection have been suggested. Fuchs heterochromic iridocyclitis, or fuchs uveitis syndrome, is an entity that is frequently overlooked. Fuchs heterochromic iridocyclitisassociated glaucoma. It is characterized by lowgrade intraocular inflammation, small stellate keratic precipitates, and iris. A syndrome characterized by corneal degeneration, heterochromia of the iris, iridocyclitis, keratic precipitates, and cataract.
This condition is usually unilateral, and its symptoms vary from none to mild blurring and discomfort. The epidemiologic association of fuchs heterochromic iridocyclitis and ocular toxoplasmosis. Fuchs heterochromic iridocyclitis and posnerschlossman. Abnormal vessels may bridge the angle on gonioscopy. Although patients may be aware of their iris heterochromia, they are often asymptomatic for many years prior to presentation and, unlike other uveitides, typically will have no pain, redness, or photophobia. First described by fuchs in 1906, fuchs heterochromic iridocyclitis fhi is a chronic, unilateral iridocyclitis characterized by iris heterochromia. Fuchs heterochromic iridocyclitis fhi is a chronic, low grade, usually unilateral, ocular inflammatory disease with a good visual prognosis. Fuchs heterochromic uveitis fhu1 accounts for perhaps 5% of all cases of uveitis,2 and secondary cataract is an expected complication. It is characterized by a classic triad of iris heterochromia, cataract and keratic precipitates. Fuchs heterochromic iridocyclitis fuchs heterochromic. It can be discovered while diagnosing heterochromia or other similar ailments. Abstract fuchs heterochromic iridocyclitis is an unusual form of uveitis of unkown aetiology that continues to be poorly understood and underdiagnosed. Very often however the unilateral inflammation is a chance discovery during a routine ophthalmological examination. The prognosis is good in most cases even though the inflammation persists for decades.
Fuchs heterochromic cyclitis fuchs syndrome fuks sindrom a syndrome characterized by corneal degeneration, heterochromia of the. Fuchs uveitis and posnerschlossman syndrome 323 with a diffuse distribution on the endothelial surface figure 3. This often overlooked uveitis syndrome is characterised by chronic low grade anterior chamber and anterior vitreous inflammation, diffusely distributed stellate keratic precipitates kps, full thickness iris atrophy, posterior. Fuchs heterochromic iridocyclitis revisited fearnley. Get a printable copy pdf file of the complete article 1. It occurs more commonly in the third and fourth decades of life with an equal gender distribution.
Aimsbackgroundthe aetiology of fuchs heterochromic uveitis fhu is unknown although it can occur in combination with a number of different ocular conditions. Between 2% and 3% of patients referred to uveitis clinics have fuchs heterochromic iridocyclitis. Usually, no symptoms are displayed by patients with this condition. Fuchs heterochromic iridocyclitis fhic is a rare form of uveitis. Ophthalmology, cataract fuchs heterochromic iridocyclitis. Between 2% and 3% of patients referred to uveitis clinics. Fuchs heterochromic iridocyclitis fhi, an unusual form of uveitis of unknown etiology, is frequently misdiagnosed. Several authors described an association of fhc with ocular toxoplasmosis. A patient had fuchs heterochromic iridocyclitis of approximately 17 years duration. Fuchs heterochromic iridocyclitis fhi is an unusual form of chronic longlasting uveitis. Fuchs heterochromic iridocyclitis and secondary glaucoma. 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 etiology of fuchs heterochromic iridocyclitis remains unclear. Fuchs heterochromic cyclitis fhc is a chronic anterior segment inflammatory syndrome that accounts for 2 to 3% of all uveitis cases.
Fuchs heterochromic iridocyclitis in blacks jama network. Men and women are equally affected, and the disease tends to come to medical attention between the ages of 20 to 40. Fuchs heterochromic cyclitis and ocular toxocariasis. Patients are often asymptomatic and the disease is often discovered through investigation of the cause of the heterochromia or cataract. The academy uses cookies to analyze performance and provide relevant personalized content to users of our website. Department of ophthalmology, first faculty of medicine, charles university and general university hospital in prague, czech r epu blic. Fuchs heterochromic iridocyclitissyndrome, disease, or both. Fuchs heterochromic iridocyclitis fhic was first clearly defined by ernest fuchs in 1906.
Nowadays fhi is one of the most common forms of anterior uveitis. We present the clinical features observed in 77 patients 89 eyes seen in the uveitis clinic at leicester royal infirmary over a 2. The term also includes inflammation that involves the other tunics the sclera and cornea, and the retina. Five patients with fhu who show an association with sarcoidosis were studied. Infrequent variants of rp such as inverse rp have not. Fuchs heterochromic iridocyclitis fhi is a chroniclow grade anterior uveitis characterized by iris heterochromia. Pdf the clinical description of fuchs heterochromic uveitis was made in 1906. To report the analysis of 26 patients 27 eyes with fhi who were diagnosed and observed over a period of 17 years mean. Histologically, keratic precipitates, iris atrophy, rubeosis iridis, discontinuous rubeosis of the anterior chamber angle, a chronic nongranulomatous iridocyclitis, and trabeculitis were noted. Fhi often affects young adults and most often involves a. The unusual association of inverse retinitis pigmentosa. Uveitis is an ophthalmic emergency and requires a thorough examination by an ophthalmologist or optometrist. We believe, for example, that most patients with jiaassociated iridocyclitis and patients with pars planitis or uveitis that has been characterized by great vitritis or multiple recurrences affecting the posterior segment very much benefit from primary. It is also known as fuchs uveitis syndrome, fuchs heterochromic uveitis or simply fhi.
Fuchs heterochromic iridocyclitis in an italian tertiary. To compare the safety and efficacy of a primary trabeculectomy with mitomycin c t and an ahmed glaucoma valve agv implantation in patients with fuchs heterochromic iridocyclitis fhic related glaucoma, a rare complication of an uncommon form of uveitis. Fuchs heterochromic iridocyclitis is a chronic form of iritis of unknown cause. Fuchs syndrome fuks, mim6800 a syndrome characterized by corneal degeneration, heterochromia of the iris, iridocyclitis, keratic precipitates, and cataract. Clinical characteristics of fuchs uveitis syndrome ncbi. Classic retinitis pigmentosa rp and other syndromic variants have previously been associated to fuchs heterochromic iridocyclitis fhi. Fuchs speculated that an unknown process leads to the development of abnormal uveal pigment with chronic lowgrade inflammation, eventually causing iris atrophy and secondary glaucoma. It is a unilateral iridocyclitis of chronic nature and is most often accompanied by iris heterochromia. Amslerverrey sign during cataract surgery in fuchs. Fuchs heterochromic iridocyclitis fhi is one of the most common types of anterior uveitis and is thought to account for approximately 8% of all cases of endogenous uveitis. Fuchs heterochromic iridocyclitis presentation initially described in 1906 by austrian ophthalmolo. We believe that fhi may be overlooked in blacks because of a. A study of glaucoma in a cohort of 103 patients with fuchs heterochromic uveitis fhu is reported.
The uvea consists of the middle layer of pigmented vascular structures of the eye and includes the iris, ciliary body, and choroid. Diagnosis of fhi is considered in people with floaters, vision loss, and heterochromia. Iris heterochromia in a patient with fuchs heterochromic iridocyclitis. Fuchs heterochromic iridocyclitis is a rare but significant cause of visual impairment.
Neovascularization of the iris and the anterior chamber ac angle radial and circumferential occurs in 622% of cases. This is a chronic type of iridocyclitis, which is mostly unilateral and is bilateral in rare cases. This condition is usually unilateral, and its symptoms vary from. Fuchs heterochromic iridocyclitis and the rubella virus. Fuchs heterochromic iridocyclitis genetic and rare. Fuchs heterochromic cyclitis medigoo health medical. Fuchs heterochromic iridocyclitis fhi is type of lowgrade chronic uveitis that makes up between 2 and 7 % of all cases of uveitis wakefield and chang in int ophthalmol clin 452. This is the reason why it is diagnosed through other investigations. Note the lighter colored iris in the affected right eye. The varying clinical appearance within a group of 103 patients with fuchs heterochromic uveitis fhu is discussed.
176 14 603 246 898 185 981 1171 892 941 693 636 380 1279 1153 401 1498 64 1254 1383 389 517 1406 962 270 329 1251 782 1268 1109 1530 1200 1444 1022 948 37 1481 1091 1105 722 1145 612 539 172 250 17 339 105 265