Fuchs Heterochromic Iridocyclitis Research Articles

Fuchs’ uveitis syndrome: a 20-year experience in 466 patients

Fuchs Uveitis Syndrome (FUS), also known as Fuchs Heterochromic Iridocyclitis, is a chronic form of uveitis characterized by mild inflammation primarily affecting one eye. This study aimed to investigate the clinical and epidemiological features of FUS in an Iranian population. A retrospective analysis was conducted on 466 patients diagnosed with FUS at an ophthalmology center affiliated with Isfahan University of Medical Sciences between 2003 and 2021. The Kimura et al. criteria were used for FUS diagnosis. Demographic data, clinical characteristics, misdiagnosed cases, concurrent diseases, and associated ocular findings were analyzed. The study included 507 eyes of 466 FUS patients, with a mean age of 34.01 ± 11.25 years. Iris atrophy, keratic precipitates, and vitritis were common clinical findings. Heterochromia was an infrequent feature. Initial misdiagnosis occurred in 13 patients, with pars planitis being the most common incorrect diagnosis. Toxoplasmosis and multiple sclerosis were common concurrent diseases. Pediatric FUS cases were noted, possibly attributed to early-onset manifestations. Differences in clinical characteristics were observed when compared to other populations. This study provides insights into the clinical and epidemiological aspects of FUS in an Iranian population. Variations in clinical features, misdiagnosis patterns, and concurrent diseases were noted. Attention to specific clinical parameters can aid in accurate FUS diagnosis. Understanding these differences contributes to a better understanding of FUS presentation and its relationship with other diseases.

Association of Fuchs Heterochromic Iridocyclitis with Multiple Sclerosis.

To draw attention to the higher proportion of Fuchs heterochromic iridocyclitis (FHI) cases in patients with multiple sclerosis (MS). Retrospective study of data collected at the Center for the Diagnosis and Treatment of Uveitis. An analysis of the medical records of 3016 patients with uveitis (in the years 2003-2020) was performed with a focus on MS. MS-associated uveitis was diagnosed in 90 patients (3%): anterior uveitis (n = 7), intermediate uveitis (n = 23), retinal vasculitis (n = 24), and panuveitis (n = 36). A clinical examination revealed signs of FHI in the anterior segment in 11 out of 90 cases (12%). Atypical manifestations of FHI included a higher incidence of bilateral involvement (45%), retinal vasculitis (27%), and vitreous snowballs (18%). The diagnosis of FHI preceded the diagnosis of MS in 4 cases. The median latency was 10.5 (range 8-15) years. In 4 patients, the diagnosis of demyelinating disease was established within one year of the diagnosis of FHI. We recommended a neurological examination for optic neuritis (n = 1), paresthesia (n = 3), relapse of motor deficit (n = 1), and screening of etiology in cases with involvement of the posterior segment (n = 3). In the other 3 cases, the diagnosis of MS preceded the diagnosis of FHI, with a median latency of 13 (range 8-19) years. We detected clinical symptoms of FHI in 12% of uveitis cases associated with MS, more often in bilateral manifestations of intraocular inflammation. Based on our experience, we recommend an investigation of the medical history of patients with FHI for manifestations of sensitive, sensory and motor deficits, especially in bilateral cases.

Multiple sclerosis-associated uveitis - A series from South India.

To report clinical profiles of multiple sclerosis (MS)-associated uveitis in seven cases from a single tertiary eye care center in South India. Retrospective case series 2013-2023. Seven cases of MS-associated uveitis were retrieved from our databases. There were five females and two males. The diagnosis of MS was made by the neurologist in all cases. Bilaterality was seen in all cases. Intermediate uveitis was the most common presentation (five cases). It was associated with peripheral retinal vasculitis (two cases) and disc pallor (two cases). Fuchs heterochromic iridocyclitis (one case) and incomplete Vogt-Koyanagi-Harada (VKH)-like presentation with a peripapillary choroidal neovascular membrane (one case) were the other presentations. In the case with incomplete VKH-like presentation, whole genome sequencing revealed a heterozygous non-synonymous variation (c.1228C>T) in exon 10 of TNFRSF1A, suggestive of susceptibility to multiple sclerosis 5 due to mutation in the TNFRS1A gene on chromosome 12p13.31. All cases received systemic steroids. Azathioprine (three cases) and rituximab (three cases) were the commonly used immunomodulatory drugs. The visual outcome was good in all cases at the last follow-up. MS-associated uveitis is underreported in India. This series highlights the clinical profile of MS-associated uveitis in India.

Fuchs heterokromik iridosiklit hastalarında kornea biyomekaniği ve kornea değerlendirilmesi

Amaç: Bu çalışmanın amacı Fuchs heterokromik iridosiklit (FHİ) tanılı gözler ile sağlıklı diğer gözlerin kornealarının ve kornea biyomekanik özelliklerinin karşılaştırılmasıdır. Gereç ve Yöntem: Fuchs heterokromik iridosiklit tanılı 14 göz (Grup 1) ve sağlıklı diğer gözler (Grup 2) çalışmaya dâhil edildi. Tüm hastalara detaylı bir oftalmolojik bakıyı takiben Ocular Response Analyzer korneal biyomekanik özellikler ve speküler mikroskobi ile santral korneal kalınlık (SKK), korneal endotelyal hücre dansitesi (KEHD) değerlendirildi. Bulgular: En iyi düzeltilmiş görme keskinliği ve intraoküler basınç istatistiksel olarak benzerdi (p değerleri sırasıyla 0,077 ve 0,557). Korneal biyomekanik parametreleri olan korneal histerezis, korneal resiztans faktör, IOPcc ve IOPg değerleri her iki grupta istatistiksel olarak benzerdi (p değerleri sırasıyla; 0,521, 0,817, 0,980 ve 0,980 idi). Ortalama santral korneal kalınlık Grup 1’de 555,57±42,95 (467-626) mikron ve Grup 2’de 556,5±37,04 (480-623) mikron olarak saptandı (p=0,959). Ortalama korneal endotel hücre dansitesi Grup 1’de 2313±420,22 (1271-2717) ve Grup 2’de 2404,42±326,75 (1566-2834) hücre/mm2 saptandı (p=0,626). Sonuç: Sağlıklı gözler ile Fuchs Heterokromik İridosiklit tanılı gözler karşılaştırıldığında kornea biyomekanik parametreleri ve korneal endotel hücre dansitesi arasında anlamlı fark saptanmamıştır. Bu sonuçlar literatürdeki birçok çalışma ile çelişmekte olup daha geniş vaka serileri ile yeni çalışmalara ihtiyaç vardır.

Optic coherence tomography angiography findings in fuchs heterochromic iridocyclitis.

To evaluate macular capillary perfusion in patients with fuchs heterochromic iridocyclitis (FHI) by using optical coherence tomography angiography (OCTA). A total of 19 eyes of 19 patients with unilateral FHI underwent detailed eye examination. OCTA (RTVue-XR Avanti) images were obtainedfrom both eyes. OCTA parameters, including foveal avascular zone, superficial capillary plexus and deep capillary plexus vessel densities, were compared betweenthe involved and fellowcontrol eyes. The median age of the patients (11 females, 8 males) was 42.0 ± 9.63 (range 24-57) years. DCP and SCP densities at the parafoveal and perifoveal area were significantly lower in the FHI eyes compared to the control eyes (44.80 ± 5.24% vs. 54.70 ± 3.76% and 43.30 ± 5.10% vs. 53.70 ± 2.73%, respectively; p < 0.05). The median FAZ was 0.29 ± 0.12 (0.11-0.42) mm2 in the FHI eyes and 0.26 ± 0.09 (0.10-0.40) mm2 in the control eyes. This difference did not reach statistical significance (p = 0.199). Macular capillary perfusion was significantly reduced in both SCP and DCP in the eyes with FHI. FHI, which is known to affect the choroid layer, could also compromise macular capillary perfusion of the retina.

A Rare Case of Hyphema in Fuchs Heterochromic Iridocyclitis After Peri-bulbar Block for Cataract Surgery

We report a case of 47 year old male patient with Fuchs heterochromic iridocyclitis and cataract in left eye who developed hyphema after peri-bulbar block for cataract surgery. The patient developed Amsler like Sign. It appeared few minutes after Peri-bulbar block following digital massage before any instrumentation of the globe. Surgery was post-poned to rule out globe perforation or any other pathology for Anterior Chamber bleed. All possible causes were ruled out and patient was eventually taken up for Cataract Surgery which was uneventful without any intra-op or post-op complications.

Cytokine Changes in the Aqueous Humor in Rubella-Related Fuchs Heterochromic Iridocyclitis.

This retrospective study is aimed at determining the correlation between cytokine levels and virus status in the aqueous humor of 38 patients with Fuchs heterochromic iridocyclitis (FHI) with/without a viral presence between May 2017 and January 2020. The levels of cytokines were analyzed in the groups with and without virus-related FHI. Among the patients, 50% had rubella virus, 5.26% had cytomegalovirus, and 2.63% had herpes simplex virus infections. The expression of interleukin-6 (IL-6) and IL-8 was significantly higher, and that of basic fibroblast growth factor (bFGF) was significantly lower in the virus-positive group than in the virus-negative group (P = 0.015, P = 0.001, and P = 0.001, respectively). Although there was no significant difference in the mean expression of vascular cell adhesion protein 1 (VCAM-1), IL-10, and vascular endothelial growth factor (VEGF), that of VCAM-1 and IL-10 was higher (M = 1338 and M = 1390, respectively; M = 6.225 and 10.600, respectively) and that of VEGF was lower (M = 134.5 and M = 38.70, respectively) in the virus-positive group than in the virus-negative group. Similar findings were observed for the expressions of IL-6, IL-8, and bFGF in the rubella-positive and rubella-negative groups. Viral presence was highly related to FHI, especially that of the rubella virus. High levels of inflammatory cytokines and low levels of neovascularization-related factors are involved in rubella-related FHI. These study findings could be helpful in the diagnosis and treatment of FHI.

Novel disease-causing variants and phenotypic features of X-linked megalocornea.

The aim of the study was to describe the phenotype and molecular genetic causes of X-linked megalocornea (MGC1). We recruited four British, one New Zealand, one Vietnamese and four Czech families. All probands and three female carriers underwent ocular examination and Sanger sequencing of the CHRDL1 gene. Two of the probands also had magnetic resonance imaging (MRI) of the brain. We identified nine pathogenic or likely pathogenic and one variant of uncertain significance in CHRDL1, of which eight are novel. Three probands had ocular findings that have not previously been associated with MGC1, namely pigmentary glaucoma, unilateral posterior corneal vesicles, unilateral keratoconus and unilateral Fuchs heterochromic iridocyclitis. The corneal diameters of the three heterozygous carriers were normal, but two had abnormally thin corneas, and one of these was also diagnosed with unilateral keratoconus. Brain MRI identified arachnoid cysts in both probands, one also had a neuroepithelial cyst, while the second had a midsagittal neurodevelopmental abnormality (cavum septum pellucidum et vergae). The study expands the spectrum of pathogenic variants and the ocular and brain abnormalities that have been identified in individuals with MGC1. Reduced corneal thickness may represent a mild phenotypic feature in some heterozygous female carriers of CHRDL1 pathogenic variants.

Resident Perspectives 56–3

Resident Perspectives 56–3

Fuchs Heterochromic Iridocyclitis: Clinic, Diagnosis, and Treatment

Fuchs heterochromic iridocyclitis (FHI) is a mild grade, chronic, non-granulomatous uveitis. Due to the asymptomatic course and variation of clinical findings, FHI is the uveitis with the highest number of late and wrong diagnosis. Decreased visual acuity due to floaters and cataracts are the most frequent complaints at presentation. It has characteristic features like unilateral involvement, diffuse, small stellate keratic precipitates, iris atrophy leading to heterochromia, abnormal angle vessels, and lack of posterior synechia or macular edema. Etiology is still uncertain however acquired rubella infection is the most approved. Visual prognosis is excellent in FHI. Cataract and glaucoma are among the major complications leading to visual loss. For the treatment, steroids might be used for the short term. Unnecessary long term use of steroids increases the risk for cataract and glaucoma

Causes of Iris Depigmentation in Young and Middle Age. Clinical Example

The spontaneous change of eyes’ color is a quite rare complain in ophthalmological clinical practice for young and middle-aged patients. We consulted the 18-year-old patient complained that a month ago she noticed a change of the iris’ color. It was symmetrical in both eyes, which had lightened and acquired a “spotty appearance”. Before the appearance of these complaints, the patient had eyes of a rich dark brown color. After a comprehensive examination, the bilateral acute iris depigmentation (BADI) was diagnosed. BADI is bilateral symmetrical iris depigmentation followed by focal or diffuse stromal atrophy and pigment dispersion in the anterior segment. It is more common for young and middle-aged women, and is often asymptomatic. BADI has bilateral symmetrical iris depigmentation followed by focal or diffuse stromal atrophy and pigment dispersion in the anterior eye segment of the eye, which may affect the trabecular network. A feature of this disease is the absence of transillumination, changes in the shape of the pupil, and an inflammatory reaction. In some cases, repigmentation may occur spontaneously during the next few years. Therefore, from a prognostic point this disease has a favorable prognosis. However, patients with BADI syndrome should undergo regular ophthalmological examinations, including measurement of intraocular pressure to exclude the development of secondary open-angle pigmented glaucoma, and use sunglasses when the sun is bright. BADI syndrome must be differentiated with the following diseases: Fuchs heterochromic iridocyclitis, viral iridocyclitis caused by HSV and CMV, pigment dispersion syndrome and pseudo exfoliative glaucoma, as well as bilateral acute iris transillumination (BAIT) syndrome to make a correct diagnosis and apply adequate treatment tactics.

Characteristics of uveitic glaucoma in Turkish patients.

To evaluate the prevalence of uveitic glaucoma (UG) in the Turkish population and investigate the primary underlying diseases. This multicenter, cross-sectional, prospective study included patients who presented to the glaucoma units of 10 tertiary ophthalmology departments in Ankara, Turkey from 15th March to 16th May 2015 and fulfilled the criteria of UG. Patients were inspected for age, sex, medical history, best corrected visual acuity, biomicroscopic findings, intraocular pressure values, and visual field results. During the study period, 4604 eyes of 2541 patients with glaucoma were screened and 145 eyes of 104 patients (4.1%) were identified as having UG. One hundred and thirty-four eyes (92.4%) had open-angle glaucoma and 11 eyes (7.6%) had closed-angle glaucoma. The mean patient age was 47 ± 16 (6-90) years. Idiopathic uveitis (54 eyes), Behçet's disease (26 eyes), Fuchs heterochromic cyclitis (21 eyes), Herpes Simplex virus infectious uveitis (14 eyes), and ankylosing spondylitis (six eyes) were the leading types of uveitis associated with glaucoma. Acute anterior uveitis was the most common type of uveitis diagnosed in 72 patients (105 eyes), whereas 21 patients (27 eyes) had panuveitis, eight patients (nine eyes) had intermediate uveitis, and three patients (four eyes) had posterior uveitis. The need for surgical intervention was 37.2% among all cases and the most common surgery was trabeculectomy in 45 eyes. UG is a vision-threatening complication commonly seen in patients with uveitis. This study demonstrates the epidemiological features and underlying etiologies of UG in the Turkish population. The most common primary causes of UG were Behçet's disease and Fuchs heterochromic cyclitis.

Analysis of retinal microvasculature in Fuchs’ uveitis syndrome. Retinal microvasculature in Fuchs’ uveitis

The objective of this study is to quantitatively analyse the foveal microvasculature in eyes with Fuchs' Uveitic Syndrome (FUS), or Fuchs' Heterochromic Iridocyclitis (FHI), by Optical coherence tomography angiography (OCTA). Thirty patients with FUS and 30 healthy volunteer patients (control group) were enrolled in the study. Vascular density (VD) in the superior and deep capillary plexuses (SCP, DCP) were reported and compared between eyes with FUS (FU), fellow eyes (FE) and the control group. Foveal VD and parafoveal VDs in all quadrants of the SCP were significantly lower in the FU group than the FE group and normal eyes (P<0.05). Foveal VDs in the DCP were similar between the three groups (P>0.05); however, parafoveal VDs in all quadrants of the DCP were significantly lower in the FU group than in the FE and control eyes (P<0.05). Foveal and parafoveal VDs in both the SCP and DCP were similar between fellow eyes and the control group. Fuchs' Uveitic Syndrome (Fuchs' Heterochromic Iridocyclitis) affects not only the anterior uvea and vitreous but also the retinal microvasculature. Analysis by OCT-A may enable us to understand the extent of this disease.

Corneal endothelium in unilateral Fuchs heterochromic iridocyclitis.

Purpose:To assess the corneal endothelium in patients with Fuchs heterochromic iridocyclitis (FHI) and compare it with the normal fellow eye.Methods:Retrospective, observational, cross-sectional study of 31 patients seen between Jan 2016 to Dec 2018, with clinical diagnosis of Fuchs heterochromic iridocyclitis, was performed. Specular microscopic examination was documented in both eyes. The affected eyes formed the study group and the fellow healthy eyes served as controls.Results:The mean age of the patients was 29.9 ± 8.2 years. The endothelial cell density (P = 0.0001) was significantly lower, whereas average cell size (P = 0.0001), coefficient of variation (P = 0.004), and maximum cell area (P = 0.01) were significantly higher in the affected eye compared to the control eye. In three patients, the affected eye showed guttae, while the healthy fellow eye revealed a normal specular mosaic.Conclusion:Specular microscopic analysis shows endothelial alterations in the affected eyes in FHI.

Clinical Features and Diagnosis of Anterior Segment Inflammation Related to Cytomegalovirus in Immunocompetent African, Asian, and Caucasian Patients

ABSTRACT Objective: To report the clinical features and treatment outcomes in immunocompetent patients with anterior segment inflammation (ASI) related to human cytomegalovirus (HCMV) depending on their ethnic origin. Material and Methods: Multicenter retrospective study of 38 patients with at least one test, either HCMV-positive PCR or GWc. Results: Features of Posner-Schlossman syndrome were observed in 50% of the eyes, Fuchs heterochromic iridocyclitis in 13% of the eyes, chronic nonspecific anterior uveitis in 21% of the eyes, and corneal endotheliitis in 18% of the eyes. PCR and GWc were positive for HCMV in 50% and 96.2% of the eyes, respectively. Glaucoma was diagnosed in 50% of eyes. Treatment was oral valganciclovir in about half of the patients. Other treatments were intravenous ganciclovir and/or ganciclovir topical ointment and/or intravitreal ganciclovir. Conclusions: No obvious association of specific clinical features with individual ethnicity could be identified. We found a high rate of glaucoma in all ethnic groups. There was a delay in diagnosis and specific treatment of HCMV in most patients.

Cornea Findings of Spectral Domain Anterior Segment Optical Coherence Tomography in Uveitic Eyes of Various Etiologies.

To evaluate the morphologic appearance of keratic precipitates (KPs) with spectral domain anterior segment optical coherence tomography (AS-OCT) for the diagnosis of uveitic eyes of various etiologies. In all, 153 eyes of 153 patients with different types of uveitis were examined to investigate the morphologic appearance of KPs. The study included 63 eyes with herpetic iridocyclitis/endotheliitis, 58 eyes with ocular sarcoidosis, 5 eyes with Fuchs heterochromic iridocyclitis, and 27 eyes with masquerade syndrome with primary intraocular lymphoma (PIOL). AS-OCT was used to analyze the different KP types among various uveitic groups. KPs were observed in 30 eyes (51.7%) from the ocular sarcoidosis group and in 15 eyes (55.6%) from the PIOL group. In the herpetic iridocyclitis/endotheliitis group, all cases showed KPs (16 cases were positive for herpes simplex virus, 22 cases for varicella zoster virus, and 27 cases for cytomegalovirus). The reflectivity and morphology of the KPs in the AS-OCT images demonstrated different patterns depending on the causative virus. Hemispheric and relatively large KPs were observed in ocular sarcoidosis cases. KPs with Fuchs heterochromic iridocyclitis appeared as small dots. By contrast, relatively high-intensity and dense KPs were observed in PIOL cases. The morphologic features of the KPs in different types of uveitis also showed other patterns such as hemispheric and rectangular-shaped KPs protruding from the retrocornea. AS-OCT images showed characteristic and specific morphological patterns. AS-OCT examination is a noninvasive and repeatable method for the diagnosis of uveitis by evaluating the morphologies of KPs and treatment effectiveness.

Predictive Factors of Intraocular Pressure Level Evolution Over Time and Glaucoma Severity in Fuchs' Heterochromic Iridocyclitis.

To investigate the clinical and virologic-associated and predictive factors of intraocular pressure (IOP) evolution over time and its severity in Fuchs' heterochromic iridocyclitis (FHC). Consecutive patients with both clinical FHC and intraocular synthesis of rubella virus (RV)-specific antibodies were included in this study. Specific ocular production of RV antibodies was confirmed using the quotient of serum/aqueous humor ratio of RV IgGs (Crv) and control antiviral IgGs (Cctl), using quantitative serology methods. Epidemiologic, clinical, biological, and virologic data at referral were collected and correlated with IOP values over time, occurrence, and severity of glaucoma. Sixty-eight eyes of 68 patients were included. Mean age at diagnosis was 40.7 ± 11.1 years. Mean follow-up was 4.3 ± 4.3 years. Mean baseline Crv and Cctl values were 12.34 ± 14.67 and 216.70 ± 98.4, respectively. Mean baseline IOP was 17.2 ± 7.2 mm Hg (range, 9-40) and 15.6 ± 5.6 (range, 3-30) 5 years after referral. The predictive factors for pejorative IOP evolution over time and glaucoma severity were male sex (P = 0.03) and decreased Crv (P = 0.04) and presence of iris nodules (P < 0.001) and decreased Cctl (P = 0.02), respectively. Diagnostic delay was associated with increased likelihood of undergoing glaucoma surgery (P = 0.02). Time to diagnosis, male sex, presence of iris nodules at baseline, and decreased Crv and Cctl ratios were associated with increased likelihood of pejorative IOP evolution over time. Given the aggressiveness of glaucoma in FHC, these results provide interesting insight into what category of patients should need the closest screening.

Phacoemulsification in Patients with Fuchs Heterochromic Iridocyclitis

Phacoemulsification in Patients with Fuchs Heterochromic Iridocyclitis

Moth-eaten appearance of the iris in Fuchs heterochromic iridocyclitis

Moth-eaten appearance of the iris in Fuchs heterochromic iridocyclitis

Fuchs heterochromic iridocyclitis-associated glaucoma: a retrospective comparison of primary Ahmed glaucoma valve implantation and trabeculectomy with mitomycin C

Background: The aim of this study was to compare the safety and efficacy of primary trabeculectomy with mitomycin C and Ahmed glaucoma valve (AGV) implantation in patients with Fuchs heterochromic iridocyclitis (FHIC)-related glaucoma, a rare complication of an uncommon form of uveitis. Methods : In this retrospective comparative case series, 26 FHIC-associated glaucoma patients received trabeculectomy (n=12) or an AGV (n=14). Primary outcome measures were surgical success, defined as intraocular pressure (IOP) ≤21 mmHg, decreasing ≥20% from baseline, and no secondary glaucoma surgery. Secondary outcome measures were the number of glaucoma medications, complications, best corrected visual acuity (BCVA), and IOP. Results: The follow-up was 34.0±17.7 months in patients that received trabeculectomy and 33.4±18.6 months in AGV (P= 0.837). The cumulative probability of success rate was 41.7% for trabeculectomy and 85.7% for AGV, with no significant difference in complications (P>0.05). The IOP in patients that received trabeculectomy dropped from 23.4±3.3 mmHg to 21.6±5.2 mmHg at the final visit (P= 0.041). In patients that received AGV, the IOP decreased from 24±7.8 to 17.1±2.6 mmHg (P= 0.003). The number of glaucoma medications at baseline were 3.3±0.5 in those that received trabeculectomy and 3±0.6 in those that received AGV (P=0.233), and decreased to 2.4±1.0 (P=0.008) and 1.7±0.6 (P=0.002), respectively. BCVA was equal in both groups and did not change (P>0.05). Conclusion: Primary AGV had a higher success rate than trabeculectomy, with patients also needing fewer medications for the management of FHIC-associated glaucoma.