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.

Astigmatismo y aberraciones corneales en ojos con Iridociclitis Heterocrómica de Fuchs / Corneal astigmatism and aberrations in patients with Fuchs heterochromic iridocyclitis

Introducción: La Iridociclitis Heterocrómica de Fuchs (IHF) es la causa del 2 a 7% de las uveítis anteriores, el astigmatismo corneal como condición de la IHF y como posible causa de la disminución de la visión, ha sido una entidad muy poco estudiada hasta el momento, tiene una gran importancia debido a que por la anisometropía que podría generar, puede llevar a alteraciones de la agudeza visual. Objetivo: Evaluar la existencia de astigmatismo corneal y/o aberraciones corneales inherentes a los ojos con Iridociclitis Heterocrómica de Fuchs. Diseño del estudio: Estudio transversal, analítico, de fuentes primarias. Método: Se realizó muestreo por conveniencia, en el cual se incluyeron 44 ojos de 22 pacientes con diagnóstico de IHF unilateral o bilateral. Para cada caso, se realizó tomografía corneal (Pentacam® AXL - OCULUS) y aberrometria (OPD-Scan III® - NIDEK) y se compararon ojos enfermos con los ojos sanos. Resultados: Se encontró una mediana de 1.35 (RIC: 0.50 ­ 1.70) para el astigmatismo de la cara anterior de los ojos enfermos .vs. 0.8 (RIC: 0.7 ­ 1.1) para el astigmatismo de la cara anterior de los ojos sanos (p= 0.135). Se identificó un astigmatismo en cara anterior >1.00 D en el 61.5% de los ojos enfermos .vs. 27.8% de los ojos sanos (p= 0.027) con un RR de 2.15. No se encontró una diferencia estadísticamente significativa entre los ojos enfermos y los ojos sanos para las aberraciones analizadas. Conclusión: El presente estudio no demuestra la existencia de astigmatismo o aberraciones inherentes a la IHF, pero evidencia una tendencia de mayor astigmatismo en los ojos con IHF, que posiblemente podría lograr significancia estadística con una muestra mayor.

Background: Fuchs heterochromic iridocyclitis (FHI) represents 2 to 7% of cases of anterior uveitis. To date, few studies have addressed corneal astigmatism as an inherent condition and as possible cause of decreased visual acuity in FHI patients. This may be relevant since the generated anisometropia may lead to alterations in visual acuity. Objective: To evaluate the presence of corneal astigmatism and/or corneal aberrations in patients with FHI. Study design: Analytic cross sectional study. Method: A convenient sampling was performed. The sample included 44 eyes of 22 patients with unilateral or bilateral FHI. All participants were studied by corneal tomography (Pentacam® AXL ­ OCULUS) and aberrometry (OPDScan III® - NIDEK); measurements of diseased and healthy eyes were compared. Results: Twenty-two patients were enrolled in this study. The mean age at diagnosis was 46.9 +/- 12 years, and 40.9% were men. FHI eyes exhibited a median astigmatism of the anterior surface of 1.35D (IQR: 0.50 ­ 1.70), compared to 0.8D (IQR: 0.7 ­ 1.1) observed in healthy eyes (p= 0.135). Anterior surface astigmatism >1.00 diopters (D) was found in 61.5% of diseased eyes compared to 27.8% of healthy eyes, with a risk ratio of 2.15. Regarding corneal aberrations, no statistically significant differences were identified between diseased and healthy eyes for the analyzed aberrations. Conclusion: While our current data does not show statistically signifi cant differences between groups that support that corneal astigmatism or aberrations are inherent to FHI-aff ected eyes, our study does show a trend that suggests increased presence of clinically relevant astigmatism in eyes with FHI. Statistical significance may be achieved with a larger sample size.

Outcomes of phacoemulsification in patients with Fuchs' heterochromic iridocyclitis / 国际眼科杂志(Guoji Yanke Zazhi)

@#AIM: To evaluate the clinical outcomes of phacoemulsification in patients with Fuchs' heterochromic iridocyclitis(FHIC). <p>METHODS: In this noncomparative case series,18 eyes of 18 patients clinically diagnosed with FHIC and cataract were studied. All of them received phacoemulsification combined with intraocular lens implantation.Preoperative and postoperative visual acuity, intraocular pressure, corneal endothelial cells density were measured. Intraoperative and postoperative complications were evaluated. <p>RESULTS: The mean follow-up period was 9.8±3.3mo. The only intraoperative complication was peripheral iris bleeding(17%). Compared with preoperative, the best corrected visual acuity improved 6mo postoperatively(0.98±0.85 <i>vs</i> 0.08±0.14, <i>P</i><0.001). There was no significant difference in intraocular pressure between preoperative and postoperative(15.11±2.63mmHg <i>vs</i> 14.94±2.49mmHg, <i>P</i>>0.05).The mean endothelial cells counting loss rate postoperatively was 10%±6%. Fibrin formation in the anterior chamber occurred in 2 eyes postoperatively. During follow-up, 1 eye developed anterior capsule contraction, and 4 eyes developed posterior capsule opacification. <p>CONCLUSION: Phacoemulsification is a safe procedure with good visual outcomes in patients with FHIC and cataract.

Revisión con aguja tras implante de válvula de Ahmed en la ciclitis heterocrómica de Fuchs / Needle revision after Ahmed valve implantation in the management of Fuch´s heterochromic cyclitis

La ciclitis heterocrómica de Fuchs es una uveítis crónica que puede ser asintomática por años o expresar solo la heterocromía antes que aparezca cualquier otro signo. El glaucoma se considera una de las complicaciones más difíciles de tratar, y requiere cirugía en múltiples ocasiones. Los dispositivos de drenaje están siendo cada vez más utilizados como alternativa de tratamiento quirúrgico en estos casos. Asiste a la consulta médica una paciente de 36 años de edad, con antecedentes de uveítis crónica unilateral del ojo izquierdo asociado a catarata y glaucoma descompensado, a pesar del tratamiento médico. Se presenta con 50 VAR de visión y presión intraocular de 32 mmHg. Se realizó cirugía combinada: facoemulsificación e implante de válvula Ahmed modelo S2 con mitomicina C (0,2 mg/mL) durante cinco minutos. Se diagnostica ampolla de filtración encapsulada en la octava semana. Se realiza revisión con aguja y subconjuntival de 1 mg de bevacizumab (avastin) subtenoniano en área de la filtrante. La inyección se repite días alternos hasta completar tres dosis según protocolo institucional. Se logran cifras de presión intraocular de 17 mmHg y agudeza visual mejor corregida de 95 VAR a los 18 meses posoperatorios(AU)

Fuch´s heterochromic cyclitis is chronic uveitis that may be asymptomatic for years or may express heretochromia just before the onset of any other sign. Glaucoma is considered one of the most difficult complications to be treated and requires multiple surgeries. Drainage devices are increasingly used as a surgical treatment option in these cases. Here is the case of a 36 years-old woman with a history of unilateral chronic uveitis in her left eye associated to cataract and decompensate glaucoma despite the medical therapy. She presented with 50 VAR visual acuity and 32 mmHg intraocular pressure. She underwent combined surgery based on phacoemulsification and S2 model Ahmed valve implantation with mitomycin C (0,2 mg/mL) for 5 minutes. The diagnosis was encapsulated filtering bleb at the 8th week of operation. Subconjuctival needle revision with one mg of subtenial bevacizumab (avastin) in the bleb area was performed. Injection was administered every other day till completion of three doses according to the institutional protocol. Eighteen months after the surgery, the intraocular pressure was 17nnHg and the best corrected visual acuity reached 95 VAR best corrected visual acuity(AU)

Corneal Endothelial Cell Loss after Tube Shunt Surgery in Fuch's Heterochromic Iridocyclitis

PURPOSE: To report a case of corneal failure after implantation of the Ahmed glaucoma valve occurring in a patient diagnosed with Fuchs' heterochromic iridocyclitis. CASE SUMMARY: A 53-year-old male who complained of ocular pain and suddenly decreased visual acuity in his right eye visited our clinic. His visual acuity was 0.15 and intraocular pressure (IOP) was 55 mm Hg. The slit-lamp examination revealed edematous cornea, fine round or stellate keratic precipitates connected with fine filaments on the endothelium and depigmentation of the iris. The corneal endothelial cell density was 2,958 cells/mm2. There was no specific finding in his left eye. The IOP did not improve with medical treatment, therefore, an Ahmed glaucoma valve was implanted in his right eye. At every follow-up exam the tube was well positioned and the IOP was maintained between 8 and 13 mm Hg. Eight months postoperatively, the patient complained of decreased visual acuity and the cell density was decreased to 1,408 cells/mm2. Posterior subcapsular cataract opacity was observed as well as progression of depigmentation and distortion of the iris. Seventeen months after the surgery, the cell density was 700 cells/mm2. On follow-up examination, his visual acuity was decreased to FC10 cm with the cataract progressing, therefore cataract surgery was performed. One month postoperatively, his vision improved to 0.1. However, the visual acuity deteriorated due to progression of the corneal edema and penetrating keratoplasty was performed. CONCLUSIONS: Aggravation of the corneal complication after Ahmed glaucoma valve implantation should be considered in patients with Fuchs' heterochromic iridocyclitis-induced glaucoma.

Iridociclitis heterocrómica de Fuchs: una uveítis subdiagnosticada / Fuchs heterochromic iridocyclitis: an underdiagnosed uveitis

Objetivo: caracterizar clínicamente 20 de casos de Iridociclitis Heterocrómica de Fuchs subdiagnosticados por su cuadro clínico. Metodología: estudio observacional, descriptivo y retrospectivo de reporte de 20 casos con evaluación clínica y registro fotográfico. Resultados: fueron evaluados 20 casos referidos a la consulta de uveítis por oftalmología general, de los cuales 13 fueron mujeres (65%) con una edad promedio al momento de la evaluación de 52 años (R= 33-80 años). Diecisiete casos tuvieron compromiso unilateral (85%) y 3 bilateral (15%) para un total de 23 ojos comprometidos. La uveítis fue clasificada como anterior en 20 ojos (86,95%), crónica en 20 ojos (86,95%) y no granulomatosa en el 100% de los casos. El tiempo promedio de evolución de la enfermedad fue de 82 meses (R= 4 - 240 meses), y el tiempo promedio de diagnóstico por especialista en uveítis fue de 1,75 meses (R=0-18 meses). El 100% de los pacientes consultaron por cambios en la agudeza visual, sin manifestación de ojo rojo o dolor. La agudeza visual inicial promedio fue de 20/40 (rango 20/1000-20/20), con una reacción inflamatoria de bajo grado con promedios por debajo de 1+ en todos los signos inflamatorios. La evidencia de precipitados queráticos estrellados difusos típicos se presentó en el 100% de los casos y en el iris se encontró heterocromía en un 50% (10 casos), atrofia del iris en 80% (18 casos) y nódulos (Koeppe y Busacca) en 8 ojos (34,78%). Como complicaciones asociadas se encontró catarata en 10 ojos (43.47%) y glaucoma en 8 ojos (34.78%). Conclusión: las características clínicas de la Iridociclitis Heterocrómica de Fuchs, especialmente lo discreto de sus síntomas y lo sutil de sus cambios biomicroscópicos, específicamente la heterocromía del iris que se sugiere como un marcador clínico importante de esta condición, han llevado a un subdiagnóstico o a un diagnóstico retardado de esta patología, siendo identificada en muchas ocasiones como cuadros de uveítis anterior sin etiología esclarecida y generando tratamientos crónicos con corticoesteroides que a la larga se convierten en factor de riesgo para complicaciones tales como catarata de presentación temprana y glaucoma.

Objetive: clinical characterization of 20 underdiagnosed cases of Fuchs Heterochromic Irido cyclitis. Methods: observational, descriptive and retrospective study report of 20 cases with clinical evaluation and photographic record. Results: a total of 20 cases referred to uveal specialist from general ophthalmology were evaluated. It was found that the group of women with 13 cases (65%) predominated with respect to men 7 cases (35%), the average age at evaluation was 52 years (range 33-80). Twenty three eyes were diagnosed, 17 cases were unilateral (85%) and 3 cases were bilateral (15%). 100% of patients complaint for changes in AV, without red eye or pain. Uveitis was classifi ed as anterior in 20 eyes (86,95%), chronic in 20 eyes (86,95%) and non-granulomatous in 23 eyes (100%). The average time evolution was found in 82 months (R 4-240 months), and the time of diagnosis by uveitis specialist was 1,75 months (0-18 months). Initial AV average found was 0.48 (0,02-1), with a low mean anterior chamber reaction cellularity of 0,80+ cells; fl are 0,69+, average cellularity at anterior vitreous of 0,26+ cells and vitreous opacity was 0,45+. Typical diffuse stellate keratic precipitates were present in 100% cases and heterochromia was found in 10 of cases (50%), iris atrophy in 18 cases (80%) and nodules (Koeppe and Busacca) in 8 eyes (34.78%). Th e average intraocular pressure was found in 15.74 mmHg. Cataract associated complications found in 10 eyes (43.47%) and glaucoma in 8 eyes (34.78%). Conclusión: clinical features in Fuchs Heterochromic Iridocyclitis, has discrete symptoms and subtle biomicroscopic changes; heterochromia is an important clinical marker of this condition, lack of identifi cation of this signs may delay the timely and proper diagnosis. This entity has been confused as anterior uveitis without known etiology and for this reason many ophthalmologists treat these cases with corticosteroids that eventually become a risk factor for complications such as early cataract and glaucoma.

Implantation of iris-claw Artisan intraocular lens for aphakia in Fuchs’ heterochromic iridocyclitis.

Implantation of iris-claw Artisan intraocular lens (IOL) is a surgical option for correction of aphakia; however, these IOLs have not been used in eyes with uveitis including Fuchs’ heterochromic iridocyclitis (FHI) due to possible risk of severe postoperative intraocular inflammation. In the case reported here, we secondarily implanted an Artisan IOL in a 28-year-old man with FHI who had aphakia with no capsular support due to a previous complicated cataract surgery. Enclavation was easily performed and no intraoperative complication was noted. Postoperative course was uneventful with no significant anterior chamber inflammation during 12 months of follow-up. Although there were few deposits on the IOL surface, the patient achieved a best-corrected visual acuity of 20/20 without developing glaucoma or other complications. Therefore, Artisan IOL may be considered for correction of aphakia in patients with FHI. However, studies on large number of patients are required to evaluate safety of the procedure.

A Case of Waardenburg Syndrome Type 4

PURPOSE: To report the first case of Waardenburg syndrome type 4 in Korea. CASE SUMMARY: A 3-year-old boy visited our clinic to have his abnormal right eye iris color checked. The patient had a previous operation Hirschsprung's disease. In addition, his older sister and aunt showed similar ocular findings. A general physical examination, hearing test, and fundus examination were performed. On examination, hypochromic heterochromic iridum, albinism of the posterior pole upon ipsilateral fundus, and dystopia canthorum were found. There was no abnormal finding in the hearing test. CONCLUSIONS: The patient showed hypochromic heterochromic iridum, dystopia canthorum, and albinism of ipsilateral fundus. He also had a family history of Waardenburg syndrome and had surgery associated with congenital megacolon. The patient was diagnosed with Waardenburg syndrome type 4.

Glaucoma secundário à iridociclite heterocrômica de Fuchs / Secondary glaucoma to fuchs' heterochromic iridocyclits

A iridociclite heterocrômica de Fuchs ou Síndrome de Fuchs é um tipo de uveíte relativamente incomum. Afeta igualmente ambos os sexos, na faixa etária dos 20-45 anos, tendo no quadro clássico uma inflamação não granulomatosa crônica unilateral na úvea anterior, de início insidioso, baixo grau de atividade, e não sendo responsiva aos corticóides. Normalmente tem um bom prognóstico, exceto quando ocorre o desenvolvimento de catarata e glaucoma, patologias que podem estar associadas à síndrome. Nesse caso, temos um paciente masculino, de 68 anos, que teve como primeira manifestação da síndrome o glaucoma.

Fuchs' heterochromic iridocyclits or Fuchs' Syndrome is a kind of uveitides relatively uncommon. Affect equally both genders, between 20-45 years old, in the classic features include a chronic unilateral nongranulomatous inflammation in anterior uvea, insidious onset, low-grade in activity, and unresponsive to corticosteroid therapy. Usually has a good prognosis, except for the development of cataract and glaucoma, pathologies associated with syndrome. In this case, there are a male pacient, 68 years, with glaucoma as the first manifestation of the Fuchs'Syndrome.

Chikungunya virus iridocyclitis in Fuchs’ heterochromic iridocyclitis.

We are reporting a case of bilateral Fuchs’ heterochromic iridocyclitis with chikungunya virus infection in the left eye. A 20-year-old female was presented with a past history of fever suggestive of chikungunya with bilateral Fuchs’ heterochromic iridocyclitis and complicated cataract. She had a tripod dendritic pattern of keratic precipitates by confocal microscopy in the left eye with a stippled pattern of keratic precipitates in both eyes. The real-time polymerase chain reaction (RT-PCR) assay in the aqueous humor detected 98 copies/ml of chikungunya virus RNA. The patient underwent clear corneal phacoemulsification with in-the-bag intraocular lens implantation in the left eye with a good visual outcome. This is the first report where the presence of chikungunya virus RNA has been associated with a case of bilateral Fuchs’ heterochromic iridocyclitis.

A Case of Waardenburg Syndrome Type 2 With Anisocoria

PURPOSE: To report a case of Type 2 Waardenburg syndrome with anisocoria. CASE SUMMARY: A 53-year-old woman with congenital deafness visited the clinic complaining of photophobia and heterochromic iris in the left eye. Her best-corrected visual acuity was 1.0 in the right eye and 0.7 in the left eye. The patient's left eye showed complete iris hypopigmentation, and the right eye showed segmental iris hypopigmentation. Fundus examination demonstrated generalized decrease in retinal pigment with focal hypopigmented lesions in both eyes. The pupil sizes were 3 mm in the right eye and 5 mm in the left eye. A pure tone audiogram showed profound bilateral sensorineural hearing loss. CONCLUSIONS: In the present case, mydriasis was found to exist in a completely hypopigmented iris. Therefore, the neural crest from which the pupillary constrictor muscle originates may play a role in the pathogenesis of Waardenburg syndrome.

Type 3 Waardenburg Syndrome

PURPOSE: We report a case of Type 3 Waardenburg syndrome accompanied by intermittent exotropia, which had not previously been encountered in Korea along with the literature study. METHODS: A 5-year-old boy visited as a result of hypochromic heterochromic iridum in the right eye. Suspecting Type 3 Waardenburg syndrome, plain radiography, a hearing test, an alternate prism cover test and fundus examinations were performed and the presence of lateral displacement of the lacrimal puntum was evaluated. RESULTS: The presence of hypochromic heterochromic iridum, dysplasia of the left thumb, sensorineural deafness, dystopia canthorum along with a lateral displacement of the lacrimal puntum, albinism of the posterior pole upon a fundus examination, and intermittent exotropia with a 20 prism diopters of deviation were found. CONCLUSIONS: This patient showed the 4 criteria for a diagnosis of Type 3 Waardenburg syndrome including hypochromic heterochromic iridum, dystopia canthorum along with lateral displacement of the lacrimal puntum, sensorineural deafness, and a muscular system abnormality, i.e., dysplasia of the left thumb. He also showed intermittent exotropia.

A Case of Waardenburg`s Syndrome

Waardenburg`s syndrome is a rare hereditary disease, which is characterized by dystopia canthorum, hypochromic heterochromic iridum, sensorineural deafness,high and broad nasal bridge, white forelock and premature graying. We present a case of 27 year old woman with Waardenburg`s syndrome. She has characteristic features such as dystopia canthorum, broad and high nasal bridge, confluent eyebrow(synophrys), hypochromic heterochromic iridum, depigmented fundus and premature graying.