[Congenital Simple Hamartoma of the Retinal Pigment Epithelium]. / Kongenitales einfaches Hamartom des retinalen Pigmentepithels.

BACKGROUND: Congenital simple hamartoma of the retinal pigment epithelium (CSHRPE) is an uncommon benign lesion with characteristic clinical features. Ophthalmoscopically it appears as a small localized, well circumscribed, pigmented tumor in the foveal region. In contrast to the more common flat congenital hypertrophy of the RPE the CSHRPE has an elevated nodular appearance. PATIENTS AND METHODS: Retrospective case series of three patients with CSHRPE. Clinical morphological features using different imaging techniques are presented. RESULTS: A typical dark lesion was incidentally noted in the macula of two patients. Optical coherence tomography (OCT) demonstrated a nodular preretinal hyperreflectivity with shadowing of deeper structures. In one patient the CSHRPE was hypofluorescent throughout the angiogram. The third patient presented with a reduced visual acuity of 0.3. A characteristic lesion was found at the foveal center. OCT revealed a hyperreflective preretinal lesion with associated moderate disruption of the foveal architecture. Amblyopia treatment slightly improved visual acuity in this case. The lesions remained stationary in two patients (follow-up 8 - 14 months). CONCLUSIONS: CSHRPE are usually detected as an incidental finding. Given its benign character and typically asymptomatic presentation an observational treatment approach is generally recommended. The lesions generally remain stationary and are not known to grow. In cases with visual impairment due to foveal involvement amblyopia treatment should be initiated.

[A case of familial pupillary iris pigment epithelium cysts]. / Przypadek rodzinnego wystepowania zrenicznych cyst nablonka barwnikowego teczówki.

Iris cysts are rare lesions. In some cases, they may mimic melanoma and cause diagnostic difficulties. Through imaging tests such as ultrasound biomicroscopy we can evaluate the structure changes and determine the location for easy diagnosis. Iris cysts often require only observation. In some cases specialist treatment is necessary. One of the methods is transpupillary cystotomy perform using the Nd:YAG laser. The paper presents the case of familial pupillary cysts of the iris pigment epithelium in a father and a daughter.

Treatment of peripheral pigment epithelium iris cyst with SLT laser.

PURPOSE: To report a case of a peripheral pigment epithelium iris cyst treated with selective laser trabeculoplasty laser. CASE REPORT: A 15-year-old girl applied to our clinic with the complaint of painless blurred vision in the right eye. Gonioscopy through a widely dilated pupil revealed a clear, oval, pale brown, semitransparent cyst that was located in the inferotemporal aspect of the iris. The cyst was touching the lens, and the lens was pushed superonasally by the cyst. The iris cyst was treated with selective laser trabeculoplasty laser. A small iris notch emerged at the 8-o'clock position of the pupillary margin. Only mild flare and minimal pigment dispersion were documented postoperatively, which resolved in a few days with a topical corticosteroid. CONCLUSIONS: Selective laser trabeculoplasty laser may be considered as a potentially useful and relatively less invasive technique in the treatment of peripheral pigment epithelium iris cysts.

COVID-19 Associated Bilateral Acute Iris Transillumination.

Purpose: To report a case of bilateral acute iris transillumination (BAIT) in association with coronavirus disease 2019 (COVID-19).Case report: A 44-year-old woman patient presented with decreased visual acuity, pain, photophobia, and redness in both eyes. The patient reported that she had recent close contact with severe acute respiratory syndrome - coronavirus-2 (SARS-CoV-2) case; also, she mentioned that she was hospitalized for bilateral pneumonia for 14 days. On examination, visual acuity of both eyes was 20/40. Slit-lamp biomicroscopy showed bilateral pigment deposition on the corneal endothelium, 4+ pigment dispersion in the anterior chamber, iris depigmentation with iris transillumination defects. Intraocular pressure was measured as 32 mmHg in right eye and 38 mmHg in left eye. The patient was started on bilaterally topical anti-inflammatory and anti-glaucomatous therapy.Conclusion: It is important to keep in mind that ocular manifestations associated with COVID-19 may include rare entities such as BAIT.

Relationship of Topographic Distribution of Geographic Atrophy to Visual Acuity in Nonexudative Age-Related Macular Degeneration.

PURPOSE: To investigate the topographic distribution of geographic atrophy (GA) and to identify an anatomic endpoint that correlates with visual acuity (VA) in eyes with GA. DESIGN: Retrospective analysis of a multicenter, prospective, randomized controlled trial. PARTICIPANTS: The Age-Related Eye Disease Study participants with GA secondary to nonexudative age-related macular degeneration. METHODS: We manually delineated GA on 1654 fundus photographs of 365 eyes. We measured GA areas in 9 subfields on the Early Treatment Diabetic Retinopathy Study (ETDRS) grid and correlated them with VA via a mixed-effects model. We determined the optimal diameter for the central zone by varying the diameter from 0 to 10 mm until the highest r2 between GA area in the central zone and VA was achieved. We estimated the VA decline rate over 8 years using a linear mixed model. MAIN OUTCOME MEASURES: Geographic atrophy area in macular subfields and VA. RESULTS: The percentage of area affected by GA declined as a function of retinal eccentricity. GA area was higher in the temporal than the nasal region (1.30 ± 1.75 mm2 vs. 1.10 ± 1.62 mm2; P = 0.005) and in the superior than the inferior region (1.26 ± 1.73 mm2 vs. 1.03 ± 1.53 mm2; P < 0.001). Total GA area correlated poorly with VA (r2 = 0.07). Among GA areas in 9 subfields, only GA area in the central zone was associated independently with VA (P < 0.001). We determined 1 mm as the optimal diameter for the central zone in which GA area correlated best with VA (r2 = 0.45). On average, full GA coverage of the central 1-mm diameter zone corresponded to 34.8 letters' decline in VA. The VA decline rate was comparable between eyes with initial noncentral and central GA before GA covered the entire central 1-mm diameter zone (2.7 letters/year vs. 2.8 letters/year; P = 0.94). CONCLUSIONS: The prevalence of GA varies significantly across different macular regions. Although total GA area was associated poorly with VA, GA area in the central 1-mm diameter zone was correlated significantly with VA and may serve as a surrogate endpoint in clinical trials.

Complete ring cyst of iris pigment epithelium documented by ultrasound biomicroscopy.

The authors report a complete ring cyst of the iris pigment epithelium confirmed by ultrasound biomicroscopy. In this case, ultrasound biomicroscopy effectively excluded the differential diagnoses of ring melanoma of the iris and multiple separate cysts of the iris pigment epithelium.

Pigment deposits in the retrolental space of Berger: a rare feature of pigment dispersion syndrome? / Depósitos de pigmento no espaço de Berger: uma característica rara de síndrome de dispersão pigmentar?

ABSTRACT Pigment dispersion syndrome is associated with clinical features such as Krukenberg's spindles, trabecular pigmentation, Scheie's stripe and Zentmayer's ring. Another less common feature of this syndrome is retrolental pigment deposits due to anterior hyaloid detachment or a defect in the Wieger's ligament. We present two cases of pigment deposits on the posterior lens capsule. In both cases, there is bilateral dispersion of pigment throughout the anterior segment. The retrolental deposits are unilateral in the first case and bilateral in the second. Both patients report a history of ocular trauma. This is a possible important clinical sign of pigment dispersion syndrome, rarely described.

RESUMO A síndrome de dispersão pigmentar associa-se a sinais clínicos característicos como fuso de Krukenberg, hiperpigmentação da malha trabecular, linha de Scheie e anel de Zentmeyer. Um sinal menos comum dessa síndrome é o depósito de pigmento posterior ao cristalino, que ocorre por um descolamento da hialoide anterior ou um defeito no ligamento de Wieger. Apresentamos dois casos de depósitos de pigmento posterior à cápsula posterior do cristalino. Em ambos os casos, existia dispersão bilateral de pigmento por todo o segmento anterior. No primeiro caso, os depósitos eram unilaterais e, no segundo, estavam presentes em ambos os olhos. Este pode corresponder a um sinal potencialmente importante da síndrome de dispersão pigmentar, raramente descrito.

Unilateral acute posterior multifocal placoid pigment epitheliopathy in a patient with a strongly positive purified protein derivative test.

A 27-year-old man presented with a complaint of decreased visual acuity in the right eye. Best-corrected visual acuity was 20/60 in the right eye and 20/20 in the left eye. Right eye fundoscopy revealed mild vitritis, multifocal yellowish lesions, and macular serous retinal detachment. Left eye evaluation was normal. Acute posterior multifocal placoid pigment epitheliopathy was diagnosed in the right eye. Complementary exams revealed a strong reaction to the Mycobacterium tuberculosis purified protein derivative test, thus treatment for tuberculosis was initiated. Baseline fluorescein angiography of the right eye revealed early hypofluorescence and late staining of the lesions. Optical coherence tomography of the right eye demonstrated the accumulation of subretinal and intraretinal fluid associated with cystoid macular edema. During follow-up, the retinal fluid and cysts disappeared, which was followed by disorganization of foveal interdigitation and ellipsoid zones. This is the second described case of unilateral acute posterior multifocal placoid pigment epitheliopathy in a patient with a strong positive result to the M. tuberculosis purified protein derivative test.

Dome-shaped macula in eyes with myopic posterior staphyloma.

PURPOSE: To describe an unusual feature in myopic eyes responsible for visual loss, which we call a dome-shaped macula. DESIGN: Retrospective, observational case series. METHODS: After observing isolated cases of dome-shaped macula, we analyzed optical coherence tomography (OCT) scans of 140 highly myopic eyes present in our OCT database to find similar cases. Fifteen eyes of 10 patients had a dome-shaped macula. These patients all had undergone fluorescein angiography (FA), indocyanine green angiography (ICGA), and B-scan ultrasonography examinations. RESULTS: The mean refractive error of the affected eyes was -8.25 diopters (D; range, -2 to -15 D). Median visual acuity was 20/50. Recent visual impairment was noted in 11 of the 15 eyes studied, and metamorphopsia was noted in eight eyes. Four eyes were asymptomatic. FA showed atrophic changes in the macular retinal pigment epithelium (RPE) in all eyes, combined with focal points of leakage in seven of the 15 eyes. The dome-shaped appearance of the macula was visible on both B-scan ultrasonography and OCT: a characteristic bulge of the macular retina, RPE, and choroid within the concavity of the moderate posterior staphyloma was present in all eyes. In 10 eyes, OCT also showed a shallow foveal detachment at the top of the dome-shaped macula. CONCLUSIONS: A dome-shaped macula within a myopic staphyloma is an unreported type of myopic posterior staphyloma. The dome-shaped macula often is associated with RPE atrophic changes and foveal retinal detachment, which may explain the visual impairment in these eyes.

In-vivo PET imaging of implanted human retinal pigment epithelium cells in a Parkinson's disease rat model.

BACKGROUND AND AIM: Researchers find that monitoring the differentiation of implanted cells in vivo is difficult. This study was designed to show that it is possible to track the efficacy of transplanted human retinal pigment epithelial cells (RPE cells) in a rat model of Parkinson's disease by using positron emission tomography (PET). METHODS: RPE cells or normal saline were injected into striatum of the injured side of the rat model in treated and control groups, respectively. PET imaging of both groups was undertaken before transplantation and at intervals afterwards, using C-raclopride and C-beta-CFT as the markers. Observation of the rats' behaviour and immunofluorescence confocal microscopy were also used to prove the PET results. RESULTS: PET studies showed increased accumulation of C-raclopride and decreased C-beta-CFT in the injured side of striatum in both groups. C-raclopride decreased along with a concomitant increase of C-beta-CFT after transplantation in the treated group. The changes shown by the PET studies paralleled the behavioural states and confocal microscopy observations in the treated animals. CONCLUSION: These results suggest that even a clinical PET scanner could, to a certain extent, provide some information on the existence and in-vivo differentiation of RPE cells in a rat model of Parkinson's disease.

Time Course Changes of the Mechanical Properties of the Iris Pigment Epithelium in a Rat Chronic Ocular Hypertension Model.

BACKGROUND: The flow field of aqueous humor correlates to the stiffness of iris pigment epithelium (IPE) which acts as a wall of posterior chamber. We focus on the variations of IPE stiffness in a rat ocular hypertension (OHT) model, so as to prepare for exploring the mechanism of duration of OHT. METHODS: Episcleral venous cauterization (EVC) was applied on one eye of male adult Sprague-Dawley rats to induce chronic high intraocular pressure. According to the duration of OHT (0, 1, 2, 4, and 8 weeks), rats were randomly divided into Gw0, Gw1, Gw2, Gw4, and Gw8. Atomic force microscope (AFM) analysis was applied to test IPE stiffness in three regions: iris root, mid-periphery, and pupillary-margin in each group. Histological changes of IPE were also examined in Gw4 and Gw8. RESULTS: There was an overall growing tendency of IPE stiffness in EVC eye. IPE in EVC eye was significantly stiffer than fellow eye in Gw2, Gw4, and Gw8 (in iris root, mid-periphery, and pupillary-margin, p<0.05). IPE in EVC eye in pupillary-margin was significantly stiffer than iris root in Gw4 and Gw8 (p<0.05). In EVC eye, IPE becomes thinner and IPE cell density decreases. CONCLUSION: IPE stiffness increases gradually with the duration of chronic high intraocular pressure.

Peripheral pigmentary iris cyst: evaluation and differential diagnosis.

Peripheral iris pigment epithelial cysts are benign lesions of the anterior segment. They are located at the iridociliary junction and can produce a localised distortion and/or displacement of the iris. Patients who have these lesions are usually asymptomatic and in the absence of a careful slitlamp examination will often remain undetected. The aetiology of these cysts is unknown and they tend to be unilateral and solitary in nature. The cyst walls are composed of iris pigment epithelium and the lumen contains clear fluid. Women are three times more likely to develop these cysts, which must be differentiated from malignancies of the iris or ciliary body. We report a case involving a peripheral pigmentary iris cyst and discuss methods of differentiating this benign lesion from serious ocular tumours.

Detection of an Iris Pigmented Epithelium Cyst in a Child by High Frequency Ultrasonography.

An 18-month-old boy presented with an iris pigmented epithelium cyst. The diagnosis was confirmed using high frequency ultrasonography. The cyst was determined to be in an intermediate location, thus needing no treatment provided that any potential complications were noted. However, continued monitoring is required to detect any changes to adapt management. [J Pediatr Ophthalmol Strabismus. 2017;54:e31-e33.].

[OCT angiography findings in acute posterior multifocal placoid pigment epitheliopathy (APMPPE)]. / OCT-Angiographie-Befund bei akuter posteriorer multifokaler plakoider Pigment epitheliopathie (APMPPE).

This article reports the case of a 32-year-old female patient who presented with severe spasmodically occurring retrobulbar pain, headaches and light sensitivity. The neurological examination confirmed cephalalgia but no evidence of other neurological symptoms was found. Ophthalmoscopy revealed yellowish patchy lightened spots in the middle periphery. Fluorescein angiography (FLA) and indocyanine green angiography (ICGA) revealed findings typical for acute posterior multifocal placoid pigment epitheliopathy (APMPPE). Hypofluorescent areas visible using ICGA in the early and late phases showed corresponding hypoperfused areas by optical coherence tomography angiography (OCT-A). To the best of our knowledge this is the first description of these findings in OCT-A.

Transpupillary argon laser photocoagulation and Nd:YAG laser cystotomy for peripheral iris pigment epithelium cyst.

PURPOSE: To report the use of a combination of transpupillary argon laser photocoagulation and Nd:YAG laser cystotomy in the treatment of peripheral iris pigment epithelium (IPE) cyst. DESIGN: Case report. METHODS: A 23-year-old man presented with a one-month history of blurred vision and pain in the right eye. Slit-lamp biomicroscopy and gonioscopy revealed, in the iridociliary sulcus, a large clear IPE cyst, which was demonstrated by ultrasound biomicroscopy (UBM). A combination of transpupillary photocoagulation to devitalize the epithelial lining and neodymium-doped yttrium aluminum garnet (Nd:YAG) laser cystotomy to drain the cyst was engaged. RESULTS: Laser treatment resulted in vanishing of the cyst and disappearance of the subject symptoms. Two months postoperatively, no evidence existed of the iris pigment cyst regrowth. CONCLUSIONS: The minimal invasiveness of the combination of transpupillary laser photocoagulation and cystotomy yielded a favorable result. This procedure may be an alternative to conservative regimen if IPE cyst is clearly defined and readily seen.

Ultrasonographic characteristics and treatment outcomes of surgery for vitreous hemorrhage in idiopathic polypoidal choroidal vasculopathy.

PURPOSE: To describe the ultrasonographic characteristics and treatment outcomes of surgery in vitreous hemorrhage (VH) associated with idiopathic polypoidal choroidal vasculopathy (IPCV). DESIGN: Retrospective interventional and observational case series. METHODS: Clinical, ultrasound, and surgical data of 10 consecutive patients operated for VH due to IPCV in a tertiary eye institute was studied by chart review. Data were analyzed to determine the clinical features, ultrasonographic characteristics, and surgical outcomes. An additional five patients with IPCV without VH were evaluated by ultrasound in various stages of the disease. RESULTS: Between January 1998 and March 2005, 10 eyes of 10 patients underwent vitreous surgery for VH associated with IPCV. Characteristic ultrasonographic features that helped the diagnosis preoperatively included focal choroidal thickening without excavation or acoustic hollowing with associated low reflective echoes of dispersed VH, or diffuse choroidal thickening and low-intensity echoes of dispersed hemorrhage on either side of the retinal spike, often without vitreous detachment spike. Oral corticosteroids were provided preoperatively to patients with associated exudative retinal detachment. Indocyanine green angiography (ICGA) confirmed IPCV postoperatively. Focal lesions were treated with laser photocoagulation. Anatomical success was seen in nine of 10 eyes. Visual acuity improved in five of 10 eyes but was limited by macular pathology in other five eyes. The most common complication was iatrogenic tears. Some eyes had recurrent IPCV lesions in follow-up. CONCLUSIONS: Characteristic ultrasonographic features could identify IPCV in eyes with VH. Anatomical and visual outcomes of our management approach were encouraging and need further study.

Adenoma of the nonpigmented ciliary epithelium.

PURPOSE: To report the clinicopathologic features of an adenoma of the nonpigmented ciliary epithelium. DESIGN: Single interventional case report. METHODS. A nonpigmented iris and ciliary body tumor was diagnosed in a 66-year-old woman who complained of blurred vision related to a unilateral cataract. A combined cataract surgery and partial lamellar sclerouvectomy was performed. RESULTS: Histopathologic findings disclosed an adenoma of the nonpigmented ciliary epithelium. CONCLUSIONS: Unilateral cataract in an adult patient can be rarely related to a tumor growing from the ciliary epithelium. Adenoma of the non pigmented ciliary epithelium may mimic an amelanotic melanoma. Partial lamellar sclerouvectomy is an effective method to manage this condition and to confirm the diagnosis.