The International Council of Ophthalmology Ophthalmic clinical evaluation exercise.

Purpose: Fifteen years after the publication of the Ophthalmic Clinical Evaluation Exercise (OCEX), it was deemed necessary to review and revise it, and to validate it for an international audience of ophthalmologists. This study to revise the OCEX and validate it for international use. Methods: The OCEX rubric was changed to a modified Dreyfus scale; a behavioral descriptor was created for each category. An international panel of ophthalmic educators reviewed the international applicability and appropriateness of the tool. Results: A tool for assessing and giving feedback on four aspects of clinical competence during the ophthalmic consultation (interview skills, examination, interpersonal and communication skills, and case presentation) was revised. The original scoring tool was improved to a new behavioral one, and relevant comments and suggestions from international reviewers were incorporated. The new tool has face and content validity for an international audience. Conclusion: The OCEX is the only tool for workplace assessment and feedback specifically for ophthalmology residents and the ophthalmic consultation. This improved and simplified version will facilitate its use and implementation to diverse programs around the world.

Bilateral congenital macular coloboma: Swept-source optical coherence tomography findings.

A 15-year-old male presented with decreased vision and nystagmus from childhood. Best-corrected visual acuity was limited to 0,05/10 in both eyes. Fundus examination revealed a well-demarcated macular excavation of 2 discs diameter, baring of the underlying sclera, surrounded by a pigmented rim and hypopigmented retinal areas. Autofluorescence images showed a central hypoautofluorescent lesion with patchy hypoautofluorescent areas corresponding to retinal pigmented epithelium (RPE) and photoreceptor atrophy. Swept-source optical coherence tomography showed a crater-like depression with atrophy of the neurosensory retina and the absence of RPE and choroid except some large choroidal vessels. Anti-toxoplasma antibody was negative. These findings are suggestive of a congenital macular coloboma. Similar lesions have to be excluded such as toxoplasmosis macular scar and North Carolina dystrophy.

Multimodal imaging of foveoschisis and macular pseudohole associated with gyrate atrophy: a family report.

BACKGROUND: To report the results of multimodal imaging of a biochemically confirmed case of a family with gyrate atrophy (GA) associated with foveoschisis and macular pseudohole. CASE PRESENTATION: Two sisters presented to us with progressive bilateral decreased vision. The 26-year old sister had a best corrected visual acuity (BCVA) of 20/32 in the right eye (RE) and 20/100 in the left eye (LE). Dilated fundus examination revealed multiple bilateral chorioretinal atrophy areas in the midperipheral and peripheral retina. Fluorescein angiography did not show any leak in the macular area. Swept-source optical coherence tomography (SS-OCT) showed increased central macular thickness in both eyes with foveoschisis. Optical coherence tomography angiography (OCTA) showed petaloid non-reflective areas and some perifoveal microvascular alterations similar to telangiectasias in the deep capillary complex. The 30-year-old sister had a BCVA of 20/20 in the RE and 20/32 in the LE. SS-OCT was normal in the RE and demonstrated a macular pseudohole with a fine epiretinal membrane in the LE. The persistent retinal tissue at the base of the pseudohole was disorganised. Blood tests showed hyperornithinemia in the 2 cases. Based on these observations, the patients were diagnosed with gyrate atrophy of the choroid and retina and were treated with a pyridoxine supplement and an arginine-restricted diet. CONCLUSIONS: Foveoschisis and macular pseudohole may be associated in GA, increasing the risk of rapid vision loss. OCTA is an interesting imaging tool that can help to better understand the pathophysiological mechanism of these macular involvements in GA.

Atrophic tear retinal detachment: clinical characteristics and surgical treatment results at long term.

AIM: to analyse clinical and epidemiological characteristics of atrophic tear retinal detachment (ATRD) and evaluate anatomical and functional results. METHODS: Retrospective study of 48 cases underwent primary scleral buckling for ATRD. Mean follow up was 80,52 months. RESULTS: ATRD represented 7% of all reghmatogenous RD. Mean age of patients was 38 years and 7 months. Age was less than 40 years in 62,5% of patients. Male predominance was noted. Myopia was noted in 67% of cases and 78,12% presented high myopia. Mean delay of consultation was 7 months ranging from 1 and 60 months. Visual acuity was ranged between light perception to 10/10. The AT number in eye varied between1 to 15 (mean 3 tears). The seat of AT was preferentially in inferior temporal quadrants than superior one. Maculae was detached in 87,5% of cases. All patients underwent scleral buckling: longitudinal buckle was performed in 27% of cases, radial sponge was put on in 8,3% of eyes and circumferential one was made in 64,5% of cases. Sub-retinal fluid was punctured in 75% od cases. Retinal reattachment was obtained in all cases; after one surgery in 95,83% of cases. Redetachment was observed in 2 cases: it was linked with unknown AT. Visual acuity was improved in 83,3% of cases. It was more than 5/10 in 14,6% of cases. Postoperative hypertonia was observed in 18,75% of cases. Choroidal detachment was observed in one case and it was resolved spontaneously. Scleral bukle rejection was observed in one case. CONCLUSION: ATRD was not associated at posterior vitreous detachment. It was observed in young myopic patients. There is a chronic form of retinal detachment with many tears. Scleral buckle was the surgical treatment of this type of RD with good anatomical prognostic.

Azathioprine for glucocorticoid resistant noninfectious uveitis.

BACKGROUND: the systemic steroids represent the first line treatment in the majority of the noninfectious uveitis, except some specific indications as the Behcet disease. Nevertheless, this treatment may be ineffective and immunosuppressive therapy is mandatory. PURPOSE: to evaluate effectiveness and side effects of azathioprine (AZA) in corticosteroid resistant noninfectious uveitis (CRNIU). METHODS: This prospective study (2002- 2009), concerned 21 patients (mean age 37 years), 37 eyes, with CRNIU. Patients received oral AZA 2,5mg/kg/day, initiated in association with high dose steroids, with an end-point of 12 months. Response was defined as complete, partial response and failure, for each of the 3 following out-come measurements: improvement of BCVA, improvement of inflammation, steroids-sparing. Statistical analysis was considered significant if p value < 0,05. RESULTS: side effects occurred in 42,8% (9/21) of patients, in which 5/9 patients stopped the treatment. Regarding BCVA, complete success was observed in 62,5%, partial response in 20,9% , and failure in 16,6% of cases. Regarding inflammation, complete success was noted in 70,8%, partial response in 29,1% and failure in 16,6 % of cases. Complete response of steroid sparing was observed in 85,7% of cases without failure. Complete success of the 3 criteria was observed in 57,1% of patients / 62,5% of eyes. Cataract (p=0,013) and pallor of optic nerve head (p=0,013) were associated to poor visual prognosis, BCVA of 20/40 or more (p=0,003, RR=2,38)) and papilledema (p=0,022, RR=2) to good visual prognosis. BCVA of 20/200 or less (p=0,001) was associated to failure of AZA on inflammatory response. CONCLUSION: AZA is safe and effective in corticosteroid-sparing and controlling inflammation in CRNIU. Its low cost and availability allow proposing it as a first-line option, especially when new biological treatments are difficult to obtain.

Serum IL-33 levels and skin mRNA expression in Behçet's disease.

OBJECTIVES: Behçet's disease (BD) is an autoimmune/inflammatory disease characterised by abnormal production of proinflammatory cytokines. Interleukin-33 (IL-33) is a novel cytokine of the IL-1 cytokine family that has been recently implicated in several inflammatory and autoimmune diseases and the association of IL-33 with BD has remained unknown. Here we document for the first time, IL-33 level and its association with BD. METHODS: Serum IL-33 levels were measured in 46 BD patients (20 patients in active stage) and compared to multiple sclerosis (MS), rheumatoid arthritis (RA) patients and to healthy controls. In parallel, the transcription factor NF-κB that mediates IL-33 transcription was also measured. IL-33 mRNA was also quantified in freshly isolated PBMCs and in skin biopsies by real-time RT-PCR analysis. IL-6 and IL-17 were measured by ELISA. RESULTS: Serum IL-33 level was significantly higher in active BD patients [159.65 ± 61.7 pg/mL] compared to inactive BD patients [85.57 ± 21.07 pg/mL] (p<0.0001) and healthy controls [70.03±25.95 pg/mL] (p<0.0001). Active BD patients expressed lower IL-33 levels than the control disease group, RA and MS patients [p=0.00021]. The serum IL-33 level in active BD patients was corroborated by IL-33 mRNA expression in fresh PBMC. Patients with active BD with retinal vasculitis showed the highest serum IL-33 level. We further stimulated cultured PBMCs with phorbol myristate acetate (PMA) and ionomycin and macrophages with LPS for 24 h. Following stimulation the levels of IL-33 were increased similarly in PBMC [92.35±24.81 pg/ml] and macrophages [93.10±21.58 pg/ml] in active BD patients compared to healthy controls. NF-κB DNA binding activity was significantly increased in PBMCs of active BD patients particularly in LPS-stimulated macrophages compared to healthy controls. IL-33 mRNA expression in the skin lesions of patients with active BD was significantly increased compared to that in healthy skin biopsies [p=0.00016]. A significant relationship was found between the levels of IL-33 and IL-17 [r=0.533; p=0.0024] and IL-33 and IL-6 [r=0.661, p=0.0015] in 20 active BD patients. CONCLUSIONS: Elevated IL-33 level in active BD patients was found to correlate with disease activity. Targeting IL-33 should be approached with caution.

Fluffy white iris precipitates in Fuchs uveitis: a new sign for an old disease.

To describe particular iris precipitates in a series of five eyes from six patients with Fuchs uveitis (FU). Iris precipitates were noted by four independent examiners during routine physical examination of the angle by gonioscopy with Goldmann's three-mirror lens in patients with FU. The result was confirmed by examination, using the same method, of five other consecutive patients with FU and compared to 10 normal control eyes from five healthy individuals. Other causes of anterior uveitis were excluded. There were no iris precipitates in the healthy eyes. In eyes with FU, there were fluffy white iris precipitates, not visible by full-face examination or by classic slit-lamp examination. They were similar to keratic precipitates described in FU: starry, blurry and transparent with a tendency towards the white. Situated on the surface of the iris, they were visible only with the particular diffusion of the light from the gonioscopy's glass on the darkly pigmented iris of patients from North Africa. Fluffy white iris precipitates, seen in FU patients, appear to represent an additional clinical sign and may improve our diagnostic accuracy in this disease. Its visibility requires a specific technique during clinical examination. The validity of this new clinical sign based on this fact is yet to be determined.

Bilateral retinal pigment epithelium tears in acute Vogt-Koyanagi-Harada disease.

PURPOSE: To report an original case of retinal pigment epithelial (RPE) tears in acute Vogt-Koyanagi-Haradadisease. METHODS: Observational case report of a patient who presented with RPE tears in acute Vogt-Koyanagi-Harada disease, who underwent clinical examination, fluorescein and infracyanine green angiographies, and optical coherence tomography evaluation. RESULTS: A 42-year-old woman presented with bilateral serous retinal detachment related to Vogt-Koyanagi-Harada disease. Eight days after high-dose systemic corticosteroids, she developed bilateral RPE tears. At the 15-month follow-up, visual acuity was 20/20 in both eyes. CONCLUSION: The RPE tears are rarely observed in acute Vogt-Koyanagi-Harada disease. The RPE tears were likely caused by acute inflammation of the choroidal stroma.