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Purpose: Treatment of persistent ocular discomfort in patients with Cogan's epithelial basement membrane dystrophy (EBMD) is a challenge for ophthalmologists. This study aimed to determine...

Purpose To describe the clinical features of secondary glaucoma associated with transthyretin (TTR)-related Familial Amyloid Polyneuropathy (FAP). Methods In this retrospective monocentric study, 5 patients with FAP associated secondary glaucoma were seen at the ophtalmologic consultation of the french national center for FAP between 2011 and 2012. The mutation of the amyloidogenic TTR variants was analysed for all patients. All patients had a complete ophthalmic examination including BCVA, IOP, slit lamp and optic disc photographs, gonioscopy, pachymetry, automated perimetry, and OCT-RNFL. Glaucomatous optic neuropathy was diagnosed based on the presence of visual field abnormalities, neuroretinal rim thining, excavation or RNFL defects. Medical and surgical treatments were analysed for all patients. Results All cases had bilateral involvement except 2 monophtalmic patients. All patients were of portuguese origin and carriers of the Val30Met mutation. There were 4 women and 1 man with a mean age of 58.8±7.7 years. Mean BCVA was 0,83±0.9 LogMAR. Three eyes had a BCVA below 20/400. Mean IOP was 26.6±6.6 mmHg. Mean deviation was -17.6±11.2dB. Fringed pupil and anterior chamber amyloid deposition were noted in all affected eyes. Three patients had concomitent vitreous involvement. Patients were treated with 1.2±1.5 ocular hypotensive drugs. Four eyes had been treated with at least 1 filtrating surgery. Conclusion FAP-associated secondary glaucoma is a very severe disease, associated with amyloid deposits in the anterior chamber and characteristic pupil deformation. Systematic and comprehensive eye examination should be performed in all patients affected with FAP in order to improve early detection of glaucoma.

Purpose To assess the quality of tear secretion in eyes of patients with a history of unilateral and recurrent herpetic keratitis Methods 33 patients with a history of recurrent herpetic keratitis (either archipelago keratitis, KA, or kerato-uveitis, KU, or neurotrophic keratitis, KN) were compared with 33 normal subjects. A complete ophthalmologic examination was performed, with successively the assessment of tear osmolarimetry, tears break-up time (TBUT), Schirmer I test, and corneal sensitivity. Patients with other potential causes of abnormal tears were excluded, and all tests were performed at least 3 months after the last relapse of keratitis. Controls were selected among asymptomatic patients scheduled for cataract surgery or refraction disorders, and were matched for age and gender with patients. Results The patients group (19 men, 14 women, aged 52 ± 7 years) included 16 patients with KA, 13 with KU, and 6 with KN. In the control group (similar sex ratio and mean age), all tests were symmetrical between the two eyes. In the 3 groups of patients, tear osmolarimetry was significantly greater in affected eyes than in controls, as well as TBUT was significantly reduced. In contrast, Schirmer I test was reduced only in eyes of patients with a history of KU or KN. Finally, only KN eyes were statistically less sensitive than healthy eyes. Conclusion Recurrent herpes keratitis induced changes in lacrimal secretion, even when the disease is apparently quiescent, and the abnormalities are more important as the corneal disease is progressing In the context of our study, tear hyperosmolarity appeared to be a particularly sensitive test to detect impairment of tear secretion.

Cholesterol crystal embolism (CCE) is a rare and severe multisystemic disorder. It results from a massive release of cholesterol crystals from widespread atherosclerotic disease. The main difference with atherosclerosis is the severity and the quantity of the embolic events that occur during the course of the disease, eventually leading to multivisceral failure and death. The symptoms are multiple and make it a diagnostic challenge. Fundoscopic examination can be of great help, showing retinal emboli in up to 25% of the cases, and has been rarely described in the ophthalmologic literature. We report the case of a 77-year-old man with acute renal failure after coronarography. Retinal emboli seen in the fundus confirmed the diagnosis of cholesterol crystal embolism and thus prevented any further invasive investigations. In this case, anticoagulants must be stopped and any further endovascular procedure proscribed. Although impossible for this patient, peritoneal dialysis should be preferred to hemodialysis because it does not need any anticoagulation. Systemic corticosteroid can be used in the acute phase. Fundoscopic examination should be performed each time cholesterol crystal embolism is suspected. When typical emboli are seen in the retina, it permits avoiding invasive investigations and saving precious time for the management of this potentially lethal disease.

Purpose Patients with history of recurrent herpetic keratitis very often complain about their vision despite normal visual acuity (VA). The purpose of this study was to assess the optical aberrations and the quality of vision of the affected eye in patients with unilateral recurrent herpetic keratitis with preserved VA, and to compare these results with those of the non-affected eye. Methods Patients (n=15) with unilateral recurrent herpetic keratitis and an normal VA (Best corrected VA≤0 logMAR) were included in this study. Corneal optical aberrations of both eyes were assessed using dynamic skiascopy with OPD Scan II® (Nidek™). Wavefront analysis was performed during a quiescent period of the herpetic disease. The fellow eye was used as control. A blinded examiner retrospectively analyzed all the datas. Results The root mean square was significantly increased for high order aberrations (p=0.004) in the affected eye and particularly for trefoil and tetrafoil (p=0.004 and 0.02, respectively). In addition, The modulation transfer function (area under the curve) and the Strehl’s ratio were significantly lower in the affected eyes compared to the non affected eyes (p=0.02 and 0.02). Conclusion Our data show that herpetic keratitis induces wavefront abnormalities which could explain visual discomfort reported by patients with normal VA. A prospective study including more patients should provide further informations on the rate and the clinical significance of these optical aberrations.

Purpose A narrow iridocorneal angle (ICA) is a risk factor for glaucoma progression. However, many patients treated for primary open angle glaucoma (POAG) have never been assessed with dynamic gonioscopy. In this study, we performed dynamic gonioscopy in patients referred for progressing POAG and suspicion of glaucoma (GS) to evaluate the rate of misdiagnosed narrow ICA. Methods We retrospectively analysed the clinical data of consecutive glaucoma or GS patients referred for evaluation from November 2009 to October 2010. All patients had been previously diagnosed with open ICA. Patients were examined by a single ophthalmologist. The evaluation included detailed medical history, comprehensive ophthalmologic examination, diurnal hourly monitoring of the intraocular pressure, static automated perimetry, retinal nerve fiber layer thickness analysis, central pachymetry and dynamic gonioscopy (Possner® lens). Results 135 patients were included. The mean age was 53.6 years (+/- 6.2 years). Prior to our evaluation, glaucoma and GS patients had been followed 5.2 years (+/- 3.4 years) with an average of 4.2 visits and 2.2 visual fields. 58 patients (43%) had never undergone gonioscopy. A narrow angle was diagnosed in 18 patients (13.3%), including 5 patients with plateau-iris configuration confirmed by ultrasonic biomicroscopy (27.7% of narrow angles). Conclusion A narrow ICA is not a rare finding among patients diagnosed with POAG or GS patients. Dynamic gonioscopy should be performed systematically for all glaucoma and glaucoma suspect patients, especially in cases of glaucoma progression despite an efficient and well conducted treatment. Lens size evolution with time requires repeated evaluations.

Purpose To assess the osmolarity of tears in eyes affected by recurrent herpetic stromal keratitis. Methods All the patients referred for a recurrent unilateral stromal keratitis infection by either Herpes simplex virus (VZV) or varicella-zoster virus (VZV). All the patients have been finally tested for dry eye conditions when all clinical signs of ongoing keratitis had resolved. The osmolority was assessed using the TearLab® and then the corneal sensitivity was tested with the Cochet-Bonnet esthesiometer (Luneau™). Values were compared using non-parametric tests, and statistical significance was defined as p

Purpose To assess optical aberrations using wavefront analysis in the affected eye of patients with unilateral herpetic keratitis with no apparent visual disturbance (visual acuity : 20/20), and to compare these results with those of the non-affected eye Methods Corneal optical aberrations from 5 patients with unilateral herpetic keratitis and normal visual acuity (20/20 OU) have been assessed using dynamic skiascopy with OPD Scan II® (Nidek™). Both eyes in all patients were apparently normal using slit-lamp examination, and wavefront analysis was performed in a quiescent period of the herpetic disease. The fellow eye was used as control. Data were retrospectively analyzed by a blinded examiner. Results Zernike’s polynoms and MTF curve analysis showed bilateral abnormalities in 4 patients, with no specific difference between the two eyes. In contrast, one patient had wavefront disturbances in the affected eye, whereas the non-affected eye was normal. Conclusion Moderate herpetic keratitis may induce wavefront abnormalities in some patients despite a normal visual acuity and an apparently normal cornea. This could explain visual discomfort that is sometimes described in such patients. . Further studies are needed to unerstand waevfront aberrations induced by herpetic keratitis with normal examination.

Purpose Define the interest of QuantiFERON®-TB Gold test (Cellestis) in patients with positive Tuberculin Skin Test (TST) to improve etiologic and therapeutic work-up in uveitis. Methods All patients followed in the Ophthalmology Department of Bicetre Hospital between April 2007 and March 2009 for uveitis had an extensive work-up including Tuberculin Skin Test (TST). Among them, 14 patients had a very positive TST making suspect an active tuberculosis infection. Then all of these patients had systematically a quantiFERON®-TB Gold test. Finally, charts from clinical exam and therapeutic management were retrospectively analysed. Results The mean age at the time of presentation was 58 years (+/- 9) and sex ratio (F/M) was 43%. Uveitis was bilateral in 43% of cases, and the location of inflammation was anterior in 43% of the cases, intermediate (21.4%), posterior (21.4%) or total (7%). The average of PPD test was 21.75mm (+/- 4mm, 15-65mm) and minimal delay between PPD and quantiFERON®-TB Gold test was 3 days (mean: 42 days +/- 17). QuantiFERON®-TB Gold test was positive in 9 patients (64.3%) enjoining introduction of a full anti-tuberculosis therapeutic treatment in 8 patients (57.1%), that preceded oral steroids regimen in 42.8% of cases. Visual acuity increased dramatically (6 lines) in 4 patients and was unmodified for the others. Conclusion QuantiFERON®-TB Gold test seems usefull in systematic diagnostic strategy in uveitis; it stays negative in 35.7% of the case suggesting that positive results are significant. However, further studies are necessary to define its place in diagnostic procedure in uveitis.