To study the presence of certain proteins - EGF (epidermal growth factor), KGF (keratinocyte growth factor), IL-10 (interleukin 10), HGF (hepatocyte growth factor), Alpha2-macroglobulin and IL-1RA (interleukin 1 receptor antagonist) in cryopreserved amniotic membranes at 1 and 18 months and, as a secondary objective, to detect mRNA corresponding to KGF, IL-1Ra, Alpha2-macroglobulin, Fas Ligand, TGF beta (transforming growth factor beta) and Lumican by RT-PCR in membranes preserved at 1 and 18 months.Four samples of amniotic membrane were divided into 2 groups: the first group (N = 2) cryopreserved for 1 month and the second group (N = 2) cryopreserved for 18 months, in order to be studied by RT-PCR and ELISA.RT-PCR detected KGF, IL-1Ra, Alpha2-macroglobulin, Fas Ligand, and Lumican. Of these, FAS Ligand mRNA was found in samples preserved for 1and 18 months. KGF, Lumican, and alpha2-microglobulin mRNA were found only at 1 month, and IL-1Ra mRNA was absent in both sample groups. RT-PCR for TGF-beta was inconclusive. ELISA was performed for detection and quantification of 6 proteins (EGF, KGF, IL-10, HGF, Alpha2-macroglobulin and IL-1Ra) in both amniotic membrane groups. All 6 proteins were found in all samples, with a lower concentration at 18 months compared to 1 month of preservation.This study shows that membranes cryopreserved in 50% glycerol for 18 months do retain the proteins necessary for regeneration of the corneal surface, giving these membranes their biochemical properties.Étudier la présence protéique par ELISA de l’EGF (facteur de croissance épidermique), du KGF (facteur de croissance des kératinocytes), de l’IL-10 (interleukine 10), de l’HGF (facteur de croissance hépatocytaire), de l’Alpha2-macroglobuline et de l’IL-1RA (antagoniste des récepteurs de l’interleukine 1) dans des membranes amniotiques cryoconservées à 1 et 18 mois ; et, comme objectif secondaire, de détecter la présence de l’ARNm correspondant au KGF, à l’IL-1Ra, à l’Alpha2-macroglobuline, au Fas Ligand, au TGF (transforming growth factor beta) et au Lumican par RT-PCR dans des membranes conservées à 1 et 18 mois.Quatre échantillons de membrane amniotique ont été répartis en 2 groupes: le premier groupe (n = 2) cryoconservé pendant 1 mois et le deuxième groupe (n = 2) cryoconservé pendant 18 mois à fin d’être étudiés par RT-PCR et ELISA.La RT-PCR a détecté la présence du KGF, de l’IL-1Ra, de l’Alpha2-macroglobuline, du Fas Ligand et du Lumican. Parmi ceux-ci, l’ARNm du Fas Ligand était positif dans les deux groupes de membranes amniotiques. L’ ARNm de KGF, du Lumican et de l’Alpha2-microglobuline étaient présents uniquement dans le premier groupe, et l’ARNm de l’IL-1Ra était absent dans les deux groupes. La RT-PCR du TGF-bêta n’était pas concluante. L’ELISA a été réalisée pour la détection et la quantification des 6 protéines (EGF, KGF, IL10, HGF, Alpha2-macroglobuline et IL-1Ra) dans les 2 groupes de membranes amniotiques. Les 6 protéines ont été trouvées dans tous les échantillons avec une concentration plus faible dans le groupe 2 par rapport au groupe 1.Cette étude a montré que les membranes amniotiques cryoconservées dans 50 % de glycérol pendant 18 mois conservaient les protéines nécessaires à la régénération de la surface cornéenne tout en préservant leurs propriétés biochimiques.

La cystinose est une maladie autosomique récessive rare liée à une mutation du gène CTNS, codant pour la cystinosine (transporteur lysosomal de cystine). Cette affection s’accompagne d’une accumulation de cystine intra-lysosomale au niveau de différents organes dont les reins. Trois formes sont décrites (infantile, juvénile et adulte). La forme la plus sévère est la forme infantile, survenant dans 95 % des cas (1/100 000 à 1/200 000 naissances). La forme juvénile, plus rare, peut toucher l’adolescent ou l’adulte jeune. La forme adulte (oculaire) est considérée comme bénigne 1, 2, 3, 4. M. A.J., âgé de 25 ans, sans antécédent personnel médical notable, est admis aux urgences le 28 février 2018 pour asthénie, pâleur cutanée et prurit évoluant depuis 3 mois. Le bilan biologique met en évidence une insuffisance rénale sévère (urée : 290 mg/dL et créatinine : 15,8 mg/dL), associée à une anémie normocytaire marquée. On note une protéinurie à 2,4 g/24 h, sans hématurie ni leucocyturie. Les reins sont de petite taille à l’échographie, signant l’insuffisance rénale terminale (IRT). Des séances d’hémodialyse itérative sont d’emblée instaurées. La biopsie rénale montre une glomérulosclérose avancée et une fibrose interstitielle avec un infiltrat lymphoplasmocytaire. Une étude en microscopie électronique est en cours. Une anamnèse familiale retrouve alors une notion de cystinose chez un cousin et une consanguinité est détectée (arrières grands-pères cousins). Une mise au point complémentaire est demandée : le dosage de cystine intra-leucocytaire est élevé (1,75 nmol de cystéine par mg de protéines [N < 0,4]) et l’examen ophtalmologique met en évidence des dépôts bilatéraux de cristaux cornéens. Malgré une biopsie rénale peu informative, la cause de l’IRT de notre patient est le plus probablement une cystinose juvénile. Une recherche de la mutation du gène CTNS est en cours. Ce cas souligne l’importance d’une anamnèse familiale approfondie chez un adulte jeune présentant une IRT inexpliquée par un bilan de première intention. Un dépistage de la fratrie nous paraît justifié. Un traitement par cystéamine est envisagé afin de prévenir les complications extrarénales. La perspective d’une transplantation rénale est retenue, étant donné l’absence de récidive sur le greffon.

Purpose. - To evaluate long-term results of low-fluence photodynamic therapy (PDT) with verteporfin in the treatment of chronic central serous chorioretinopathy (CCSC).Methods. - Retrospective medical record review of 38 eyes (34 patients) who received low-fluence PDT for the treatment of CCSC. Visual acuity (VA), fundus biomicroscopy, fluorescein angiography (FA), indocyanine green angiography (ICG) and optical coherence tomography (OCT) were analyzed.Results. - Thirty-eight eyes (34 patients) with CCSC received low-fluence PDT. Mean follow-up after PDT was 43.97 months. Mean logMar best corrected VA (BCVA) improved significantly from 0.33 to 0.11 at the last follow-up which corresponds to a gain of 2.2 lines. At 3 months, complete resolution of central subretinal fluid was achieved on OCT after 1 PDT in 37 eyes and after 2 PDTs in 1 eye (retreated at 3 months after first PDT). One patient developed choroidal neovascularization (CNV) 4 years after his low-fluence PDT and received anti-vascular endothelial growth factor (VEGF) injections.Conclusion. - Low-fluence PDT with verteporfin for CCSC seems efficacious and safe in the long-term. (C) 2015 Elsevier Masson SAS. All rights reserved.

Age-related macular degeneration (AMD) is the leading cause of irreversible blindness in industrialized countries in individuals over 65 years of age. It is characterized by a progressive degenerative disorder of the macula, resulting in a loss of the central vision. There are two forms of AMD, the atrophic and the exudative form. A number of risk factors have been implicated in the onset of this condition. With the ageing of the population, the prevalence of AMD is steadily raising and is leading to a growing social and economical burden. Much research has been conducted to improve the diagnosis and management of people at risk of AMD and to develop new treatments. In this review we will discuss the risk factors associated with AMD, the clinical forms and their diagnosis, as well as the current and future therapeutic options.

Age-related macular degeneration (AMD) is the leading cause of irreversible blindness in industrialized countries in individuals over 65 years of age. It is characterized by a progressive degenerative disorder of the macula, resulting in a loss of the central vision. There are two forms of AMD, the atrophic and the exudative form. A number of risk factors have been implicated in the onset of this condition. With the ageing of the population, the prevalence of AMD is steadily raising and is leading to a growing social and economical burden. Much research has been conducted to improve the diagnosis and management of people at risk of AMD and to develop new treatments. In this review we will discuss the risk factors associated with AMD, the clinical forms and their diagnosis, as well as the current and future therapeutic options.

PURPOSE:To document for the first time intrinsic retinal pigment epithelium (RPE) fluorescence in occult macular dystrophy (OMD). This entity is characterized by a central cone dysfunction leading to a decline of visual acuity without visible fundus and fluorescein angiography abnormalities. A great variability in clinical findings and in the pattern of inheritance have been reported suggesting probably several etiologies of which some are well known but seen too early to detect significant changes.METHODS:Fundus autofluorescence imaging is a recent method to detect early retinal pigment epithelial alterations. It may visualise disease specific abnormalities in the retinal pigment epithelium often not yet visible on ophthalmoscopy such as Stargardt disease, rod-cone dystrophy. This method was applied in a member of a family with OMD.RESULTS:The normal fundus autofluorescence observed in our patient allowed the distinction between well-known maculopathies not yet visible on ophthalmoscopy but showing abnormal autofluorescence, and genuine occult macular dystrophy.CONCLUSION:Fundus autofluorescence imaging in our case of dominant autosomal OMD suggests a healthy and functional RPE. This examination of RPE should therefore be added to the work-up of suspected OMD.

Central serous chorioretinopathy (CSC) is a relatively frequent ocular disorder. Its pathophysiology remains however unclear. This disease typically affects young men with type A behaviour within a context of stressful events. Recently, endogenous or exogenous hypercortisolism has been associated with development, prolongation and exacerbation of CSC. Exogenous hypercortisolism has been induced by any route: intravenous, cutaneous, or nasal spray. Some clinical features such as bilaterality of lesions, an atypical form of the presentation can evoke this association. Although a few years ago glucocorticoids were still used as treatment of CSC, experimental and clinical observations suggest that they are contra-indicated in the context of CSC.

Crystalline retinopathy is characterized by intraretinal crystalline deposits that, according to their etiology, can be localized in the macular area or, indeed, be found in the entire retina. These deposits can be associated or not to visual loss and electrophysiological perturbations. Among the toxic drugs leading to this retinopathy are tamoxifen, canthaxanthine, methoxyflurane, talc and nitrofurantoin. A detailed description of tamoxifen and canthaxanthine toxicity is reported in this chapter.

Purpose: Diffuse unilateral subacute neuroretinitis (DUSN) is well known in endemic areas of the southeastern United States, South America, and the northern Midwestern United States. Two different categories of nematodes, according to their length, are related to endemic areas. We report the first case of DUSN caused by a small nematode in Africa. Methods: We describe the case of a 12-year-old Senegalese girl who presented a long-standing diffuse unilateral subacute neuroretinitis and in whom the worm could be localized. Results: The length of the unidentified worm measured using the software of the fundus camera was approximately 600 mu m. This measurement corresponds to the smaller nematode usually found in patients from the southeastern United States and South America. Conclusion: Diffuse unilateral subacute neuroretinitis can also be observed in patients living in Africa.

Inherited retinal venous beading is a rare autosomal dominant disorder with variable expressivity. It is characterized by irregular, segmented beading of the retinal veins. Some patients have arteriolar tortuosity. The conjunctiva can be affected by saccular vascular changes. Episodes of acute retinal vascular decompensation are also described. Low to normal leucocyte and neutrophil counts may be an extraocular associated condition. We describe the isolated case of a woman presenting retinal venous beading and conjunctival vascular aneurysms. She has no systemic anomaly and her family members examined are unaffected.

PURPOSE:Cytomegalovirus (CMV) retinitis is the most common ocular opportunistic infection associated with AIDS. It usually affects the peripheral retina, sparing the macula. We describe an atypical CMV retinitis exclusively confined to the macula.METHODS:A 43-year-old man with the diagnosis of AIDS developed a white retinal lesion confined to the macula of the right eye. Two weeks later, a more typical granular appearance was observed leading to presumption of CMV retinitis.RESULTS:The patient was treated with ganciclovir without success. With foscarnet, a good response was obtained, leading to total healing of the lesion.CONCLUSIONS:CMV retinitis has to be taken into consideration in all lesions confined to the macula in immunodepressed patients. An early diagnosis is crucial to avoid blindness.

BACKGROUND:To report the long term follow-up of a case of Birdshot Chorioretinopathy treated with steroids and cyclosporine during three years and followed for twenty years.METHODS:The patient was monitored with Snellen visual acuity, slit lamp examination, perimetry, colour vision test, fluorescein angiography, electroretinogram (ERG) and electrooculogram (EOG).RESULTS:The retinal alterations progressed despite minimization of the intraocular inflammation. Vision dropped, perimetric and severe colour vision alterations appeared and the patient complained of nyctalopia.CONCLUSIONS:The retinal findings in our case treated during three years resemble twenty years later tapetoretinal dystrophy and evoke the natural evolution of Birdshot Chorioretinopathy. The therapeutic approach was either inadequate due to early interruption or to inefficacy on the mechanisms of the retinal alterations.

Diabetic retinopathy remains today a leading cause of blindness. Dramatic progress during the past three decades led to the classification of diabetic retinopathy, to provide guidelines of screening and follow-up, to determine risk factors of progression of retinopathy and to propose treatment strategies. This paper reviews clinical and therapeutical knowledge about this topic.

The main clinical activities developed during the first 25 year of the department are described. Topics of fundamental and also clinical research are briefly reviewed.

The vitreomacular relationship has been demonstrated to play a role in the physiopathology of the diabetic macular edema. We review the functional and anatomic results of vitrectomy performed on four eyes of three patients with diabetic cystoid macular edema unresolved by photocoagulation. The preoperative visual acuity was "finger counting" at one meter to 1/20. After surgery, for three eyes, the visual acuity raised between 2/10 and 4/10. There was no change for one eye, which presented a subretinal fibrosis. The macular edema resolved in three eyes and improved in one eye. The mean postoperative follow-up is 13 months. The vitrectomy may be effective in cases of diabetic macular traction and edema without posterior vitreous detachment.

Although the macular hole surgery with gas tamponade obtains good results, the face-down positioning for minimum 5 days remains constraining and is often a cause of refusal or abstention concerning the operation. We describe 7 patients who underwent the macular hole surgery by pars plana vitrectomy with internal limiting membrane peeling and fluid-silicone exchange without position restrictions. The silicone oil has been removed 2 to 3 months later. All the holes have been closed except in one case where the internal limiting membrane had not been peeled with certainty. A second surgery with peeling of this membrane has permitted the hole closure. We don't report any complications induced by the silicone oil. Silicone oil tamponade for macular hole surgery with internal limiting membrane peeling shows a good efficiency without particular positioning and without additional complications.

Although the macular hole surgery with gas tamponade obtains good results, the face-down positioning for minimum 5 days remains constraining and is often a cause of refusal or abstention concerning the operation. We describe 7 patients who underwent the macular hole surgery by pars plana vitrectomy with internal limiting membrane peeling and fluid-silicone exchange without position restrictions. The silicone oil has been removed 2 to 3 months later. All the holes have been closed except in one case where the internal limiting membrane had not been peeled with certainty. A second surgery with peeling of this membrane has permitted the hole closure. We don't report any complications induced by the silicone oil. Silicone oil tamponade for macular hole surgery with internal limiting membrane peeling shows a good efficiency without particular positioning and without additional complications.

We report the case of a 21 year old man who has severe headache and blurred vision since 2 weeks. Ophthalmologic examination discloses typical lesions of acute posterior multifocal placoid pigment epitheliopathy and an homonymous right inferior quadrantanopsia. An inflammatory syndrome and a cerebrospinal fluid lymphocytosis are found. Cerebral imagery is normal. Headache improves only with corticotherapy. We conclude that the neurological attack associated with this acute posterior multifocal placoid pigment epitheliopathy is most likely due to a cerebral vasculitis.

PURPOSE:To describe a case of herpes simplex virus primary infection after a corneal transplantation and to analyse different possible ways by which the infection could have been transmitted.METHODS:A corneal transplanted patient for a Fuchs dystrophy complains 10 days postoperatively of a flu-like syndrome with keratouveitis. Donor and recipient viral serologies and a polymerase chain reaction analysis of aqueous humor were performed.RESULTS:An herpetic seroconversion is observed with presence of type I herpes simplex virus (HSV I) nucleic acids in the recipient's aqueous humor. The donor herpetic serology is negative.CONCLUSION:In this patient, the most probable routes of transmission of the virus are a viral reactivation from the trigeminal ganglion of the recipient, the presence of the virus in the corneal transplant or an external contamination.

An algerian woman presents with a panuveitis of the right eye. Main features of this uveitis are exudative retinal detachments. That happened several months after a corneal ulcer of unknown aetiology on the left eye. Immunologic, neurologic and infections workup associated with previous ocular injury points to the diagnosis of sympathetic ophthalmia. However, the diagnosis of Harada disease can also be considered in this case because of similarities regarding the clinical and the histologic aspects which are exposed. If corticosteroids are the drug of choice, enucleation is still indicated for sympathetic ophthalmia.