[Bilateral diffuse uveal melanocytic proliferation : a rare paraneoplasic syndrome to be diagnosed early]. / Cas clinique. Prolifération mélanocytique uvéale bilatérale diffuse : un syndrome paranéoplasique rare à reconnaître précocement.

Diffuse bilateral uveal melanocytic proliferation is a rare paraneoplastic ophthalmologic syndrome. The increase in life expectancy in oncology partly explains the gradual increase in its incidence. In almost half of the cases, the syndrome manifests itself before the diagnosis of primary neoplasia. It should be suspected in cases of bilateral uveal pigmented lesions that do not meet the clinical criteria for other known eye pathologies. Legal blindness occurs for the majority of patients during the first year after the initial clinical presentation. Death, due to the severity of the underlying pathology, occurs statistically within three years. Here we display the case of such a diffuse bilateral uveal melanocytic proliferation in a patient with relapsed colonic adenocarcinoma, initially treated for age-related macular degeneration.

La prolifération mélanocytique uvéale bilatérale diffuse est un syndrome paranéoplasique ophtalmologique rare. L'augmentation de l'espérance de vie en oncologie explique, en partie, la majoration progressive de son incidence. Dans près de la moitié des cas, le syndrome se manifeste antérieurement au diagnostic de la néoplasie primaire. Il doit être suspecté en cas de lésions pigmentées uvéales bilatérales ne répondant pas aux critères cliniques d'autres pathologies oculaires connues. La cécité légale s'établit chez la majorité des patients durant la première année suivant la présentation clinique initiale. Un décès, dû à la sévérité de la pathologie sous-jacente, survient presque toujours dans les trois ans. Nous rapportons ici le cas d'une telle prolifération mélanocytique uvéale bilatérale diffuse, chez une patiente atteinte d'un adénocarcinome colique en rechute, traitée initialement pour une dégénérescence maculaire liée à l'âge.

[What's new in diabetic retinopathy ?] / Actualités dans la rétinopathie diabétique.

Diabetic retinopathy is a public health issue and the leading cause of blindness in adults in industrialized countries. Many advances have been made during the last few years in the diagnosis, the understanding of the pathophysiology and the treatment of diabetic retinopathy, especially with the rise of anti-angiogenic treatments. We bring here an overview of the last advances, the current therapeutic algorithms and future perspectives.

La rétinopathie diabétique est un problème de santé publique et est la première cause mondiale de cécité dans les pays industrialisés. Les dernières années ont été marquées par des progrès dans le diagnostic, la compréhension de la physiopathologie et le traitement de la rétinopathie diabétique, notamment avec l'introduction des traitements visant à réduire l'angiogenèse rétinienne. Cet article passe en revue les dernières avancées dans ces domaines, l'algorithme thérapeutique appliqué actuellement et les perspectives futures.

[Post-traumaticaniridia long after cataract surgery]. / Cas clinique. Aniridie post-traumatique à distance d'une opération de cataracte.

Post-traumatic aniridia is an uncommon complication of blunt ocular trauma. When it is completed, it represents a 360-degree tear from the root of the iris to its insertion into the ciliary body. Since the introduction of the use of corneal micro-incisions in cataract surgery, some cases of complete tear of iris root have been observed in patients who suffered from violent trauma. We report the case of a patient who has a complete aniridia after a fall, 15 years after cataract surgery.

L'aniridie post-traumatique est une complication peu fréquente d'un traumatisme oculaire à globe fermé. Lorsqu'elle est complète, il s'agit d'une déchirure sur 360° de la racine de l'iris à son insertion au corps ciliaire. Depuis l'avènement de l'utilisation de micro-incisions cornéennes en chirurgie de cataracte, la déchirure complète de la racine de l'iris a été observée sur plusieurs patients ayant subi un traumatisme violent. Nous rapportons le cas d'un patient, opéré de cataracte 15 ans auparavant, qui présente une aniridie complète suite à une chute.

[ETHICAL ASPECTS OF INFORMED CONSENT IN REFRACTIVE SURGERY]. / ASPECTS ÉTHIQUES DU CONSENTEMENT ECLAIRÉ EN CHIRURGIE RÉFRACTIVE.

With the introduction of the use of Laser assisted surgery, refractive eye surgery knows a very large success. Surgery of well being, it requires that an extensive information is delivered to the patient concerning the benefit and possible side-effects of the available treatments. This information process may reduce the frequency of negligence claims relating to Laser eye surgery.

[Recent advances in the treatment of presbyopia]. / Les nouvelles possibilités de traitement de la presbytie.

Everyone will experience presbyopia between the age of 45 and 50 years. The classical solution to help these patients is to offer spectacles or multifocal contact lenses. Recent developments in corneal or intraocular surgery may provide new solutions for selected patients to prevent them from always wearing glasses to read.

[Updates in corneal transplantation]. / Nouvelles approches des greffes de cornée.

Corneal transplantation or keratoplasty has rapidly developed over the last 10 years. Penetrating keratoplasty, a well-known operation consisting of full thickness replacement of the cornea, has remained the dominant procedure for a long time. It allows appropriate therapy of most causes of corneal blindness. However, this technique is currently evolving toward slamellar keratoplasties which selectively treat the specific affected layers: deep anterior lamellar keratoplasty replaces the diseased corneal stromal layers; endothelial keratoplasty replace the affected endothelium. This article will present these techniques, and briefly discuss their advantages.

[Clinical case of the month: non-familial vitreous amyloidosis]. / Amylose vitréenne non familiale.

Vitreous amyloidosis is characterized by the presence of amyloid deposition in the vitreous cavity. It is frequently associated with dominantly inherited familial amyloidosis of the transthyretin mutation. The nonfamilial form is rare, and only a dozen of cases have been reported in the ophthalmological literature.

ADAMTS-2 functions as anti-angiogenic and anti-tumoral molecule independently of its catalytic activity.

ADAMTS-2 is a metalloproteinase that plays a key role in the processing of fibrillar procollagen precursors into mature collagen molecules by excising the amino-propeptide. We demonstrate that recombinant ADAMTS-2 is also able to reduce proliferation of endothelial cells, and to induce their retraction and detachment from the substrate resulting in apoptosis. Dephosphorylation of Erk1/2 and MLC largely precedes the ADAMTS-2 induced morphological alterations. In 3-D culture models, ADAMTS-2 strongly reduced branching of capillary-like structures formed by endothelial cells and their long-term maintenance and inhibited vessels formation in embryoid bodies (EB). Growth and vascularization of tumors formed in nude mice by HEK 293-EBNA cells expressing ADAMTS-2 were drastically reduced. A similar anti-tumoral activity was observed when using cells expressing recombinant deleted forms of ADAMTS-2, including catalytically inactive enzyme. Nucleolin, a nuclear protein also found to be associated with the cell membrane, was identified as a potential receptor mediating the antiangiogenic properties of ADAMTS-2.

Retinitis pigmentosa and bronchiectasis: a case report on a rare association suggestive of a common underlying primary ciliary dyskinesia (PCD).

We describe a case of retinitis pigmentosa, associated with bronchiectasis, as the first sign of primary ciliary dyskinesia (PCD). Only a few cases were described in the literature and the association of both diseases is not obvious at first sight, although a common ciliary dysfunction of both respiratory epithelium and photoreceptors of the retina seems to be the common factor. It is important to recognize the association and to question patients with retinitis pigmentosa about their respiratory functions, because an early diagnosis of PCD can prevent recurrent infections and development of bronchiectasis with daily physiotherapy.

[Clinical case of the month. A case of von Hippel-Lindau disease]. / Le cas clinique du mois. A propos d'un cas de maladie de von Hippel-Lindau.

von Hippel-Lindau disease is an inherited multisystemic familial cancer syndrome caused by mutations of the VHL gene. The spectrum of clinical manifestations is broad and includes central nervous system hemangioblastomas and visual benign and malignant tumors. The various manifestations can be demonstrated by means of different imaging techniques such as magnetic resonance imaging, computed tomography, and fluorescein retinal hemangiography. A systematic approach must be followed for repeated screening in patients at risk, since many lesions in VHL disease are treatable.

[VEGF inhibitors in ophthalmology]. / Utilisation des anticorps anti-VEGF en ophtalmologie.

The exsudative form of age-related macular degeneration (AMD) is characterized by the development of choroidal neovascularization (CNV) under the retina driven in part by the vascular endothelial growth factor. Several VEGF inhibitors have recently been produced for ocular administration. This review will focus on ranibizumab which is a specific antibody neutralizing all VEGF isoforms. The development, pharmacokinetics, clinical efficacy, current and future indications of ranibizumab are discussed.

[Bilateral optic atrophy in a drug addict]. / Atrophie papillaire bilatérale chez un patient héroïnomane.

Bilateral optic neuropathy in a young man is suggestive of hereditary or toxic damage. We describe the case of a 38-year-old man with bilateral optic neuropathy. His best corrected visual acuity was light perception OD and 20/125 OS. The patient's history revealed an addiction to heroin. Three drugs - heroin, quinine, and cocaine - were considered as a possible cause, which is discussed further. We suggest that the condition reported here may be attributed to the use of heroin. This underlines the importance of meticulous history taking in diagnosing a young man with bilateral loss of vision.

[Overview of a decade of advances in ophtalmology]. / Resume d'une décennie de progrès ophtalmologiques.

This review summarizes the most important advances that occurred in ophthalmology during the last decade, with a focus on corneal pathology, cataract surgery, glaucoma and retinal diseases.

[Eye and ultraviolet light]. / Oeil et lumière ultraviolette.

All the ocular structures, from cornea to retinal layer, can be damaged by exposure to the light radiations. Acute keratitis combined with conjunctivitis will result from an intensive photic exposure. On the other hand, pterygion more likely represents the consequence of chronic exposure to the ultraviolet rays. Exposure to the ultraviolet rays has been involved in the appearance of the typical yellow pigments of the nuclear cataract. Indeed, the physiopathological mechanisms are now progressively better understood. The progression of retinal degenerations might be influenced by uncontrolled exposure to ultraviolet rays, particularly during childhood. It is therefore useful to protect the eyes against ultraviolet sunrays. Filtering glasses seem to be one of the most effective ways to reach that objective.

Role of plasminogen activator-plasmin system in tumor angiogenesis.

New blood formation or angiogenesis has become a key target in therapeutic strategies aimed at inhibiting tumor growth and other diseases associated with neovascularization. Angiogenesis is associated with important extracellular remodeling involving different proteolytic systems among which the plasminogen system plays an essential role. It belongs to the large serine proteinase family and can act directly or indirectly by activating matrix metalloproteinases or by liberating growth factors and cytokines sequestered within the extracellular matrix. Migration of endothelial cells is associated with significant upregulation of proteolysis and, conversely, immunoneutralization or chemical inhibition of the system reduces angiogenesis in vitro. On the other hand, genetically altered mice developed normally without overt vascular anomalies indicating the possibility of compensation by other proteases in vivo. Nevertheless, they have in some experimental settings revealed unanticipated roles for previously characterized proteinases or their inhibitors. In this review, the complex mechanisms of action of the serine proteases in pathological angiogenesis are summarized alongside possible therapeutic applications.

Presence of oestrogen receptor type beta in human retina.

BACKGROUND/AIMS: Recent studies have demonstrated the existence of two oestrogen receptor subtypes alpha (ORalpha) and beta (ORbeta) with significant differences of expression among organs. Since important pathologies of human eye could be linked to hormonal status, the expression of ORbeta in ocular posterior segment was sought. METHODS: Immunohistochemical localisation of ORbeta and ORalpha protein and detection of OR mRNAs by reverse transcription-polymerase chain reaction (RT-PCR) were performed in macular and extramacular regions of the retina and in the choroid on male and female donors eyes. RESULTS: ORbeta protein was localised in the ganglion cell layer and in the choroid. At the transcriptional level, mRNA for ORbeta and for ORalpha were both present. Local differences in the expression level were observed, however, suggesting the possibility of variation in the ratio of ORalpha v ORbeta. CONCLUSIONS: The coexistence of two oestrogen receptor subtypes in the human ocular posterior segment raises acute questions about their potential physiological role, but offers a perspective for preferential targeting of a specific receptor subtype.

Influence of plasminogen activator inhibitor type 1 on choroidal neovascularization.

High levels of the plasminogen activators, but also their inhibitor, plasminogen activator inhibitor 1 (PAI-1), have been documented in neovascularization of severe ocular pathologies such as diabetic retinopathy or age-related macular degeneration (AMD). AMD is the primary cause of irreversible photoreceptors loss, and current therapies are limited. PAI-1 has recently been shown to be essential for tumoral angiogenesis. We report here that deficient PAI-1 expression in mice prevented the development of subretinal choroidal angiogenesis induced by laser photocoagulation. When systemic and local PAI-1 expression was achieved by intravenous injection of a replication-defective adenoviral vector expressing human PAI-1 cDNA, the wild-type pattern of choroidal angiogenesis was restored. These observations demonstrate the proangiogenic activity of PAI-1 not only in tumoral models, but also in choroidal experimental neovascularization sharing similarities with human AMD. They identify therefore PAI-1 as a potential target for therapeutic ocular anti-angiogenic strategies.

Lens epithelial cell proliferation in human posterior capsule opacification specimens.

In previous in vitro studies on capsular bags it was shown that, after a sham extracapsular cataract extraction (ECCE) on human donor eyes, lens epithelial cells (LECs) show, in the short term, a dramatically elevated mitotic activity as compared to that in the intact lens. The long term in vivo proliferation of LECs in human lenses after ECCE and intraocular lens (IOL) implantation has not been studied until now. In the present study, the mitotic activity of LECs in human post-mortem eyes with posterior capsule opacification (PCO) was investigated. Human lenses with signs of PCO were dissected from donor eyes and incubated in MEM, supplemented with fetal calf serum, for 1 day (n = 10) or 7 days (n = 9). Six additional specimens were cultured for 7 days after removal of the IOL and lens fibres. After the incubation period, mitotic activity was estimated using the BrdU procedure and the Ki67 proliferating cell marker. The mean number of BrdU-positive nuclei in the intact PCO specimens was at a level of 7.5 (day 1) and 6.5 (day 7). Removal of the IOL and the lens fibres leads to a ten-fold increase in BrdU positive cells (mean = 84.5). No correlation with donor age was found. The Ki67 observations corroborate the BrdU results. The results demonstrate that after an initial rise in proliferative activity, as shown in the capsular bag model, the mitotic activity of LECs returns to a rate comparable to that in intact cultured non-cataractous lenses. As in control lenses, removal of lens fibres significantly elevated the proliferative activity of the remaining LECs. Suppression by newly formed differentiated lens fibres in the in vivo capsular bag may be responsible for this return to control levels of mitotic activity of LECs in the PCO specimens.

Lens cell populations studied in human donor capsular bags with implanted intraocular lenses.

PURPOSE: Posterior capsule opacification is an ongoing cellular redistribution process. The level of viable cell coverage was therefore determined in human donor capsular bags with implanted intraocular lenses, and cellular morphology and ultrastructure were investigated in relation to cell type and level of differentiation. METHODS: Donor capsular bags, retrieved at intervals of 4 months to 13 years after surgery, were investigated by phase optics before fixation. Postfixation techniques included scanning electron microscopy and transmission electron microscopy of sections and immunofluorescent staining of cytoskeletal proteins in wholemounts. RESULTS: All the capsular bags contained a large population of viable cells on the capsular surfaces. Cells on the anterior face of the anterior capsule and in the spaces around the intraocular lens had an elongated morphology and expressed alpha-smooth muscle actin. The cells formed light-scattering, multilayered aggregates and strands that were surrounded by layers of extracellular matrix. The regions between the intraocular lens and the equator of the bags were populated by monolayers of epithelial cells of normal morphology and ultrastructure, on both the anterior and posterior capsules. In some regions the apical surfaces of the two epithelial monolayers were in contact, and in some parts of the equatorial regions, differentiation of cells into well-organized fiberlike cells was evident. CONCLUSIONS: Human capsular bags contain a large population of viable cells for many years after cataract surgery. Cells in the regions around the intraocular lens undergo transition to a mesenchymal type. Cells peripheral to these regions can form a stable closed microenvironment in which both normal epithelial morphology and differentiation to fiberlike cells are maintained.