[Tear your eyes out ! A case of bilateral auto-enucleation]. / «Arrache-toi les yeux !¼ À propos d'un cas d'auto-énucléation bilatérale.

Bilateral autoenucleation is an extremely rare form of ocular self-mutilation. This gesture usually occurs in psychotic patients. In a moment of madness, a 28-year-old man brutally tore out both of his eyes. He was in acute relapse of schizophrenia after having interrupted all neuroleptic treatment for 6 months. Four days after admission, surgical enucleation was the only possible outcome. Facing the complexity of this clinical case, the ophthalmologist has a central role in the organization of long-term surgical, neurological and psychiatric care.

L'auto-énucléation bilatérale est une forme d'automutilation oculaire rarissime. Ce geste est observé presque toujours chez des patients psychotiques. Dans un moment de folie, un homme de 28 ans, s'est brutalement arraché les deux yeux. Il se trouvait en rechute aiguë de schizophrénie après avoir interrompu tout traitement neuroleptique depuis 6 mois. Quatre jours après son admission, l'énucléation chirurgicale fut la seule issue possible. Face à la complexité de ce cas clinique, l'ophtalmologue aura un rôle central dans l'organisation des soins chirurgicaux, neurologiques et psychiatriques au long cours.

[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.

[Unilateral pigmentary retinopathy : about 2 cases]. / Le cas clinique du mois. Rétinopathie pigmentaire unilatérale.

We present the case of two patients in whom unilateral retinal involvement with pigmentary lesions on the fundus examination was observed. Apart from the unilateral nature of the lesions, a diagnosis of retinitis pigmentosa could have been made in view of the morphological and functional aspects of the retina. However, in these two clinical cases, an association between retinal lesions and Bartonella in one case, and pre-existing multiple sclerosis in the other case, has been proposed with a final diagnosis of unilateral pigmentary retinopathy. Nevertheless, a sufficiently long period of patient follow-up is necessary to rule out delayed onset in the unaffected eye, suggesting an asymmetrical bilateral retinitis pigmentosa.

Nous présentons le cas de deux patientes chez qui une atteinte rétinienne unilatérale comportant des lésions pigmentaires à l'examen du fond de l'œil a été observée. Malgré le caractère unilatéral des lésions, un diagnostic de rétinite pigmentaire d'origine génétique aurait pu être évoqué au vu des aspects morphologiques et fonctionnels de la rétine. Cependant, une origine infectieuse (infection à Bartonella) a pu être proposée pour un cas, alors que, pour l'autre cas, une atteinte inflammatoire secondaire à une sclérose en plaques a été retenue. Pour cette raison, le diagnostic final est celui d'une rétinopathie pigmentaire unilatérale. Néanmoins, un suivi à long terme est obligatoire de façon à écarter une apparition retardée des lésions dans l'œil indemne, ce qui pourrait indiquer la présence d'une rétinite pigmentaire bilatérale asymétrique.

[Painful Horner's syndrome revealing carotid artery dissection : about four cases]. / Claude Bernard-Horner douloureux, de la clinique au diagnostic de dissection carotidienne : à propos de quatre cas décrits par l'ophtalmologue.

We report four cases of painful Horner's syndrome due to carotid artery dissection. A lesion on oculosympathic pathway causes Horner's syndrome. A painful Horner's syndrome needs a prompt exploration by tomodensitometry or magnetic resonance imaging to exclude carotid artery dissection. Actually, carotid artery dissection leads to stroke or cerebral ischemic symptoms, mainly in the first two weeks. The treatment is based on anticoagulants or antiplatelet agents.

Nous rapportons quatre cas de dissections carotidiennes suspectées par la présence d'un syndrome de Claude Bernard-Horner douloureux. Ce syndrome est dû à une lésion au niveau de la voie oculosympathique. Lorsqu'il est accompagné de douleurs, une exploration par tomodensitométrie avec injection de produit de contraste, ou mieux, par résonance magnétique, est nécessaire afin d'exclure une dissection carotidienne. Etant donné la possible apparition d'un accident vasculaire ischémique endéans les deux premières semaines, un diagnostic précoce ainsi qu'un traitement par antiagrégant plaquettaire ou par anticoagulant sont primordiaux.

[Ocular vascular pathologies : keep an eye open!] / Les pathologies vasculaires oculaires : ouvrez l'œil !

Vascular ocular pathologies are common and usually diagnosed in the emergency department by the ophtalmologist. However, it is very important for the physicians and the general practitioners to know these different diseases for improving the visual and vital prognosis. This paper describes the most important ocular and cerebrovascular pathologies.

Les pathologies vasculaires oculaires sont fréquentes et leur diagnostic est souvent posé aux urgences ophtalmologiques. Néanmoins, pour les médecins traitants et les médecins spécialistes, il est important de les reconnaitre afin d'améliorer le pronostic visuel et vital du patient. Cet article décrit les pathologies vasculaires oculaires et cérébrovasculaires les plus importantes.

[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.

[Posterior scleritis]. / La sclérite postérieure : cas clinique.

Posterior scleritis, a severe and painful inflammation of the sclera, is an often misdiagnosed pathology due to its clinical polymorphism. An accurate diagnosis is however needed in order to propose an appropriate treatment of the ophthalmologic symptoms and to exclude an associated systemic inflammatory or auto-immune pathology.

La sclérite postérieure est une inflammation sévère et douloureuse localisée au niveau de la sclère. Cette pathologie méconnue est souvent sous-diagnostiquée en raison de son polymorphisme clinique. Il est néanmoins important de la reconnaître de façon à proposer un traitement adéquat des symptômes oculaires et de rechercher les différentes pathologies systémiques inflammatoires ou auto-immunes pouvant lui être associées.

[Diagnostic approach of vision loss occurring in a white eye]. / La vignette diagnostique de l'étudiant. Evaluation d'une baisse de vision avec un œil blanc.

Vision loss is a major symptom in ophthalmology. We report a clinical observation of acute unilateral non ischemic optic neuropathy in a 55 year old patient to illustrate the diagnostic approach of vision loss occurring in a white eye. Several algorithms are proposed to facilitate the diagnostic approach.

Résumé : La perte d'acuité visuelle est un symptôme majeur en ophtalmologie. Nous rapportons le cas d'une neuropathie optique aiguë unilatérale non ischémique survenant chez une patiente de 55 ans. Ce cas clinique permet d'illustrer la démarche diagnostique devant une chute d'acuité visuelle survenant avec un œil blanc. Plusieurs algorithmes sont fournis pour faciliter l'approche diagnostique.

[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.

[How I treat...a red eye]. / Comment je traite...un oeil rouge.

Before any initiation of treatment for a red eye, an accurate differential diagnosis must absolutely be done, as considered in a previous article. Red eye can result from benign diseases, but also from serious pathologies which shouldn't be neglected, as they require a rapid ophtalmologist management. This is the case for keratitis, uveitis, acute glaucoma attack, or endophtalmitis. Treatment of red eye will just be symptomatic in benign diseases but, in contrast, will absolutely need an etiological diagnosis and therapeutic approach in more serious cases. Generally speaking, topical steroids should never be prescribed in doubt of infectious keratitis. Moreover, local anaesthetics, because of their toxicity for the cornea, should not be prescribed under any circumstances.

[How I explore ... differential diagnosis of a red eye]. / Comment j'explore ... le diagnostic différentiel d'un oeil rouge.

As a common reason for consultation, red eye can be a symptom of benign diseases, such as conjunctivitis, subconjunctival hemorrhage, dry eye syndrome,..., which can be managed by the general practitioner or internist. Conversely, more serious pathologies require a ophthalmologist management: these are keratitis, uveitis, acute glaucoma attack, or endophtalmitis. This article is intended to help practitioner to guide diagnosis when he's faced with a red eye.

[Clinical case of the month. Acute post-traumatic panophthalmitis]. / Panophtalmie post-traumatique fulgurante.

Panophtalmitis is an intra-ocular and intra-orbital rare infection, but its consequences are often horrendous, with loss of both function and anatomy of the eye. The surgical approach and the antibiotherapy should be given as soon as possible, both by intra-vitreal and intra-venous injections. We report the case of a 49-year-old man who developed a Bacillus cereus panophtalmitis. A delay in the diagnosis lead to the loss of vision and the evisceration of the sick eye. Bacillus cereus panopthalmitis should be suspected in all cases of post-traumatic endophtalmitis with a soiled metallic foreign body, in patients with important local inflammatory signs as well as systemic signs. Vitrectomy, surgical extraction of the foreign body, intra-vitreal and systemic antibiotherapies should be given as soon as possible, even before the results of the bacteriological analysis.

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.