Treatment of cystoid macular edema in homozygous twins with glutathione synthetase deficiency and retinal dystrophy.

Monozygotic twins with glutathione synthetase deficiency, progressive retinal dystrophy and cystoid macular edema were followed for foveal changes on optical coherence tomography under different treatment modalities. The purpose of the study is to show the effect of topical dorzolamide in conjunction with systemic acetazolamide in terms of decreasing macular edema in this specific disease. The results showed that systemic acetazolamide alone or in combination with topical dorzolamide decreased CME in both patients for a certain period of time. The result can be temporary sustained after treatment discontinuation. In conclusion, topical dorzolamide, in conjunction with systemic acetazolamide, could reduce cystoid macular edema in GSSD.

[Endovascular treatment of idiopathic intracranial hypertension. Analysis of eight consecutive patients]. / Traitement endovasculaire de l'hypertension intracrânienne bénigne étiquetée "idiopathique". Analyse de huit cas consécutifs.

INTRODUCTION: Optic nerve sheath fenestration or ventricular shunting are classically proposed after failure of medical treatment. Idiopathic intracranial hypertension is caused by venous sinus obstruction in an unknown percentage of cases. Recently, endoluminal venous sinus stenting was proposed as an alternative treatment. PATIENTS AND METHOD: Between September 2003 and December 2004, eight consecutive patients with a diagnosis of idiopathic intracranial hypertension underwent MRI venography and direct retrograde cerebral angiography. RESULTS: There were five females and three males aged 22 to 55 years. All patients had vision disorders. The cerebrospinal fluid pressure ranged from 27 to 45 mmHg with normal composition. All patients presented at least one sinus stenosis. Endovascular stenting of the stenotic venous sinus was performed under intravenous heparin administration. Anti-platelet therapy was administered for 3 months post treatment. Intra-sinus pressures were invariably reduced by stenting. Mean follow-up was 18 months. All patients improved clinically. The cerebrospinal fluid pressure had normalized at 3-month follow-up in all patients. In all patients, multidetector row CT-angiography or MRI venography was performed at 3-, 6- and 12-month follow-up and demonstrated the patency of the stent. DISCUSSION: The importance of venous sinus disease as a cause of idiopathic intracranial hypertension is probably underestimated. Patients with idiopathic intracranial hypertension should be evaluated with direct retrograde cerebral venography and manometry. In patients with venous sinus lesions, treatment by an endoluminal venous sinus stent is a safe and effective alternative for amenable lesions.

Metabolic factors and the foveal avascular zone of the retina in diabetes mellitus.

AIM: To study the foveal avascular zone (FAZ) of the central retina in diabetic patients with retinopathy having undergone metabolic evaluation. METHODS: One hundred and ten digital fluorescein angiograms were chosen from our digital image bank after cross matching diabetic patient lists of the ophthalmology and endocrinology departments of our institution. The patients had undergone day visits with systemic, biological and ophthalmologic evaluation, including digital fluorescein angiography. RESULTS: Sex ratio was M 62/F 48. Average age was 52.4 years (+/- 13.8) with 44 type 1 diabetics and 66 type 2. Retinopathy was present in all patients (54 background (BDR), 30 pre-proliferative (PPDR), 26 proliferative (PDR)). Age was positively correlated with FAZ grade (47.3 years +/- 13.2 for normal FAZ, 53.8 years +/- 13.7 for abnormal FAZ, P=0.03). Lipid profile showed a protective tendency of the Apo A1 fraction of cholesterol on macular vascularization (1.7 gr./l in normal FAZ patients vs 1.43 gr./l in abnormal FAZ patients, P=0.004). Body mass index was negatively correlated with macular ischemia (28.11 if FAZ not severely altered, 25.97 if FAZ severely altered, P=0.03). CONCLUSIONS: We found possible relations between BMI and Apo A 1 cholesterol and macular vascularization which may warrant further investigation.

[Quantitative and spatial analysis in image processing: study of drusen distribution from the foveal center in age-related macular degeneration]. / Méthode d'analyse quantitative et spatiale dans le traitement d'image: étude de la distribution radiale des drüsen dans la dégénérescence maculaire liée à l'âge.

Angiofluorograms were obtained from 58 patients presenting posterior pole drusen due to age-related macular degeneration. These frames underwent image processing by morphological mathematics algorithms. The detection sensitivity of this algorithm is 78%. We present a quantitative and spatial analysis of drusen distribution from the foveal center in the form of a graph. This technique may allow for multi-date comparisons, by using a graphic representation of this risk factor for age-related macular degeneration.

Caterpillar setae-induced acute anterior uveitis: a case report.

PURPOSE: To report uveitis secondary to ocular penetration of caterpillar hairs (setae). METHODS: Case report. A documented attack of acute anterior uveitis was caused by initially overlooked penetration of caterpillar setae. RESULTS: A 66-year-old man presenting with unilateral hypertensive keratouveitis was treated with antiherpes simplex medication (along with local anti-inflammatory and cycloplegic agents) after anterior chamber paracentesis and serologic testing. Laboratory testing was negative. Resolution occurred after 5 days, and corneal clearing showed a predescemetic caterpillar seta. CONCLUSION: Patient history taken in an anterior uveitis setting should include gardening habits and searching for possible exposure to insects or arachnids.

Mathematical morphology in computerized analysis of angiograms in age-related macular degeneration.

This paper deals with image processing of numerical retinal angiograms in order to facilitate diagnosis and follow-up in age-related macular degeneration (AMD) which is currently the main cause of blindness in industrialized countries. A computerized scheme using principally mathematical morphology operators is proposed for detecting and counting drusen, which are precursor lesions of the ocular fundus. In order to check the feasibility of this approach, results relative to 58 retinal images are compared with those given by three retinal specialists independently. From manual counting measures it is found that interobserver correlation coefficients lie in the range 0.71-0.78. On the other hand, a correlation coefficient of 0.89 is obtained when the average of the three expert countings is compared with the drusen number given by the computerized method. This coefficient is improved from 0.89 to 0.93 by processing only frames captured immediately after the appearance of a dye consecutive to intravenous sodium fluorescein injection. Compared to the manual analysis which is, among other things, tedious and time consuming, the computerized analysis is both quicker and more objective. Validation by the practitioner is however necessary, given possible detection errors. The proposed computerized scheme for detecting and counting drusen may be easily automated and so should prove useful in clinical studies which involve high volume analysis of retinal angiograms.

The short-wavelength mechanisms of Stiles in age-related macular degeneration.

Clinical measurements by the increment-threshold technique of W.S. Stiles are reported in five cases of age-related macular degeneration. Measurements were made on a modified Tübingen perimeter using 1 degree, short-wavelength targets presented on a red field.

Molecular effects of cyclosporine and oncogenesis: a new model.

Cyclosporine A is the most commonly used immunosuppressive agent during organ transplantation. One of the most feared adverse effects of cyclosporine A is the appearance of de novo cancers. The mechanisms that lead to the genesis of such cancers are thought to be only related to a side effect of cyclosporine A: a depressed immune system. Here, we review different molecular effects induced by cyclosporine A (inhibition of DNA repair, synthesis of TGF Beta, induction of apoptosis of activated T cells, inhibition of apoptosis through the inhibition of the opening of the mitochondrial Permeability Transition Pore) and propose that cyclosporine A can promote the genesis and the spread of cancer not only because of immunosuppression but also because of its ability to facilitate DNA mutations accumulation, to diminish the clearance of altered cells and to transform cancer cells into aggressive cancer cells. This new insights into the mechanisms of genesis of cyclosporine A-related cancers should be taken into account to develop preventive strategies or new immunosuppressive strategies.

Comparison of different materials for interposition arthroplasty in treatment of temporomandibular joint ankylosis surgery: long-term follow-up in 25 cases.

A variety of interposition materials have been used to prevent recurrence after arthroplasty in treatment of temporomandibular joint ankylosis. The purpose of this retrospective study of our experience was to compare the different materials (skin, temporal muscle, homologous cartilage) used for interposition arthroplasty over a period of 22 years. A total of 25 patients (32 joints) with at least 3 years of follow-up were included. Good results were achieved in 92% of cases using total full thickness skin graft and 83% of cases using temporal muscle flap. Homologous cartilage gave poor results.

[Indocyanine green angiography in age-related macular degeneration]. / Intérêt de l'angiographie au vert d'indocyanine dans la dégénérescence maculaire liée à l'âge.

Indocyanine green ocular angiography was developed more than 30 years ago, but has been used commonly for less than 10 years. In exudative age-related macular degeneration, certain cases of occult neovascularization may be converted into well-defined cases, in theory allowing treatment of more patients. Neovascularization is observed as either focal hyperfluorescence (hot spot) or diffuse hyperfluorescence (plaque). Scanning laser ophthalmoscopy imaging allows for better visualization of feeder vessels, allowing even more selective treatment. Even though a multicentric, prospective, randomized study has not yet been conducted, a number of pilot studies are very encouraging concerning indocyanine green angiography-guided laser treatment.

[Description of palpebral involvement in Fraser's syndrome]. / Description des atteintes palpébrales au cours du syndrome de Fraser.

GOAL: Fraser's syndrome is a rare multiple malformation involving the eyes, with cryptophthalmos. In its complete form, cryptophthalmos is a serious malformation with absence of eyelids and palpebral fissure. A review of ophthalmological signs is presented. CASE REPORT: A case of Fraser's syndrome is presented. Ophthalmological and somatic examination as well as evolution are described. RESULTS: Incomplete cryptophthalmos with symblepharon and bilateral palpebral coloboma are present. Surgical treatment with palpebral reconstruction allowed preservation of visual acuity in one eye. DISCUSSION: Ophthalmological signs are present in 93% of cases. Eyelid involvement such as symblepharon, and partial or complete coloboma are relatively frequent, whereas cryptophthalmos in its complete form are much more rare. CONCLUSION: Treatment involves palpebral reconstructive surgery in order to preserve visual function.

[Branch retinal vein occlusion reveals Takayasu's arteritis]. / Occlusion de branche veineuse rétinienne révélatrice d'une maladie de Takayasu.

We report a case of branch retinal vein occlusion in a 28-year-old woman that revealed Takayasu's arteritis. Somatic examination showed humeral blood pressure asymmetry and an abdominal murmur; a biological inflammatory syndrome was noted. Abdominal aortography showed marked stenosis of the superior mesenteric artery. Three of the six diagnostic criteria of Takayasu's arteritis were present in our patient, sufficing to make the diagnosis. This is the first case reported of branch retinal vein occlusion revealing Takayasu's arteritis. It is to be added to the List of atypical initial manifestations of Takayasu's arteritis such as anterior ischemic optic neuropathy. Branch retinal vein occlusion is a rare enough occurrence in a 28-year-old patient to warrant further clinical investigation.

[Brown syndrome: current status]. / Syndrome de Brown: actualités.

PURPOSE: Brown's syndrome is a form of anatomical strabismus, or retraction syndrome. It is defined by active and passive limitation of upward gaze in adduction in the field of action of the inferior oblique muscle. The etiology of Brown's syndrome remains unknown. The defect lies at the level of the superior oblique's tendonis trajectory via the trochlea. We studied the frequency of clinical signs and results after surgery in patients presenting congenital Brown's syndrome. PATIENTS AND METHODS: Our study involved 18 children. They all underwent complete ophthalmological examination with orthoptic testing, pre and postoperatively. RESULTS: Neither sidedness nor predominance of sex was noted. Compensatory head posture was noted in 7 of 18 cases. Limitation of upward gaze in adduction was a constant finding, with a positive duction test. Eleven cases underwent superior oblique recession. Results of surgery were satisfactory, with resolution of compensatory head posture in over 80% of cases. CONCLUSION: The etiology of congenital Brown's syndrome remains unknown. The different surgical techniques give inconstant results. Operative indication is decided only when in the presence of well defined clinical manifestations: CHP, deviation in primary position with alteration of binocular vision.

[Penicillium chrysogenum endophthalmitis: a case report]. / Endophtalmie à Penicillium chrysogenum: à propos d'un cas.

We report a case of Penicillium chrysogenum endophthalmitis after penetrating ocular trauma in a 9-year-old child, describing the initial management and the therapeutic adaptation after biopsy culture. After a review of endophthalmitis treatment, we discuss mycotic endophthalmitis treatment and recommend the use of intravitreal antibiotics. In this case, we used amphotericin B to treat the fungal disorder with success.

[Ocular metastasis of cutaneous melanoma]. / Métastase oculaire d'un mélanome cutané.

We report a case of vitreal metastases from cutaneous melanoma. We describe the clinical findings and the histological aspects of the lesions, which allows us to discuss the diagnosis of masquerade syndrome and highlight the diagnostic importance of vitreous biopsy.

[Surgical follow-up and importance of information and consent on behalf of patients undergoing esthetic blepharoplasty]. / Suites opératoires et importance de l'information et du consentement éclairé des patients dans les blépharoplasties esthétiques.

INTRODUCTION: Plastic surgery of the eyelids aims to improve the cosmetic aspect while correcting either an excess of palpebral skin, or an orbital septum dehiscence with fat porf2p4on in the palpebral spaces, after evaluation of horizontal and vertical palpebral laxity. PATIENTS AND METHODS: The authors describe follow-up of upper and lower lid blepharoplasty by cutaneous and conjunctival approaches respectively, with case descriptions and a review of the literature. RESULTS: A "calendar" is made of the different phases of healings, to the final aesthetic aspect desired. DISCUSSION: The patients wishes as to technique and results of blepharoplasty must be discussed and understood. The surgeon must forewarn of eventual per and postoperative complications (hemorrhage, infection), but also the usual follow-up (hematoma, edema, scarring ...). This allows to avoid aggravation of the patients "legitimate" worrying when he/she looks in the mirror! CONCLUSION: The authors underline the importance of patient information concerning the pre and postoperative aspects of aesthetic blepharoplasty.

[Teaching phacosection in a tropical setting]. / Formation à la phacosection en milieu tropical.

PURPOSE: The aim of this study was to prospectively evaluate the training of a Malagasy ophthalmologist, already proficient in classic manual extracapsular cataract extraction (ECCE), in a small incision manual technique (phacosection). MATERIALS AND METHODS: Within the activity of the Sight First program to fight blindness in Madagascar, the ophthalmologist of Majunga recruited patients presenting total white cataracts. These patients were operated by phacosection using the local hospital's usual instrumentation along with two specific phacosection instruments and extra single-use material (a precalibrated 3.2 mm knife and viscoelastic products). Peribulbar anesthesia with eye pressure was provided. Postoperative follow-up (visual acuity and corneal status) was conducted on days 1, 7 and 30. RESULTS: Forty-four eyes were operated, the first seven by the instructor assisted by the local ophthalmologist, the next 36 by the local ophthalmologist assisted by the instructor. Five postoperative complications were noted: two cases of posterior capsular rupture (one during implantation) and three cases needing reoperation with aspiration of residual cortical masses. Induced astigmatism was low and visual rehabilitation was good, with all corneas clear at day 30. DISCUSSION: The results are quite acceptable given the training period, with only two marked complications (one not related to the operative technique) and good visual rehabilitation in less than 2 weeks. This small incision technique allowed reduction of induced astigmatism and risk-free management of total white cataracts (independent of operator). CONCLUSION: Small incision manual ECCE by phacosection is a safe, possible management option for difficult cataracts in a tropical setting with only a small increase in cost. Moreover, the training of an ophthalmologist mastering classic manual ECCE appears rapid and risk-free for patients.

Evaluation of biological stress markers in police officers.

This study was performed on 18 police officers, in order to evaluate relations between behavior and the activity of the main hormonal systems implicated in stress management. All subjects were male volunteers with more than 2 years on the job, a type A psychological profile according to Friedman and Rosenman, suffering from no mental or physical illness. The experimental setting used to study the role of different stress factors consisted of a series of tests that re-created stressful situations that the subject might encounter in his job. The study showed that during stressful states, not only did peripheral secretion of catecholamines increase, but that the ratio of epinephrine to norepinephrine was a determinant factor. Epinephrine secretion which was elevated at the beginning, decreased when the stressful situation was clarified or controlled, whereas norepinephrine secretion remained elevated as long as the subject had to remain alert. Cortisol secretion seemed to depend upon anticipation of the stressful event more than its actual happenning.

[Kyrieleis arteritis: report of two cases and literature review]. / Artérite de Kyrieleis : présentation de deux cas et revue de la littérature.

We report the cases of two patients presenting with unilateral Kyrieleis arteritis complicating severe posterior uveitis, one secondary to toxoplasmosis and the other in the context of Behçet's disease. The treatment response was favorable with complete visual recovery in both cases. Kyrieleis arteritis has been described since 1933, but its pathogenesis is still unknown. However, it appears to be more of a periarteritis than an actual arteritis. It is classically associated with posterior uveitis without a direct correlation with disease severity.