[Epiretinal membranes]. / Les membranes épirétiniennes maculaires.

Idiopathic epiretinal membranes represent a common condition, and are present in approximately 10% of people over the age of 70 years. They are idiopathic in 80% of cases, or may be secondary to various conditions such as a prior retinal detachment, or vascular or inflammatory retinal diseases. The main symptoms are visual loss and metamorphopsia. The diagnosis of epiretinal membrane is currently facilitated by OCT, which provides prognostic and therapeutic decision-making assistance. Surgery for epiretinal membranes is currently well codified through sutureless vitrectomy and dyes. Dissection of the membrane (with or without associated peeling of the internal limiting membrane) ensures good anatomical and functional results, while being relatively minimally invasive.

[Current uses and indications for indocyanine green angiography]. / Intérêt et indications actuelles de l'angiographie au vert d'indocyanine (ICG).

A full interpretation of indocyanine green angiography images involves not only optical issues but also pharmacokinetic and biochemical aspects. These issues may involve biochemical changes in the fluorescence yield and the affinity of the molecule for lipoproteins and phospholipids. For age related macular degeneration (AMD), the advent of photodynamic therapy and especially anti-VEGF drugs has increased the use of OCT in assessing treatment response and guiding retreatment. The ease and advantages of OCT have become increasingly associated with a decreasing interest in ICG angiography, which is becoming less well suited for the current management of AMD. An aging population, the efficacy of anti-VEGF drugs and the relative rarity of polypoidal choroidal vasculopathy (PCV) in Europe are factors contributing to our proportional increase in AMD patients. However, aside from AMD, the indications for ICG angiography remain little changed over the last decade: it remains important in diagnosing PCV and choroidal hemangiomas, since their prognosis and treatment are specific. Similarly, for certain inflammatory conditions such as Multiple Evanescent White Dot Syndrome (MEWDS) or Birdshot chorioretinitis, the value of ICG angiography remains significant. In addition, for the treatment of chronic Central Serous Chorioretinopathy, ICG angiography helps to find sites of leakage which otherwise might have been missed. The ICG angiographic appearance in this setting may also have prognostic value. Although the indications for ICG angiography are currently decreasing for AMD, these other conditions represent a large enough number of patients to justify the continued use of this original test, which remains complementary to other chorioretinal imaging techniques.

[Unusual retinal abnormality: retinal hemorrhages related to scurvy]. / Une cause inhabituelle d'anomalies rétiniennes, hémorragies rétiniennes révélatrices de scorbut.

A diet restricted to rice and boiled fruit and vegetables leads to vitamin C deficiency. We describe the third case, to our knowledge, of retinal hemorrhages related to scurvy. Reduced bilateral visual acuity in a 50-year-old patient was associated with macrocytic anemia, denutrition, and cutaneous ecchymoses. Oral vitamin C treatment provided subjective clinical improvement and regression of the retinal hemorrhages on fundus examination, with no side effects. Vitamin C plays an important role in collagen stability in vascular and bone walls.

[Intracameral cefuroxime injections in prophylaxis of postoperative endophthalmitis after cataract surgery: implementation and results]. / Injections intracamérulaire de céfuroxime dans la prophylaxie des endophtalmies après chirurgie de cataracte: organisation et résultats.

INTRODUCTION: The European Society for Cataract and Refractive Surgery (ESCRS) endophthalmitis study demonstrated a significant decrease in the rates of postoperative endophthalmitis with the use of intracameral cefuroxime during cataract surgery. We report the organization of the implementation of this procedure in our departments and our results after 2 years. METHODOLOGY: All patients operated on for cataract between January 2007 and December 2008 in the ophthalmology departments of the Pellegrin University Hospital in Bordeaux and Lariboisiere University Hospital in Paris received an intracameral injection of cefuroxime at the end of the surgery. The cefuroxime was prepared in ready-for-use syringes by the hospital's central pharmacy. RESULTS: A convenient method of preparation and conditioning cefuroxime with stable preservation lasting longer than 1 week was set up in our hospitals. Between January 2007 and December 2008, out of 3316 patients who had a cataract surgery, two presented an endophthalmitis (0.06%). CONCLUSION: The intracameral cefuroxime injection at the end of the cataract surgery is a means to consider to prevent endophthalmitis. It can be implemented as a part of regular practice with appropriate preparation rules. The major barrier to its further use appears to be the lack of a commercially available preformulated preparation.

A hereditary moyamoya syndrome with multisystemic manifestations.

OBJECTIVE: We report a detailed description of a family affected by a hereditary multisystem disorder associated with moyamoya syndrome. METHODS: In this family case report, we evaluated 9 members of the same family originating from Algeria. Investigations included neuroimaging, cardiologic and ophthalmologic evaluation, hormonal testing, hemoglobin electrophoresis, chromosomal karyotyping, muscle biopsy for morphology, immunohistochemistry and enzyme assays, mtDNA mutation screening, and haplotype analysis of 2 loci previously linked to moyamoya, on chromosomes 10 (ACTA2) and 17. RESULTS: Five males related through a maternal lineage were affected, suggesting an X-linked inheritance. Four of them had symptomatic moyamoya syndrome with an onset of acute neurologic manifestations between 4 and 32 years. Hypergonadotropic hypogonadism, azoospermia, short stature of postnatal onset (-2 to -4 SD in adulthood), premature graying of hair, and dysmorphism were present in all patients. The other features of the disease included early cataract in 4, dilated cardiomyopathy in 3, and partial growth hormone deficiency in 2 members. Muscle biopsy data did not reveal signs of a mitochondrial disorder. All conditions known to be associated with moyamoya syndrome such as Down syndrome, neurofibromatosis, and sickle cell disease were excluded. We also excluded linkage to the 2 loci previously reported to be involved in autosomal dominant syndromic and nonsyndromic moyamoya. Carrier females had normal phenotype and clinical history. CONCLUSIONS: These data strongly suggest that this family is affected by a hereditary moyamoya multisystem disorder with X-linked recessive pattern of inheritance.

[Macular edema without fluorescein leakeage]. / OEdèmes maculaires sans diffusion angiographique.

PURPOSE: To study the OCT (optical coherence tomography) features of cases of unexplained macular edema, which were not combined with fluorescein leakage on angiography. PATIENTS AND METHOD: We report a retrospective series of three patients who presented with visual acuity impairment due to unilateral macular thickening visible on OCT examination. All eyes studied underwent OCT and angiography and electrophysiological examinations. None of the patients had a medical record and none of the eyes studied was highly myopic. RESULTS: OCT profiles shared characteristics of myopic foveoschisis or X-linked retinoschisis. No fluorescein leakage was detected on angiography. The electrooculogram was impaired in one eye while other electrophysiological investigations were normal in all eyes. DISCUSSION: Most frequently, macular edemas are associated with a fluorescein leakage on angiography. Macular edemas without angiographic leakage may correspond to unilateral idiopathic retinoschisis or macular edemas, with an atypical mechanism of intraretinal fluid accumulation.

[Severe disseminated toxoplasmosis with atypical chorioretinitis. A case of primary infection]. / Toxoplasmose disséminée sévère avec choriorétinite atypique : à propos d'un cas de primo-infection.

The clinical diagnosis of ocular toxoplasmosis is based on clinical features and biological tests: polymerase chain reaction (PCR) and the determination of intraocular specific antibody secretion (Goldmann-Witmer coefficient) on aqueous humor. Older patients may have a higher prevalence of ocular involvement and more severe ocular disease during the acute phase of recently acquired systemic infection because of altered cell-mediated immunity. Moreover, the genotype of the infecting parasite (particularly involving neotropical Type I Toxoplasma gondii strain), appears to be an important determinant of disease severity.

Elicited repetitive daily blindness: a new phenotype associated with hemiplegic migraine and SCN1A mutations.

OBJECTIVE: Familial hemiplegic migraine (FHM) is a genetically heterogeneous disorder in which three genes, CACNA1A, ATP1A2, and SCN1A, are currently known to be involved. FHM is occasionally associated with other neurologic symptoms such as cerebellar ataxia or epileptic seizures. A unique eye phenotype of elicited repetitive daily blindness (ERDB) has also been reported to be cosegregating with FHM in a single Swiss family. METHODS: We report an additional family in whom the proband had, in addition to FHM, typical ERDB. In this family and the previously reported Swiss family, the whole coding region of the SCN1A gene was screened after exclusion of mutation in CACNA1A and ATP1A2 genes. RESULTS: We identified two novel SCN1A mutations (c.4495T>C/p.Phe1499Leu and c.4467G>C/p.Gln1489His missense substitutions) in exons 24 and 23, respectively, segregating with the disease in all living affected members. Both mutations were absent from 180 healthy Caucasian controls and were located in an intracellular loop highly conserved throughout evolution. CONCLUSION: We report new clinical data supporting cosegregation of familial hemiplegic migraine and the new eye phenotype of elicited repetitive daily blindness and two novel SCN1A mutations as the underlying genetic defect in two unrelated families. SCN1A encodes the voltage-gated sodium channel Nav1.1 that is highly expressed in the CNS including the retina. This remarkably stereotyped new eye phenotype has clinical characteristics of abnormal propagation of the retinal electrical signal that may be a retinal spreading depression. These results suggest that SCN1A mutations, which alter neuronal brain excitability, may occasionally alter retinal cell excitability.

Idiopathic intracranial hypertension: a comparison between French and North-American white patients.

OBJECTIVE: To compare French and American white patients with idiopathic intracranial hypertension (IIH), and to determine prognostic factors associated with visual loss. METHODS: Medical records of all consecutive white patients with definite IIH seen between 2001 and 2006 in three French tertiary care medical centers and one American tertiary medical center were reviewed. Demographics, associated clinical features, and visual function at presentation and follow-up were collected. French white patients were compared to American white patients. RESULTS: One hundred and thirty-four patients (66 French, 68 American) were included. American patients were 8.7 times more likely than French patients to have visual acuity 20/60 or worse or visual field constriction (95% CI: 2.1-36.1, p=0.0001). American patients were treated more aggressively than French patients. French patients were older (31 vs. 28 years, p=0.02) and more likely to have anemia (20 vs. 2%, p<0.001). American patients had a longer duration of symptoms prior to diagnosis (12 vs. 4 weeks, p=0.01) and longer follow-up than French patients (26 vs. 11 months, p=0.001). Multivariable analysis found that nationality was an independent risk factor for visual loss. French and American patients did not differ regarding gender proportion, frequency of obesity, sleep apnea, endocrine diseases, or systemic hypertension. Cerebrospinal fluid (CSF) opening pressures were similar in both groups. CONCLUSION: American patients with IIH had worse visual outcomes than French patients despite more aggressive treatment. These differences are not explained by differences in previously known risk factors.

Macular full-thickness and lamellar holes in association with type 2 idiopathic macular telangiectasia.

PURPOSE: To describe patients with full-thickness macular holes (FTMHs) and lamellar macular holes (LMHs) in association with type 2 idiopathic macular telangiectasia (type 2 IMT). METHODS: Six patients with either FTMH or LMH and type 2 IMT were evaluated by means of optical coherence tomography (OCT) imaging, funduscopy, and fluorescein angiography. RESULTS: The age of the examined patients ranged from 57 to 70 years (mean 62.5+/-5.2), and best-corrected visual acuity of the affected eyes ranged from 20/50 to 20/200 (mean 20/100). All eyes showed macular abnormalities typical for nonproliferative type 2 IMT except for one eye with a proliferative disease stage. Three patients had an FTMH, one presenting with bilateral FTMH, and three had an LMH on OCT. In all cases of FTMH, the macular holes did not have elevated margins. Surgery was performed in two patients with a FTHM without subsequent functional improvement. CONCLUSIONS: The altered foveal anatomy with progressive atrophic changes within the neurosensory retina in type 2 IMT may predispose to the development of FTMH and LMH. Type 2 IMT should be considered in the differential diagnosis in patients presenting with macular holes. The association between the two may reflect alternative pathogenetic mechanisms in the development of macular holes.

[Evolving intravitreous injection technique]. / Evolution des pratiques pour la réalisation des injections intravitréennes.

The technique of intravitreous injections has been well documented for several years. Recently, a descriptive article on the intravitreous injections procedure was published in the Journal Français d'Ophtalmologie, and the AFSSAPS (French agency for drug safety) released recommendations concerning this matter on the occasion of commercial launch of pegaptanib. Since that time, the number of intravitreal injections has considerably increased, because anti-VEGF drugs had been made available to ophthalmologists, and several teams have performed a large number of procedures, allowing them to better comprehend intravitreous injections. The present paper describes our current practice of intravitreous injections. Several specialists have exchanged their experiences and issued a common synthesis. Detailed modifications of the initial recommendations have been suggested, with such basic changes such as abandoning preoperative pupil dilatation and easing postsurgical monitoring. Follow-up examinations should be adapted to each patient rather than being systematic. The suggested modifications do not change the procedure of intravitreous injections substantially, but they simplify many steps and detail the various procedures when consensus is lacking.

[Age-related macular degeneration and risk of stroke]. / Dégénérescence maculaire liée à l'âge et risque d'accident vasculaire cérébral.

Age-related macular degeneration (ARMD) and stroke are common causes of disability in subjects over 40 years of age. The etiology of ARMD remains unknown and numerous epidemiological studies have suggested a relationship between vascular risk factors and ARMD. A direct relationship between stroke and ARMD has also been suggested. An update on the association between ARMD and stroke is discussed in this article.

[Monitoring anti-VEGF drugs for treatment of exudative AMD]. / Injections intra-vitréennes d'anti-VEGF pour la dégénérescence maculaire liée à l'âge exsudative: place des examens complémentaires dans les décisions de retraitement.

AIM: Anti-VEGF drugs are the most recent treatments for choroidal neovascularization, the most severe complication of the age-related macular degeneration (AMD). These drugs are administered by intravitreal injections. Several clinical studies have demonstrated their advantages. However, these same studies have not established the modalities for monitoring their efficacy. The aim of this paper is to provide an in-depth look at the existing practices in monitoring anti-VEGF therapy among different French specialists. METHODS: Several meetings were held with specialists coming from both the hospital setting and private practice to share their practices in monitoring anti-VEGF therapy and to propose more rational monitoring to standardize practices. RESULTS: The physicians attached an increasing importance to optical coherence tomography (OCT) in monitoring anti-VEGF drugs after intravitreal injection. They acknowledged the value of fluorescein angiography when initiating the treatment and 3 months after beginning anti-VEGF therapy. They advise using fluorescein angiography every time that functional results are not at the level expected by the physician or the patient. The authors provide a flow chart that should help in deciding on retreatment. CONCLUSION: The authors suggest the use of a flow chart aimed to define indications of retreatment according to clinical response and OCT results.

Evaluation of the effect of JPEG and JPEG2000 image compression on the detection of diabetic retinopathy.

AIMS: To compare the effect of classic Joint Photographic Experts Group (JPEG) and JPEG2000 compression algorithms on detection of diabetic retinopathy (DR) lesions. METHODS: In total, 45 colour fundus photographs obtained with a digital nonmydriatic fundus camera were saved in uncompressed Tagged Interchanged Files Format (TIFF) format (1.26 MB). They were graded jointly by two retinal specialists at a 1 month interval for soft exudates, hard exudates, macular oedema, newvessels, intraretinal microvascular abnormalities (IRMA), and retinal haemorrhages and/or microaneurysms. They were compressed to 118, 58, 41, and 27 KB by both algorithms and 24 KB by classic JPEG, placed in random order and graded again jointly by the two retina specialists. Subjective image quality was graded, and sensitivity, specificity, positive and negative predictive values, and kappa statistic were calculated for all lesions at all compression ratios. RESULTS: Compression to 118 KB showed no effect on image quality and kappa values were high (0.94-1). Image degradation became important at 27 KB for both algorithms. At high compression levels, IRMA and HMA detection were most affected with JPEG2000 performing slightly better than classic JPEG. CONCLUSION: Performance of classic JPEG and JPEG2000 algorithms is equivalent when compressing digital images of DR lesions from 1.26 MB to 118 KB and 58 KB. Higher compression ratios show slightly better results with JPEG2000 compression, but may be insufficient for screening purposes.

[Efficacy and efficiency of preoperative mydriasis: drops versus ocular insert]. / Mydriase préopératoire obtenue par insert ophtalmique versus collyres.

OBJECTIVE: The primary objective of this study was to compare eye drops and inserts in terms of the quality of preoperative mydriasis. The secondary objective was to evaluate the safety and ease of use of each modality. PATIENTS AND METHODS: Prospective randomized and simple blinded study. Patients were randomized into two groups: the insert protocol (atropine 1%, diclofenac 0.1% collyrium and Mydriasert) or the eye drop protocol (atropine 1%, diclofenac 0.1%, tropicamide 0.5%, and phenylephrine 10% collyrium). Fifty-one patients were included: 25 in the insert protocol and 26 in the drop protocol. Mydriasis quality was evaluated by nurses and surgeons. The ease of use was estimated by the number of gestures required to obtain mydriasis and the difficulties encountered. Safety was evaluated at the patient's bedside. RESULTS: Mydriasis of at least 6 mm was obtained in 92% of the patients in the insert protocol (23/25) and 85% of the patients in the drop protocol (22/26) (NS). Four patients lost their insert during the study. Of the patients in the insert group, 16% (4/25) experienced stinging in their eyes, as did 23% (6/26) of patients in the group using drops (NS). To reach effective mydriasis, the insert protocol required fewer nurse gestures per patient than the drop protocol (3.0 versus 8.3; p<0.001). CONCLUSION: With the protocol using an insert, preoperative mydriasis is not inferior to that obtained with drops only and is more efficient in terms of nurse time and number of gestures. Nurses should be trained in appropriate handling of the insert and avoiding the loss of the device.

Relationship between macular hole size and the potential benefit of internal limiting membrane peeling.

AIM: To investigate the relationship between the size of macular holes and the possible benefit of internal limiting membrane (ILM) peeling. METHODS: 84 consecutive cases of idiopathic macular hole followed up for at least 3 months were included in this retrospective study. Surgery comprised pars plana vitrectomy, peeling of any epiretinal membrane, 17% C2F6 (hexafluoroethane) gas filling and 10 days of positioning. 36 eyes had ILM peeling. The main outcome measure was the macular hole closure rate checked by optical coherence tomography. RESULTS: The overall postoperative closure rate was 90.5%. For macular holes > or =400 microm in diameter, the rate was 100% with ILM peeling versus 73.3% without (p = 0.015). For smaller macular holes, the rates were 100% in both groups. Postoperative gain in visual acuity was not significantly different in eyes with ILM peeling and those without. CONCLUSIONS: ILM peeling does not seem to be useful for macular hole <400 mum in diameter. Its likely benefit has to be investigated for larger macular hole sizes, for which the failure rate is higher.

[Neuro-ophthalmologic initial presentation of sarcoidosis]. / Manifestations neuro-ophtalmologiques révélatrices d'une neuro-sarcoïdose.

PURPOSE: To describe different forms of neuro-ophthalmologic onset of sarcoidosis: clinical signs, means of diagnosis, treatment, and progression. PATIENTS AND METHODS: Retrospective study of 13 patients with neuro-ophthalmologic initial onset of sarcoidosis diagnosed in three departments between 1997 and 2003. RESULTS: There were ten women and three men, with a mean age of 36 years. Six patients suffered from diplopia. In three cases, the cavernous sinus was involved; the three other patients with diplopia had meningoradiculitis. Nine patients had infiltration of the anterior visual pathway: the optic nerve was involved in five cases, the chiasm in two cases, and two patients had papilledema. Two patients also had both symptoms. The dosage of the angiotensin-converting enzyme level was evaluated in 11 patients and was elevated in six cases. Nine patients underwent a lumbar puncture; the cerebrospinal fluid protein was high in seven cases. Chest radiography and CT were abnormal in nine cases of 11. Ten patients had histological proof of sarcoidosis; the three others had enough evidence to support this diagnosis. All of them were treated with systemic corticosteroids. The diplopia improved for the six patients. Among the seven patients with optic nerve or chiasmal infiltration, one recovered completely, two were partially improved, and four remained stable. CONCLUSIONS: Diplopia and anterior visual pathway abnormalities can be the manifestation of initial onset of sarcoidosis; therefore this diagnosis must be kept in mind when these frequent neuro-ophthalmologic signs are encountered. Complementary exams, mainly biopsy of the involved areas with histological analysis, are needed to confirm this diagnosis. Corticosteroid treatment is generally followed by improvement, but relapses may occur.

[Acute macular neuroretinopathy]. / Neurorétinopathie aiguë maculaire.

Acute macular neuroretinopathy (AMNR) is a rare disease of unknown origin, usually occurring in young women. It could cause visual loss, photopsia, and paracentral scotomas. It often occurs after a flu-like syndrome. Tiny changes may be observed in the fundus: reddish-brown ovoid dots around the fovea. Fluorescein angiography and indocyanine green angiography are usually normal. The present report describes a typical case of AMNR and emphasizes visual field changes during the follow-up.