Long-term functional outcomes and vision-related quality of life after vitrectomy for epiretinal membrane: a prospective cohort study.

To investigate the long-term effect of unilateral idiopathic epiretinal membrane (uiERM) removal on monocular and binocular visual function, and on vision-related quality of life (VR-QoL). Prospective, single-center study. The following data were collected before and after surgery: distance monocular and binocular best-corrected visual acuity (BCVA), horizontal and vertical metamorphopsia, horizontal and vertical aniseikonia, stereoacuity and National Eye Institute Visual Function Questionnaire-25 item (NEI VFQ-25). Forty-two patients (mean age: 72.7 ± 7.4 years; 24 men) were included. At 6 months postoperatively, distance monocular BCVA (p < 0.001), horizontal metamorphopsia (p = 0.001) and the composite score of NEI VFQ-25 (p < 0.001) significantly improved, in comparison to baseline. At 2 years postoperatively, distance monocular (p < 0.001) and binocular (p = 0.01) BCVA, horizontal (p < 0.001) and vertical (p = 0.02) metamorphopsia, vertical aniseikonia (p = 0.01), stereoacuity (p < 0.001) and 3 subscales scores of the NEI VFQ-25 (p < 0.05) ("general vision", "mental health", "driving") significantly improved in comparison to baseline. Removal of uiERM improves VR-QoL and achieves good visual outcomes on both monocular and binocular visual parameters over long-term. Visual symptoms induced by macular contraction have different improvement kinetics after surgery. Stereopsis, the highest level of binocular vision, can be improved in some cases.

Strabismus outcomes after surgery: the nationwide SOS France study.

PURPOSE: To describe the types of strabismus operated on, the surgical procedures performed, and the 2-year reoperation rate in France. METHODS: Entire population 5-year cross-sectional analysis of a national medico-administrative database in France between January 2013 and December 2017 included all patients who underwent a first strabismus surgery, with a 2-year follow-up. Patient identification was based on the diagnostic codes of the 10th International Classification of Diseases and surgical procedures on the codes of the Common Classification of Medical Acts. A subgroup analysis comparing non-paralytic and paralytic strabismus was performed. RESULTS: Among the 56,654 patients included (women: 50.8%), 26,892 (47.5%) patients were under 10 years old. Overall, 52,711 (93%) were diagnosed with non-paralytic strabismus and 3,943 (7%) with paralytic strabismus. Among the non-paralytics, the most frequent diagnosis was esotropia (21,282, 37.6%), followed by exotropia (14,392, 25.4%) and vertical strabismus (2,017, 3.6%). Among the paralytics, fourth cranial nerve palsy (1,499, 2.6%) was more frequent than sixth cranial nerve palsy (691, 1.2%) and third cranial nerve palsy (431, 0.8%). The 2-year reoperation rate was 7.7% (4,362 patients), the lowest for non-paralytic (7.4%) and the highest for paralytic (11.4%). CONCLUSION: This first French population-based study about strabismus will contribute to the evaluation of practices at a national level and permit comparisons between countries. Although the 2-year reoperation rate was found to be 1 out of 13 patients, it should be interpreted with caution. Long-term follow-up is still warranted due to considerable variability of the type and severity of strabismus as well as surgical practices.

Monocular and binocular visual parameters associated to vision-related quality of life in patients with epiretinal membrane: a prospective cohort.

PURPOSE: Binocular visual impairment related to unilateral idiopathic epiretinal membranes (uiERM) and its association with vision-related quality of life (VR-QoL) has not yet been investigated. This study aimed to explore binocular visual parameters (distance binocular best-corrected visual acuity (BCVA), aniseikonia, stereopsis) and VR-QoL in patients with uiERM. METHODS: We carried out a prospective single-centre cohort study. The following data were collected: VR-QoL (NEI VFQ-25), distance monocular and binocular BCVA, horizontal and vertical metamorphopsia (M-charts test), horizontal and vertical aniseikonia (NAT TEST) and stereoacuity (TNO test). Multiple linear regression analyses were performed to evaluate factors influencing VR-QoL. RESULTS: Forty-six patients with uiERM and visual complaints were included. Aniseikonia was found in 40 (80%) participants. Stereoacuity was poor (above 120 s of arc) in 46 (100%) participants. Distance monocular BCVA, horizontal metamorphopsia and horizontal aniseikonia were the factors associated with VR-QoL in patients with uiERM. CONCLUSIONS: In subjects presenting uiERM, aniseikonia is frequently found and stereopsis is constantly impaired. We advocate quantitative testing of metamorphopsia and aniseikonia in addition to BCVA for the assessment of global visual function. Further investigations are needed to evaluate the effect of vitrectomy on these parameters and VR-QoL. TRIAL REGISTRATION: EudraCT Number/ID RCB: 2016-A00252-49.

Predictive values of initial semi-quantitative assessment of relative afferent pupillary defect for neovascularization in central retinal vein occlusion.

PURPOSE: To measure the predictive values of relative afferent pupillary defect (RAPD) assessed semi-quantitatively, and visual acuity (VA) at onset of central retinal vein occlusion (CRVO), for neovascularization. METHODS: Retrospective analysis of the TROXHEMO trial that included patients with CRVO within 30 days after the onset. Inclusion criteria were as follows: semi-quantitative RAPD assessment at diagnosis and/or at one month. RAPD was 'severe' if ≥ 0.9 log. Exclusion criteria were as follows: prophylactic panretinal photocoagulation (PRP) before neovascularization. RESULTS: Among the 119 patients enrolled in the main centre, 101 were analysed. 26 had a neovascular complication during the twelve months of follow-up: rubeosis (19), glaucoma (7) and posterior neovascularization (15). The mean time to onset of a neovascular complication was 4.7 months (1 to 12, median 3 months). All the patients who had a neovascular complication had RAPD at first examination or at one month (negative predictive value (NPV) = 100%) but the positive predictive value (PPV) was low (31%, 95% CI [21%; 42%]). The association 'severe RAPD or VA < 35 letters (ETDRS) at inclusion or at one month' was the best compromise between PPV (53%, [39%; 68%]) and NPV (96%, [92%; 100%]). CONCLUSION: To predict neovascularization, RAPD should be routinely evaluated with filters: the risk of neovascular complication is (a) almost nil if there is no RAPD, (b) very low if there is no severe RAPD and if VA is higher than 35 letters, and (c) higher than 50% if RAPD is ≥ 0.9 log or if VA is less than 35 letters.

Perioperative risk of bleeding with antithrombotic agents in macular surgery: a national, prospective, multicentre study.

PURPOSE: To compare the risk of haemorrhagic complications in elective macular surgery between patients with no antithrombotic (AT) treatment (defined as patients with no history of AT therapy or who discontinued AT therapy) and patients who continued AT treatment during the surgery. METHODS: E-case report forms were prospectively recorded in a database before vitreoretinal surgery and 1 month after. Data on patient characteristics, surgical techniques, haemorrhagic complications and antithrombotic status were collected. Patients with retinal detachment, proliferative diabetic retinopathy and previous retinal haemorrhage were excluded. RESULTS: A total of 748 procedures (single procedure in one eye per patient) were performed between January and May 2019. Among them, 202 patients (27.0%) were treated with antithrombotic therapy at the time of surgery: 19.5% with antiplatelet agents (n = 146), 6.3% with anticoagulants (n = 47) including 3.2% (n = 24) patients treated with novel oral anticoagulants, 0.8% (n = 6) with anticoagulants and antiplatelet agents, and 0.4% (n = 3) with heparin. Overall, 92 patients (12.3%) developed one or more haemorrhagic complications, of which 63 (11.5%) and 29 (14.4%) were in the non-AT and AT group, respectively. The multivariate logistic regression model showed no difference between AT treatment groups regarding ocular bleeding complications (odds ratio [OR] 1.2, 95% confidence interval (CI) [0.7-2.2], p = 0.54). CONCLUSION: No cases of uncontrolled or severe perioperative haemorrhage in patients continuing antithrombotic agents were reported in this selected population. For the majority of the patients taking antiplatelets or anticoagulants, these agents could be safely continued during macular surgery.

Ophthalmological findings in children with autism spectrum disorder.

PURPOSE: Eye pathology could be related to atypical visual behaviours and impaired social communication through visual cues in children with autism spectrum disorder (ASD). The main purpose of this prospective study was to assess ophthalmological disorders in children with ASD and to investigate the relationships with intellectual disability (ID) and ASD severity. METHODS: In this prospective study, comprehensive ophthalmological and oculomotor examinations were performed. ASD severity and verbal and performance intelligence quotients were determined using adapted scales. These clinical data were compared between groups of children based on the presence or absence of ophthalmological disorders and the achievement or not of visual acuity (VA) testing by using non-parametric statistical tests. RESULTS: Amongst a sample of 51 children, ophthalmological disorders were found in 39% of cases, with 35% having significant refractive errors and 10% presenting with strabismus. Children with ASD and ophthalmological disorders had significantly lower verbal (29.8 ± 14.7 compared with 44.3 ± 21.5; p = 0.010) and performance quotients (57.8 ± 18.3 compared with 67.59 ± 20; p = 0.049) but no significant result was found between the presence of ophthalmological disorders and ASD severity, level of communication and social contact, or modulating behaviour when changes occur. Children who did not achieve monocular VA testing (39%) had significantly lower verbal (25.1 ± 9.7 compared with 46.1 ± 20.9; p < 0.001) and performance quotients (52.7 ± 17 compared with 69.8 ± 18.8; p = 0.001), also presented higher social interaction impairment (p = 0.002), and expressed more important behavioural signs (p = 0.007). CONCLUSIONS: Ophthalmological disorders are frequently found in children with ASD, especially in those with ID. Ophthalmologists and child psychiatrists should pay attention to perform ophthalmological examination in children with ASD since eye disorders might remain undetected. A comprehensive examination by a paediatric ophthalmologist would help to improve the individual clinical description and the global intervention. TRIAL REGISTRATION: Clinical trial registration number: NCT02444117.

Reduced visual evoked potential amplitude in autism spectrum disorder, a variability effect?

Atypical sensory behaviours represent a core symptom of autism spectrum disorder (ASD). Investigating early visual processing is crucial to deepen our understanding of higher-level processes. Visual evoked potentials (VEPs) to pattern-reversal checkerboards were recorded in ASD children and age-matched controls. Peak analysis of the P100 component and two types of single-trial analyses were carried out. P100 amplitude was reduced in the ASD group, consistent with previous reports. The analysis of the proportion of trials with a positive activity in the latency range of the P100, measuring inter-trial (in)consistency, allowed identifying two subgroups of ASD participants: the first group, as control children, showed a high inter-trial consistency, whereas the other group showed an inter-trial inconsistency. Analysis of median absolute deviation of single-trial P100 (st-P100) latencies revealed an increased latency variability in the ASD group. Both single-trial analyses revealed increased variability in a subset of children with ASD. To control for this variability, VEPs were reconstructed by including only positive trials or trials with homogeneous st-P100 latencies. These control analyses abolished group differences, confirming that the reduced P100 amplitude results from increased inter-trial variability in ASD. This increased variability in ASD supports the neural noise theory. The existence of subgroups in ASD suggests that the neural response variability is not a genuine characteristic of the entire autistic spectrum, but rather characterized subgroups of children. Exploring the relationship between sensory responsiveness and inter-trial variability could provide more precise bioclinical profiles in children with ASD, and complete the functional diagnostic crucial for the development of individualized therapeutical projects.

Objective ocular torsion outcomes after unilateral horizontal rectus surgery in infantile esotropia.

PURPOSE: To analyse objective ocular torsion among patients with infantile esotropia and to determine the effects of unilateral horizontal rectus surgery. METHODS: Sixty-eight patients (136 eyes) (range 4 to 16 years) who underwent unilateral horizontal rectus surgery for infantile esotropia participated in this retrospective single-centre study. Objective ocular torsion using fundus photography was assessed before surgery and 1 year later. We defined three groups of patients based on preoperative qualitative objective ocular torsion: physiological extorsion and pathological extorsion and intorsion. For each group, the disc-foveal angle was measured and analysed both before and after surgery. We looked for possible correlations between amount of esodeviation and disc-foveal angle size. RESULTS: Preoperatively, 28 (41%) patients had + 6.73 (± 2.66) degrees of physiological extorsion. Thirty-one (46%) patients had + 12.94 (± 3.67) degrees of pathological extorsion. Nine (13%) patients had - 1.99 (± 2.52) degrees of intorsion. After surgery, the number of subjects with physiological extorsion increased to 45 (66%). The number of patients with pathological extorsion decreased to 17 (25%) and the mean disc-foveal angle was significantly reduced by 1.80°. Six (9%) patients presented intorsion and the mean disc-foveal angle was significantly reduced by 2.28°. For the pathological extorsion group, the size of the disc-foveal angle before surgery was positively correlated to its reduction after surgery. Disc-foveal angle variation and distance esodeviation variation after surgery were positively correlated. CONCLUSIONS: These results highlight that pathological objective ocular torsion can be frequently found in infantile esotropia and is decreased after unilateral recession-plication surgery.

Dietary, environmental, and genetic risk factors of Extensive Macular Atrophy with Pseudodrusen, a severe bilateral macular atrophy of middle-aged patients.

EMAP (Extensive Macular Atrophy with Pseudodrusen) is a maculopathy we recently described that shares pseudodrusen and geographic atrophy with Age-related Macular Disease (AMD). EMAP differs from AMD by an earlier age of onset (50-55 years) and a characteristic natural history comprising a night blindness followed by a severe visual loss. In a prospective case-control study, ten referral centers included 115 EMAP (70 women, 45 men) patients and 345 matched controls to appraise dietary, environmental, and genetic risk factors. The incidence of EMAP (mean 2.95/1.106) was lower in Provence-Côte d'Azur with a Mediterranean diet (1.9/1.106), and higher in regions with intensive farming or industrialized activities (5 to 20/1.106). EMAP patients reported toxic exposure during professional activities (OR 2.29). The frequencies of common AMD complement factor risk alleles were comparable in EMAP. By contrast, only one EMAP patient had a rare AMD variant. This study suggests that EMAP could be a neurodegenerative disorder caused by lifelong toxic exposure and that it is associated with a chronic inflammation and abnormal complement pathway regulation. This leads to diffuse subretinal deposits with rod dysfunction and cone apoptosis around the age of 50 with characteristic extensive macular atrophy and paving stones in the far peripheral retina.

Treatment by rituximab on six Grave's ophthalmopathies resistant to corticosteroids.

OBJECTIVES: Graves' ophthalmopathy occurs in 50% of Graves' disease cases. Treatment is based on smoking cessation, and control of the euthyroidism and ocular repercussions associated with the disease. The active orbital forms are treated with glucocorticoids. Non-validated therapies have also been recently tested. Rituximab has been effectively used several times to treat corticosteroid-resistant Graves' ophthalmopathy associated with an optic neuropathy, but its use could be proposed only in inflammatory ophthalmopathies after failure of the corticosteroids. We present six cases treated since early 2012 at the University Hospital Center of Tours, France. METHODS: Six patients were treated at the University Hospital Center of Tours, France, between September 2012 and April 2014. The patients had a Mourits' score greater than three after treatment with corticosteroids and/or a severe NOSPECS score and/or orbital inflammation resistant to maximal treatment with intravenous injections of methylprednisolone and an optic neuropathy. They twice received one gram of rituximab by slow intravenous injection two weeks apart. Efficacy was assessed by a decrease of the orbital inflammatory clinical Mourits' score, and visual acuity and visual field testing. RESULTS: The inflammatory score of patients improved and treatment helped to stop the progression of the sequelae due to neuropathy. The orbital inflammatory clinical score, and the visual acuity and visual field improved but orbital decompression was necessary to complete the treatment. CONCLUSION: Rituximab has been used for the treatment of active corticosteroid-resistant Graves' ophthalmopathies. We also had positive results on patients with visual threat and optic neuropathy, when combined with surgical decompression.

Clinical Characteristics and Risk Factors of Extensive Macular Atrophy with Pseudodrusen: The EMAP Case-Control National Clinical Trial.

PURPOSE: To assess the association of clinical and biological factors with extensive macular atrophy with pseudodrusen (EMAP) characterized by bilateral macular atrophy occurring in patients aged 50 to 60 years and a rapid progression to legal blindness within 5 to 10 years. DESIGN: A national matched case-control study. PARTICIPANTS: Participants were recruited in 10 French Departments of Ophthalmology and their associated clinical investigation centers. All 115 patients with EMAP had symptoms before the age of 55 years due to bilateral extensive macular atrophy with a larger vertical axis and diffuse pseudodrusen. Three controls without age-related macular degeneration (AMD) or retinal disease at fundus examination were matched for each patient with EMAP by gender, age, and geographic area (in total 415). METHODS: Subjects and controls underwent an eye examination including color, red-free autofluorescent fundus photographs and spectral-domain optical coherence tomography with macular analysis. The interviews collected demographic, lifestyle, family and personal medical history, medications, and biological data. Associations of risk factors were estimated using conditional logistic regression. MAIN OUTCOME MEASURES: Extensive macular atrophy with pseudodrusen status (cases vs. controls). RESULTS: Extensive macular atrophy with pseudodrusen most frequently affected women (70 women, 45 men). After multivariate adjustment, family history of glaucoma or AMD was strongly associated with EMAP (odds ratio [OR], 2.3, P = 0.008 and OR, 1.5, P = 0.01, respectively). No association was found with cardiac diseases or their risk factors. Mild and moderate kidney disease and higher neutrophil rate were associated with a reduced risk of EMAP (OR, 0.58, P = 0.04; OR, 0.34, P = 0.01; and OR, 0.59, P = 0.003, respectively). On the contrary, eosinophilia (OR, 1.6; P = 0.0002), lymphocytosis (OR, 1.84; P = 0.0002), increased erythrocyte sedimentation rate (OR, 6.5; P = 0.0005), decreased CH50 (P = 0.001), and high plasma C3 level (P = 0.023) were significantly associated with a higher risk of EMAP. CONCLUSIONS: This study documents an association between EMAP and family history of AMD and glaucoma, a clear female predominance, and a systemic inflammatory profile. The reduced CH50 and increased C3 plasma values could reflect a more severe complement pathway dysfunction than in AMD, leading to early pseudodrusen and rapid development of geographic atrophy. There is no association of EMAP with AMD cardiac diseases or cardiac risks, including cigarette smoking.

Long-Term Assessment of Suturectomy in Trigonocephaly and Anterior Plagiocephaly.

INTRODUCTION: Techniques for treating trigonocephaly and anterior plagiocephaly have evolved from the initial suturectomy technique to frontal cranioplasty. The authors evaluated the suturectomy technique in adolescent patients with these craniosynostoses, by carrying out a retrospective, long-term assessment at the end of growth. METHODS: Patients with anterior plagiocephaly or trigonocephaly were included. All had undergone coronal or metopic suturectomy with bilateral orbitofrontal bandeau resection between 1997 and 2005. The primary endpoint was the Whitaker classification. The secondary endpoints were anthropometric measurement, assessments of the bone defects on computed tomography scan, and the comments of patients and their relatives concerning the final skull outcome. Patients with anterior plagiocephaly also attended an ophthalmological consultation. RESULTS: Seventeen patients were included in the study: 8 with anterior plagiocephaly and 9 with trigonocephaly. Mean age at the time of surgery was 6.91 months. Mean age at the time of craniofacial consultation was 14 years. Fourteen patients (82%) were classified as Whitaker Class III and IV, corresponding to poor esthetic results and persistent bone defects. Seven patients requested further surgery. CONCLUSION: This study shows that suturectomy seems to yield poor esthetic results in the long term and patients should be followed up throughout adolescence to correct any craniofacial deformities.

Conversion from nonischemic to ischemic retinal vein occlusion: prediction by venous velocity on color Doppler imaging.

PURPOSE: To identify color Doppler imaging (CDI) parameters and other prognostic factors of a conversion from nonischemic to ischemic retinal vein occlusion (RVO) in a large population with a long follow-up. METHODS: This was a retrospective observational study. Data were collected for patients who had been admitted to the ophthalmologic department of the Hospital of Tours because of nonischemic central RVO (CRVO) or branch RVO (BRVO). We analyzed the relation between time until conversion into ischemic RVO and several prognostic factors of conversion, mainly vein velocities as measured by CDI. RESULTS: Analyses involved 162 patients. One year after inclusion, conversion into ischemic RVO occurred in 25.0% of the 113 CRVO and in 28.6% of the 49 BRVO cases. For CRVO, an increase of the minimal central retinal venous velocity (CRV), measured by CDI before and after treatment by hemodilution, diminished the risk of conversion into an ischemic form (p=0.048). For BRVO, an elevated maximal CRV on diagnosis was a protector (p=0.004). Age was associated with a high risk of ischemic evolution for CRVO (p=0.023) but not BRVO. Initial visual acuity was not associated with the conversion, for BRVO or CRVO. Increased retinal hemorrhages highly increased the risk of conversion both for CRVO (p<0.0001) and BRVO (p=0.010). CONCLUSIONS: Risk of ischemic evolution for BRVO and CRVO treated by isovolemic hemodilution was associated with central venous velocities. CDI might be useful for identifying risk of ischemic conversion and individualizing the follow-up of patients.

Acute and late-onset optic atrophy due to a novel OPA1 mutation leading to a mitochondrial coupling defect.

PURPOSE: Autosomal dominant optic atrophy (ADOA, OMIM 165500), an inherited optic neuropathy that leads to retinal ganglion cell degeneration and reduced visual acuity during the early decades of life, is mainly associated with mutations in the OPA1 gene. Here we report a novel ADOA phenotype associated with a new pathogenic OPA1 gene mutation. METHODS: The patient, a 62-year-old woman, was referred for acute, painless, and severe visual loss in her right eye. Acute visual loss in her left eye occurred a year after initial presentation. MRI confirmed the diagnosis of isolated atrophic bilateral optic neuropathy. We performed DNA sequencing of the entire coding sequence and the exon/intron junctions of the OPA1 gene, and we searched for the mitochondrial DNA mutations responsible for Leber hereditary optic atrophy by sequencing entirely mitochondrial DNA. Mitochondrial respiratory chain complex activity and mitochondrial morphology were investigated in skin fibroblasts from the patient and controls. RESULTS: We identified a novel heterozygous missense mutation (c.2794C>T) in exon 27 of the OPA1 gene, resulting in an amino acid change (p.R932C) in the protein. This mutation, which affects a highly conserved amino acids, has not been previously reported, and was absent in 400 control chromosomes. Mitochondrial DNA sequence analysis did not reveal any mutation associated with Leber hereditary optic neuropathy or any pathogenic mutations. The investigation of skin fibroblasts from the patient revealed a coupling defect of oxidative phosphorylation and a larger proportion of short mitochondria than in controls. CONCLUSIONS: The presence of an OPA1 mutation indicates that this sporadic, late-onset acute case of optic neuropathy is related to ADOA and to a mitochondrial energetic defect. This suggests that the mutational screening of the OPA1 gene would be justified in atypical cases of optic nerve atrophy with no evident cause.

Unusual pharmacokinetics of intravitreal and systemic voriconazole in a patient with Scedosporium apiospermum endophthalmitis.

In this paper, we report a case of a post-traumatic Scedosporium endophthalmitis treated with a posterior vitrectomy, followed by intravitreal injections and systemic voriconazole. This is the second documented case of S. apiospermum endophthalmitis treated with voriconazole and the first case with intravitreal injections of voriconazole. A 29-year-old man developed endophthalmitis after being struck in the left eye by a chip from a swimming pool pump. Despite 3 weeks of prophylactic antibiotherapy, his visual acuity remained only for the perception of light and vitreous inflammation increased. A creamy-white fungal mass grew at the inferior peripheral retina and pars plana. The fungus was identified as S. apiospermum. Oral voriconazole, at 200 mg twice-daily, did not bring the infection under control, considering the low plasma and intravitreal concentrations. Before steady-state plasma voriconazole concentrations reached an efficacy level greater than minimum inhibitory concentration of Scedosporium, intravitreal injections of 64 ug/0.1 mL of voriconazole were initiated twice-weekly for 3 weeks. Administration of higher intravenous voriconazole doses (6 mg/kg b.i.d.) for 6 weeks was needed to achieve an antifungal effect without systemic dissemination.

Increased prevalence of factor V Leiden in patients with retinal vein occlusion and under 60 years of age.

Retinal vein occlusion (RVO) is a multifactorial disease involving vessel damage, stasis, viscosity and thrombosis. Conflicting findings on hereditary thrombophilic risk factors have been reported and their impact on RVO features remains to be defined. The aim of the present study was to evaluate the prevalence of hereditary thrombophilic risk factors (HTRF) and characteristics of RVO in patients with or without HTRF. The design of the study was a prospective, observational case series. Two hundred and thirty-four patients with RVO were included consecutively. A French healthy population of the same region was studied as control group. The HTRF studied were protein C (PC), protein S (PS) and antithrombin (AT) deficiencies, factor V Leiden (FVL) and factor II 20210A polymorphisms. Chi-Square was used for comparison with the healthy subjects and between RVO patient with and without HTRF according to localisation (branch vs. central), type of RVO (ischemic or non-ischemic), recurrence, age at first event and classical vascular risk factors. Twenty-two patients had HTRF (12 FV Leiden heterozygotes, 9 FII 20210A heterozygotes and 1 PS deficiency). No AT or PC deficiency was detected. Frequencies of PS deficiency, FVL and FII 20210A allele were similar to the reference population as well as to published data in the general caucasian population. Eighty-six patients experienced their first episode before the age of 60 years. Systemic hypertension, glaucoma and angina were significantly less frequent in patients with RVO before 60 years. Fourteen of the 22 patients with one HTRF (64%) experienced their first episode of RVO before the age of 60 years compared to 72 of 212 without HTRF (34%) (p = 0.006). Heterozygote status for FV Leiden was significantly more frequent in patients who had experienced their first episode of RVO before 60 years (p = 0.027). In conclusion, this study suggests a role of FV Leiden in the occurrence of RVO in patients younger than 60 years who exhibit fewer acquired vascular risk factors than in older patients.

Color Doppler imaging of orbital vessels: personal experience and literature review.

Many Doppler imaging studies have been performed in recent years in a large number of ocular disorders because of improvements in the Doppler equipment used for detecting and measuring the low blood-flow velocities that are a requisite for the quantitative evaluation of blood flow in the orbital vessels. The ophthalmic artery, central retinal artery and vein, posterior ciliary arteries, and the superior ophthalmic vein can be easily identified using color Doppler sonography. The changes in local blood flow in these vessels assessed by spectral analysis pulsed Doppler sonography have been used to characterize and to obtain new insights into different nontumoral vascular disorders including carotid artery stenosis, central retinal vein occlusion, giant cell arteritis, glaucoma, diabetes, fistulas, and tumoral processes of the eye and orbit. Our experience has confirmed the important role of Doppler sonography in the assessment of subclinical changes in the vascular bed, in the understanding of different processes, for following up after specific treatments, and for determining the long-term prognosis of these various conditions.