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We report a case of a 28-year-old otherwise healthy female patient who presented with blurred vision in her right eye related to multiple branch retinal artery occlusions confirmed by fluorescein angiography. Investigation revealed positive antinuclear antibodies and an interatrial septal aneurysm on transthoracic echocardiography. The patient was treated with oral prednisolone and aspirin. Two months after her initial presentation, she developed hearing loss and tinnitus. Ophthalmological examination revealed temporal inferior and nasal inferior branch retinal artery occlusions in the left eye. Magnetic resonance imaging of the brain showed multifocal T2 hyperintense lesions in cortical and subcortical areas as well as the corpus callosum consistent with Susac’s syndrome. The diagnosis of Susac’s syndrome should be kept in mind in young patients presenting with multiple or recurrent retinal artery occlusions even in the absence of associated systemic symptoms to not delay appropriate management.

Purpose: To describe optical coherence tomography angiography (OCTA) findings in eyes with Behcet uveitis (BU) and to compare these findings with those of fluorescein angiography (FA). Methods: Prospective, comparative, cross-sectional study. Patients presenting with clinically active BU involving the posterior segment were evaluated using FA, spectral domain optical coherence tomography (SD-OCT), and OCTA. Optical coherence tomography angiograms were reviewed and analyzed. Foveal avascular zone areas and vessel densities were also reported. Results: Twenty-five patients (44 eyes) were included. Perifoveal microvascular changes were more frequently observed on OCTA than on FA (95.5 vs 59.1%; P < 0.001). Disruption of the perifoveal capillary arcade, areas of retinal capillary nonperfusion/hypoperfusion, and perifoveal capillary abnormalities, including rarefied, dilated, or shunting vessels were observed more frequently using OCTA than FA (40.9 vs 25%; P = 0.039, 86.4 vs 34.1%; P < 0.001, and 84.1 vs 36.4%; P < 0.001, respectively). Areas of retinal capillary nonperfusion/hypoperfusion were more frequently observed in the deep than in the superficial capillary plexus (81.8 vs 63.6%; P = 0.039). Capillary abnormalities and disorganization of the normal architecture of the capillary network were more frequent in the deep than in the superficial capillary plexus (P < 0.001). Foveal avascular zone area was not significantly larger in eyes with BU than in control group in both the superficial and the deep capillary plexuses (0.4 vs 0.34 mm; P = 0.23 and 0.72 vs 0.53 mm; P = 0.053, respectively). Capillary vessel density was significantly lower in eyes with BU than in control group in the deep capillary plexus (13.7 vs 17.2 mm 21; P = 0.004). Conclusion: OCTA allows better visualization and characterization of perifoveal microvascular changes than FA in eyes with active BU. The deep capillary plexus seemed to be more severely involved than the superficial capillary plexus.

The purpose of this study is to compare the effect of topical diclofenac and topical dexamethasone on anterior chamber flare and postoperative pain following rhegmatogenous retinal detachment (RRD) surgery. This prospective study included 40 eyes of 40 patients treated for RRD. Twenty-eight patients underwent scleral buckling and 12 patients underwent 20-gauge pars plana vitrectomy (PPV). For each surgical procedure, patients were postoperatively randomly divided into two groups: the first group received topical dexamethasone phosphate 0.1 % four times daily for 28 days; the second group received topical diclofenac sodium 0.1 % three times daily for 28 days. The inflammatory reaction in the anterior chamber was measured with laser flare photometry preoperatively and 1, 7, 14, 28, and 90 days postoperatively. Pain level was evaluated with Scott's visual analog scale at day 1, 7, 14, and 28 postoperatively. For patients treated with scleral buckling, there was no significant difference between the two groups regarding mean aqueous flare at day 1 (p = 0.096), day 7 (p = 0.435), day 14 (p = 0.510), day 28 (p = 0.583), and day 90 (p = 0.423). The group who received diclofenac had significantly lower pain score at days 7, 14, and 28 (p = 0.048, p = 0.017, and p = 0.028, respectively). For patients treated with PPV, there was no significant difference between the two groups regarding mean aqueous flare at day 1 (p = 0.400), day 7 (p = 0.728), day 14 (p = 0.843), day 28 (p = 0.939), and day 90 (p = 0.568). Patients who received diclofenac had significantly lower pain score at days 7, 14, and 28 (p = 0.032, p = 0.030, and p = 0.023, respectively). Topical diclofenac seems to be as potent as topical dexamethasone in managing postoperative inflammatory response induced by surgery for RRD with better analgesic effect. Both of them are consequences of blood-aqueous barrier and blood-retinal barrier breakdown.

The purpose of this study was to determine final diagnosis of patients referred with a diagnosis of neuroretinitis. A retrospective study of 40 patients with optic disc oedema with macular star (ODOMS) referred with a diagnosis of neuroretinitis was conducted. The final diagnosis was neuroretinitis in 26 patients (65%), with most of these patients (96.1%) having unilateral involvement. Main underlying aetiologies included cat scratch disease (30.8%), rickettsiosis (19.2%), and idiopathic neuroretinitis (23.1%). The remaining 14 patients (35%) had ODOMS that had been mistaken for neuroretinitis. Of these patients, 42.8% were found to have a previously unknown malignant systemic hypertension in association with bilateral ODOMS. Neuroretinitis, usually unilateral, should be differentiated from other causes of unilateral or most often bilateral ODOMS that may masquerade as neuroretinitis, mainly malignant systemic hypertension. This is essential to avoid inappropriate work-up and management and subsequent potential visual or systemic morbidity.

Background. Uveitisisamajorcauseofvisualimpairmentthroughouttheworld.Analysisofcytokineprofilesin aqueous humor specimens may provide insight into the physiopathological processes that underly retinal damage in this context. Methods. Usingamultiplexassay,wedeterminedtheconcentrationsof17cytokinesandchemokinesinaqueous humor specimens obtained from patients with ocular toxoplasmosis or viral uveitis and compared these concentrations with those in specimens obtained from patients with noninfectious intermediate uveitis or cataract. Results. Five mediators (interleukin [IL]‐8, monocyte chemoattractant protein‐1, tumor necrosis factor‐, IL-4, and IL-10) were detected in 50% of patients in all groups. In contrast, IL-5 and IL-12 were specific for ocular toxoplasmosis, and granulocyte monocyte colony-stimulating factor and IL-1 were specific for viral uveitis; these mediators could present specific markers for diagnostic purposes. Interferon-, IL-6, and macrophage inflammatory protein-1 were common markers of ocular toxoplasmosis and viral uveitis. IL-17 was a common marker of ocular toxoplasmosis and intermediate uveitis. Conclusions. Wefoundspecificcytokineprofilesforeachtypeofuveitis,withlargeinterindividualvariationsand no etiological or clinical correlations. Ocular cytokine mapping contributes to a better understanding of the physiopathology of specific forms of uveitis and provides guidance for new targeted treatment. Uveitis is a potentially blinding inflammatory disease that affects individuals of any age. It is the third leading causeofblindnessworldwide,accountingfor10%‐15%

To report optical coherence tomography angiography findings in a patient with rickettsial retinitis.A 29-year-old man complaining of acute blurring vision in the right eye associated with Rickettsia conorii infection underwent a comprehensive ocular examination, fluorescein angiography, spectral-domain optical coherence tomography, and swept-source optical coherence tomography angiography.Funduscopy showed two large areas of retinitis in the inferior macula along the distribution of the inferotemporal artery with associated retinal hemorrhages, retinal edema, and serous retinal detachment. Fluorescein angiography showed early hypofluorescence and late staining of white retinal lesions and associated adjacent retinal vascular leakage and optic disc hyperfluorescence. Optical coherence tomography angiography showed hypointense dark areas in the superficial capillary plexus and larger hypointense areas in the deep capillary plexus, outer retina, and choriocapillaris layer. The patient was treated with doxycycline and prednisone. Six weeks after presentation, retinal changes seen at the acute phase had resolved, leading to mild residual retinal pigment epithelial changes. Fluorescein angiography showed retinal capillary nonperfusion within areas of resolved retinitis. Spectral-domain optical coherence tomography findings included inner retinal atrophy, intraretinal cysts, and disruption of ellipsoid zone and interdigitation zone. Swept-source optical coherence tomography angiography showed well-delineated hypointense greyish areas of retinal capillary nonperfusion in both the superficial and deep capillary plexuses. Visual field testing revealed the presence of a corresponding paracentral defect.Optical coherence tomography angiography may be a valuable noninvasive imaging technique for detecting and analyzing occlusive complications associated with rickettsial retinitis.

West Nile virus (WNV) infection is an emerging and resurgent arthropod vector-borne disease caused by WNV, which belongs to the Flaviviridae family. WNV infection in humans usually manifest as a mild febrile illness, but a severe potentially lethal neurologic involvement may occur in patients who are elderly or debilitated. A bilateral multifocal chorioretinitis with typical linear clustering of lesions is the most common ocular finding in patients with acute WNV infection associated with neurologic illness. Other less common findings may include retinitis, anterior uveitis, retinal vasculitis, and optic neuropathy. Diabetes mellitus and advanced age appear to be a potential risk factor for developing severe neurologic disease and also severe chorioretinitis. Diagnosis is based on epidemiologic data, pattern of ocular and systemic involvement, and is usually confirmed by serologic testing and/or PCR. Treatment of WNV infection is entirely supportive, and prevention is the mainstay of infection control.

Purpose: To describe the pattern of acute retinal necrosis (ARN) syndrome in a referral center in Tunisia.Methods: Retrospective review of the charts of 12 patients (12 eyes).Results: Necrotizing retinitis involved >50% of the retina in 33.3% of eyes. Polymerase chain reaction on aqueous humor sample identified herpes simplex virus-1 in 4 eyes (33.3%) and varicella zoster virus in 3 eyes (25%), and was negative in 5 eyes (41.7%). All patients received intravenous acyclovir, followed by oral antivirals. Follow-up ranged from 6 to 16 months. Retinal detachment (RD) occurred in 3 eyes (25%), but no patient developed bilateral ARN. Final visual acuity was 20/200 or worse in 7 eyes (58.3%). Delay in diagnosis (p = 0.015), macular involvement (p = 0.045), development of RD (p = 0.018), and 25–50% of retinal involvement (p = 0.045) were associated with a worse visual outcome.Conclusion: ARN carries poor visual prognosis in Tunisia.

Summary Primary congenital glaucoma (PCG) is responsible for a significant proportion of childhood blindness in Tunisia. Early prevention based on genetic diagnosis is therefore required. This study sought to determine the frequency of CYP1B1 (cytochrome P450, family 1, subfamily B, polypeptide 1) mutations in 18 PCG patients, recruited from Central and Southern of Tunisia. Genomic DNA was extracted and the coding regions of CYP1B1 were analysed by direct sequencing. A phylogenetic network of CYP1B1 haplotypes was drawn using the median-joining algorithm. Sequence analysis revealed a “tetra-allelic mutation” (two novel mutations, p.F231I and p.P437A in the homozygous state) in one patient. The healthy members of his family carried those variations on the same allele. Two previously described mutations p.G61E and c.535delG were also identified in the homozygous state in seven and two probands, respectively. Seven single-nucleotide polymorphisms were identified and used to generate haplotypes. Our results showed that the CYP1B1 mutations were present in 55% of Tunisian PCG patients’ alleles. Haplotype analysis allowed us to define the proto-haplotype and to confirm historical migratory flows. Establishment of PCG genetic aetiology in Tunisia will improve genetic diagnosis and counselling.

Purpose: To characterize and analyze ocular involvement associated with visual loss in a cohort of patients with rickettsial disease.Methods: Retrospective study of 16 eyes of 14 patients.Results: Mean initial visual acuity (VA) was 20/63 (range, 20/800–20/25). White retinal lesions infiltrating inner retina was the most common finding occurring in 14 eyes (87.5%). It was associated with a serous retinal detachment (SRD), accurately detectable by optical coherence tomography, in 11 eyes (78.6%). Other findings included optic neuropathy in 7 eyes (43.75%), cystoid macular edema in 1 eye (6.25%), branch retinal artery occlusion in 1 eye (6.25%), and choroidal neovascularization in 1 eye (6.25%). Thirteen patients were treated with a 2-week course of oral doxycycline 200 mg/day. Mean final VA was 20/40.Conclusions: Inner retinitis, associated with mild vitritis and SRD, and optic neuropathy are the most common vision-threatening ocular manifestations of rickettsial disease.

Human herpesviruses (HHVs) are involved in the pathogenesis of different types of uveitis. Cytokine response plays an important role in virus-induced immunopathology. This study aimed to investigate the incidence of HHVs in aqueous humor samples of immunocompetent patients with suspected viral uveitis and cytokine (IL-10, IL-6, and IFN-γ) expression profiling. Forty-seven aqueous humor samples were collected from immunocompetent patients with viral uveitis. Samples were assayed for HHV-1 to HHV-8 by in-house real-time polymerase chain reactions. IL-6, IL-10, and IFN-γ were quantified with a cytometric bead array. Relations between viral detection, cytokine profiles, and clinical data were studied. At least one viral genome was detected in 21 aqueous humor samples analyzed. Varicella-zoster virus (VZV) was detected in 14 of the positive samples, cytomegalovirus (CMV) in 8, HSV-1 in 1, Epstein–Barr virus (EBV) in 4, and HHV-6 in 2. More than one viral genome was detected in seven aqueous humor samples. Aqueous humor samples positive for HHV-DNA contained significant levels of IL-6, IL-10, and IFN-γ, compared to HHV-DNA negative samples. High levels of IL-6 were detected in patients with CMV-DNA in their aqueous humor samples. Significantly higher levels of IL-10 and IFN-γ were found in positive samples for VZV, EBV, and HHV-6 DNA than in negative aqueous humor ones. VZV was the principal etiologic agent of uveitis in this Tunisian series, with CMV the second most common agent. Knowledge of immunoregulatory interactions and dynamic changes in viral uveitis may be a key to understand the pathogenesis leading to more-effective treatments. J. Med. Virol. 85:2079–2086, 2013. © 2013 Wiley Periodicals, Inc.

To report the results of multimodal imaging of acute outer retinitis associated to mumps infection. A patient with mumps-associated outer retinitis evaluated by color fundus photography, spectral domain optical coherence tomography (SD-OCT), optical coherence tomography angiography, fundus autofluorescence (FAF), fluorescein angiography (FA), and indocyanine green angiography (ICGA). We report a case of a 12-year-old boy who developed bilateral outer retinitis related to mumps. Ophthalmoscopy showed confluent areas of outer retinitis involving the posterior pole and the periphery with a centrifugal gyrate pattern. SD-OCT revealed a marked disorganization of the outer retinal layers with multiple highly reflective spicules. FA shows diffuse late hyperfluorescence with optic disk staining. ICGA shows macular and peripheral hyperfluorescent lesions with a geographical pattern in the late phases. The patient was treated with acyclovir and oral prednisone. Four weeks after presentation visual acuity remained unchanged, and retinal changes seen at the acute phase had resolved leading to extensive retinal atrophy and optic disk pallor. SD-OCT showed atrophy of the retinal pigment epithelial and outer retinal layers. FAF revealed scattered hyperautofluorescent lesions. Electrophysiology showed generalized retinal dysfunction. Mumps infection should be considered in the differential diagnosis of bilateral necrotizing outer retinitis in children and young adults. A multimodal imaging approach may help distinguish mumps-associated retinitis from other causes of viral retinitis and facilitate appropriate management.

Orbital infections (OI) are a serious disorder that may results in visual and systemic morbidity.To study epidemiologic data and outcome of OI in a tertiary eye care center.Retrospective study of 28 patients (28 eyes) with OI. All patients underwent detailed ophthalmic examination, otolaryngology examination, and cranio-orbital computed tomography scan. All patients received parenteral probabilistic antibiotic treatment then adapted depending on the sensitivity of antibiogram and clinical evolution, associated or not to corticosteroid treatment. The mean follow-up was 14 months.The mean age of our patients was 35.9 years. Fifteen patients (54%) were male. Of 28 cases, retroseptal cellulitis was the most common, noted in 67.8% of cases. subperiosteal or orbital abscess was recorded in 28.6% of the cases. Sinusitis was the most common etiology, found in 39.3% of cases. Treatment was based on empirical broad spectrum systemic antibiotics in all cases. Surgical management was performed in 39.3% of cases. Bacteriological samples were obtained for 28.5% of patients, with Staphylococcus aureus the most frequently isolated pathogen (5 cases; 62.5%). The evolution was favorable with complete resolution without sequelae in 92.8% of the cases and improvement of visual acuity in 82% of cases. Causes of poor visual outcome were corneal perforation, cataract and retinal detachment.In our series, OI affects children and young adults. Their prognosis is generally favorable provided rapid and appropriate management.

Rift Valley fever is an arthropod-borne viral disease caused by Bunyaviridae, primarily affecting domesticated cattle. It is transmitted to humans through the bite of infected mosquitoes or through direct contact with infected animals. Systemic involvement typically consists of febrile, influenza-like illness. Macular or paramacular retinitis is the most common ocular manifestation, and may lead to permanent visual impairment. The diagnosis is easy in the setting of an outbreak. Serologic tests may be used to detect specific antibodies.

Emergent and resurgent arthropod vector-borne diseases are major causes of systemic morbidity and death and expanding worldwide. Among them, viral and bacterial agents including West Nile virus, Dengue fever, Chikungunya, Rift Valley fever, and rickettsioses have been recently associated with an array of ocular manifestations. These include anterior uveitis, retinitis, chorioretinitis, retinal vasculitis and optic nerve involvement. Proper clinical diagnosis of any of these infectious diseases is based on epidemiological data, history, systemic symptoms and signs, and the pattern of ocular involvement. The diagnosis is usually confirmed by the detection of a specific antibody in serum. Ocular involvement associated with emergent infections usually has a self-limited course, but it can result in persistent visual impairment. There is currently no proven specific treatment for arboviral diseases, and therapy is mostly supportive. Vaccination for humans against these viruses is still in the research phase. Doxycycline is the treatment of choice for rickettsial diseases. Prevention, including public measures to reduce the number of mosquitoes and personal protection, remains the mainstay for arthropod vector disease control. Influenza A (H1N1) virus was responsible for a pandemic human influenza in 2009, and was recently associated with various posterior segment changes.

To analyze the pattern of intermediate uveitis (IU) in a referral center in Tunisia, North Africa. A retrospective, descriptive study of 87 consecutive patients (145 eyes) examined at the Department of Ophthalmology of Monastir (Tunisia) from January 1996 to August 2008. All patients underwent an extensive ocular and systemic history, a complete ophthalmic examination, and fluorescein angiography. Standard diagnostic criteria of IU were employed. The mean follow-up period was 43.2 months (range, 4-65 months). The mean age at diagnosis of IU was 29.1 years (range, 5-54 years). The male-to-female ratio was 1:1.6. Both eyes were affected in 58 patients (66.7%). The best-corrected visual acuity at onset of uveitis ranged from light perception to 20/20 (mean, 20/32). The most frequent ocular findings were vitritis (100%), snowballs (53.8%), and retinal vasculitis (28.3%). Concurrent anterior uveitis was noted in 38.6% of affected eyes. Intermediate uveitis was most commonly idiopathic (86.2%). Systemic associations were found in 13.8%; sarcoidosis (9.2%) and multiple sclerosis (2.3%) were the most common systemic diseases. The ocular complications that developed during the follow-up period included cystoid macular edema (39.3%), cataract (31.7%), inflammatory optic disc involvement (26.2%), and glaucoma or ocular hypertension (9%). One hundred-twelve affected eyes (77.2%) had a final visual acuity more than or equal to 20/40. Factors of poor visual outcome were initial VA less than 20/40 (P = 0.00011), CME (P = 0.0016), and vitritis more than 3+ cells (P = 0.023). In a hospital population in Tunisia, intermediate uveitis started frequently at the 3rd decade. This type of uveitis was most commonly idiopathic. Cystoid macular edema and cataract were the most frequent complications. Visual prognosis appeared to be fairly good.

The aim of this retrospective study is to assess the frequency of HLA-B27 and HLA-B51 in healthy subjects from the center of Tunisia and to investigate their usefulness in the diagnosis of ankylosing spondylitis (AS) and Behçet's disease (BD), respectively. Microlymphocytotoxicity test was used to perform serologic HLA typing in a group of 124 healthy volunteers and a group of 365 patients suffering from clinical manifestations of AS and/or BD. HLA-B27 was found in 3.2% of healthy subjects and in 42.9% of patients with AS (P0.00006). HLA-B51 is, however, found in 16.1% of healthy subjects and in 30.0% of patients with BD (P0.05). Unlike HLA-B51, which seems to be as frequent in Tunisian patients with BD as in healthy subjects, HLA-B27 is more frequent in patients with AS than in controls. This highlights the usefulness of HLA-B27, rather than that of HLA-B51, in the diagnosis of the respective diseases.

Purpose: To describe eight patients with active toxoplasmic retinochoroiditis (RC) who had features suggestive of acute choroidal ischemia. Methods: A retrospective review of the clinical records of 23 consecutive patients with acute toxoplasmic RC was performed. All patients underwent detailed ophthalmic examination at presentation and throughout follow-up, including dilated biomicroscopic fundus examination, fundus photography, fluorescein angiography, and indocyanine green (ICG) angiography. Results: Of 23 patients, 8 (34.8%) had a large area of retinal whitening surrounding a small focus of RC. Fluorescein as well as ICG angiography showed a well demarcated geographic area of early choroidal hypofluorescence that extended beyond the clinical borders of the white retinal lesion, particularly by ICG angiography. Associated findings for these 8 patients included old retinochoroidal scars (7 [87.5%]), serous retinal detachment (3 [37.5%]), retinal hemorrhages (1 [12.5%]), and multiple satellite dark dots by ICG angiography (6 [75%]). Seven of eight patients were treated using a combination of antitoxoplasmic drugs and corticosteroids. All findings seen at the acute stage resolved in 2 weeks to 6 weeks. A small atrophic retinochoroidal scar replaced the active toxoplasmic lesion and was surrounded with mild or moderate retinal pigment epithelium changes that were associated with decreased final visual acuity in 2 patients (25%). Conclusion: Patients with toxoplasmic RC may develop features suggestive of choroidal ischemia that can result in a transient or permanent decrease in vision. Choroidal ischemia can only be suspected clinically, and fluorescein angiography and ICG angiography are required to establish the definitive diagnosis.

Objective To analyze the clinical profile of Vogt-Koyanagi-Harada (VKH) disease in Tunisia, North Africa. Methods We retrospectively reviewed the clinical records of 49 patients diagnosed with VKH disease at the Department of Ophthalmology of Fattouma Bourguiba University Hospital, Monastir, Tunisia, between January 1994 and September 2005. Results Of all the uveitis cases diagnosed during the study period, VKH disease was the fourth most commonly occurring type (7.4%). Thirty-two patients (65.3%) were female, and 17 patients (34.7%) were male. The mean age at disease onset was 35 years (range: 16–54 years). The majority of patients (51%) had probable VKH disease, followed by incomplete type (47%); only 2% had the complete type. At presentation, 47 patients (96%) had bilateral ocular involvement. Clinical presentation was a panuveitis in 25 patients (51%) and a posterior uveitis in 24 patients (49%). Visual acuity (VA) at presentation ranged from less than 20/200 (40.8%) to more than 20/40 (28.5%). All patients were treated with systemic corticosteroids for 2–19 months (mean: 10.5 months). Four patients (8%) were treated with cyclosporin because of serious systemic side effects of corticosteroids. Complications included cataract in 18 eyes (33.9%), glaucoma in nine eyes (16.96%) and choroidal neovascularization in one eye (1%). Fifty-eight eyes (59%) had a final VA of 20/40 or better. Factors associated with a poor VA at the final follow-up were the presence of a poor VA at presentation (P = 0.02), the occurrence of complications (P = 0.001) and/or recurrences (P = 0.02). Conclusion In Tunisia, VKH disease is a common cause of uveitis that predominantly affects young women. The overwhelming majority (98%) have probable or incomplete VKH disease, presenting as panuveitis or posterior uveitis. More than 50% of patients undergoing treatment with corticosteroids will maintain a VA of 20/40 or better.

Marshall syndrome is a rare autosomal dominant skeletal dysplasia. It associates a particular facial dysmorphism with midface hypoplasia, ocular abnormalities and sensorineural hearing loss. It is caused by heterozygous mutations in COL11A1 gene coding the 1 chain of collagen XI. Stickler syndrome is the principal differential diagnosis of Marshall syndrome.Clinical and radiological study of Marshall syndrome in a Tunisian family with a linkage study of the COL11A1 gene to this disease.We report the clinical and the radiological findings of a Tunisian family including 8 members affected by Marshall syndrome. The linkage of the COL11A1 gene to this disease was tested using the polymorphic microsatellite markers of DNA.A variability of the clinical expression of Marshall syndrome was reported. Specific Marshall phenotype and an overlapping phenotype between the Marshall and Stickler syndromes were observed among the affected members of this family. The ocular manifestations were also heterogeneous. Marshall syndrome's specific radiological signs were found. The linkage study supports the linkage of the abnormal phenotype to the COL11A1 gene.There is a variability of the clinical expression among the affected members of the study's family. We will continue searching the causative mutation to establish a clear genotype- phenotype correlation.

International audience; Usher syndrome (USH) is an autosomal recessive disorder characterized by combined deafness-blindness. It accounts for about 50% of all hereditary deafness blindness cases. Three clinical subtypes (USH1, USH2, and USH3) are described, of which USH1 is the most severe form, characterized by congenital profound deafness, constant vestibular dysfunction, and a prepubertal onset of retinitis pigmentosa. We performed whole exome sequencing in four unrelated Tunisian patients affected by apparently isolated, congenital profound deafness, with reportedly normal ocular fundus examination. Four biallelic mutations were identified in two USH1 genes: a splice acceptor site mutation, c.2283-1G>T, and a novel missense mutation, c.5434G>A (p.Glu1812Lys), in MYO7A, and two previously unreported mutations in USH1G, i.e. a frameshift mutation, c.1195_1196delAG (p.Leu399Alafs*24), and a nonsense mutation, c.52A>T (p.Lys18*). Another ophthalmological examination including optical coherence tomography actually showed the presence of retinitis pigmentosa in all the patients. Our findings provide evidence that USH is under-diagnosed in Tunisian deaf patients. Yet, early diagnosis of USH is of utmost importance because these patients should undergo cochlear implant surgery in early childhood, in anticipation of the visual loss.

Purpose To characterize and analyze the chorioretinal manifestations of West Nile virus (WNV) infection. Design Prospective, noncomparative case series. Participants Twenty-nine consecutive patients with serologically confirmed WNV infection in the setting of an outbreak of the disease. Methods The average duration of systemic symptoms before ophthalmic examination was 10 days (range, 2–30 days). All participants underwent a detailed ophthalmic examination at presentation and regularly throughout follow-up, including dilated biomicroscopic fundus examination, fundus photography, and fluorescein angiography. Results A typical multifocal chorioretinitis was observed in 20 of 29 patients (69%) bilaterally at presentation and developed later during follow-up in 3 patients (10.3%), bilaterally (n = 1) or unilaterally (n = 2). Multifocal chorioretinitis was associated with mild vitreous inflammatory reaction in all cases. Other findings included intraretinal hemorrhages (21 patients [72.4%]), white-centered retinal hemorrhages (7 patients [24.1%]), focal retinal vascular sheathing (3 patients [10.4%]), marked diffuse retinal arterial sheathing (1 patient [3.4%]), retinal vascular leakage (5 patients [17.2%]), optic disc swelling (2 patients [6.9%]), optic disc staining (6 patients [20.7%]), segmental zones of retinal pigment epithelium changes (1 patient [3.4%]), and nonproliferative diabetic retinopathy (7 patients [24.1%]). The posterior segment findings related to WNV disease had a self-limited course in all patients. Conclusions Chorioretinal involvement, frequently asymptomatic and self-limited, is common in patients with acute WNV infection. The unique pattern of multifocal chorioretinitis in patients with systemic symptoms suggestive of WNV can help to establish the diagnosis while serologic testing is pending. Therefore, a systematic ocular evaluation, including dilated fundus examination and fluorescein angiography in selected cases, is recommended in patients with clinically suspected WNV infection.

Purpose To characterize and analyze the posterior segment manifestations of Mediterranean spotted fever (MSF), an infectious disease caused by Rickettsia conorii . Design Prospective, noncomparative case series. Participants Thirty patients (60 eyes) with serologically proven MSF at the acute stage. Methods Patients underwent complete ophthalmic examination, including dilated biomicroscopic fundus examination, fundus photography, and fluorescein angiography. Sequential follow-up examinations were performed in patients with evidence of posterior segment involvement. Results Of 30 patients, 25 (83.3%) had unilateral (n = 5) or bilateral (n = 20) posterior segment involvement related to MSF. Of those 25 patients, 16 (64%) had no ocular symptoms, and 9 (36%) had ocular complaints. Findings included mild vitreous inflammation (45 eyes [75%]), white retinal lesions (18 eyes [30%]), focal vascular sheathing (5 eyes [8.3%]), multiple arterial plaques (1 eye [1.7%]), intraretinal hemorrhages (14 eyes [23.3%]), white-centered retinal hemorrhages (2 eyes [3.3%]), subretinal hemorrhages (2 eyes [3.3%]), serous retinal detachment (3 eyes [5%]), macular star (2 eyes [3.3%]), cystoid macular edema (1 eye [1.7%]), optic disc edema (1 eye [1.7%]), branch retinal artery occlusion (1 eye [1.7%]), optic disc staining (30 eyes [50%]), retinal vascular leakage (27 eyes [45%]), delayed filling in a branch retinal vein (1 eye [1.7%]), and multiple hypofluorescent choroidal dots (10 eyes [16.7%]). One eye (1.7%) had retinal neovascularization at the 6-month follow-up examination. All posterior segment findings at the acute stage resolved in 3 to 10 weeks, and the final visual acuity was 20/20 in 42 of 45 affected eyes (93.3%). Retinal pigment epithelium changes developed in 9 eyes (15%), with resolved full-thickness white retinal lesions. No other abnormalities were noted in the eye with retinal neovascularization over a further follow-up of 6 months. Conclusion Posterior segment involvement, frequently asymptomatic, is common in patients with acute MSF. Because the diagnosis can be easily overlooked, a careful dilated funduscopic examination, complemented by fluorescein angiography in selected cases, is recommended. Mild vitritis, retinal vasculitis, optic disc staining, white retinal lesions, retinal hemorrhages, and multiple hypofluorescent choroidal dots are the most common manifestations of MSF. Posterior segment changes in a patient with fever and/or skin rash living in or returning from a specific endemic area, especially during the spring or summer, strongly suggest R. conorii infection.

Purpose: To report 2 cases of branch retinal artery occlusion (BRAO) associated with Behcet disease (BD).Design: Small case series.Methods: Review of two patients’ charts, fluorescein angiography, and optical coherence tomography (OCT).Results: The authors report two young patients, diagnosed with BD, who developed decreased vision secondary to BRAO for the first patient and cilioretinal artery occlusion associated with branch retinal vein occlusion for the second patient. In the two cases, artery occlusion was associated with active intraocular inflammation.Conclusions: BRAO, with subsequent transient or permanent visual impairment, is an additional ocular complication of BD.

The purpose of this study was to describe the clinical and multimodal imaging findings in acute Vogt–Koyanagi–Harada (VKH) disease without clinically evident exudative retinal detachment (ERD). We retrospectively reviewed the charts of 18 patients (36 eyes), diagnosed with acute VKH disease without clinically evident ERD. All patients underwent complete ophthalmic examination, fundus photography, optical coherence tomography (OCT), B-scan ultrasonography, fluorescein angiography (FA), and indocyanine green angiography (ICGA). Of 18 patients, twelve (66.7 %) were female and 6 (33.3 %) were male. Mean age was 39 years (range, 23–60). Ten patients had been referred with an erroneous diagnosis of primary optic nerve disorder (8; 44.4 %) or isolated anterior uveitis (2; 11.1 %). Anterior chamber or vitreous inflammatory reaction was noted in 22 eyes (61.1 %), each. Fundus findings included optic disc swelling in 30 eyes (83.3 %), retinal striae in 20 eyes (55.5 %), and yellowish deep lesions in 3 eyes (8.3 %). OCT showed a shallow, localized subclinical ERD in 18 eyes (50 %), and retinal pigment epithelial folds in 23 eyes (63.9 %). B-scan ultrasonography showed diffuse, low- to medium-reflective choroidal thickening in all eyes. FA disclosed delayed choroidal perfusion in at least one eye of all patients (100 %), mild pinpoint leakage in 21 eyes (58.3 %), optic disc hyperfluorescence in 35 eyes (97.2 %) and choroidal folds in 13 eyes (36.1 %). ICGA findings included delayed choroidal perfusion in 24 eyes (66.7 %), decrease in the number of large choroidal vessels in 36 eyes (100 %), fuzzy choroidal vessels in 35 eyes (97.2 %), and hypofluorescent dark dots in 28 eyes (77.8 %). The association of bilateral optic disc edema with retinal striae and intraocular inflammatory reaction highly suggests acute VKH disease. A multimodal imaging approach including fundus photography, OCT, B-scan ultrasonography, FA, and ICGA provides important clues for the definite diagnosis and help differentiate VKH disease from primary optic nerve disorders.

International audience; Primary congenital glaucoma (PCG) is responsible for a significant proportion of childhood blindness in Tunisia. Early prevention based on genetic diagnosis is therefore required. This study sought to determine the frequency of CYP1B1 (cytochrome P450, family 1, subfamily B, polypeptide 1) mutations in 18 PCG patients, recruited from Central and Southern of Tunisia. Genomic DNA was extracted and the coding regions of CYP1B1 were analysed by direct sequencing. A phylogenetic network of CYP1B1 haplotypes was drawn using the median-joining algorithm. Sequence analysis revealed a "tetra-allelic mutation" (two novel mutations, p.F231I and p.P437A in the homozygous state) in one patient. The healthy members of his family carried those variations on the same allele. Two previously described mutations p.G61E and c.535delG were also identified in the homozygous state in seven and two probands, respectively. Seven single-nucleotide polymorphisms were identified and used to generate haplotypes. Our results showed that the CYP1B1 mutations were present in 55% of Tunisian PCG patients' alleles. Haplotype analysis allowed us to define the proto-haplotype and to confirm historical migratory flows. Establishment of PCG genetic aetiology in Tunisia will improve genetic diagnosis and counselling.

We report a case of a 14-year-old otherwise healthy patient who developed acute retinal periphlebitis mimicking frosted branch angiitis inferotemporally and associated exudative retinal detachment in the left eye following blunt trauma. Fluorescein angiography revealed delayed filling of inferotemporal branch retinal vein and late leakage of sheathed retinal venules, and late pooling in the area of exudative retinal detachment. Indocyanine green angiography showed a crescent-shaped hypofluorescent streak concentric to the optic disk inferiorly highly suggestive of choroidal rupture. The patient was treated with oral prednisone, with gradual tapering over a period of 15 days. One month after presentation, retinal vein sheathing and exudative retinal detachment had resolved, with the development of peripapillary subretinal fibrosis, macular atrophy, pseudomacular hole, and epiretinal membrane. The acute perivenular sheathing in our patient might be related to autoimmune-mediated reaction induced by retinal vascular damage caused by severe ocular trauma. Fluorescein angiography and indocyanine green angiography findings might suggest that the retinal detachment could be caused by leakage from choroid through Bruch’s membrane and retinal pigment epithelium rupture or by transient dysfunction of the outer or inner blood-retinal barrier.

Arthropod vector-borne diseases are among the most important emergent and resurgent infections. They are transmitted to humans by the bite of mosquito, tick, or other arthropod. Most of them are prevalent in warm regions, but they tend to spread into new regions around the world mainly due to climate changes and globalization. Most human infections are subclinical or manifest as a mild febrile illness, but a severe, potentially lethal systemic involvement can occur. Several emergent infections including West Nile virus infection, dengue fever, Chikungunya, Rift Valley fever, and rickettsioses have been recently associated with an array of ocular manifestations. These include anterior uveitis, retinitis, chorioretinitis, retinal vasculitis, and optic nerve involvement. Diagnosis of any of these infectious diseases is primarily based on epidemiological data, history, systemic symptoms and signs, and the pattern of ocular involvement. The diagnosis is usually confirmed by serology and/or detection of the causative agent.

Herein a case of a 35-year-old woman with a history of Behcet's disease, who presented with swelling and redness of her right eye with increasing pain, is reported. Computed tomography and magnetic resonance imaging showed enlargement of the right lacrimal gland and contrast enhancement of the extraocular muscles. A diagnosis of orbital inflammation was made and the patient was treated with corticosteroids, with prompt resolution of symptoms and clinical signs. Orbital inflammation should be considered as an ophthalmic manifestation of Behcet's disease.

A 60-year-old man with diabetes mellitus had a sudden decrease in vision in his right eye 3 weeks after confirmed West Nile virus infection. Visual acuity in the right eye was 20/400. Fundus examination showed bilateral multifocal chorioretinitis, which was associated with proliferative diabetic retinopathy in the right eye and severe nonproliferative diabetic retinopathy in the left eye. There were deep, dense retinal hemorrhages, retinal opacification, and retinal arterial sheathing in the macula of the right eye. Fluorescein angiography revealed extensive capillary nonperfusion in the macular area of the right eye. Six months later, vision remained unchanged and a choroidal neovascularization developed over a chorioretinal scar in the same eye. [Ophthalmic Surg Lasers Imaging 2006;37:240-242.] AUTHORS From the Department of Ophthalmology, Fattouma Bourguiba University Hospital, Monastir, Tunisia. Accepted for publication December 4, 2005. Address reprint requests to Moncef Khairallah, MD, Department of Ophthalmology, Fattouma Bourguiba University Hospital, 5019 Monastir, Tunisia.

The aim of this study was to evaluate and compare 10-MHz and 20-MHz ultrasonography in the assessment of patients with optic nerve head drusen (ONHD). The design of the study was prospective, comparative and cross-sectional. Ultrasonographic examination with a 10 and 20 MHz probe was performed in 45 eyes with suspected ONHD. The 20 MHz probe showed drusen in 43 eyes (95.5 %), while the 10 MHz probe revealed drusen in only 33 eyes (73.3 %, p = 0.0001). The 10 MHz probe showed surface drusen in 10 eyes (22.2 %), while the 20 MHz probe showed surface drusen in 14 eyes (31.1 %) (sensitivity 71.4 %; 95 % CI [47.6–95.1 %]). The 10 MHz probe showed buried drusen in 23 eyes (23.1 %), while the 20 MHz probe showed buried drusen in 29 eyes (64.4 %) (sensitivity 79.3 %; 95 % CI [56.6–86.2 %]). The sensitivity was 76.7 % with 10 MHz probe compared to a 20 MHz as gold standard. The use of 20 MHz probe increased the sensitivity of buried disc drusen by 1.5 times and surface disc drusen by nearly 2 times. Using the 10 MHz probe alone the false negative error rate was 83.3 %. The 20 MHz probe has shown itself to be an excellent method for the diagnosis of ONHD; it is more sensitive and reliable than 10 MHz probe and should be considered in the management of patients with clinical evidence of ONHD.

Serum soluble receptor for advanced glycation end product (sRAGE) may reflect the activity of the advanced glycation end product (AGE)–receptor for advanced glycation end product (RAGE) axis, which has been proposed as a potential mechanism linking hyperglycaemia to vascular complications in diabetes. We have investigated whether serum AGEs, sRAGE and pentosidine levels were increased and correlated with microvascular complications in type 2 diabetes mellitus (DM). We included 30 healthy control subjects, and 200 diabetic patients were divided into two subgroups: 100 patients with diabetic retinopathy and 100 patients with diabetic nephropathy. AGEs, sRAGE and pentosidine were measured in serum by enzyme-linked immunosorbent assay (ELISA). Serum AGEs, sRAGE and pentosidine levels were significantly increased in diabetic patients with retinopathy and in diabetic patients with nephropathy compared to control subjects ( p < 0.001). Serum AGEs, sRAGE and pentosidine levels are positively associated with microvascular complications in type 2 DM. Multiple regression analysis reveals serum pentosidine as an independent determinant of the presence of diabetic retinopathy ( p = 0.004) and the presence of hypertension ( p = 0.018) and hyperlipidaemia ( p = 0.036). Pentosidine levels may be a biomarker for microvascular complications in type 2 diabetic patients.

Purpose The purpose of this study is to assess macular involvement in patients with Behçet’s uveitis. Methods The study included 65 patients (120 eyes) with Behçet’s uveitis. All patients underwent detailed ophthalmic examination, including dilated biomicroscopic fundus examination, fundus photography, fluorescein angiography, and optical coherence tomography. Follow-up ranged from 6 to 46 months (mean 20 months). Results At initial examination, 29 eyes (24.1 %) had macular involvement including macular edema (16 eyes, 13.3 %), serous retinal detachment (SRD; five eyes, 4.1 %), active retinitis (three eyes, 2.5 %), macular hole (three eyes, 2.5 %), macular atrophy (two eyes, 1.6 %), macular ischemia (one eye, 0.8 %), epiretinal membrane (one eye, 0.8 %), branch retinal vein occlusion involving the macula (three eyes, 2.5 %), and branch retinal artery occlusion involving the macula (two eyes, 1.6 %). During follow-up, 22 eyes (18.3 %) developed macular complications including macular edema (ten eyes, 8.3 %), SRD (four eyes, 3.3 %), active retinitis (two eyes, 1.6 %), severe macular atrophy (two eyes, 1.6 %), macular ischemia (three eyes, 2.5 %), macular hole (one eye, 0.8 %), epiretinal membrane (two eyes, 1.6 %), and subretinal fibrosis (one eye, 0.8 %). Branch retinal vein occlusion involving the macula developed in two eyes (1.6 %). Final best corrected visual acuity in patients with macular involvement ranged from 20/400 to 20/25 (mean 20/80). Conclusions Macular edema and other vision-threatening macular complications are common in Behçet’s uveitis. Macular damage is often irreversible, causing permanent visual impairment. Early and appropriate treatment of Behçet’s uveitis is mandatory to reduce the risk of visual impairment due to macular involvement.

Purpose: To describe the clinical, optical coherence tomographic, and angiographic findings in patients with acute toxoplasmic retinochoroiditis (RC) associated with serous retinal detachment (SRD). Methods: The study included 60 eyes with acute toxoplasmic RC. Results: Of 60 eyes, 14 (23.3%) were found to have SRD. The SRD was visible on fundus examination in 6 cases and detectable only by optical coherence tomography (OCT) in the 8 remaining cases. It involved the fovea in 9 eyes. There was evidence of associated choroidal ischemia on fluorescein angiography and indocyanine green angiography in 5 eyes. Findings seen at the acute stage gradually resolved over a period of 2–6 weeks in all patients. Conclusions: SRD, accurately detected by OCT, is a common complication of acute toxoplasmic RC that should be considered as a potential cause of visual loss. Choroidal ischemia might contribute to the development of such complication.

To evaluate the efficacy and safety of supratarsal injection of triamcinolone acetonide in the treatment of refractory allergic keratoconjunctivitis in childhood. Thirty-five patients (70 eyes) with severe allergic keratoconjunctivitis were included in this study. Fifteen patients (42.8%) had atopic keratoconjunctivitis (AKC) and 20 (57.2%) had vernal keratoconjuncivitis (VKC). All patients underwent a bilateral supratarsal injection of 20 mg triamcinolone acetonide. Mean follow-up was 28 months (range 14–38). All patients showed a significant symptomatic improvement and partial resolution of clinical features of keratoconjunctivitis within the first 2 weeks. Corneal complications related to keratoconjunctivitis disappeared in all patients. One patient with VKC experienced ocular hypertension. No patient with AKC showed side-effects. Severe relapse of the disease was noted in 10% of eyes with VKC and in 6.7% of eyes with AKC after a mean follow-up of 9 and 11 months, respectively. Supratarsal injection of triamcinolone acetonide is effective and safe in patients with severe chronic allergic keratoconjunctivits to relieve severe inflammation associated with this disease.

Microspherophakia seems to be the most specific feature of the Weill-Marchesani Syndrome, which could be due to mutations within the ADAMTS10 gene. As the locus responsible for isolated microspherophakia is still unknown, because the reported cases are rare, we checked whether the ADAMTS10 gene is involved in isolated microspherophakia in a Tunisian family. A consanguineous family (MSP-M), including six family members and two patients, presented with decreased vision secondary to bilateral isolated microspherophakia. A linkage analysis was carried out using microsatellite markers flanking the ADAMTS10 candidate gene. In the MSP-M family, isolated microspherophakia is likely inherited as an autosomal-recessive disease. Using a homozygosity-mapping strategy, haplotypic analysis using four STRs showed an exclusion of linkage between the ADAMTS10 gene and the disease locus in this family. Our study suggests that isolated microspherophakia and the Weill-Marchesani Syndrome are not allelic to the ADAMTS10 gene.

Background Treatment modalities for choroidal neovascularization due to ocular toxoplasmosis include laser photocoagulation, surgery, corticosteroids, and verteporfin therapy. Intravitreal injection of bevacizumab in the treatment of choroidal neovascularization due to other conditions appears to be an effective and safe therapeutic option. Case reports We report two young patients with choroidal neovascularization secondary to ocular toxoplasmosis who received a single intravitreal injection of bevacizumab as primary or rescue therapy. After a follow-up of 12 and 10 months, respectively, visual acuity improved, and features of active neovascularization resolved with no recurrence. No adverse events were recorded. Conclusion Intravitreal injection of bevacizumab appears to be an effective and safe treatment modality in patients with choroidal neovascularization secondary to ocular toxoplasmosis. Further evaluation with a longer follow-up period is needed to confirm these findings.

Purpose: To identify factors associated with the development and severity of chorioretinitis (CR) in patients with West Nile virus (WNV) infection. Methods: Prospective study of 38 patients with WNV infection. Results: Simple analysis showed that diabetes (p = 0.027) and age older than 45 years (p = 0.03) were significantly associated with CR. When controlling for age, only association between diabetes and CR remains statistically significant. Diabetic patients' eyes were more likely to have macular involvement (p < 0.0001), 20 or more chorioretinal lesions (p < 0.001), and lesion size 500 μ m or more (p < 0.01). Conclusions: Diabetes was a risk factor for the development and severity of WNV-associated CR.

The purpose of this study was to analyze the pattern of uveitis in Behcet’s disease in Tunisia, North Africa. We retrospectively reviewed the clinical records of 62 patients (111 eyes) diagnosed with Behcet’s uveitis (BU) at the Department of Ophthalmology, Fattouma Bourguiba University Hospital, Monastir, Tunisia, between January 1995 and December 2006. Forty-seven of 62 patients (75.8%) were men and 15 (24.2%) were women. Mean age at onset was 29.3 years. The average follow-up was 75.6 months. The most common extraocular clinical manifestations were recurrent oral ulcer in all patients and pseudofolliculitis in 52 patients (83.9%). Uveitis was bilateral in 79%. Initial best-corrected visual acuity was ≥20/40 in 41 affected eyes (36.9%) and

Case report: An 11-year-old girl diagnosed with Fanconi anemia was referred to us for redness and pain in her right eye. Findings in the right eye included visual acuity of counting fingers, neovascular glaucoma, vitreous hemorrhage, optic disc neovascularization, and features of peripheral ischemic retinopathy. Findings in the left eye included peripheral retinal neovascularization and areas of retinal capillary nonperfusion. Comments: Patients with Fanconi anemia may develop ocular neovascularization with subsequent severe visual loss due to vitreous hemorrhage or neovascular glaucoma. Regular ophthalmic examination, including ophthalmoscopy and fluorescein angiography in selected cases, is recommended in such patients.

Purpose: To describe a case of orbital sarcoidosis manifesting with enophthalmos. Methods: A 60-year-old woman presented with right enophthalmos. She had a palpable mass in her anterior orbit inferiorly. She underwent a complete ophthalmic evaluation, magnetic resonance imaging, and histopathologic examination. Results: A magnetic resonance imaging scan disclosed an orbital, extraconal mass, with displacement of the orbital floor. A biopsy specimen of the mass demonstrated a granulomatous inflammation without caseation necrosis. Workup revealed hilar lymphadenopathy, pleural nodule, and splenomegaly. The patient was treated with corticosteroids, with subsequent involution of the mass and disappearance of enophthalmos. Conclusion: Although orbital sarcoidosis usually manifests with proptosis or palpebral swelling, enophthalmos should be considered as ophthalmic manifestation of sarcoidosis.

To analyze the pattern of childhood uveitis in a referral center in Tunisia, North Africa.The study included 64 patients with uveitis examined at the Department of Ophthalmology of Monastir (Tunisia) from January 1994 to July 2005. All patients had a comprehensive ocular and systemic history, including an extensive review of medical systems. Complete ophthalmic examination was performed in all cases, including best-corrected Snellen visual acuity, slit-lamp examination, applanation tonometry, and dilated fundus examination with 3-mirror lens. Standard diagnostic criteria were employed for all syndromes or entities of uveitis.The mean age at onset of uveitis was 12.4 years. The male-to-female ratio was 0.68. The process was unilateral in 51.6% of patients. Mean follow-up was 43.2 months. Anterior and intermediate uveitis each represented 31.25% of cases, posterior uveitis 20.3%, and panuveitis 17.2%. Noninfectious uveitis (75%) was the most frequent type of inflammation. Idiopathic uveitis was found in 50% of patients. Infectious uveitis was responsible for 25% of the cases, with toxoplasmosis (14.1%) being the most frequent cause. Twenty percent of the patients had systemic associations; juvenile idiopathic arthritis was found in 6.25%. Ocular complications occurred in 74.7% of affected eyes, of which the most common were posterior synechiae (28.4%), cataract (17.9%), cystoid macular edema (19%), and optic disc edema (32.6%). Fifty-seven affected eyes (60%) had a final visual acuity more than 20/40 and nine (9.5%) had a final visual acuity less than 20/200.In a hospital population in Tunisia, a specific cause of uveitis in children was found in half the patients. Idiopathic intermediate uveitis was the leading cause of uveitis in our study, followed by idiopathic anterior uveitis and toxoplasmosis. Uveitis associated with juvenile idiopathic arthritis was rare. Visual prognosis appeared to be good.