Atypical presentation of bilateral iridoschisis with abnormal visibility of iris vessels: Role of anterior segment optical coherence tomography.

PURPOSE: To describe clinical and anterior segment optical coherence tomography (AS-OCT) findings in a patient with bilateral iridoschisis and unilateral angle closure glaucoma (ACG) associated with abnormal visibility of iris vessels. CASE PRESENTATION: A 67-year-old male patient with a history of red and painful left eye (LE) one year earlier, presented to our ophthalmology department for a routine examination.Ophthalmic examination of the right eye revealed narrow anterior chamber with sectorial iris atrophy associated to abnormal visibility of an iris vessel. Intraocular pressure (IOP) was 12 mmHg with normal optic disc appearance. LE anterior chamber was narrow with diffuse iris atrophy and abnormal vessels visibility. IOP was 28 mmHg with an important optic disc excavation. On gonioscopy, angle was narrow without neovessels nor synechiae. AS-OCT of both eyes revealed shallow angles, iris splitting with material release in the anterior chamber, while pigmented epithelium was preservedAnti-glaucoma eye drops were prescribed and peripheral laser iridotomy was performed in both eyes with decreased IOP at 14 mmHg in the LE. CONCLUSION: Iridoschisis is a rare ocular condition characterized by a separation between the anterior and posterior layers of iris stroma with several clinical presentations, and may be associated with abnormal visibility of iris vessels in some cases. The diagnosis of iridoschisis may be challenging and AS-OCT can be a very useful tool to confirm the diagnosis in atypical presentations and to detect associated angle closure.

Choroidal features in non-neovascular and neovascular pachychoroid diseases.

PURPOSE: To evaluate choroidal findings on multimodal imaging in eyes within pachychoroid spectrum diseases and to compare quantitative and qualitative choroidal features between non-neovascular (NNV-PDS) and neovascular (NV-PDS) pachychoroid diseases. METHODS: Retrospective cross-sectional study comparing between NV-PDS and NNV-PDS. All patients underwent multimodal imaging including infracyanine green angiography (IFCGA) and swept source optical coherence tomography (OCT) and angiography (OCT-A). The following parameters were analyzed: subfoveal choroidal thickness (SFCT), choroidal vascular index (CVI), presence of pachyvessels and choroidal vascular interconnections (CVIC), presence of choroidal neovascularization and choriocapillaris density. RESULTS: Of the 87 eyes included in the study, 63 eyes (73%) had NNV-PDS and 24 eyes (27%) had NV-PDS. Mean SFCT and CVI were significantly higher in NNV-PDS group (p = 0.01; p = 0.022). Pachyvessels were more diffusely distributed in NNV-PD group and more focally distributed in NV-PDS group (p = 0.029). CVIC were more frequently noted in NV-PDS group (p = 0.024). A higher CVI was associated to a thicker choroid (p < 0.001), with significant negative correlations between the presence of CVIC and both SFCT (p = 0.015) and CVI (p = 0.002). We also observed a lower choriocapillaris vascular density and higher number of choriocapillaris flow voids in eyes with NNV-PDS (p = 0.24; p = 0.61). CONCLUSION: NNV-PDS eyes had a significantly thicker SFCT, higher CVI and a lower rate of detected CVIC than eyes with NV-PDS. These highlighted choroidal vascular changes might lead to a better understanding of pachychoroid disease pathophysiology. More frequently observed in NV-PDS group, CVIC are believed to assess the development of vortex vein anastomoses as a remodelling process for vascular decongestion.

Optic disc and choroidal metastases secondary to breast cancer: A case report.

AIM: To report an uncommon case of optic disc and multiple choroidal metastases secondary to breast cancer, assessed with swept source optical coherence tomography (SS-OCT), fluorescein (FA), and infracyanine (ICGA) angiographies. METHODS: Observational case report. CASE PRESENTATION: A 40-year-old woman with history of left breast carcinoma presented with blurred vision in her right eye (RE). Her visual acuity was 1/20 in the RE and 10/10 in the left eye. Fundus examination of the RE showed a large yellowish elevation of the posterior pole and a particular whitish nodular papillary cluster protruding from the optic disc into the vitreous. Infrared imaging enhanced the papillary nodular infiltrates. Characteristic findings of choroidal metastasis were noted within the macular lesion on SS-OCT and ICGA. SS-OCT showed specific "lumpy bumpy" irregularity of the anterior surface of the choroid and elevated hyperreflective nodular lesions of the optic disc associated to peripapillary subretinal fluid. The papillary lesions appeared as a bunch of hypofluorescent dots on both FA and ICGA, and ultra-wide field FA was helpful clearly delimiting the large macular lesion. Besides, comprehensive imaging and especially ICGA could detect two asymptomatic choroidal metastases in a systematic assessment of the fellow eye. CONCLUSION: Optic disc metastases are extremely rare. Their diagnosis can be easily done on fundus examination when presenting with characteristic whitish cluster nodular infiltrates of the optic disc. However multimodal imaging remains very useful for the assessment of the local extension of the lesion and for diagnosing associated asymptomatic choroidal lesions gone unnoticed at the fundus examination.

Multimodal imaging of bilateral immunogammopathy maculopathy associated to Waldenström's macroglobulinemia.

AIM: Our aim is to report a case with bilateral Waldenström's macroglobulinemia (WM) associated maculopathy, assessed with multimodal imaging including swept source optical coherence tomography (SS-OCT) and OCT-Angiography (OCT-A). METHODS: Observational case report. CASE PRESENTATION: A 61-year-old diabetic woman with history of treated WM currently in remission, presented with progressive bilateral visual loss. Best-corrected visual acuity was 20/100 in the right eye (RE) and 20/200 in the left eye (LE). Fundus examination showed bilateral microaneurysms and retinal punctuate hemorrhages and a large macular serous detachment in the LE. There was no retinal ischemia on FA nor macular dye leakage. SS-OCT showed a significant schisis-like intraretinal fluid accumulation in the RE and a large prominent macular detachment with significant subretinal fluid accumulation in the LE. Retinal and choriocapillaris vascular densities were normal on OCT-A. CONCLUSION: Our case illustrated characteristic multimodal imaging findings in WM associated maculopathy such as schisis-like intraretinal fluid accumulation and angiographically silent serous macular detachment. OCT-A could non-invasively analyze macular vascular densities layer-by-layer, without noticing any vascular anomaly.

Case Report: Swept-source Optical Coherence Tomography and Angiography Findings in Lipemia Retinalis.

SIGNIFICANCE: Lipemia retinalis is a very rare ocular manifestation of severe hypertriglyceridemia. It is usually symptomatic and regresses after normalization of triglycerides levels. Early recognition is important to prevent ocular and life-threatening complications. PURPOSE: This study aimed to report a case of marked lipemia retinalis secondary to type V hypertriglyceridemia assessed with swept-source optical coherence tomography (OCT) and OCT angiography (OCT-A), with follow-up after dietary lipid restriction. METHODS: Observational case report of lipemia retinal findings on color fundus photography, swept-source OCT and OCT-A, initially and after triglycerides lowering. CASE REPORT: A 32-year-old pregnant patient with gestational diabetes and a history of hypertriglyceridemia was referred for diabetic retinopathy screening. Fundus examination revealed bilateral milky-white discoloration of retinal vessels with a "salmon-colored" retina. Swept-source OCT and OCT-A revealed extremely hyperreflective and dilated retinal vessels and multiple high-flow retinal hyperreflective dots, corresponding to dilated retinal capillaries. Choroidal vessels were enlarged and engorged, and choriocapillaris layer appeared thickened and hyperreflective with dilated and tortuous capillaries. Serum triglycerides were very high (70.02 mmol/L). After 21 days of very-low-fat diet, it was lowered to 15 mmol/L. We noted a normalization of the clinical, structural, and vascular findings. However, peripheral retinal vessels remained hyperreflective, despite their clinical normalization. CONCLUSIONS: Swept-source OCT and OCT-A were beneficial in assessing lipemia retinalis noninvasively and monitoring choroidal and retinal vascular changes. Lipemia retinalis signs regressed initially in the posterior pole, choroidal anomalies were first to resolve, and clinical normalization preceded tomographic resolution.

Irregular vascular network identified with OCT-A in angioid streaks: A probable predictor of active choroidal neovascularization (case series).

PURPOSE: To evaluate the risk of active choroidal neovascularization (CNV) in presence of deep irregular vascular network (IVN) in eyes with angioid streaks (AS). METHODS: Observational case series including three treatment-naive eyes with angioid streaks and IVN, without CNV. Patients were followed-up during 18 months with multimodal imaging including structural optical coherence tomography (OCT) and OCT Angiography (OCT-A) to detect possible neovascular complication. RESULTS: On OCT-A, IVN was detected as a peripapillary and/or macular high-flow lesion, filling the spaces between the angioid streaks in the outer retina slab. Repeated OCT-A could detect an active CNV emerging from the IVN, as a high-flow rich anastomotic vascular network with a perilesional dark halo. Patient was treated with intravitreal injections of Bevacizumab on a Pro Re Nata regimen, with a decreased CNV area and lower vascular density on control OCT-A. CONCLUSION: OCT-A shown to be helpful in detecting the presence of IVN in asymptomatic eyes with AS during a routine examination. In our series, the IVN seems to be predictor of active CNV, needing a close surveillance and frequent follow-up to allow early treatment upon CNV activation.

Swept source OCT-Angiography assessing a case of aneurysmal type 1 neovascularization secondary to high-myopic posterior staphyloma.

AIM: To report an uncommon case of aneurysmal type 1 neovascularization (polypoidal choroidal vasculopathy) secondary to high-myopic staphyloma in a Caucasian patient, assessed with multimodal imaging including swept source OCT-Angiography. METHODS: Observational case report. RESULTS: About 73-year-old Caucasian male patient with high myopia (axial length = 27.24 mm). Fundus examination showed a myopic conus and a deep orange-brownish nodular lesion at the edge of a deep haemorrhage and connected to a large choroidal vessel. ICGA showed a circular hyperfluorescent lesion in mid-phase, without any branching vascular network. OCT-Angiography could detect the aneurysmal lesion non-invasively as a small circular high-flow lesion in the outer retina slab, with a shadowing in the choriocapillaris slab. At the level of the aneurysmal lesion, structural OCT showed a high bilobed PED, without any subretinal fluid. A vascular flow was noted within the PED on cross-sectional OCT-A, confirming the vascular aneurysmal nature of this lesion. Additionally, swept source OCT highlighted the presence of an abrupt change in choroidal thickness, from 62 µm in the peripapillary area to 120 µm underneath the polypoidal lesion, with dilated choroidal vessels. CONCLUSION: To our knowledge, this is the first report of OCT-A findings in aneurysmal (polypoidal) dilation secondary to high-myopic staphyloma. We could demonstrate the usefulness of OCT-A detecting non-invasively the aneurysmal dilation and the usefulness of swept source OCT assessing the choroidal structure to better understand the pathophysiology of this uncommon finding.

E-learning for Ophthalmology Training Continuity During COVID-19 Pandemic: Satisfaction of residents of Hédi Raies Institut of Ophthalmology of Tunis.

INTRODUCTION: COVID-19 pandemic created great challenges for the continuity of medical education. At the Hédi Raies Institut of Ophthalmology of Tunis (HRIO), the need to ensure continuity in the teaching of ophthalmology has stimulated the development of a new e-learning resource based on clinical case studies. AIM:   To evaluate level of satisfaction of HRIO residents in regard to clinical case-study-based e-learning of ophthalmology. METHODS: Cross-sectional survey including 40 ophthalmology residents doing their internship at the ROHI during the first half of 2020. Learners were tutored in e-learning via the Moodle online learning platform and using a problem-solving format based on clinical case studies describing various ophthalmologic conditions. Data collection was carried out through an online survey after four months of training, designed to assess learners' satisfaction with the e-learning. RESULTS: Mean age of participants was 29.95 ± 1.73 years. The majority had found the navigation easy, the content relevant to their training objectives, and were satisfied with the discussion forums as a method of communication. All respondents were satisfied with clinical cases presented through the platform and felt that they helped them to better understand the content. Among them, 97.5% considered that this teaching method corresponded to their training needs. There was a statically significant difference in the level of knowledge before and after e-learning teaching, taking into account the residents' appreciation (p<0.001). CONCLUSIONS: This study highlights the importance of virtual learning in ophthalmology in the era of the COVID-19 pandemic. E-learning is well appreciated by ophthalmology residents, relatively easy to integrate to their training program, and reduces issues of time, patient availability and case exposure.

Swept Source Optical Coherence Tomography Angiography: Characteristic Findings in Type 3 Macular Neovascularization.

We report a typical illustration of Swept source OCT-Angiography (SS-OCT-A) findings in type 3 macular neovascularization(MNV) or retinal angiomatous proliferation (RAP). This is a case of a 70-year-old Caucasian male patient presenting with an exudative type 3 neovascular age-related macular degeneration. En face SS-OCT-A could non-invasively detect a tiny perifoveal circular "clew-like" high-flow neovascular lesion, visible in the deep capillary plexus, the outer retina and communicating with the choriocapillaris, with a perilesional dark halo and associated to no-flow macular cysts in the deep capillary plexus slab. En face SS-OCT-A could also highlight the presence of a telangiectatic capillary dilation in the superficial capillary plexus appearing to be at the origin of the retinal neovascularization. Cross-sectional SS-OCT-A showed an intra-retinal vertical high vascular flow within the hyper-reflective neovascular lesion, with a typical hyperreflective "kissing sign" and associated to subretinal and intraretinal fluid. In conclusion, en face OCT-A is useful tool to diagnose type 3 MNV or RAP non-invasively and associated cross-sectional OCT-A scan is very helpful highlighting the linear vascular high-flow within the retinal neovascularization.

Contribution of ultra-wide field fluorescein angiography in diabetic retinopathy in a Tunisian population.

AIM: To assess the contribution of ultra-wide field (UWF) fluorescein angiography (FA) in clinically non proliferative diabetic retinopathy (DR) and to study the relationship between peripheral vascular lesions and the presence of diabetic macular edema (DME). METHODS: Retrospective study of consecutive UWF-FA obtained using a wide-field contact lens system. DME was detected on both FA and spectral-domain optical coherence tomography (SD-OCT). RESULTS: A total of 71 eyes of 39 diabetic patients with clinically non proliferative DR (NPDR) was included. DR was clinically graded as severe NPDR in 52 eyes (73%), moderate NPDR in 15 eyes (21%) and mild NPDR in 4 eyes (6%). On UWF-FA, DR was predominantly anterior in 14% of cases (10/71), predominantly posterior in 48% of cases (34/71) and diffuse in 38% of cases (27/71). Retinal non perfusion was present in 87% of eyes (62/71), predominating in superior-temporal areas. Peripheral vessel leakage was present in 85% of cases (60/71) and retinal neovascularization was noted in 14% of cases (10/71), unpgrading DR severity from NPDR to proliferative DR in 10 eyes. DME was present on SD-OCT in 53% of cases. Central macular thickness was significantly higher in eyes with retinal non-perfusion (353 µm vs. 254 µm, p=0,006) and retinal non-perfusion was associated with macular edema (97% vs. 76%, p=0,01) and poor visual acuity (p<0.001). Peripheral vessel leakage was associated with retinal non-perfusion (p<0.001) and retinal neovascularization (53% vs. 35%, p=0.01), but it was not associated with the presence of DME (p=0.449). CONCLUSION: UWF-FA was of great help assessing DR and evaluating peripheral retinal lesions in order to refine DR staging and to guide laser treatment. Besides, it allows better understanding of DME pathophysiology.

Valsalva retinopathy occurring after general anesthesia.

PURPOSE: To report a case of patient who presented with valsalva retinopathy after genral anesthesia for the treatment of ruptured intracranial aneurysm. OBSERVATION: A forty year-old man presented, after a general anesthesia for treatment of a ruptured intracranial aneurysm, with a severe decrease of the visual acuity in the left eye. Ophthalmic examination, performed one month after surgery showed a retrohyaloid macular hemorhage. After failure of laser Nd-YAG hyaloidotomy, vitrectomy allowed drainage of the hematoma with good visual outcome. CONCLUSION: Valsalva retinopathy is a rare complication that can occur after genral anesthesia. Vitrectomy may be needed if Nd-Yag laser hyaloidotomy fails.

Unilateral infiltration of the optic nerve revealing relapse of an acute lymphoblastic leukemia.

INTRODUCTION: Ocular infiltration of leukemia can involve orbit, uveal tract, retina and optic nerve. It may result from direct ocular infiltration by leukemic cells or indirect ocular involvement resulting from secondary hematologic changes, opportunistic infections and complications of various modalities of therapy. We report a case of unilateral infiltration of the optic nerve revealing a relapse of acute lymphoblastic leukemia. CASE REPORT: forty eight years-old woman in a remission of acute B lymphoblastic leukemia presented with headaches and blurred vision in the left eye. Ophthalmic examination showed a visual acuity reduced to 20/200 in the left eye, and a voluminous disc edema with papillary mass surrounded by retinal hemorrhages, exudates and important serous retinal detachment. CT scan showed a thickened left optic nerve and excluded true papillary edema due to intracranial hypertension secondary to central nervous system involvement. Myelogram and lumbar punction demonstrated blast infiltration and confirmed ocular relapse of the leukemia. CONCLUSION: The incidence of ocular involvement lymphoblastic acute leukemias decreased since the introduction of a systematic prophylactic treatment of central nervous system. Periodic ophthalmic examination is necessary to allow early diagnosis and treatment.

Association of carotido-cavernous fistula and controlateral optic neuropathy secondary to cranial trauma.

PURPOSE: to report a case of a woman who had a severe cranial trauma complicated by two ophalmologic potentially cecitating complications: right carotido-cavernous fistula and left traumatic optic neuropathy. OBSERVATION: A 56 years-old woman, without any medical history, referred, one month after fall from a height of 3 meters, for right exophthalmos. Ophthalmic examination of the right eye completed by retinal angiography suspected carotid-cavernous fistula which was confirmed by angio-MRI. In the left eye, the visual acuity was decreased to no light perception and fundus examination showed optic nerve head palor secondary to traumatic optic neuropathy. Arterial embolization was performed and allowed closing of the fistula. CONCLUSION: Optic neuropathy and carotido-cavernous fistula are two severe complications that can occur simultaneously in the same patient. The prognosis of the optic neuropathy may be compromised, and the treatment of carotido-cavernous fistula benefited from progress in interventional neuro-radiology.

Complement Component C3 Variant (R102G) and the Risk of Neovascular Age-Related Macular Degeneration in a Tunisian Population.

Purpose To explore the association between the polymorphism (S/F) p.R102G in the complement component 3 (C3) gene and age-related macular degeneration (AMD) in a Tunisian population. Methods The molecular study was performed by polymerase chain reaction using sequence-specific primers (PCR-SSP) in 207 control subjects free of any eye disease (fundus normal) and 145 patients with exudative AMD. The CH50 activity and quantification of C3 and C4 have been made by technical home method and nephelometry, respectively. Results The prevalence of C3 GG genotype polymorphism was significantly higher in AMD patients compared to controls (OR: 2.41, IC 95% [1.90-3.05], p = 0.0007). However, no correlation was found between this allelic variant and the type of neovascularization. Similarly, there is no association between this polymorphism and the presence of functional and/or quantitative hypocomplementemia. Conclusions The C3 GG genotype of the gene could be a susceptibility factor for AMD in the Tunisian population. However, it does not seem to influence the clinical profile of the disease.

Ophthalmic artery occlusion with total ophtalmoplegia and anisocoria revealing cavernous sinus thrombosis.

AIM: To describe a case of ophthalmic artery occlusion associated with anisocoria and revealing a cavernous sinus thrombosis due to sinusitis. OBSERVATION:   A 48-year-old man with a history of diabetes presented acutely with loss of vision and proptosis in the left eye. Ophthalmologic examination concluded in a left ophthalmic artery occlusion with anisocoria and total ophthalmoplgia. Cardiac assessment was normal. Magnetic resonance imaging (MRI) revealed left ophthalmic artery and internal carotid occlusions, left cavernous and transverse sinus thrombosis and sphenoid sinusitis. The patient underwent extensive haematological and medical assessment to search for embolic sources and disease causing thrombophilia. The patient recovered from the thrombosis episode, but sustained permanent blindness. CONCLUSION: Ophthalmic manifestations may be the only signs revealing cavernous sinus thrombosis which must be usually suspected. Visual prognostic was very poor.

Vascular endothelial growth factor genetic polymorphisms and susceptibility to age-related macular degeneration in Tunisian population.

PURPOSE: Three VEGF SNPs (-2578) C/A, (+405) G/C and (+936) C/T were investigated in Tunisian exudative AMD patients in order to determine their association with the disease susceptibility and their influence to intravitreal bevacizumab therapy response. METHODS: 145 AMD patients and 207 age-matched controls were included. 68 patients were treated with intravitreal bevacizumab. SNPs genotyping were performed using direct sequencing. The serum VEGF was assayed by ELISA (R&D). RESULTS: The (+405) CC and (+936) TT genotypes were higher in AMD patients than in controls (p = 5 × 10(-6) and p = 0.021, respectively). The mean plasma levels of VEGF were statistically higher in AMD patients (84.22 pg/ml) than in controls (15 pg/ml). Three months after bevacizumab treatment, 52 patients (85.6%) were classified as good responders (GR) and 16 (14.4%) as poor responders (PR). The mean plasmatic-VEGF levels in GR patients was higher (86.61 ± 80.30 pg/ml) than in PR patients (47.12 ± 45.74 pg/ml) (p = 0.086). The patients with genotype homozygous TT (+936) would be PR compared to those carrying CT and CC genotypes. Whereas, those with AA (-2578) genotype would be GR compared with others genotypes (p = 0.014; p = 0.042 respectively). CONCLUSIONS: Our results show that VEGF genetic variants may contribute to the susceptibility to neovascular AMD in Tunisian patients.

An unusual clinical presentation of ocular trauma in a child.

To report an unusual clinical presentation of ocular trauma in a child. Observational case report used in this study. A 12 year-old previously healthy boy presented with decreased vision and corneal opacity in the right eye following a punch in the face three years earlier. At presentation, his vision in the right eye was counting fingers. Ophthalmologic examination of the right eye revealed paracentral Descemet's membrane detachment and slit-lamp examination showed corneal opacity occupying almost 80 % of the corneal surface. In addition, there was a corneal white liquid collection communicating with the anterior chamber. The contralateral eye was within normal limits. The patient underwent penetrating keratoplasty. Bacteriological cultures of the corneal liquid did not reveal the presence of germs. The post-operative course was uneventful, the graft was clear and there was no evidence of graft rejection or failure. Visual acuity in the operated eye was 5/10. At present, the patient is still being followed up. The authors believe that this case is unique since Descemet's membrane detachment with liquid collection and corneal opacification has never been reported in literature to date.