Peripapillary retinal neovascularization and vitreous hemorrhage secondary to peripapillary pachychoroid syndrome.

Purpose: To report a case of peripapillary pachychoroid syndrome (PPS) complicated with peripapillary retinal neovascularization causing vitreous hemorrhage. Observation: A 42-year-old man, with a history of a visual loss of the right eye (RE) for 4 years, presented for ophthalmological examination. Best-corrected visual acuity was «counting fingers¼ in the RE and 20/25 in the left eye. Fundus examination showed irregular pattern of hypopigmentation of the retinal pigmented epithelium in both eyes with retinal neovessels in the peripapillary region of the RE. Swept-source optical coherence tomography demonstrated a central serous pigment epithelial detachment with intraretinal cysts and serous retinal detachment in the nasal macula extending from the temporal disc margin in the RE. Fluorescein angiography showed multiple areas of hyperfluorescence without clear distinction of retinal neovessels. Indocyanine green angiography showed patches of choroidal hyperpermeability predominant in the peripapillary region in both eyes. Optical coherence tomography angiography provided a good visualization of the papillary and retinal neovessels without signs of choroidal neovascularization. Two months after initial examination, the patient presented with vitreous hemorrhage associated to juxtapapillary preretinal hemorrhage in the RE. After vitreous injections of bevacizumab, we observed a total resolution of the vitreous hemorrhage a partial decrease of the intraretinal and subretinal fluid. Conclusions and importance: We report an unusual case of peripapillary retinal neovascularization and vitreous hemorrhage complicating a PPS. OCTA was useful to detect retinal neovessels and peripapillary retinal and choriocapillaris hypoperfusion, supporting the understanding of the pathogenic mechanism of neovascularization in PPS.

Case Report: Complete tomographic resolution of focal choroidal excavation complicated with choroidal neovascularization after anti-VEGF treatment.

Purpose We report a case of focal choroidal excavation (FCE) that resolved after intravitreal injection of anti-vascular endothelial growth factor (VEGF) for choroidal neovascularization (CNV) and we describe its tomographic features. Case report A 43-year-old female presented with blurred vision and metamorphopsia in her left eye (LE) evolving for 10 years. The best corrected visual acuity (BCVA) was 20/20 in the right eye and 20/32 in the LE. Fundus examination revealed the presence of a yellowish foveal lesion which corresponded to a conforming FCE associated to a pachychoroid on swept-source optical coherence tomography (OCT). The OCT-Angiography showed a foveal flow void in the choriocapillaris layer corresponding to the FCE area. Three years later, the patient complained of visual impairment, more metamorphopsia with a BCVA of 20/80 on her LE. The OCT showed intraretinal fluid with a foveal retinal pigment epithelium (RPE) detachment. The OCT-angiography confirmed the presence of CNV. Two months after one intravitreal bevacizumab injection, the OCT documented the complete resolution of macular edema, the regression of the CNV tissue and the restoration of a normal aspect of the fovea without any FCE. Her BCVA improved to 20/32 with resolution of the metamorphopsia. The OCT aspect remained stable during 3 years of follow-up. Conclusion CNV can develop in FCE and anti-VEGF therapy is a good option treatment. After treatment, FCE pattern can change et may completely resolve.

Optical coherence tomography angiography analysis of microvascular abnormalities and vessel density in treatment-naïve eyes with diabetic macular edema.

BACKGROUND: The aim of this study was to evaluate the structural retinal vascular integrity using optical coherence tomography angiography (OCTA) in treatment-naïve eyes with diabetic macular edema (DME) and to compare it with findings in diabetic eyes without DME. METHODS: In this prospective study, 70 eyes with diabetic retinopathy were included (37 eyes with DME and 33 eyes without DME). The medical records, including swept-source optical coherence tomography and 9 × 9 mm swept-source OCTA images were reviewed and compared between DME and non-DME groups. Microaneurysms, intraretinal microvascular abnormalities (IRMA), areas of capillary non perfusion, foveal avascular zone (FAZ), and capillary vascular density (CVD) were analyzed in the superficial capillary plexus (SCP) and the deep capillary plexus (DCP). RESULTS: Compared to the non-DME eyes, DME eyes had more microaneurysms in the SCP and the DCP (p = 0,039 and p = 0,024 respectively), more IRMA in the SCP (p = 0,005), larger areas of capillary non perfusion in the SCP and the DCP (p = 0,026 and p = 0,02 respectively) and larger FAZ in both plexuses (p = 0,048 in the SCP and p = 0,012 in the DCP). The CVD in the DCP was lower in DME eyes compared to non-DME eyes (p = 0,007). The severity of DME was significantly correlated to the number of microaneurysms and to the FAZ surface. Central macular thickness was significantly correlated with the number of microaneurysms in the DCP, the surface of capillary non perfusion areas and the FAZ area in both plexuses. CONCLUSIONS: OCTA with a 9 × 9 mm field of view showed that the retinal vascular integrity regarding the number of microaneurysms, the number of IRMA, the surface of capillary non perfusion areas, the FAZ area and the CVD, was significantly more impaired in DME eyes compared to diabetic eyes without DME. The DCP seemed to be more affected in diabetic eyes with and without DME than the SCP.

Evaluation of the effect of the severity of diabetic retinopathy on microvascular abnormalities and vascular density using optical coherence tomography angiography.

PURPOSE: To quantify and to study the relationship between retinal microvascular changes and different stages of diabetic retinopathy (DR) using optical coherence tomography angiography (OCTA). METHODS: This prospective observational study included the eyes of patients with different stages of DR. OCTA was performed in all eyes using a 9 × 9 mm protocol. We analyzed the superficial and deep retinal capillary plexuses, for the following OCTA parameters: number of microaneurysms (MA), number of intraretinal microvascular abnormalities (IRMA), the total surface of capillary non-perfusion (CNP) areas, and vascular density (VD). The association between those parameters and the severity of DR was studied. RESULTS: A total of 70 eyes of 45 patients were included. The number of MA in the superficial capillary plexus (SCP) showed a significant association with DR severity (p = 0.03). The number of IRMA and the total surface of CNP areas were associated with the severity of DR in both plexuses (p = 0.019 in the SCP and p = 0.044 in the DCP for IRMA; p < 0.001 in the SCP and p = 0.001 in the DCP for CNP areas). The VD decreased significantly with the severity of DR in the DCP (p = 0.04). CONCLUSIONS: The number of vascular abnormalities and the surface of CNP areas were significantly associated with the severity of DR. The decrease in vascular density in the DCP with increasing disease severity suggests that the DCP is more vulnerable to vascular changes than the SCP. Those parameters might be tools for a future DR severity scale based on OCTA. HIGHLIGHTS: In addition to its capability of detecting vascular changes in DR non-invasively, OCTA may also serve as a valuable tool to graduate DR.

Bilateral congenital macular coloboma: Swept-source optical coherence tomography findings.

A 15-year-old male presented with decreased vision and nystagmus from childhood. Best-corrected visual acuity was limited to 0,05/10 in both eyes. Fundus examination revealed a well-demarcated macular excavation of 2 discs diameter, baring of the underlying sclera, surrounded by a pigmented rim and hypopigmented retinal areas. Autofluorescence images showed a central hypoautofluorescent lesion with patchy hypoautofluorescent areas corresponding to retinal pigmented epithelium (RPE) and photoreceptor atrophy. Swept-source optical coherence tomography showed a crater-like depression with atrophy of the neurosensory retina and the absence of RPE and choroid except some large choroidal vessels. Anti-toxoplasma antibody was negative. These findings are suggestive of a congenital macular coloboma. Similar lesions have to be excluded such as toxoplasmosis macular scar and North Carolina dystrophy.

Multimodal imaging of foveoschisis and macular pseudohole associated with gyrate atrophy: a family report.

BACKGROUND: To report the results of multimodal imaging of a biochemically confirmed case of a family with gyrate atrophy (GA) associated with foveoschisis and macular pseudohole. CASE PRESENTATION: Two sisters presented to us with progressive bilateral decreased vision. The 26-year old sister had a best corrected visual acuity (BCVA) of 20/32 in the right eye (RE) and 20/100 in the left eye (LE). Dilated fundus examination revealed multiple bilateral chorioretinal atrophy areas in the midperipheral and peripheral retina. Fluorescein angiography did not show any leak in the macular area. Swept-source optical coherence tomography (SS-OCT) showed increased central macular thickness in both eyes with foveoschisis. Optical coherence tomography angiography (OCTA) showed petaloid non-reflective areas and some perifoveal microvascular alterations similar to telangiectasias in the deep capillary complex. The 30-year-old sister had a BCVA of 20/20 in the RE and 20/32 in the LE. SS-OCT was normal in the RE and demonstrated a macular pseudohole with a fine epiretinal membrane in the LE. The persistent retinal tissue at the base of the pseudohole was disorganised. Blood tests showed hyperornithinemia in the 2 cases. Based on these observations, the patients were diagnosed with gyrate atrophy of the choroid and retina and were treated with a pyridoxine supplement and an arginine-restricted diet. CONCLUSIONS: Foveoschisis and macular pseudohole may be associated in GA, increasing the risk of rapid vision loss. OCTA is an interesting imaging tool that can help to better understand the pathophysiological mechanism of these macular involvements in GA.

Atrophic tear retinal detachment: clinical characteristics and surgical treatment results at long term.

AIM: to analyse clinical and epidemiological characteristics of atrophic tear retinal detachment (ATRD) and evaluate anatomical and functional results. METHODS: Retrospective study of 48 cases underwent primary scleral buckling for ATRD. Mean follow up was 80,52 months. RESULTS: ATRD represented 7% of all reghmatogenous RD. Mean age of patients was 38 years and 7 months. Age was less than 40 years in 62,5% of patients. Male predominance was noted. Myopia was noted in 67% of cases and 78,12% presented high myopia. Mean delay of consultation was 7 months ranging from 1 and 60 months. Visual acuity was ranged between light perception to 10/10. The AT number in eye varied between1 to 15 (mean 3 tears). The seat of AT was preferentially in inferior temporal quadrants than superior one. Maculae was detached in 87,5% of cases. All patients underwent scleral buckling: longitudinal buckle was performed in 27% of cases, radial sponge was put on in 8,3% of eyes and circumferential one was made in 64,5% of cases. Sub-retinal fluid was punctured in 75% od cases. Retinal reattachment was obtained in all cases; after one surgery in 95,83% of cases. Redetachment was observed in 2 cases: it was linked with unknown AT. Visual acuity was improved in 83,3% of cases. It was more than 5/10 in 14,6% of cases. Postoperative hypertonia was observed in 18,75% of cases. Choroidal detachment was observed in one case and it was resolved spontaneously. Scleral bukle rejection was observed in one case. CONCLUSION: ATRD was not associated at posterior vitreous detachment. It was observed in young myopic patients. There is a chronic form of retinal detachment with many tears. Scleral buckle was the surgical treatment of this type of RD with good anatomical prognostic.

[Biological assay for galactose-1 phosphate measurement application in subjects with galactosemia]. / Dosage du galactose-1 phosphate et application chez des sujets galactosémiques.

Congenital galactosemia is a hereditary, autosomal recessive and metabolic disease. It is linked to an enzyme deficiency, more commonly known by the deficiency of galactose-1- phosphate uridyltransferase (GALT), which is responsible for an accumulation of galactose-1- phosphate in the blood. Clinical symptoms appear early in infancy from the second week of life. They generally manifested by some disorders within liver, kidney, eye, gastrointestinal, neurological and also with cataracts. Currently, the clinical diagnosis remains difficult hence the importance of further investigations based on effective biological assessments to highlight the disease. The diagnosis of galactosemia is made by the laboratory test. The latter includes the determination of Gal-1-P which is done by a fluorometric method spot test. This study was conducted in order to assess the repeatability, reproducibility, accuracy, and effectiveness of the techniques used. We have found the CV for a repeatability (CV = 5 %), reproducibility (CV = 4 %) which confirms the accuracy of the method proceeded in this study. This method allows us to have a degree of inaccuracy less than 1%. According to the study of the effectiveness of "spot test", we found that our technique is specific (Sp = 93 %) and sensitive (Se = 83 %).