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 diffuse uveal melanocytic proliferation: Report of a rare Ocular Paraneoplastic Syndrome.

Bilateral diffuse uveal melanocytic proliferation is a rare paraneoplastic disorder where bilateral blindness is caused by uveal thickening, serous retinal detachment, and rapid cataract formation. Several different malignancies have been associated with bilateral diffuse uveal melanocytic proliferation, but ovarian carcinoma in women and lung and pancreatic carcinoma in men are the most common. The underlying mechanism is thought to be related to a an endogenous factor wich regulates the proliferation of uveal melanocytes. We present the case of a 75-year-old man with bilateral diffuse uveal melanocytic proliferation secondary to pulmonary adenocarcinoma.

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.

Abducens nerve palsy in pre eclampsia : case report.

Cranial nerves palsy associated with preeclampsia is reported in literature. Facial and abducens palsies were the most nerve disorders described. Only eleven cases have been reported in the literature. We report the case of a 27-years-old patient who presented abducens nerve palsy in immediately after the delivery of severe pre-eclampsia. No specific pathology was found. Symptoms of abducens nerve palsy have resolved spontaneously by controlling blood pressure after delivery.

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.

Azathioprine for glucocorticoid resistant noninfectious uveitis.

BACKGROUND: the systemic steroids represent the first line treatment in the majority of the noninfectious uveitis, except some specific indications as the Behcet disease. Nevertheless, this treatment may be ineffective and immunosuppressive therapy is mandatory. PURPOSE: to evaluate effectiveness and side effects of azathioprine (AZA) in corticosteroid resistant noninfectious uveitis (CRNIU). METHODS: This prospective study (2002- 2009), concerned 21 patients (mean age 37 years), 37 eyes, with CRNIU. Patients received oral AZA 2,5mg/kg/day, initiated in association with high dose steroids, with an end-point of 12 months. Response was defined as complete, partial response and failure, for each of the 3 following out-come measurements: improvement of BCVA, improvement of inflammation, steroids-sparing. Statistical analysis was considered significant if p value < 0,05. RESULTS: side effects occurred in 42,8% (9/21) of patients, in which 5/9 patients stopped the treatment. Regarding BCVA, complete success was observed in 62,5%, partial response in 20,9% , and failure in 16,6% of cases. Regarding inflammation, complete success was noted in 70,8%, partial response in 29,1% and failure in 16,6 % of cases. Complete response of steroid sparing was observed in 85,7% of cases without failure. Complete success of the 3 criteria was observed in 57,1% of patients / 62,5% of eyes. Cataract (p=0,013) and pallor of optic nerve head (p=0,013) were associated to poor visual prognosis, BCVA of 20/40 or more (p=0,003, RR=2,38)) and papilledema (p=0,022, RR=2) to good visual prognosis. BCVA of 20/200 or less (p=0,001) was associated to failure of AZA on inflammatory response. CONCLUSION: AZA is safe and effective in corticosteroid-sparing and controlling inflammation in CRNIU. Its low cost and availability allow proposing it as a first-line option, especially when new biological treatments are difficult to obtain.

Immunohistochemical study of vascular endothelial growth factor (VEGF), tumor suppressor protein (p53) and intercellular adhesion molecule (ICAM-1) in the conjunctiva of diabetic patients.

The expression pattern of VEGF, p53 and ICAM-1 was studied in conjunctiva of diabetic patients with and without retinopathy. All patients underwent a complete ophthalmic examination, including retinal fluorescein angiography. Indirect immunoperoxidase method was performed on 20 eyes of 20 patients with type II diabetes without DR and on 5 eyes of 5 patients with PDR. A control study was performed on 6 normal conjunctiva undertaken during cataract surgery. Immunoreactivity of VEGF, p53 and ICAM-1 was found in epithelial, fibroblast and vascular endothelial cells. For the same duration of diabetes, a strong to moderate or weak immunoreactivity was observed in the conjunctiva of patients without retinopathy. In patients with PDR, the expression was strong for all these proteins. The immunoreactivity was correlated between VEGF, p53 and ICAM-1. In the normal conjunctiva, a weak to negative immunostaining was observed. The presence of these proteins in the conjunctiva of diabetic patients without retinopathy may add new data in the pathogenesis of diabetic retinopathy. Further studies are needed to confirm this hypothesis.

[Xeroderma pigmentosum. Cutaneous, ocular, and neurologic abnormalities in 49 Tunisian cases]. / Xeroderma pigmentosum: manifestations cutanées, oculaires et neurologiques à partir de 49 patients tunisiens.

This is a retrospective study about 49 cases of xeroderma pigmentosum (XP). The goal was to determine the clinical features of XP in Tunisia. Our study revealed the predominance of a moderate form. Malignant skin tumors showed early. Squamous cell carcinoma (SCC) remainsed the most frequent skin malignancy in all clinical forms. Neurological abnormalities were more frequent in the moderate form. Mental retardation and peripheral neuropathy were the most common signs.

[Functional results of rhegmatogenous retinal and macular detachment]. / Les resultats fonctionnels du décollement de rétine rhégmatogène avec macula décollée.

The aim of our study is to evaluate the results of visual recovery after retinal detachment surgery with detached macula and to analyze factors that would influence these results. We carried out a retrospective study about 133 cases of reghmatogenous retinal detachment with detached macula. They were followed between 1990 and 1997 and had a primary classical surgery. The follow up was at least 6 months. The age of our patients varied from 9 to 79 years with an average of 43.8 years. Postoperatively, final visual acuity was equal or superior to 1/10 in 49.63% of cases. We analyzed the results of functional recovery according to the following factors: preoperative visual acuity, duration of detachment, patients history, area of detachment, break size, proliferative vitreoretinopathy stage and surgery factors (kind of buckling, subretinal fluid drainage and reoperations). We found a statistical significance between postoperative visual acuity and the following factors; preoperative visual acuity, duration and area of detachment, break size and proliferative vitreoretinopathy. It is essential to operate the patients with retinal detachment as early as possible in order to guarantee a better postoperative visual acuity. It is also important to incite patients with retinal detachment risk factors to consult in case of any ocular symptoms.

[Subretinal fluid drainage in rhegmatogenous retinal detachment]. / La ponction du liquide sous rétinien dans la chirurgie du decollement de rétine rhegmatogène.

Subretinal fluid drainage during retinal surgery is a tricky manoeuver that remains controversial. In this study, we carried out a comparative retrospective study about 265 cases with retinal detachment that had a primary episcleral surgery. We compared the results of the group operated with subretinal fluid drainage (199 cases) to the group without (66 cases). The complications met were retinal haemorrhages in 14 cases (7%) and retinal incarceration in one case (0.5%). Anatomical results were 91.45% in the group with drainage and 93.93% in the group without. There was no statistical difference between the two groups. The complications of the subretinal fluid drainage are extremely rare and have no repercussion if it is well checked. Subretinal fluid drainage remains the surgeon decision. It is achieved by most retinal surgeons (75%).