Are PHOMS (Peri papillary hyperreflective ovoid mass-like structures ) with an elevated optic disc still a diagnosis dilemma?

OBJECTIVE: To identify clinical characteristics that would help make or rule out the diagnosis of Idiopathic intracranial hypertension (IIH) in patients referred for papilledema (PE) with peripapillary hyperreflective ovoid mass-like structures (PHOMS) DESIGN: A retrospective cohort study SUBJECTS: All patient referred for PE excluding PE with Frisén grade ≥ 3, optic neuritis, ischemic optic neuropathy, compressive optic neuropathy. Patients were divided into 2 groups: Group1= isolated PHOMS Group2= PHOMS associated with IIH METHODS: We analyzed the location of PHOMS based on OCT-EDI and calculated their volume MAIN OUTCOME MEASURES: P RNFL,GCC, PHOMS' volume RESULTS: 154 patients (308 eyes) were included, mean age was 29 with female predominance (78%). PHOMS were associated to these etiologies : IIH(38.3%) isolated (35.7%), posterior uveitis (11%),optic disc drusen (ODD)(10%), and tilted optic disc (5%). An MRI was obtained in 83.1% of cases. More than half of the MRIs were interpreted as consistent with IIH. However, only 39.7% of these patients had confirmed IIH with 44.5% of sensitivity and 55.5% of sensibility. PHOMS were overrepresented in the nasal region (95.5%).The location of PHOMS in the superior and/ or inferior quadrant was significantly associated to IIH or optic dusc drusen, while their presence in the temporal or nasal sector was strongly associated to isolated lesions. PHOMS mean and median volume were 1.66 µm3 and 1.50 µm3 respectively. There was a significant difference in PHOMS volume with higher volume in IIH patients (p=0.0037). Follow up of these patients at 3 and 6 months demonstrated no significant changes in visual function, as per visual field mean deviation, and visual acuity measurements as well as Ganglion cell layer (GCL).Mean pRNFL, showed a decrease of -4.225 µm at 3 months and of -6.489 µm at 6 months, when compared to initial measurement independing of the etiology. CONCLUSION: Isolated PHOMS should be considered as a distinct entity. In asymptomatic patients, PHOMS should be carefully studied. Nasal or temporal location, small volume, stable aspect over the course of weeks or months, are so suggestive of this entity. This strategy would considerably reduce the impact on patients' anxiety and morbidity.

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.

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.

Specificities of pediatric ocular emergencies before and during the COVID-19 era: A retrospective comparative study in an eye-related emergency department in Paris.

INTRODUCTION: Epidemiological data on the use of eye-related emergency services by children are limited. The objective of this study was to determine how COVID-19 affected the epidemiological trends of pediatric ocular emergencies. METHODS: We performed a retrospective chart review of children under the age of 18 years who visited our eye-related emergency department between March 17 and June 7, 2020 and between March 18 and June 9, 2019. This was a descriptive and comparative analysis of the two study periods based on the demographic characteristics of patients and the diagnosis reported by the ophthalmologist in the digital medical charts. One of the investigators performed a second reading of the files to homogenize the diagnosis classification based on the most frequently found items. RESULTS: In total, 754 children were seen in our eye-related emergency department during the 2020 study period versus 1399 in 2019, representing a 46% decrease. In 2019, the four main diagnoses were traumatic injury (30%), allergic conjunctivitis (15%), infectious conjunctivitis (12%), and chalazion/blepharitis (12%). In the 2020 study period there was a significant decrease in the proportion of patients presenting with traumatic injuries (p < 0.001), infectious conjunctivitis (p = 0.03), and chalazion/blepharitis (p < 0.001). Consultations for chalazion/blepharitis were the most affected by the pandemic, followed by traumatic injuries (-72% and -64%, respectively). The proportion of patients who required surgery after trauma was higher in 2020 than in 2019 (p < 0.01), but the absolute number of severe trauma cases remained stable. CONCLUSIONS: The COVID-19 pandemic was accompanied by a decrease in the overall use of a pediatric eye-related emergency services in Paris. Visits due to benign causes and ocular trauma also decreased, but visits for more severe pathologies were not affected. Longer-term epidemiological studies may confirm or refute a change in eye emergency department use habits.

Isolated optic nerve infiltration as a site of relapse of acute lymphoblastic leukemia.

Optic nerve infiltration is relatively rare in acute lymphoblastic leukemia. We present a case of a -53 year-old-man who was diagnosed with T- acute lymphoblastic leukemia (ALL). The patient was treated with ALL national protocol and the central nervous system (CNS) prophylactic management. On treatment, the patient presented with sudden severe vision deterioration of both eyes. Fundoscopic examination of the eye and magnetic resonance imaging of the orbits were in favor of an infiltration of the optical nerve. An isolated extramedullary relapse of the optical nerve was retained. The patient was treated with salvage chemotherapy systematic and intrathecal. Waiting forthe beginning of radiotherapy, the patient presented a bone marrow relapse. He died of a severe hemorrhagic syndrome. Conclusion: Optic nerve leukemic infiltration has a severe prognosis. Ophthalmic assessment is essential in patients with ALL in order to diagnose an early ocular involvement and the patient's vision can be preserved if treatment is initiated promptly.