Peripapillary Lesions Research Articles

A case documenting distinct natural history of multizonal outer retinopathy and retinal pigment epitheliopathy (MORR) with longitudinal multi-modal documentation of progression

PurposeTo describe the clinical and imaging characteristics of the acute progressive phase of a recently proposed clinical entity, Multizonal Outer Retinopathy and Retinal Pigment Epitheliopathy (MORR), a variant of Acute Zonal Occult Outer Retinopathy (AZOOR) MethodsSingle observational case report. ResultsWe present the case of a 49-year-old myopic female with progressive outer retinopathy most consistent with a diagnosis of MORR. Through multimodal imaging and longitudinal follow-up, we delineate the clinical course and imaging findings of asymmetrical episodic progressive centrifugal extension of retinal pigment epithelial disturbance in both eyes, highlighting the features of an acute progressive episode not previously described. ConclusionsClinicians should be aware of the active clinical and multimodal imaging features of MORR and its distinction from other outer retinopathies due to its sight-threatening distinct clinical course, bilateral involvement with peripapillary lesions, and episodic progression into the macula. Additionally, we describe a “grass-fire” and “spot-fire” progression pattern during acute exacerbation, highlighting the need for vigilant monitoring and early intervention in MORR.

PROGRESSION OF PERIPAPILLARY AND MACULAR CHORIORETINAL ATROPHY IN MULTIFOCAL CHOROIDITIS IS ASSOCIATED WITH PERIATROPHIC INFLAMMATORY PLUMES.

To investigate peripapillary atrophy and macular chorioretinal scars in eyes affected by multifocal choroiditis and panuveitis. This retrospective cohort study reviewed the medical records, fundus photographs, and spectral-domain optical coherence tomographic scans of 31 eyes from 19 patients. Patients had a mean age of 45 years (range 24-69 years). The average follow-up duration was 7 years (range, 2.5-14.5 years), with 14 patients undergoing immunosuppressive treatment. In the group of 31 eyes, 20 showed peripapillary plumes of ill-defined hyperreflectivity at the termination border of the retinal pigment epithelium. These plumes, extending from bare Bruch membrane to the outer nuclear layer, sometimes undermined the adjacent retinal pigment epithelium. They responded to corticosteroid treatment and resembled the material under the retinal pigment epithelium in acute lesions. Among 20 eyes with these peripapillary inflammatory lesions, 16 (80%) experienced increased atrophy, in contrast to none in the eyes without these lesions ( P < 0.001). Similar patterns were observed at the edges of macular chorioretinal atrophy. This observation occurred in patients using immunosuppressive medication who were otherwise thought to be under adequate control. In patients with multifocal choroiditis and panuveitis, previously unrecognized plumes of smoldering inflammatory activity at the borders of chorioretinal atrophy appears to be linked to atrophy expansion. The recognition of this phenomenon may require a reappraisal of treatment of multifocal choroidopathies to help mitigate the expansion of atrophy in these eyes.

Multiple Evanescent White Dot Syndrome following COVID-19 Vaccination: Two Case Reports

Purpose: We present two cases of multiple evanescent white dot syndrome (MEWDS) following COVID-19 vaccination.Case summary: We present two cases of visual field defects following BNT162b2 (Pfizer-BioNTech, Kronach, Germany) COVID-19 vaccination. A 14-year-old male experienced reduced visual acuity in the temporal field of his left eye after his second dose. Similarly, a 38-year-old woman experienced decreased visual acuity in her right eye after her first dose. Notably, neither patient had pre-existing medical conditions or exhibited COVID-19 symptoms following vaccination. Funduscopic examination revealed multifocal peripapillary hyperreflective lesions in both cases. Visual field defects and hyperreflective lesions spontaneously resolved within 6 weeks, with normal visual acuity and fundus findings at the 7-month follow-up.Conclusions: The recent COVID-19 pandemic has led to a significant increase in vaccination rates. Notably, MEWDS demonstrated a significant association with previous vaccines, including influenza and hepatitis, and the cases describe herein also suggest a potential association between BNT162b2 vaccination and MEWDS.

Acute Neuro-Retinitis Secondary to a Recently Acquired Ocular Toxoplasmosis in a Young Immunocompetent Patient

A 27-year-old male patient with no medical history presented with a painless unilateral vision loss in the right eye, counting fingers at 5 meters, with active vitreous inflammation, stage II papilledema, whitish lesion on the superior-temporal border of the optic disc and a poor macular reflex, without any active or scarring chorioretinal lesion in the periphery. Multimodal imaging showed intra- and subretinal macular edema with early hypo-fluorescence followed by progressive centripetal filling of the peripapillary lesion and finally papillary diffusion. The visual field revealed an inferotemporal defect. Blood work showed hyperleukocytosis with positive toxoplasma serology, the rest of the workup was negative, especially no immunosuppression. The diagnosis of toxoplasmic anterior optic neuropathy was made due to the papillary and peripapillary involvement without a distant active or scarring lesion, with positive toxoplasma IgG and IgM serology. Trimethoprim/sulfamethoxazole antibiotic treatment with systemic corticosteroid therapy was initiated in our patient with an excellent outcome, including restoration of visual acuity to 10/10, complete vitreous clearance, complete resolution of the papillary and macular edema, and the peripapillary lesion without visible scarring, but leaving a persistent defect of the corresponding retinal nerve fibers. This clinical case thus illustrates a toxoplasmic anterior optic neuropathy with direct papillary involvement complicated by macular edema, a rare and atypical clinical entity of ocular toxoplasmosis, especially in a young immunocompetent subject, reflecting the clinical polymorphism of this pathology.

Generalizability of Deep Neural Networks for Vertical Cup-to-Disc Ratio Estimation in Ultra-Widefield and Smartphone-Based Fundus Images.

To develop and validate a deep learning system (DLS) for estimation of vertical cup-to-disc ratio (vCDR) in ultra-widefield (UWF) and smartphone-based fundus images. A DLS consisting of two sequential convolutional neural networks (CNNs) to delineate optic disc (OD) and optic cup (OC) boundaries was developed using 800 standard fundus images from the public REFUGE data set. The CNNs were tested on 400 test images from the REFUGE data set and 296 UWF and 300 smartphone-based images from a teleophthalmology clinic. vCDRs derived from the delineated OD/OC boundaries were compared with optometrists' annotations using mean absolute error (MAE). Subgroup analysis was conducted to study the impact of peripapillary atrophy (PPA), and correlation study was performed to investigate potential correlations between sectoral CDR (sCDR) and retinal nerve fiber layer (RNFL) thickness. The system achieved MAEs of 0.040 (95% CI, 0.037-0.043) in the REFUGE test images, 0.068 (95% CI, 0.061-0.075) in the UWF images, and 0.084 (95% CI, 0.075-0.092) in the smartphone-based images. There was no statistical significance in differences between PPA and non-PPA images. Weak correlation (r = -0.4046, P < 0.05) between sCDR and RNFL thickness was found only in the superior sector. We developed a deep learning system that estimates vCDR from standard, UWF, and smartphone-based images. We also described anatomic peripapillary adversarial lesion and its potential impact on OD/OC delineation. Artificial intelligence can estimate vCDR from different types of fundus images and may be used as a general and interpretable screening tool to improve community reach for diagnosis and management of glaucoma.

Disinsert, retract and rotate technique of plaque brachytherapy.

Plaque brachytherapy is commonly used in the management of choroidal melanomas. The surgical steps usually involve creating a conjunctival peritomy, fixing the recti muscles, with or without disinserting them based on the location of the lesion, and placing the plaque. The inferior oblique muscle is attached close to the macula, and in cases of perimacular or peripapillary lesions, the muscle needs to be sacrificed. The authors here demonstrate a novel technique of placing radioactive plaque without disinserting the inferior oblique muscle in cases of perimacular or peripapillary choroidal melanomas. The video demonstrates how the "disinsert, retract, and rotate technique" of brachytherapy plaque placement can be performed and what are the fundamentals behind this technique. The authors have performed this procedure multiple times and there has been no incidence of plaque tilt or migration. In perimacular and peripapillary choroidal melanoma brachytherapy plaque placement, the inferior oblique muscle can be spared. The simple technique does not lead to any tilt or migration of the radioactive plaque. https://youtu.be/YMIg3rYyp2o.

Exudative vascular anomalous complex-like lesion of the peripapillary area.

Perifoveal exudative vascular anomalous complex is an uncommon retinal lesion of idiopathic origin characteristically described as an isolated, aneurysmal lesion of the perifoveal region. We report an unusual presentation of an exudative vascular anomalous complex-like lesion affecting the peripapillary area. A 69-year-old diabetic woman presented with blurred vision in her left eye for two months. Fundus examination of the left eye showed a small, reddish-orange lesion just supratemporal to the optic disc with perifoveal hard exudates and retinal thickening. Fundus fluorescein angiography demonstrated a peripapillary hyperfluorescent lesion with minimal leakage. Ocular coherence tomography showed an oval structure extending throughout the outer and inner plexiform and nuclear layers with a hyper-reflective wall accompanied by subfoveal and intraretinal fluid suggestive of an eVAC-like lesion in the peripapillary area. Intravitreal anti-vascular endothelial growth factor injection was given, and the lesion persisted even three weeks after the injection. Later, focal laser photocoagulation of the aneurysmal lesion was done. At 6 weeks follow-up, a complete resolution of the aneurysmal lesion with a marked decrease in retinal edema and an improvement of the visual acuity was observed. Exudative intraretinal aneurysmal lesions can occur in areas other than perifoveal area. OCT is an useful investigation for knowing their characteristics and the response to treatment. These aneurysmal lesions irrespective of their location can be called by the name eVAC-like or more aptly retinal capillary macro aneurysms (RCM).

MULTIZONAL OUTER RETINOPATHY AND RETINAL PIGMENT EPITHELIOPATHY (MORR): A Newly Recognized Entity or an Unusual Variant of AZOOR?

To describe specific clinical, multimodal imaging, and natural history features of an unusual variant of acute zonal occult outer retinopathy. Retrospective, observational, longitudinal, multicenter case series. Patients exhibiting this unusual clinical condition among cases previously diagnosed with acute zonal occult outer retinopathy were included. Multimodal imaging, laboratory evaluations, and genetic testing for inherited retinal diseases were reviewed. Twenty eyes from 10 patients (8 females and 2 males) with a mean age of 54.1 ± 13.3 years (range, 38-71 years) were included. The mean follow-up duration was 13.1 ± 5.3 years (range, 8-23 years). Presenting symptoms were bilateral in 7 patients (85% of eyes) and included scotomata and photopsia. All patients had bilateral lesions at presentation involving the peripapillary and far peripheral retina. Baseline optical coherence tomography showed alteration of the retinal pigment epithelium and photoreceptor layers corresponding to zonal areas of fundus autofluorescence abnormalities. Centrifugal and centripetal progression of the peripapillary and far-peripheral lesions, respectively, occurred over the follow-up, resulting in areas of complete outer retinal and retinal pigment epithelium atrophy. Initial alteration of photoreceptors and retinal pigment epithelium and a stereotypical natural course that includes involvement of the far retinal periphery, characterize this unusual condition. It may represent a variant of acute zonal occult outer retinopathy or may be a new entity. We suggest to call it multizonal outer retinopathy and retinal pigment epitheliopathy .

Esophageal Adenocarcinoma Metastatic to the Choroid with Extrascleral Extension

A 78-year-old man with history of esophageal adenocarcinoma, in remission 9 months after resection, chemotherapy, and radiation, presented with a peripapillary choroidal lesion with leopard-spot pigmentation and disc edema (A). Extraocular extension was demonstrated on B-scan and magnetic resonance imaging (B, C). A transconjunctival orbitotomy approach was performed rather than choroidal fine-needle biopsy to maximize tissue yield. Histopathology revealed expression of keratin (D), cytokeratin 20, caudal-related homeobox transcription factor 2 (CDX-2), and cytokeratin 7, consistent with metastatic esophageal adenocarcinoma. Positron emission tomography scan showed no primary tumor recurrence or additional metastases (Magnified version of Figure A-D is available online at www.aaojournal.org).

Focal laser and intravitreal bevacizumab for choroidal neovascularization secondary to Grönblad–Strandberg syndrome

Introduction: Pseudoxanthoma elasticum is a rare genetic disorder. It has several ocular features including angioid streaks and choroidal neovascularization which can significantly affect vision. Case Report: A 50-year-old man presented with nine months history of poor vision affecting both distant and near vision. His best corrected visual acuities were 6/18 right eye and 1/60 left eye. His anterior segments were normal. Funduscopy revealed linear hyperpigmented irregular branching peripapillary lesions; angioid streaks both eyes. He had bilateral speckled retina pigment epithelial hyperpigmented lesions interspaced with dot hemorrhages in the macula region suggestive of likely choroidal neovascularization. He had “peau d’orange” skin appearance around his neck. Fundus fluorescein angiography showed features of occult subfoveal choroidal neovascularization. He was referred to a physician and had a skin biopsy which revealed histologic features of Pseudoxanthoma elasticum. He was treated with bilateral focal argon laser. However he had right intravitreal Bevacizumab after a large ipsilateral subfoveal hemorrhage. His visual acuities remained stable afterward. Conclusion: Patients with Pseudoxanthoma elasticum have to be closely monitored for ocular manifestations. Choroidal neovascularization should be detected early and promptly treated. Antivascular endothelial growth factor injections have become the preferred treatment option for choroidal neovascularization in these patients.

Seagull wings appearance on optical coherence tomography: A case of peripapillary detachment in pathologic myopia.

To report the case of a patient who presented with a peripapillary retinal detachment caused by pathological myopia. A 73-year-old female with a clinical history of high myopia was admitted to our ophthalmic department complaining of vision loss in her right eye. Her best-corrected visual acuity was 20/25 in the right eye and 20/400 in the left eye. The refractive error was -9.00 D in both eyes. The anterior segment and pupillary reflex were normal. Dilated fundus examination revealed myopic retinopathy in both eyes and a yellow peripapillary lesion, distinct from the myopic conus and encircling almost the whole optic disc, in her right eye. The left eye displayed macular atrophy with macular pigmentation due to a previous myopic choroidal neovascularization. Optical coherence tomography showed a localized retinal detachment in the peripapillary area, adjacent to the inferior edge of the optic disc, giving a strange "seagull wings" appearance. In case of peripapillary neuroepithelial detachment the presence of PDPM in high myopic patients should always be excluded. Further research is due in order to better understand this clinical entity's pathogenesis and prognosis.

Subretinal peripapillary biopsy-proven sarcoidosis: a case report

BackgroundTo report a case of a subretinal, unilateral, peripapillary granuloma that was diagnosed as sarcoidosis by a 27-gauge pars plana vitrectomy subretinal biopsy. Sarcoidosis is a chronic idiopathic granulomatous inflammatory disease, that has ocular involvement in 10–80% of patients. It is often mistaken for many other primary ocular diseases because the condition can involve any structure in or around the eye. Previous case reports of peripapillary sarcoidosis have either been limited to the choroid or presented with additional ocular and systemic signs, hence have not required an intraocular biopsy.Case presentationA 54-year-old Filipino male presented with a 6-month history of painless blurred vision in his right eye. Fundus examination revealed a large white peripapillary lesion. Enhanced-depth imaging optical coherence tomography confirmed the subretinal location of the mass. Indocyanine green angiography demonstrated absolute hypofluorescent blockage with satellite lesions. A whole-body positron emission tomography scan demonstrated widespread lymphadenopathy, but investigations including an inguinal lymph node biopsy were inconclusive. Following growth of the peripapillary lesion and worsening vision, a 27-gauge pars plana vitrectomy subretinal biopsy was performed which confirmed sarcoidosis. He was treated with oral corticosteroids and transitioned to long term immunotherapy with methotrexate.ConclusionsSarcoidosis can present in the subretinal space, around the optic nerve without other ocular findings.

Three-Dimensional Volume Calculation of Intrachoroidal Cavitation Using Deep-Learning–Based Noise Reduction of Optical Coherence Tomography

PurposeIntrachoroidal cavitations (ICCs) are peripapillary pathological lesions generally associated with high myopia that can cause visual field (VF) defects. The current study aimed to evaluate a three-dimensional (3D) volume parameter of ICCs segmented from volumetric swept-source optical coherence tomography (SS-OCT) images processed using deep learning (DL)-based noise reduction and to investigate its correlation with VF sensitivity.MethodsThirteen eyes of 12 consecutive patients with peripapillary ICCs were enrolled. DL-based denoising and further analyses were applied to parapapillary 6 × 6-mm volumetric SS-OCT scans. Then, 3D ICC volume and two-dimensional depth and length measurements of the ICCs were calculated. The correlations between ICC parameters and VF sensitivity were investigated.ResultsThe ICCs were located in the inferior hemiretina in all eyes. ICC volume (P = 0.02; regression coefficient [RC], −0.007) and ICC length (P = 0.04; RC, −4.51) were negatively correlated with the VF mean deviation, whereas ICC depth (P = 0.15) was not. All of the parameters, including ICC volume (P = 0.01; RC, −0.004), ICC depth (P = 0.02; RC, −0.008), and ICC length (P = 0.045; RC, −2.11), were negatively correlated with the superior mean total deviation.ConclusionsWe established the volume of ICCs as a new 3D parameter, and it reflected their influence on visual function. The automatic delineation and 3D rendering may lead to improved detection and pathological understanding of ICCs.Translational RelevanceThis study demonstrated the correlation between the 3D volume of ICCs and VF sensitivity.

Choroidal Osteoma with Choroidal Neovascularization and Focal Choroidal Excavation

A 28-year-old female presented with recent onset of metamorphopsia in left eye. Best corrected visual acuity was 20/20 in right eye and 20/600 in left eye. Colour fundus photograph revealed orange yellow peripapillary lesion crowning the optic disc. Fundus Fluorescein Angiography revealed presence of choroidal neovascular membrane (CNV) type 2 in juxta-foveal region. Enhanced Depth Imaging Optical Coherence Tomography confirmed presence of CNV and conforming type of focal choroidal excavation (FCE). Diagnosis of choroidal osteoma with conforming FCE and CNV type 2 was made. FCE may be seen in choroidal osteoma with CNV, primarily in areas adjacent to decalcification.

Bilateral idiopathic choroidal hyperpigmentation

To report a series of cases showing bilateral circumpapillary hyperpigmentation on routine fundus examination. In this observational case series, three patients showing bilateral circumpapillary hyperpigmentation on routine fundus examination were included. Multimodal retinal imaging was done for these cases. Multimodal imaging of the lesion showed the hyper pigmentation to be confined to the choroid. The optical coherence tomography scans described the features through the hyperpigmented area, adjacent hypopigmented area and the normally pigmented area of the fundus as suggestive of choroidal hyper melanosis. Follow-up of these lesions over a period of 2-6 years showed no change in the vision or characteristics of the lesion. Bilateral circumpapillary hyperpigmentation needs to be considered as a differential for bilateral peripapillary pigmented lesions.

Choroidal Osteoma in a Young Female – a Case Report

We present a case of23 year old female, who presented with history of decreased central vision in the right eye for 3 months. Best corrected visual acuity was 6/36 OD and 6/9 OS. Anterior segment was normal OU. Fundus examination revealed a yellowish white peripapillary lesion extending up to the macula in the right eye. A similar lesion was seen in the left eye. OCT macula showed central macular thickness of 193 – 323 µm with cystoid spaces and 290 – 436 µm with serous retinal detachment in the right and left eye respectively. CT scan showed a hyper dense opacity similar to the bony tissue OU. All lab investigations were normal. The patient was diagnosed as a case of bilateral choroidal osteoma. After 6 months no progression or complication was noted.&#x0D; Key Words: Choroid, Osteoma, Choroidal Neoplasm.

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

To evaluate the risk of active choroidal neovascularization (CNV) in presence of deep irregular vascular network (IVN) in eyes with angioid streaks (AS). 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. 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. 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.

Bilateral Peripapillary Choroidal Neovascularization Associated with Age-Related Macular Degeneration: A Case Report

Summary A 50-year-old female was diagnosed with significant peripapillary choroidal neovascularization (PCNV) in the left eye (LE). 12 months after a close follow-up, 4 intravitreal injections with bevacizumab and retinal laser photocoagulation were performed to limit the distribution of the lesion. A year after the primary diagnosis in the LE, a tiny PCNV also developed in the right eye (RE). Simultaneous activity was additionally observed in both peripapillary lesions at the time. At the last follow-up, after 43 months, the best corrected visual acuity in the RE was 0.8, but in the LE it was only 0.2. In total, the LE received 6 intravitreal injections with bevacizumab, while the RE received 3 injections. The case report concludes that early diagnosis and intervention helps maintain visual acuity better in the cases of a small lesion than in developed significant PCNV with intense activity characterised with oedema, hard exudates and haemorrhages.

Acquired choroidal osteoma

Choroidal osteoma is a choristomatous lesion postulated to be congenital in nature. Described herein is the case of a 16-year- old female presenting with a peripapillary lesion diagnosed as choroidal osteoma on multimodal imaging. Routine fundus photography 18 months before presentation demonstrated a normal retina and choroid without evidence of the lesion. Overall, this report provides evidence that choroidal osteoma may be acquired and not always congenital in origin.

Filigree Vascular Pattern in Combined Hamartoma of Retina and Retinal Pigment Epithelium on OCT Angiography

To evaluate the vascular structure within combined hamartoma of retina and retinal pigment epithelium (CHRRPE) lesions using OCT angiography (OCTA). Multicenter, retrospective, observational analysis, PARTICIPANTS: Twenty eyes of patients diagnosed with CHRRPE. Retrospective analysis of color fundus photographs, OCT, and OCTA of 20 eyes with CHRRPE. Morphologic characteristics of CHRRPE and the OCT features were correlated with the density of the filigree vascular pattern and with the published histopathologic findings of CHRRPE lesions. Density of flow signals, that is, the filigree vascular pattern seen on OCTA in the deep capillary plexus, graded as high (>20), intermediate (10-20), or low (<10). Of 20 lesions, 11 were peripapillary, 8 were macular, and 1 was equatorial in location. A high density of filigree vascular pattern was observed in most peripapillary CHRRPE lesions, which also showed full-thickness retinal involvement (8/10). A low density of filigree pattern was seen in macular lesions, which showed partial-thickness retinal involvement and preretinal fibrosis (5/6). A filigree vascular pattern on OCTA is seen in CHRRPE lesions. High density of this pattern is noted in CHRRPE lesions with a peripapillary location, full-thickness retinal disorganization, and minimal preretinal fibrosis. These findings correlate well with published histopathologic findings of CHRRPE lesions both in terms of topographic and morphologic features. OCT angiography provides a promising method for further study of these lesions.