Morning glory disc anomaly-associated maculopathy: multimodal imaging

2021 ◽  
Vol 14 (1) ◽  
pp. e237462
Author(s):  
Inae Hwang ◽  
Marta Ugarte
Keyword(s):  
Multimodal Imaging ◽  
Fluid Pressure ◽  
Morphological Characteristics ◽  
Posterior Pole ◽  
Morning Glory ◽  
Fluid Accumulation ◽  
Tomography Imaging ◽  
Morning Glory Disc Anomaly ◽  
Optical Coherence Tomography Imaging ◽  
The Right

Morning glory disc anomaly (MGDA) is most commonly found in white females in childhood with reduced vision. One in two cases have been reported to develop maculopathy or posterior pole retinal detachment as they grow older. The pathophysiology of MGDA-associated maculopathy is not well understood.We describe a 31-year-old black woman, who presented with gradual reduction of vision in the right eye due to MGDA-associated maculopathy. We identified morphological characteristics of the optic disc and macula with multicolour and optical coherence tomography imaging.We speculate that the centripetal inner retina traction and cerebrospinal fluid pressure fluctuation play an important role in inner retinal fluid accumulation in the pathology of retinoschisis in MGDA. Further studies will shed some light of a potential cause-and-effect relationship between MGDA and retinoschisis.

A triad of retinal signs in Alport syndrome: The ‘stair-case’ fovea, choroidal thinning and peripheral schisis

2019 ◽  
Vol 29 (1_suppl) ◽  
pp. 10-14
Author(s):  
NJ Ghadiri ◽  
N Stanojcic ◽  
MSA Raja ◽  
BJ Burton
Keyword(s):  
Renal Failure ◽  
Optical Coherence Tomography ◽  
Alport Syndrome ◽  
Optical Coherence ◽  
Membrane Structures ◽  
Tomography Imaging ◽  
Type Iv ◽  
Ophthalmic Manifestations ◽  
Optical Coherence Tomography Imaging ◽  
The Right

Background: Alport syndrome is an inherited Type IV collagenopathy characterised by renal failure, hearing loss and ophthalmic manifestations such as lenticonus and dot-and-fleck retinopathy. New signs have been described which can be useful both for diagnosis and for prognosticating the risk of complications. This study examines and describes a triad comprising the unusual ‘stair-case’ foveal sign, together with choroidal thinning and late-stage peripheral schisis in a patient with Alport syndrome. Case presentation: This is a case report of a 49-year-old Caucasian male with a background of X-linked Alport syndrome presenting with gradual and progressive diminution of vision in the left eye with a central blur. He had already undergone three renal allografts, was deaf and suffered from hypertension by the time of his first presentation to ophthalmology. On examination, corrected visual acuity was 6/9.5 in the right eye and 6/30 in the left eye. Optical coherence tomography imaging showed an unusual ‘stair-case’ sign of the fovea in both eyes, together with choroidal thinning. We postulate that an abnormal vitreomacular interface followed by vitreomacular traction and eventually separation, removing layers of the inner retina with the vitreous, led to this unusual appearance. Subsequently, this patient also developed schitic changes more peripherally in the retina which progressed over the following 5 years. Conclusion: The stair-case foveal sign, choroidal thinning and mid-peripheral schisis are three signs that clinicians might expect to encounter on optical coherence tomography imaging of patients with Alport syndrome. These findings can be attributed to unique mutations of collagen IV which lead to a variety of clinical phenotypes affecting basement membrane structures. Identification of these features may not only be useful diagnostically and in forecasting complications such as macular holes, but also predict mode of inheritance and likelihood of early-onset renal failure.

scholarly journals Spontaneous retinal pigment epithelial rip

2020 ◽  
Vol 2 (4) ◽  
pp. 300-304
Author(s):  
Kosyilya Arumugam ◽  
Ayesha Mohd Zain ◽  
Mushawiahti Mustapha
Keyword(s):  
Multimodal Imaging ◽  
Retinal Pigment Epithelial ◽  
Retinal Pigment ◽  
Vital Role ◽  
Posterior Pole ◽  
Large Area ◽  
Pigment Epithelial ◽  
Rare Manifestation ◽  
The Right

This is a case report of a spontaneous retinal pigment epithelial (RPE) rip in a 64-year-old woman who presented with progressive, painless, blurring of vision in her right eye for 2 weeks. Her vision was 6/60 in the right eye and 6/6 in the left eye. On fundoscopy, there was a large depigmented area over the right posterior pole. A large area of RPE rip involving the fovea was revealed via optical coherence tomography and angiogram. Spontaneous RPE rip is a rare manifestation. However, when the fovea is involved the visual prognosis is poor. This article aims to highlight the vital role of multimodal imaging in aiding the diagnosis of RPE rips or tears.

scholarly journals Optical Coherence Tomography Imaging of Choroidal Abnormalities in Neurofibromatosis Type 1

2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Shinji Makino ◽  
Hironobu Tampo
Keyword(s):  
Optical Coherence Tomography ◽  
Neurofibromatosis Type 1 ◽  
Fundus Autofluorescence ◽  
Neurofibromatosis Type ◽  
Posterior Pole ◽  
Optical Coherence ◽  
Tomography Imaging ◽  
Optical Coherence Tomography Imaging

We report a case of neurofibromatosis type 1 (NF1) examined by infrared fundus autofluorescence (IR-FAF) and optical coherence tomography (OCT) to characterize the associated choroidal abnormalities. The conventional ophthalmoscopic findings were unremarkable. However, IR-FAF revealed multiple bright patchy lesions in the choroid of the posterior pole, in both eyes. OCT demonstrated irregular hyperreflectivity at the sites of these lesions. Patients with NF1 may have typical choroidal lesions that are visible on IR-FAF, which can be confirmed through OCT.

scholarly journals Management of Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE): Insights from Multimodal Imaging with OCTA

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Mariana A. Oliveira ◽  
Jorge Simão ◽  
Amélia Martins ◽  
Cláudia Farinha
Keyword(s):  
Visual Acuity ◽  
Multimodal Imaging ◽  
Indocyanine Green Angiography ◽  
Optical Coherence Tomography Angiography ◽  
Posterior Pole ◽  
Blurred Vision ◽  
Corrected Visual Acuity ◽  
The Right ◽  
Yellowish White ◽  
Best Corrected Visual Acuity

A 28-year-old man presented to the emergency room with blurred vision in the right eye for two days. He reported a preceding flu-like illness one week earlier. His best-corrected visual acuity (BCVA) was 20/40 in the right eye and 20/25 in the left eye. There was no anterior chamber inflammation or vitritis in either eye. He presented multiple yellowish-white placoid lesions in the posterior pole, some involving the foveal area, bilaterally. General examination and systemic investigation were unremarkable. Multimodal evaluation with fluorescein angiography, indocyanine green angiography, and spectral domain and optical coherence tomography angiography (OCTA) were consistent with the diagnosis of acute posterior multifocal placoid pigment epitheliopathy. Due to centromacular involvement with decreased BCVA, treatment with oral methylprednisolone was started after infectious causes were ruled out. After two weeks, the patient presented functional and anatomical improvement. OCTA showed partial reperfusion of the choriocapillaris in the affected areas, in both eyes.

scholarly journals Case Report: Fibroglial Retinal Tissue in Contractile Morning Glory Disc Anomaly

2021 ◽  
pp. 525-530
Author(s):  
Abel Ramirez-Estudillo ◽  
Karla Torres-Navarro ◽  
Sergio Rojas-Juárez ◽  
Ximena Ramirez-Galicia ◽  
Berenice Palafox-Cornejo ◽  
...  
Keyword(s):  
Structural Changes ◽  
Retinal Pigment ◽  
Anterior Segment ◽  
Posterior Pole ◽  
Retinal Pigment Epithelial Cells ◽  
Morning Glory ◽  
Retinal Tissue ◽  
Morning Glory Disc Anomaly ◽  
History Of ◽  
Pars Plana

The purpose of the present case is to describe a patient with tractional retinal detachment (RD) associated with contractile morning glory: a 17-year-old female, with a history of failed surgery for RD when she was 2 years old in her right eye (OD), nystagmus, and a limited visual acuity in the left eye (OS). The slit lamp examination showed phthisis bulbi in OD and the anterior segment was unremarkable in OS. Dilated fundus examination revealed a tractional RD in the posterior pole and peripapillary and preretinal fibrosis without evidence of intravitreal dispersion of retinal pigment epithelial cells. After surgery treatment, the RD resolved and the posterior segment showed a staphylomatous excavation around the optic disc anomaly with irregular contractions that folded the macular area. This were unrelated to light, breathing, or eye movements. Although morning glory disc anomaly is associated with RD, the early diagnosis can reverse structural changes. In this case, the rare association with contractile movements was found posterior to the pars plana vitrectomy after all the fibroglial epiretinal tissue was removed.

scholarly journals A rare triad of morning glory disc anomaly, moyamoya vasculopathy, and transsphenoidal cephalocele: pathophysiological considerations and surgical management

2021 ◽  
Author(s):  
Marco Pavanello ◽  
Pietro Fiaschi ◽  
Andrea Accogli ◽  
Mariasavina Severino ◽  
Domenico Tortora ◽  
...  
Keyword(s):  
Surgical Management ◽  
Missense Variant ◽  
Morning Glory ◽  
Morning Glory Disc Anomaly ◽  
Long Term Follow Up ◽  
Indirect Revascularization ◽  
Peripapillary Retina

AbstractMorning glory disc anomaly is a congenital abnormality of the optic disc and peripapillary retina reported as an isolated condition or associated with various anomalies, including basal encephaloceles and moyamoya vasculopathy. However, the co-occurrence of these three entities is extremely rare and the pathogenesis is still poorly understood. Moreover, data on the surgical management and long-term follow-up of the intracranial anomalies are scarce. Here, we describe the case of a 11-year-old boy with morning glory disc anomaly, transsphenoidal cephalocele, and moyamoya vasculopathy, who underwent bilateral indirect revascularization with encephalo-duro-myo-arterio-pericranio-synangiosis at the age of 2 years, and endoscopic repair of the transsphenoidal cephalocele at the age of 6 years. A rare missense variant (c.1081T>C,p.Tyr361His) was found in OFD1, a gene responsible for a X-linked ciliopathy, the oral-facial-digital syndrome type 1 (OFD1; OMIM 311200). This case expands the complex phenotype of OFD1 syndrome and suggests a possible involvement of OFD1 gene and Shh pathway in the pathogenesis of these anomalies.

In vivo cellular optical coherence tomography imaging

1998 ◽  
Vol 4 (7) ◽  
pp. 861-865 ◽  
Author(s):  
Stephan A. Boppart ◽  
Brett E. Bouma ◽  
Costas Pitris ◽  
James F. Southern ◽  
Mark E. Brezinski ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Optical Coherence ◽  
Tomography Imaging ◽  
Optical Coherence Tomography Imaging

scholarly journals Acute exudative polymorphous paraneoplastic vitelliform maculopathy (AEPPVM) associated with choroidal melanoma

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Aluisio Rosa Gameiro Filho ◽  
Guilherme Sturzeneker ◽  
Ever Ernesto Caso Rodriguez ◽  
André Maia ◽  
Melina Correia Morales ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Serous Retinal Detachment ◽  
Central Nervous System Involvement ◽  
Choroidal Melanoma ◽  
Posterior Pole ◽  
Pancreatic Metastasis ◽  
Small Areas ◽  
History Of ◽  
The Right

Abstract Background To report a case of acute exudative polymorphous paraneoplastic vitelliform maculopathy in a patient with a history of choroidal melanoma, with metastases to the pancreas, liver, and central nervous system. Case presentation A 63-year-old patient, with a history of enucleation of the right eye due to choroidal melanoma, complained of progressive visual loss during a follow-up visit. Fundoscopic examination revealed multiple small areas of serous retinal detachment scattered throughout the posterior pole and ancillary tests confirmed the diagnosis of acute exudative polymorphous paraneoplastic vitelliform maculopathy (AEPPVM). Screening for systemic metastases showed pancreatic, hepatic, and central nervous system involvement. Conclusions We describe a rare case of acute exudative polymorphous paraneoplastic vitelliform maculopathy, which should be considered in patients with or without a history of melanoma, who have vitelliform retinal detachments. Nevertheless, no previous reviews of literature have shown a correlation between AEPPVM and pancreatic metastasis.

scholarly journals Case of peripheral fibrovascular proliferative retinopathy associated with morning glory disc anomaly

2021 ◽  
Vol 22 ◽  
pp. 101029
Author(s):  
Tomoka Ishida ◽  
Ryuki Fukumoto ◽  
Yoshihiro Wakabayashi ◽  
Yuji Itoh ◽  
Makoto Inoue ◽  
...  
Keyword(s):  
Proliferative Retinopathy ◽  
Morning Glory ◽  
Morning Glory Disc Anomaly

scholarly journals Posterior Uveal Effusion after Cataract Surgery

2021 ◽  
pp. 62-67
Author(s):  
Annegret Abaza ◽  
Özlem Dikmetas ◽  
Irmingard Neuhann ◽  
Faik Gelisken
Keyword(s):  
Cataract Surgery ◽  
Corticosteroid Therapy ◽  
Anterior Segment ◽  
Subretinal Fluid ◽  
Posterior Pole ◽  
Functional Improvement ◽  
Long Term Observation ◽  
The Right ◽  
Uveal Effusion

We report a case of posterior uveal effusion (UE) with a long-term follow-up that has occurred following cataract surgery. A 64-year-old woman presented with diminished vision of the right eye (RE) 3 weeks after an uneventful phacoemulsification and intraocular lens implantation. Complete ophthalmic examination including fluorescein angiography (FA), indocyanine green angiography (ICGA), echography and optical coherence tomography (OCT) were performed. Best corrected visual acuity (BCVA) of the RE was 20/50. Anterior segment and intraocular pressure were unremarkable. OCT revealed prominent folds of the choroid and retina, subretinal fluid and darkening of the choroid with reduced visibility of the choroidal vessels and the scleral border. The left eye (LE) was unremarkable. BCVA of the LE was: 20/20. After topical anti-inflammatory and systemic corticosteroid therapy for 5 months, no morphological change of the macula was seen. The patient was observed without any treatment. Forty-three months after the cataract surgery and 38 months after cessation of the corticosteroid therapy, OCT revealed a normal macular morphology and the BCVA improved to 20/25. Even though rare, UE at the posterior pole may occur after modern cataract surgery. OCT examination is a reliable tool in monitoring the macular morphology. Since morphological and functional improvement can be seen in long-term, observation may be considered for some cases of posterior UE with resistance to the therapy.

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