Search

Purpose: To describe a representative case and review the literature on paracentral acute middle maculopathy (PAMM) and giant cell arteritis (GCA). Methods: A review of the English-language ophthalmic literature was performed using the search terms of PAMM, giant cell arteritis, and temporal arteritis. Results: A 72-year-old woman with PAMM as the presenting ophthalmic manifestation of GCA was described with a review of the prior cases from the literature. It was found that there were 26 cases of PAMM in GCA. In 19 of 26 cases, PAMM was associated with no other fundus abnormalities and was only seen on multimodal imaging including optical coherence tomography. Conclusion: PAMM can cause acute paracentral visual loss, and GCA should be suspected in all cases of PAMM of the elderly patients, even when isolated and not associated with constitutional symptoms of GCA. [ABSTRACT FROM AUTHOR]

Purpose: To report a case of paracentral acute middle maculopathy in a pediatric patient with sickle cell trait. Methods: The patient was evaluated with a comprehensive ophthalmic examination, optical coherence tomography, optical coherence tomography angiography, and fluorescein angiography. Results: Acute loss of vision occurred immediately after an uneventful scleral buckling procedure for retinal detachment in a 16-year-old female patient. Retinal imaging studies confirmed the occurrence of paracentral acute middle maculopathy, an ischemic condition characterized by infarction of the inner nuclear layer of the retina caused by hypoperfusion of the intermediate and deep capillary plexuses. Laboratory evaluation was only remarkable for sickle cell trait. Over a course of 3 months after the loss of vision, visual acuity improved from 20/1,000 to 20/20, and optical coherence tomography lesion resolved to a parafoveal area of inner and middle retinal layer thinning. Paracentral scotoma corresponding to the atrophic area persisted. Conclusion: Dehydration because of the presurgical nil-per-os status and transient increase in intraocular pressure during the buckling surgery may have predisposed this patient with sickle cell trait to a sickling event that caused localized ischemia in the middle retina's end-capillaries. Avoiding long nil-per-os status, being mindful of transient intraocular pressure elevation during scleral buckling procedures, and presurgical hemoglobin electrophoresis in at-risk populations are recommended to prevent sickling attacks during retinal surgeries in individuals with sickle cell trait. [ABSTRACT FROM AUTHOR]

Purpose: To present a case of paracentral acute middle maculopathy (PAMM) associated with transient loss of vision from eosinophilic granulomatosis with polyangiitis (EGPA)-associated ischemic vasculitis. Methods: Case report and review of the literature. Results: A 52-year-old man presented with recurrent episodes of amaurosis of the right eye. A diagnosis of EGPA was established with the combination of late-onset asthma, peripheral eosinophilia, patchy parenchymal consolidation, and diffuse pansinusitis. Fundus examination displayed cotton wool spots in the right eye. Optical coherence tomography (OCT) of the macula showed features of paracentral focal inner nuclear layer/outer plexiform layer hyperreflectivity consistent with PAMM. He was treated with corticosteroids and cyclophosphamide. At 2 months following the treatment, his EGPA was in remission; the patient reported no recurrence of visual symptoms. Dilated fundoscopy revealed resolution of the cotton wool spots. A repeat OCT of the macula showed resolution of PAMM. Conclusion: This case supports sublethal hypoxia of the middle retinal layers as the underlying mechanism for PAMM. Ophthalmologists should be cognizant to look for PAMM on OCT in patients presenting with transient visual loss and be aware of EGPA-associated ischemic vasculitis as a rare cause of amaurosis. [ABSTRACT FROM AUTHOR]

Purpose: To report a case of bilateral paracentral acute middle maculopathy lesions on spectral domain-optical coherence tomography(OCT) secondary to severe Plasmodium falciparum malaria. Methods: Retrospective case report. Spectral domain-OCT, ultra-widefield fluorescein angiography, and OCT angiography were performed and analyzed. Results: A 54-year-old healthy man presented with acute vision loss in both eyes few days after being diagnosed with severe Plasmodium falciparum malaria. Ophthalmoscopic examination was unremarkable, but near-infrared reflectance imaging showed patchy hyporeflective areas located at the terminal tips of the venous branches. Corresponding spectral-domain OCT demonstrated alternating bands of hyperreflectivity involving the inner nuclear layer, consistent with skip paracentral acute middle maculopathy lesions. Optical coherance tomography angiography illustrated corresponding flow signal loss at the level of the deep capillary plexus. Ultra-widefield fluorescein angiography showed peripheral retinal vein staining and capillary nonperfusion. Conclusion: Paracentral acute middle maculopathy may be an OCT manifestation of malarial retinopathy associated with severe Plasmodium falciparum infection. [ABSTRACT FROM AUTHOR]

Purpose: To report the case and multimodal imaging findings of a healthy young woman who developed paracentral acute middle maculopathy 9 weeks after COVID-19. Methods: Case report. Ultra-widefield fundus photography, macular spectral domain optical coherence tomography, fluorescein angiography, and optical coherence tomography angiography were performed. Results: A 36-year-old woman went to the emergency department with sudden, painless, left eye vision loss. The only relevant medical history was COVID-19 9 weeks before. The best-corrected visual acuity was 20/200, a left eye relative afferent pupillary defect was present, and superficial hemorrhages throughout the macular area and peripheral retina were found. Nearly 4 hours after admission, the left eye best-corrected visual acuity recovered to 20/20 without relative afferent pupillary defect. Five days after presentation in the emergency department, the patient returned with recurrent left eye vision loss, with spontaneous recovery within 12 hours. Macular spectral domain optical coherence tomography revealed hyperreflectivity of the inner plexiform and inner nuclear layers, and the diagnosis of paracentral acute middle maculopathy was established. The patient started oral acetylsalicylic acid and oral prednisolone. The patient did not report any new episodes of vision loss, and there was a progressive resolution of abnormal fundus findings. Conclusion: SARS-CoV-2 infection increases the risk of vascular thrombotic events with possible involvement of the retinal circulation, and paracentral acute middle maculopathy may present as a possible complication. Ophthalmologists should be able to recognize it promptly through multimodal imaging findings. [ABSTRACT FROM AUTHOR]

Background/Purpose: To report an atypical case of ocular bartonellosis evaluated longitudinally with multimodal imaging and review the literature on the less common posterior segment manifestations of bartonella infection. Methods: Case report and review of the literature. Results: A 26-year-old man presented with focal choroiditis in the right eye and focal retinitis, a branch retinal artery occlusion, and paracentral acute middle maculopathy in the left eye after a systemic prodrome including headache and lymphadenopathy. Serologic testing revealed positive bartonella immunoglobulin G with a titer of 1:512. The patient was diagnosed with ocular bartonellosis infection and started on systemic doxycycline. The posterior segment findings resolved over the follow-up period, and their resolution was documented with optical coherence tomography. Conclusion: Neuroretinitis is the classic ocular finding in cat scratch disease, but less common presenting findings may include focal choroiditis, retinitis, and branch retinal artery occlusion. To the best of our knowledge, this is the first report of paracentral acute middle maculopathy secondary to a retinitis-associated branch retinal artery occlusion as a presenting sign in ocular bartonellosis. [ABSTRACT FROM AUTHOR]

We report a case of a healthy multiparous woman in her second trimester of pregnancy presenting with a paracentral scotoma found to have a paracentral acute middle maculopathy lesion, resulting in a new diagnosis of a possible hypercoagulable condition with elevated Factor VIII. Purpose: To report a case of paracentral acute middle maculopathy in an otherwise healthy young, multiparous woman in her second trimester of pregnancy. Methods: A case report. Results: A 38-year-old woman in her twentieth week of pregnancy presented with a four-day history of an acute paracentral scotoma in her left eye. Fundoscopic examination of the left eye was significant for a white–gray lesion inferonasal to the fovea which corresponded with spectral-domain optical coherence tomography hyperreflectivity at the outer plexiform layer–inner nuclear layer junction and optical coherence tomography angiography nonperfusion. A diagnosis of paracentral acute middle maculopathy was made. The patient was sent for a hypercoagulability workup that revealed elevated Factor VIII activity, which has been associated with increased risk of complications during pregnancy. Conclusion: Paracentral acute middle maculopathy in pregnancy may be secondary to an underlying hypercoagulable condition. We recommend systemic evaluation and referral to a high-risk pregnancy specialist if paracentral acute middle maculopathy is diagnosed during pregnancy. In addition, optical coherence tomography angiography in paracentral acute middle maculopathy may demonstrate reperfusion of the affected vessels. [ABSTRACT FROM AUTHOR]

We report the case of a 27-year-old woman with a history of sickle cell disease whose vision improved from 20 of 200 to 20 of 20 over 3 months after receiving intra-arterial tissue plasminogen activator for acute monocular painless vision loss secondary to central retinal artery occlusion presenting as paracentral acute middle maculopathy. Purpose: We report the case of a 27-year-old monocular woman with a history of sickle cell disease who received intra-arterial tissue plasminogen activator (tPA) after presenting with acute painless vision loss secondary to incomplete central retinal artery occlusion presenting as paracentral acute middle maculopathy in her left eye. Methods: Ultrawidefield fundus photography, ultrawidefield fluorescein angiography, en face optical coherence tomography (OCT), and optical coherence tomography angiography (OCTA) were obtained and reviewed, followed by cerebral angiography and infusion of intra-arterial tissue plasminogen activator. Results: A patient with a history of sickle cell disease presented with a new onset of a dense central scotoma, and the visual acuity diminished to 20 of 200 from baseline 20 of 20 in her left eye. Fluorescein angiogram was nondiagnostic. Optical coherence tomography revealed perifoveal hyper-reflective bands in the inner nuclear layer in a pattern characteristic of paracentral acute middle maculopathy. The patient received intra-arterial tissue plasminogen activator through her left ophthalmic artery shortly after presentation, resulting in a gradual restoration of the visual acuity to 20 of 20 in the three months after the procedure. Conclusion: This is the first report describing the use of intra-arterial tissue plasminogen activator to treat incomplete central retinal artery occlusion presenting as a paracentral acute middle maculopathy lesion in a patient with sickle cell disease. [ABSTRACT FROM AUTHOR]

This article describes a case of paracentral acute middle maculopathy associated with central retinal vein occlusion in a patient affected by amyloid light-chain amyloidosis. We discuss the multimodal imaging findings, including dye-based angiography, fundus autofluorescence, optical coherence tomography, and optical coherence tomography angiography, over the follow-up. Purpose: To describe a case of paracentral acute middle maculopathy associated with central retinal vein occlusion in a patient affected by amyloid light-chain amyloidosis. Methods: One patient with confirmed diagnosis of amyloid light-chain amyloidosis, displaying paracentral acute middle maculopathy and central retinal vein occlusion, was recruited. The patient underwent complete ophthalmologic examination and multimodal imaging, including: fundus autofluorescence, fluorescein angiography, indocyanine green angiography, spectral-domain optical coherence tomography, and optical coherence tomography angiography. Results: Fundus autofluorescence showed a ferning pattern, corresponding to linear hypofluorescence in late-phase indocyanine green angiography and delayed venous filling, detected by fluorescein angiography. Structural optical coherence tomography revealed a hyper-reflective line located in the outer plexiform layer, corresponding to the prominent middle limiting membrane, along with several placoid lesions. Optical coherence tomography angiography found that the superficial capillary plexus was preserved, whereas vessel density was reduced in both the deep capillary plexus and the choriocapillaris. After 1 year of follow-up, the patient achieved an almost complete morphological recovery. Conclusion: Multimodal imaging described in depth the morphological features of a case of combined paracentral acute middle maculopathy and central retinal vein occlusion in a patient affected by amyloid light-chain amyloidosis. [ABSTRACT FROM AUTHOR]

Paracentral acute middle maculopathy can cause acute paracentral scotomas because of capillary ischemia. We report the first case of paracentral acute middle maculopathy secondary to Waldenström macroglobulinemia, a hyperviscosity syndrome. This case highlights the importance of initiating a work-up for unexplained paracentral acute middle maculopathy-associated visual field defects, especially when associated with arteriolar tortuosity. Purpose: To report a case of paracentral acute middle maculopathy as the earliest sign of an undiagnosed, life-threatening hyperviscosity syndrome. Methods: A 78-year-old man with an acute paracentral scotoma and examination findings of bilateral arteriolar tortuosity and unilateral paracentral acute middle maculopathy. Results: Work-up revealed anemia and elevated serum viscosity. Protein electrophoresis demonstrated an immunoglobulin M kappa monoclonal protein spike, and bone marrow biopsy confirmed an immunoglobulin M gammopathy consistent with Waldenström macroglobulinemia. Systemic chemotherapy was initiated. Conclusion: This case demonstrates typical optical coherence tomography findings of paracentral acute middle maculopathy, which led to the diagnosis of a rare lymphoproliferative disorder. This highlights the importance of a prompt work-up for paracentral acute middle maculopathy to detect underlying systemic diseases, including hyperviscosity syndromes. [ABSTRACT FROM AUTHOR]

We describe the first two observations of paracentral acute middle maculopathy after cardiac surgery involving cardiopulmonary bypass. Bilateral cotton-wool spots and unilateral ischemic optic neuropathy were also observed. We discuss possible mechanisms of multifocal microvascular nonperfusion in the setting of extracorporeal oxygenation and circulation of blood. Purpose: To report the first two observations of bilateral paracentral acute middle maculopathy in the early postoperative period after cardiopulmonary bypass procedures. Methods: Comprehensive ophthalmic examinations were performed, including best-corrected visual acuity, Humphrey visual field testing, dilated fundus examination, and optical coherence tomography. Results: Two patients complained of unilateral vision loss after cardiac surgery involving cardiopulmonary bypass. Unilateral optic disc edema consistent with ischemic optic neuropathy was observed in the symptomatic eye of each patient, whereas scattered cotton-wool spots were identified bilaterally. In addition, optical coherence tomography imaging revealed bilateral paracentral acute middle maculopathy, correlating with visual field defects detected on automated perimetry in the asymptomatic eyes. Conclusion: Although symptomatic postoperative vision loss is a rare complication of cardiac surgery, intraoperative retinal microvascular nonperfusion as a consequence of extracorporeal circulation of blood may be a more common and under-recognized occurrence. [ABSTRACT FROM AUTHOR]

A 45-year-old man who had a sudden onset of a positive scotoma was found to have a very small paracentral acute middle maculopathy lesion that was not visible by ophthalmoscopy but was imaged successfully with the high-density optical coherence tomography scans used for angiographic imaging. Purpose: To describe a transient positive scotoma and corresponding optical coherence tomography (OCT) structural and angiographic findings. Methods: The patient was evaluated with a comprehensive ophthalmic examination to include OCT structural and angiographic imaging with two different instruments, the Zeiss Plex Elite and the Optovue RTVue XR Avanti. Results: A 45-year-old man had a sudden onset of a positive scotoma in the visual field of the left eye. No abnormalities were noted by ophthalmoscopy or fundus photography. Optical coherence tomography angiography was performed to evaluate the macular perfusion status. With each instrument, a small hyperreflective area, 175 μ m in diameter, was imaged in the inner nuclear layer. The OCT angiographic images suggested a small area of decreased perfusion in the deep capillary plexus. Except for the diminutive size, the lesion had an appearance suggestive of paracentral acute middle maculopathy. The symptoms lessened rapidly, and when examined 4 days later, the lesion was less hyperreflective. Two weeks after presentation, the positive scotoma was not present and there was no longer any hyperreflectivity in the inner nuclear layer. Conclusion: Detection of the lesion was aided by using OCT angiographic scans, which have a much higher scan density than conventional OCT evaluations. The diminutive abnormality was consistent with a paracentral acute middle maculopathy lesion, although smaller than those previously reported. Micro–paracentral acute middle maculopathy lesions should be considered in the differential diagnosis of positive scotomas. [ABSTRACT FROM AUTHOR]

Purpose: To describe a unique presentation of multifocal paracentral acute middle maculopathy in a young patient with juvenile dermatomyositis including optical coherence tomography angiography findings. Methods: Retrospective single case report. Patient: Seventeen-year-old male patient with a history of juvenile dermatomyositis presented for routine hydroxychloroquine screening. Results: Optical coherence tomography revealed undulations of the outer retinal layers and retinal thinning near the fovea in both eyes. Optical coherence tomography angiography revealed loss of flow void densities in the superficial and deep capillary plexi in both eyes. These findings were consistent with a diagnosis of paracentral acute middle maculopathy. Conclusion: To the authors' knowledge, this is the first report of the optical coherence tomography angiography findings in paracentral acute middle maculopathy associated with juvenile dermatomyositis. The defects were located in the superficial and deep capillary plexi and likely due to the known occlusive retinopathy associated with juvenile dermatomyositis. [ABSTRACT FROM AUTHOR]

We aim to present a case with paracentral acute middle maculopathy associated with branch retinal artery occlusion secondary to polycythemia, which is suggested a new precursor cause of paracentral acute middle maculopathy. We believe this report will be of interest to the readers of your journal. Purpose: To describe the occurrence of paracentral acute middle maculopathy (PAMM) associated with branch retinal artery occlusion secondary to polycythemia in a patient with tetralogy of Fallot. Methods: Case report. Results: A 30-year-old man presented with acute vision loss and superior visual deficit in his left eye for two days. His medical record had a tetralogy of Fallot. Complete blood count showed an erythrocyte count of 9.88 million/µL (4.4–5.6), hemoglobin of 17.7 g/dL (13.5–16.9), and hematocrit of 65.4% (40–49). The best-corrected visual acuity was 20/25 in the left eye, and a diagnosis of left inferotemporal branch retinal artery occlusion was made. Spectral-domain optical coherence tomography revealed a characteristic hyperreflective band-like lesion on the inner nuclear layer consistent with PAMM. Conlusion: Polycythemia may be a trigger for branch retinal artery occlusion–associated PAMM. We suggest a new precursor cause of PAMM that is previously undescribed. [ABSTRACT FROM AUTHOR]

Purpose: To describe two cases of paracentral acute middle maculopathy (PAMM) that occurred unilaterally after endovascular coil embolization. Methods: Medical records including ophthalmic examinations of two PAMM cases were retrospectively reviewed. Results: The first case was a 64-year-old woman with hyperlipidemia and chronic sinusitis. The second case was a 49-year-old man with hypertension, hyperlipidemia, tension headache, and vertigo. Both cases exhibited paracentral scotomas just after endovascular coil embolization. Ophthalmoscopy showed multiple faint white-yellow lesions in each affected eye. Optical coherence tomography showed hyper-reflective bands that were mainly observed in the inner nuclear layer and corresponded to the PAMM lesions. Optical coherence tomography angiography revealed variable degrees of abnormal vasculature from slight dilation, decreased visualization, to nonvisualization in the superficial capillary plexuses and similar capillary changes in deep capillary plexuses. Conclusion: We described two cases with PAMM that occurred after endovascular coil embolization, which was a new precursor cause of PAMM. Our results demonstrate that optical coherence tomography angiography is especially useful for not only diagnosis, but also for follow-up evaluations in patients with PAMM. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this report was to describe a case with paracentral acute middle maculopathy after oral intake of sumatriptan. Methods: Case presentation. Results: One patient showed typical findings on fundoscopic examination and optical coherence tomography consistent with paracentral acute middle maculopathy following oral intake of sumatriptan. Conclusion: Sumatriptan may be a trigger for paracentral acute middle maculopathy. [ABSTRACT FROM AUTHOR]

Purpose: We report a case of paracentral acute middle maculopathy secondary to cholesterol embolization syndrome after an endovascular aortic aneurism repair (EVAR). Methods: Functional and anatomical damage was assessed by Snellen visual acuity, fundoscopy, and optical coherence tomography. Results: A 67-year-old man presented with sudden vision loss in the right eye for one day with concomitant livedo reticularis in both feet. His medical history included an abdominal EVAR repair three days before the symptoms. The best-corrected visual acuity was 20/40 in the right eye and 20/20 in the left eye. Fundoscopy showed intraarteriolar cholesterol plaques (Hollenhorst) bilaterally, and a localized retinal whitening in the right eye. Optical coherence tomography at the lesion site revealed a hyperreflective band on the level of inner nuclear layer. The patient was referred for imaging and complete systemic evaluation. He was then closely followed up by a vascular team, without additional intervention. Retinal stigma partially regressed at six months with visual improvement. Conclusion: This case describes paracentral acute middle maculopathy as a rare ocular sign of cholesterol embolization in postoperative intraaortic operations. The diagnosis of this complication led to prompt clinical care with a multidisciplinary approach. [ABSTRACT FROM AUTHOR]

Purpose: To report a case of acute macular neuroretinopathy in a patient with Susac syndrome. Methods: Case report. Results: A 39-year-old male patient presented with severe headache, photopsias, and a sudden onset of hearing loss in the right ear. Fluorescein angiography of the right eye revealed multiple branch retinal artery occlusions. Clinical presentation of encephalopathy, hearing loss, and branch retinal artery occlusions, along with characteristic magnetic resonance imaging findings, led to a diagnosis of Susac syndrome. Despite aggressive immunosuppression for four months, the patient later presented with acute macular neuroretinopathy in the left eye. Conclusion: Acute macular neuroretinopathy and Susac is a new association of two well-defined disorders. The concurrence of both disorders supports retinal ischemia as the proximate cause of acute macular neuroretinopathy and inflammation as a potential etiology. [ABSTRACT FROM AUTHOR]

Background/Purpose: To present a novel case of paracentral acute middle maculopathy associated with bilateral optic disk swelling. Methods: Retrospective case report. Results: A 67-year-old woman presented with sudden onset of central vision loss and subsequent bilateral optic disk edema, retinal vessel attenuation, and anterior uveitis. Cerebrospinal fluid analysis showed signs of inflammation. Spectral domain optical coherence tomography demonstrated the pathognomonic hyperreflectivity of the middle retinal layers consistent with paracentral acute middle maculopathy. Swept-source optical coherence tomography angiography with custom vessel analysis demonstrated a 18.3% decrease in the deep retinal vascular density and 2.4 times increase in absent flow area in the affected eye compared with the fellow eye. Conclusion: This case demonstrates a novel association between paracentral acute middle maculopathy and bilateral optic disk swelling secondary to central nervous system inflammation and possible infection. Although spectral domain optical coherence tomography is valuable in detecting paracentral acute middle maculopathy, optical coherence tomography angiography with vessel analysis can provide additional insight into the disease mechanism. [ABSTRACT FROM AUTHOR]

Purpose: To present the first reported case of paracentral acute middle maculopathy in association with Susac syndrome. Method: Case report. Results: A young female patient presented with unilateral sudden loss of vision of her right eye to count fingers. There were numerous cotton wool spots in a multifocal pattern concentrated around the right macula and optic disk. Clinical examination and spectral domain optical coherence tomography imaging were consistent with paracentral acute middle maculopathy, and fluorescein angiography showed features of retinal arteriole vasculitis. Magnetic resonance imaging demonstrated multiple white matter lesions in a pericallosal distribution. High-dose corticosteroid therapy was commenced, and visual acuity gradually improved to 6/6 corrected. The patient reported hearing impairment and was diagnosed with Susac syndrome for which she has responded extremely well to longterm immunosuppressive therapy. Conclusion: Paracentral acute middle maculopathy can occur in association with Susac syndrome. Susac syndrome may present only with multifocal retinal ischemia, and such patients with paracentral acute middle maculopathy should be checked for characteristic magnetic resonance imaging lesions and hearing loss. [ABSTRACT FROM AUTHOR]

Purpose: Paracentral acute middle maculopathy (PAMM) is a recently identified clinical entity with numerous retinal vascular and systemic associations. To our knowledge, this is the first reported case of PAMM associated with idiopathic intracranial hypertension. Methods: A case is presented with multimodal imaging. Results: A patient with idiopathic intracranial hypertension is found to have the characteristic clinical, spectral domain optical coherence tomography, and OCT angiography findings associated with PAMM. Conclusion: Paracentral acute middle maculopathy is believed to arise from a microvascular insult to the retinal deep capillary plexus. We add a previously undescribed association with idiopathic intracranial hypertension to the list of etiologies that can cause PAMM. [ABSTRACT FROM AUTHOR]

Purpose: To report a case of primary congenital glaucoma associated with paracentral acute middle maculopathy. Methods: A case report. Results: A 14-year-old girl with undiagnosed primary congenital glaucoma was referred for the evaluation of sudden vision loss a week after the initial symptoms. Visual acuity was counting fingers at 2 m in the right eye. Examination of her right eye revealed corneal stromal scar like Haab striae, splinter hemorrhage at the temporal border of the optic disk, perivenular hemorrhage, and fern-like white area at the macula. On optical coherence tomography of the right eye, hyperreflective plaques at the level of the inner nuclear layer/ outer plexiform layer were present, corresponding to the opaque areas in fundoscopy. On optical coherence tomography angiography of the right eye, ischemic areas in deep capillary plexuses were present. These findings in optical coherence tomography angiography and optical coherence tomography were consistent with paracentral acute middle maculopathy. Conclusion: This is the first case of primary congenital glaucoma associated with paracentral acute middle maculopathy in the literature, and primary congenital glaucoma might be a predisposing factor. [ABSTRACT FROM AUTHOR]

Purpose: To report the imaging findings of a single case of paracentral acute middle maculopathy associated with bilateral Purtscher-like retinopathy in the setting of recent viral illness. Methods: Case report, multimodal imaging. Results: A 21-year-old woman who awoke with sudden-onset bilateral finger count vision in the setting of a recent viral illness. Initial examination and imaging was consistent with a bilateral Purtscher-like retinopathy with evidence of paracentral acute middle maculopathy and ellipsoid zone thickening and hyperreflectivity on spectral domain optical coherence tomography (OCT). Baseline fluorescein angiography and OCT angiography revealed normal perfusion and flow, respectively. All laboratory investigation was negative for possible causative etiology. The patient was followed for 6 months with steady visual improvement to 20/20 in both eyes. En face OCT demonstrated near-complete restoration of the ellipsoid layer. The patient still noted bilateral scotomas which were mapped out on microperimetry and found to correlate to en face OCT findings. Conclusion: Purtscher-like retinopathy in the setting of recent viral illness is a rare cause of vision loss that may be associated with the finding of paracentral acute middle maculopathy on spectral domain OCT. Despite poor presenting vision, in the presence of normal perfusion near total visual recovery is possible. [ABSTRACT FROM AUTHOR]

Purpose: To compare the spectral domain and swept source optical coherence tomography angiography findings in two cases of sickle cell maculopathy. Methods: A 53-year-old man and a 24-year-old man both with sickle cell disease (hemoglobin SS) presented with no visual complaints; Humphrey visual field testing demonstrated asymptomatic paracentral scotomas that extended nasally in the involved eyes. Clinical examination and multimodal imaging including spectral domain and swept source optical coherence tomography, and spectral domain optical coherence tomography angiography and swept source optical coherence tomography angiography (Carl Zeiss Meditec Inc, Dublin, CA) were performed. Results: Fundus examination of both patients revealed subtle thinning of the macula. En-face swept source optical coherence tomography confirmed the extent of the thinning correlating with the functional paracentral scotomas on Humphrey visual field. Swept source optical coherence tomography B-scan revealed multiple confluent areas of inner nuclear thinning and significant temporal retinal atrophy. En-face 6 × 6-mm spectral domain optical coherence tomography angiography of the macula demonstrated greater loss of the deep capillary plexus compared with the superficial capillary plexus. Swept source optical coherence tomography angiography 12 × 12-mm imaging captured the same macular findings and loss of both plexuses temporally outside the macula. Conclusion: In these two cases of sickle cell maculopathy, deep capillary plexus ischemia is more extensive within the macula, whereas both the superficial capillary plexus and deep capillary plexus are involved outside the macula likely due to the greater oxygen demands and watershed nature of these areas. Swept source optical coherence tomography angiography clearly demonstrates the angiographic extent of the disease correlating with the Humphrey visual field scotomas and confluent areas of inner nuclear atrophy. [ABSTRACT FROM AUTHOR]

Purpose: To report the occurrence of a branch retinal artery occlusion with paracentral acute middle maculopathy in an otherwise healthy young man with a history of livedo reticularis (LR). Methods: Retrospective case report. Patients: A 21-year-old man with a history of LR being treated with pentoxifylline developed an acute branch retinal artery occlusion with initial best-corrected visual acuity at presentation of 20/80. Results: A thorough diagnostic work up was negative for potential causes of branch retinal artery occlusion or LR. The patient was continued on pentoxifylline and started on aspirin 81 mg daily. At five-month follow-up, vision had improved to 20/25. Optical coherence tomography testing showed a hyperreflective band in the inner nuclear layer and outer plexiform layers in the affected eye that ultimately thinned, consistent with paracentral acute middle maculopathy. Conclusion: To our knowledge, this is the first case of branch retinal artery occlusion occurring in a patient with a history of LR. This could potentially be an early manifestation of Sneddon syndrome, a rare entity characterized by LR and cerebrovascular disease, which has been previously associated with central retinal artery occlusions. [ABSTRACT FROM AUTHOR]

Purpose: To report a case of reperfused central retinal artery occlusion causing a globular pattern of paracentral acute middle maculopathy with en face optical coherence tomography (OCT). Methods: Retrospective case report. Widefield fluorescein angiography, en face OCT, and OCT-angiography were performed. Retinal capillary plexus vessel density (mm-1) was measured using OCT-angiography analysis and was defined as total vessel length (mm) per area (mm²). Results: A 76-year-old female presented with decreased vision in the left eye for 1 day. Widefield fluorescein angiography revealed delayed venous filling in the early phase and selective arterial staining in the late phase. Spectral domain OCT demonstrated paracentral acute middle maculopathy. Optical coherence tomography-angiography showed an ischemic deep retinal capillary plexus that colocalized with a globular shaped hyper-reflective pattern identified with structural en face OCT. Quantitative vessel density analysis comparing the fellow eye to the affected eye at presentation demonstrated 43% reduction in the superficial capillary plexus vessel density and 33% reduction of the deep capillary plexus in the affected eye. At 2 months follow-up, the reduction of superficial capillary plexus vessel density improved to 33%, whereas the deep capillary plexus vessel density reduced further to 35% compared with the fellow eye. Conclusion: The authors report a case of reperfused central retinal artery occlusion leading to a globular shaped paracentral acute middle maculopathy lesion with en face OCT. Quantitative OCT-angiography vessel density analysis in the chronic phase revealed disproportionate reduction of deep capillary plexus vessel density. The globular pattern of paracentral acute middle maculopathy with en face OCT can be an important clue to the diagnosis of central retinal artery occlusion. [ABSTRACT FROM AUTHOR]

Purpose: To report a single case of paracentral acute middle maculopathy in association with retinal artery occlusion in the setting of ipsilateral facial cosmetic filler injection. Methods: Case report. Patient: A 35-year-old woman presenting with sudden vision loss to finger count vision immediately after left nasal fat pad cosmetic filler injection. Results: Dilated funduscopic examination revealed a swollen optic disc with multiple branch arterial occlusions with visible embolic material. Fluorescein angiography confirmed multiple branch arterial occlusions in addition to a focal choroidal infarction in the macula. Spectral-domain optical coherence tomography revealed middle retinal hyperreflectivity in the superotemporal macula consistent with paracentral acute middle maculopathy. En face optical coherence tomography demonstrated a superotemporal area of whitening at the level of the deep capillary plexus corresponding to the paracentral acute middle maculopathy lesion seen on spectral-domain optical coherence tomography. On twelvemonth follow-up, final visual acuity was 20/100 due to optic neuropathy. Conclusion: Emboli from cosmetic facial filler injections may rarely result in ipsilateral arterial occlusions and now have a novel association with paracentral acute middle maculopathy likely due to deep capillary plexus feeder vessel occlusion. [ABSTRACT FROM AUTHOR]

Background/Purpose: To present a novel case of paracentral acute middle maculopathy in association with birdshot chorioretinopathy. Methods: Case report. Results: A patient presented with decreased vision and findings of uveitis, vasculitis and a paracentral scotoma. Multimodal imaging was consistent with paracentral acute middle maculopathy in the setting of a multifocal chorioretinopathy and a laboratory workup was consistent with birdshot chorioretinopathy. Conclusion: Paracentral acute middle maculopathy is a newly described entity that may be associated with birdshot chorioretinopathy, and clinicians should be aware of it when patients present with new paracentral scotoma and signs of intraocular inflammation. [ABSTRACT FROM AUTHOR]

The article describes the case of a 71-year-old woman who complained of a sudden spot in the vision of her left eye for 2 weeks. Spectral domain optical coherence tomography findings suggest Purtscher-like retinopathy and the patient was later found to have metastatic pancreatic cancer. It concluded that Purtscher-like retinopathy can co-exist with a malignant pancreatic cancer.