Paracentral Acute Middle Maculopathy Research Articles

Commentary: COVID-19 disease is more than just a respiratory infection

The coronavirus disease (COVID-19), caused by the novel, highly contagious severe acute respiratory syndrome coronavirus 2, has quickly become a global health emergency. It started with an outbreak of pneumonia causing fever, cough, and shortness of breath. However, the virus has been noted to involve multiple organs and systems. The global research community has made an impressive effort in compiling the various manifestations of the disease in a short time.[1] The infected individuals are predisposed to arterial and venous thromboembolic complications like deep vein thrombosis, pulmonary embolism, myocardial infarction, and stroke. The proposed mechanisms responsible for these thromboembolic events include a transient hypercoagulable state due to systemic inflammation and cytokine storm, postinfectious immune-mediated responses, and direct viral-induced endothelitis.[2] The incidence of thrombotic complications in COVID-19 patients admitted to intensive care unit ranges between 16 to 49%.[1] Acute cardiovascular accident has been reported in 0.5–6.0% of COVID-19 patients.[3] Acharya et al.[4] first reported a central retinal artery occlusion (CRAO) in a case of severe COVID-19 disease. However, recent evidence suggests that arterial thromboembolism can occur in patients with mild disease also. Currently, the incidence is unknown due to possible underreporting of symptoms. Turedi et al. first reported CRAO within a case of moderate COVID-19 disease.[5] Early anticoagulant treatment has been reported to improve the clinical outcome of COVID-19 patients and reduce the risk of thromboembolic events.[1] Interestingly, all the patients that have been reported to develop RAO secondary to COVID-19 were already being treated with anticoagulant therapy. The occurrence of RAO in COVID-19 patients is limited to only case reports. Further studies should be planned to evaluate out the incidence of vascular occlusions in patients suffering and recovered from COVID-19. Such studies can give us insight related to the association of the ocular diseases with the stage and severity of the COVID-19 infection. Our knowledge regarding the pathophysiology and treatment of this disease is still in its preliminary stage. The fast-paced ongoing research will hopefully provide better algorithms for management of this disease and prevention of its complications. Physicians treating COVID-19 patients must be sensitized to seek an urgent retina consultation for patients complaining of symptoms like blurry vision, sudden painless visual loss, or scotomas. We congratulate the authors for reporting the first case of an isolated cilioretinal artery occlusion (CLRAO) associated with paracentral acute middle maculopathy (PAMM) in a patient with moderate COVID-19 disease and no other predisposing diseases. PAMM is reported to be present in 100% of isolated CLRAO due to hypoperfusion of the involved cilioretinal artery.[6] The authors started treating the patient 48 h after the episode and gave 20 sessions (40 h) of hyperbaric oxygen (HBO), which led to resolution of retinal edema after 3 weeks. HBO involves the inhalation of 100% oxygen at pressures greater than 1 atm. The inspired oxygen partial pressure is nearly 203 kPa, which is almost 10 times that of breathing air at normal atmospheric pressure. It is postulated that oxygen at higher pressures diffuses from the choroidal circulation to reach the ischaemic retina and increase the oxygen delivery to the ischemic tissue until spontaneous or assisted reperfusion occurs.[7] However, isolated CLRAO is associated with good prognosis and does not usually require treatment. Retinal edema usually resolves spontaneously within few weeks. The central field defect improves in nearly half of the eyes and remains stable in the other half.[8] HBO is usually reserved for patients presenting with poor vision due to CRAO and BRAO. Also, the maximum benefit of the treatment is expected in case it is started within 6–8 h of the occlusion.[7] The risk-benefit for the application of multiple sessions to gain <1 line of vision during the COVID-19 pandemic should be carefully calculated. Role of corticosteroids in COVID-19 has been evaluated by several studies. The RECOVERY trial was a prospective study where 2104 patients were randomized to dexamethasone 6 mg per day (oral or intravenous) for 10 days and compared to the 4321 patients receiving usual care. They concluded that in patients hospitalized with COVID-19, dexamethasone reduced the 28-day mortality among those receiving invasive mechanical ventilation or oxygen at randomization, but not among patients with mild to moderate COVID-19 disease.[9]

Commentary: Retinal manifestations in patients following COVID-19 infection: A consecutive case series.

Coronavirus disease-2019 (COVID-19) pandemic has created a global health challenge owing to widespread mortality and morbidity. Alterations of the immune system, activation of coagulation pathways, and direct cytopathic effect of the virus are purported as the possible mechanisms that are responsible for the pathology of COVID-19. In addition to these, side effects of drugs and complications related to prolonged hospitalization and intensive care all combine to cause systemic manifestations of this virus, including ocular complications.[12] Multiple ophthalmic manifestations related to COVID-19 have been reported. Initially, when the pandemic started, ocular surface involvement was reported with self-limiting conjunctivitis being the earliest reported ocular manifestation.[3] Subsequently, a plethora of retinal manifestations have been reported in these patients, namely cotton wool spots, flame-shaped and dot-blot hemorrhages, retinal vascular occlusions, acute macular neuroretinopathy (AMN), paracentral acute middle maculopathy (PAMM), fungal retinitis, acute retinal necrosis, and serpiginous choroiditis. Neuro-ophthalmic manifestations like optic neuritis, papillo-phlebitis, extra-ocular muscle palsies, neurogenic ptosis, intracranial hypertension, cerebrovascular accident with vision loss, and orbital manifestations like mucormycosis and histiocytic orbital mass have also been reported in patients with COVID-19.[145] In the current study, authors have enumerated varied retinal manifestations following COVID-19. Patients with candida retinitis, tubercular choroidal abscess, and central serous chorioretinopathy (CSC) have been reported for the first time in this cohort.[6] Coagulopathy and endothelial dysfunctions are known associations of COVID-19 infection. In the eye, these manifest as cotton wool spots, central retinal vein occlusion, central retinal artery occlusion, AMN, and PAMM.[57] As reported in the current article, the aforementioned entities may occur during the active phase or even after recovery from COVID-19. Presence of cotton wool spots has been shown to be a potential biomarker in identifying underlying vascular and/or neurological comorbidity in COVID-19 patients.[7] Mean retinal artery diameter and the mean retinal vein diameter (MVD), measured using the Automated Retinal Image analyzer, have been shown to be higher in COVID-19 patients compared to healthy cohorts. MVD was negatively correlated with the time from onset of symptoms and positively correlated with disease severity.[8] Herein lies the role of an ophthalmologist and ocular examination in COVID-19 patients. Fundus examination is a simple, easy to perform and non-invasive. It has been shown to predict systemic endothelial dysfunction and triggering of the inflammatory cascade in subclinical and asymptomatic patients, thereby allowing earlier institution of appropriate therapy and potentially improving long-term morbidity and mortality.

Status of Choriocapillaris in Fellow Eyes of Patients With Unilateral Retinal Vein Occlusions.

To study the status of the choriocapillaris in fellow eyes of patients with unilateral retinal vein occlusions (RVOs). Thirty-two healthy eyes of patients with unilateral RVO and 16 eyes of healthy age-matched controls were included. Choriocapillaris flow voids and device-specific choriocapillaris total flow areas were quantified based on 3-mm optical coherence tomography angiography (OCTA) scans and their correlation with the number of resolved para-central acute middle maculopathy (PAMM) lesions on 6-mm OCTA scans was calculated. In fellow eyes of unilateral RVOs and in eyes of healthy individuals, the number of choriocapillaris flow voids was 20.8 ± 5.5 and 13.4 ± 5.4, respectively (P < .001), and choriocapillaris total flow area was 6.0 ± 0.34 mm2 and 6.22 ± 0.13 mm2, respectively (P = .005). The number of resolved PAMM lesions correlates significantly with the number of choriocapillaris flow voids (r = 0.44; P = .002) and with choriocapillaris total flow area (r = -0.52; P < .001). Fellow eyes of patients with unilateral RVO demonstrate a substantial decrease of perfusion in choriocapillaris, which correlates with the prevalence of small resolved PAMM lesions. [Ophthalmic Surg Lasers Imaging Retina. 2021;52:23-28.].

Evaluation of paracentral acute middle maculopathy with multimodal imaging in a pregnant patient

Evaluation of paracentral acute middle maculopathy with multimodal imaging in a pregnant patient

Paracentral acute middle maculopathy: from diagnosis toward clinical perspectives

In this review of literature, we reviewed the current state of knowledge on paracentral acute middle maculopathy (PAMM). The diversity of clinical manifestations, a variety of related cardiovascular and ocular conditions, as well as reported idiopathic cases, allow considering PAMM as a distinct clinical phenomenon or a syndrome. Taking into account already described and potential associations with systemic and ocular morbidity, PAMM may apply for a place of an important clinical biomarker not only for ocular but for systemic disorders in a broad cohort of patients. However, the understanding of the pathophysiology of PAMM and of its actual clinical significance is far from complete, and further studies are warranted.

Vascular Microanatomy of Small Resolved Paracentral Acute Middle Maculopathy Lesions

To study microvascular characteristics of small resolved paracentral acute middle maculopathy (PAMM) lesions in fellow eyes of patients with unilateral retinal vein occlusion (RVO). Prospective cross-sectional study. Patients with prior unilateral branch or central RVO and OCT evidence of resolved PAMM in the fellow, otherwise normal, eye were recruited prospectively and imaged with OCT angiography (OCTA). The resolved PAMM lesions were identified as focal areas of inner nuclear layer thinning over an anteriorly displaced outer plexiform layer (OPL). En face OCTA projections showing the location and size of the resolved PAMM lesions were created using 2 OPL segmentation lines with -9-μm and 0-μm offsets, and the cumulative distribution was evaluated. Anterior to the resolved PAMM lesions, vessels in the superficial vascular plexus were traced to identify small arterioles supplying the affected areas. Cumulative spatial distribution on small resolved PAMM lesions. From 24 fellow eyes of 24 patients with unilateral RVO (15 men and 9 women; mean age, 62.1 ± 13.6 years), 152 resolved PAMM lesions were identified. Of these lesions, 130 (85.5%) were found within the perifoveal region, and only 12 (7.9%) were found within the temporal quadrant. Of 28 lesions analyzed, the arteriole supplying the affected area was a single side branch of a larger vessel, with only 3 supplied by a terminal branch. Small resolved PAMM lesions in fellow eyes of patients with unilateral RVO are most prevalentin perifoveal regions supplied by side branches of low-order retinal arteries.

BRANCH RETINAL ARTERY OCCLUSION WITH PARACENTRAL ACUTE MIDDLE MACULOPATHY PRESUMABLY RELATED TO HEAVY CANNABIS USE

To report a case of branch retinal artery occlusion associated with paracentral acute middle maculopathy on spectral-domain optical coherence tomography presumably related to heavy cannabis consumption. Retrospective case report. Spectral-domain optical coherence tomography, fluorescein angiography, and optical coherence tomography angiography were performed. A 21-year-old healthy man described the acute onset of superior visual field loss in his right eye. He admitted smoking approximately 15 g daily of cannabis for several weeks during COVID-19 confinement. Ophthalmoscopic examination of the right eye showed inferotemporal retinal whitening. Spectral-domain optical coherence tomography illustrated evidence of the ischemic cascade with diffuse hyperreflectivity of the inner and middle retinal layers within the central region of the retinal infarct and paracentral acute middle maculopathy at the border of the infarct. Optical coherence tomography angiography demonstrated predominant flow signal loss at the level of the deep retinal capillary plexus. Fluorescein angiography and complete systemic workup were unremarkable. Branch retinal artery occlusion and paracentral acute middle maculopathy may be related to heavy cannabis use as the result of transient arterial vasospasm.

Paracentral Acute Middle Maculopathy after Severe Headache

Paracentral Acute Middle Maculopathy after Severe Headache

Neurovascular Causes of Acute Monocular Visual Loss

Acute monocular visual loss may result from a diverse set of neurovascular and systemic disorders and requires urgent evaluation. The goal of this review is to describe the most current understanding of the clinical features, pathophysiology, diagnosis, and treatment of these disorders. 1. Acute central retinal artery occlusion (CRAO) should be treated as an equivalent to acute ischemic stroke. 2. Several recent clinical trials are evaluating the treatment of non-arteritic anterior ischemic optic neuropathy (NAION). 3. Tocilizumab is emerging as a first-line steroid-sparing immunomodulatory therapy for the treatment of giant cell arteritis (GCA). 4. Optical coherence tomography (OCT) imaging of the retina has led to the appreciation that retinal capillary ischemia in the form of paracentral acute middle maculopathy (PAMM) is a distinct form of acute monocular visual loss with relatively normal-appearing fundus examination and good prognosis for recovery. Accurate diagnosis and timely treatment of disorders resulting in acute monocular visual loss may reduce the likelihood of additional visual and neurologic complications.

Paracentral Acute Middle Maculopathy Mimicking Retrobulbar Optic Neuropathy

A 70-year-old woman presented with acute awareness of decreased color vision in her right eye. Fundus examination and a flash electroretinography (ERG) were both normal. A multifocal ERG (mfERG) however revealed foveal depression, and thinning of inner nuclear layer was noted on macular optical coherence tomography (OCT), and a diagnosis of resolved paracentral acute middle maculopathy was made. Clinicians should be aware of the complementary role of OCT and mfERG in unexplained acute central visual loss to distinguish retinal from neuro-ophthalmic etiologies. Structural ocular imaging with OCT shows features of inner, middle, and outer retinal localizations to the visual loss.

Paracentral acute middle maculopathy and the organization of the retinal capillary plexuses.

The retinal capillary vasculature serves the formidable role of supplying the metabolically active inner and middle retina. In the parafoveal region, the retinal capillary plexuses (RCP) are organized in a system of three capillary layers of varying retinal depths: the superficial capillary plexus (SCP), intermediate capillary plexus (ICP) and deep capillary plexus (DCP). While the dynamic flow through these plexuses is complex and not completely understood, current research points to a hybrid model that includes both parallel and in series components in which blood flows in a predominantly serial direction between the superficial vascular complex (SVC) and deep vascular complex (DVC). Each capillary plexus autoregulates independently, so that under most conditions the retinal vasculature supplies adequate blood flow and oxygen saturation at varying depths despite diverse environmental stressors. When the flow in the deep vascular complex (i.e. ICP and DCP) fails, an ischemic lesion referred to as Paracentral Acute Middle Maculopathy (PAMM) can be identified. PAMM is an optical coherence tomography (OCT) finding defined by the presence of a hyperreflective band at the level of the inner nuclear layer (INL) that indicates INL infarction caused by globally impaired perfusion through the retinal capillary system leading to hypoperfusion of the DVC or specifically the DCP. Patients present with an acute onset paracentral scotoma and typically experience a permanent visual defect. Lesions can be caused by a diverse set of local retinal vascular diseases and systemic disorders. PAMM is a manifestation of the retinal ischemic cascade in which the mildest forms of ischemia develop at the venular end of the DCP, i.e. perivenular PAMM, while more severe forms progress horizontally to diffusely involve the INL, and the most severe forms progress vertically to infarct the inner retina. Management is targeted toward the identification and treatment of related vasculopathic and systemic risk factors.

Differences in the multicolour imaging features between the superficial and deep vascular occlusions.

In this retrospective observational study, patients diagnosed with central retinal artery and branch retinal artery occlusion, cotton-wool spot, paracentral acute middle maculopathy and acute macular neuroretinopathy between January 2018 and June 2019 were included. Colour fundus photograph, optical coherence tomography and MI of these patients were analysed. A total of 41 eyes of 40 patients were included in this study. In eyes with central retinal artery occlusion, MI showed white areas in the retina with orange foveal centre. In eyes with branch retinal artery occlusion, MI showed white area along the affected retinal quadrant without an orange foveal centre. In pure superficial vascular plexus occlusions as in cotton-wool spots, the lesion was identified on MI as a white lesion. On MI, paracentral acute middle maculopathy showed parafoveal white areas with orange foveal centre while acute macular neuroretinopathy on MI parafoveal greyish-white areas with normal foveal centre. En-face images using MI technology can provide yet another way to identify the level of retinal vasculature involvement which complements the existing gold standard of optical coherence tomography imaging.

Парацентральная острая срединная макулопатия: особенности клинической диагностики

Парацентральная острая срединная макулопатия: особенности клинической диагностики

Paracentral acute middle maculopathy-review of the literature.

Paracentral acute middle maculopathy (PAMM) is a recently identified spectral-domain optical coherence tomography (SD-OCT) finding characterized by a hyper-reflective band spanning the inner nuclear layer (INL), which typically evolves to INL atrophy in later stages. Typical clinical features include the sudden onset of one or multiple paracentral scotomas, normal or mild reduction in visual acuity, and a normal fundus appearance or a fundus with a deep grayish lesion. Although its pathophysiology is not yet fully understood, ischemia at the level of the intermediate and deep capillary plexa has been demonstrated to play a major role. Since its first description, an increasing number of publications on PAMM have been published in ophthalmology scientific journals. The purpose of this study is to provide a review of the current literature on PAMM.

CENTRAL RETINAL VEIN OCCLUSION IN YOUNG PATIENTS: Clinical Characteristics and Prognostic Factors.

To investigate the clinical characteristics and prognostic factors of young patients with central retinal vein occlusion (CRVO). This retrospective cohort study involved treatment-naïve patients with CRVO. Medical records regarding basic demographics, predisposing factors, ocular characteristics, and treatments were reviewed and compared according to age at CRVO onset. We enrolled 263 patients, of whom 69 were younger patients. Younger patients had higher prevalence of nontraditional risk factors including physical or psychological stress (P = 0.032), hematologic abnormalities (P = 0.003), and better visual acuity at baseline and last visit (all P < 0.001) and were unlikely to undergo intravitreal injections (47.8 vs. 68.6%, P < 0.001) during follow-up. Younger patients had higher prevalence of paracentral acute middle maculopathy (28.1 vs. 4.7%, P < 0.001). Older age (odds ratio = 1.165, P = 0.028), male sex (odds ratio = 7.074, P = 0.034), coexisting renal disease (odds ratio = 7.845, P = 0.050), and poor baseline visual acuity (odds ratio = 16.069, P = 0.002) were significant risk factors for poor visual outcomes in young CRVO patients. Younger CRVO patients had a milder clinical course with fewer treatments and were more likely to have nontraditional risk factors than older patients.

Paracentral Acute Middle Maculopathy with Patent Foramen Ovale

Paracentral Acute Middle Maculopathy with Patent Foramen Ovale

Subclinical Retinal versus Brain Findings in Infants with Hypoxic Ischemic Encephalopathy.

To detect retinal features and abnormalities on optical coherence tomography (OCT) without pupil dilation and relate these to brain injury in infants with a clinical diagnosis of hypoxic ischemic encephalopathy (HIE). Under an institutional review board-approved protocol, we imaged eight infants without pharmacologic mydriasis, using handheld, non-contact spectral-domain (Leica Microsystems, IL) or investigational swept-source OCT at the bedside in an intensive care nursery, after birth (depending on primary clinical care team permission based on health status) and weekly until discharge. The newborn infant with HIE is neurologically unstable; therefore, pharmacologic mydriasis and stimulation with visible light for retinal examination are usually avoided. We analyzed images for retinal pathologies, central foveal thickness, and retinal nerve fiber layer (RNFL) thickness at the papillomacular bundle and compared them to historical controls and published normative data, HIE clinical assessment, and abnormalities on brain magnetic resonance imaging (MRI). On OCT, three of eight infants had bilateral multiple small macular and perimacular cystoid spaces; two of these three infants also had pronounced retinal ganglion cell layer thinning and severe brain injury on MRI and the third had bilateral paracentral acute middle maculopathy and mild brain injury on MRI. Other findings in HIE infant eyes included abnormally thin fovea and thin RNFL and markers of retinal immaturity such as the absence of sub-foveal photoreceptor development and sub-foveal fluid. Bedside handheld OCT imaging within the first 2 weeks of life revealed retinal injury in infants with HIE-related brain injury. Future studies may determine the relationship between acute/subacute retinal abnormalities and brain injury severity and neurodevelopmental outcomes in HIE.

Hyperacute Retinal Arterial Wall Dynamics in Embolic Paracentral Acute Middle Maculopathy

ABSTRACT To report the hyperacute compensatory vascular responses to retinal artery occlusion using spectral-domain optical coherence tomography (SD-OCT) and OCT-Angiography (OCT-A).

Visual improvement in a patient with paracentral acute middle maculopathy treated with prostaglandin E1

The authors present the use of prostaglandin E1 (PGE1) for the treatment of an acute paracentral acute middle maculopathy (PAMM). A 78-year-old white female was seen with a sudden loss of vision in her left eye (OS) to 20/200 noted upon awakening. The right eye (OD) saw 20/20. A complete eye exam was done and an ocular coherent tomography revealed retinal thickening and a whitening of the inner nuclear layer in the area of the macula OS. A diagnosis of PAMM in the OS was made. Treatment was immediately started with 70 μg of PGE1 administered over 1.5 hours in the form of a skin cream. A volume of 3.5 cc of skin cream was applied in divided doses to the inner surface of the forearm, rubbed into the skin and allowed to dry. The same 70 μg of PGE1 in 3.5 cc of skin cream was repeated once the next morning. The patient began to see better the second day of treatment with a final visual acuity of 20/20. The OD was unchanged. After 14 months she was stable with no further treatment. PAMM is an ischemic process of the inner retina. PGE1, a potent vasodilator of the microcirculation, when given immediately seemed to be useful in restoring vision in this form of retinal ischemia. Treatment was immediately started with PGE1 in the form of a skin cream with visual improvement. The authors normally use PGE1 intravenously for acute ocular ischemia and would have preferred that here. Intravenous PGE1 was not available and was substituted with the skin cream of PGE1 that worked well for the patient.

BILATERAL PARACENTRAL ACUTE MIDDLE MACULOPATHY AFTER CARDIOPULMONARY BYPASS.

To report the first two observations of bilateral paracentral acute middle maculopathy in the early postoperative period after cardiopulmonary bypass procedures. Comprehensive ophthalmic examinations were performed, including best-corrected visual acuity, Humphrey visual field testing, dilated fundus examination, and optical coherence tomography. 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. 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.