Deep Capillary Ischemia Research Articles

Case Series: Paracentral Acute Middle Maculopathy as a Sign of Severe Systemic Vasculopathy

Paracentral acute middle maculopathy represents deep capillary ischemia that can cause acute symptoms of scotoma. This case series describes two cases of symptomatic paracentral acute middle maculopathy that reflect the more severe spectrum of associated etiologies. Case 1: A 65-year-old male with recent history of hypertensive encephalopathy presented with sudden onset central visual disturbance described as amorphic in shape. Dilated fundus exam revealed severe hypertensive retinopathy with paracentral acute middle maculopathy. The patient died one year after the initial discovery of the retinal lesions. Case 2: A 87-year-old male presented with paracentral vision loss in one eye for three days. Clinical examination revealed multiple Hollenhorst plaques and paracentral acute middle maculopathy at presentation, and a subsequent branch retinal artery occlusion two days later. The patient was found to have 76% left carotid artery stenosis with numerous brain infarcts. The detection of paracentral acute middle maculopathy indicates an investigation through ocular and systemic risk factors to determine an attributable etiology. The search for possible etiologies should be tailored to the particular patient, based on age, associated ocular findings, systemic history, and symptomatic versus incidental presentation. The presence of symptomatic paracentral acute middle maculopathy, particularly in at-risk vasculopathic patients, should be addressed urgently as it may be an indicator of an underlying severe systemic disease process. CE Notification: This article is available as a COPE accredited CE course. You may take this course for 1-hour credit. Read the article and take the qualifying test to earn your credit. Click here to Enroll (https://www.crojournal.com/case-series-paracentral-acute-middle-maculopathy-as-a-sign-of-severe-systemic-vasculopathy)

RETINAL DEEP CAPILLARY PLEXUS ISCHEMIA IN A CASE WITH ANTIPHOSPHOLIPID SYNDROME

To report visualization of a case of retinal deep capillary plexus ischemia with antiphospholipid syndrome. A 53-year-old woman was referred with a 1-week history of sudden onset of decreased vision in the right eye. Her symptoms were evaluated by clinical examination, infrared reflectance, fundus autofluorescence, fluorescein angiography, spectral-domain optical coherence tomography, microperimetry, and multifocal electroretinography. The patient's visual acuity on presentation was 0.4 (Snellen) in the right eye and 0.7 in the left eye. Spectral-domain optical coherence tomography showed increased reflectivity of inner nuclear and inner plexiform layers of the right eye. Laboratory evaluation revealed abnormal titers of lupus anticoagulant antibodies. After 6 months, her visual acuity was finger counting at 1 m in the right eye and 0.4 in the left eye. Spectral-domain optical coherence tomographic image demonstrated a diffuse thinning of those retinal layers in the right eye. These findings are consistent with the clinical characteristics of retinal deep capillary ischemia in association with antiphospholipid syndrome. Deep capillary ischemia has some characteristic findings, and on spectral-domain optical coherence tomography, outcomes in conjunction with multimodal imaging are helpful for the diagnosis of the acute and the chronic stages of retinal deep capillary plexus ischemia.

OCT Angiography of Paracentral Acute Middle Maculopathy Associated With Central Retinal Artery Occlusion and Deep Capillary Ischemia

An 82-year-old man presented with acute, painless vision loss in the left eye associated with headaches, jaw claudication, and scalp tenderness. Clinical examination and fluorescein angiography confirmed the diagnosis of a central retinal artery occlusion of the left eye. Spectral-domain optical coherence tomography (OCT) of the left eye showed paracentral acute middle maculopathy (PAMM), and OCT angiography showed severe attenuation of the deep capillary plexus. This is the first case report of OCT angiography of PAMM associated with central retinal artery occlusion confirming the presence of ischemia of the deep retinal capillary plexus.

Could Progressive Outer Retinal Necrosis Begin With Retinal Deep Capillary Ischemia?—Reply

Could Progressive Outer Retinal Necrosis Begin With Retinal Deep Capillary Ischemia?—Reply

The Spectrum of Superficial and Deep Capillary Ischemia in Retinal Artery Occlusion

To describe the spectrum of retinal capillary ischemia, including superficial and deep capillary ischemia, as identified with spectral-domain optical coherence tomography (SD OCT), that occurs in retinal arterial occlusive disease. Retrospective observational case series. Clinical charts, color fundus photography, red-free fundus photography, fluorescein angiography, near-infrared reflectance, and SD OCT imaging in 40 eyes of 35 patients with retinal arterial occlusive disease were studied in both the acute and chronic phases in multicenter clinical practices. SD OCT imaging analysis was employed to characterize the presence of superficial and deep capillary ischemia in each eye. Of the 40 eyes, 15 eyes had central retinal artery occlusion (CRAO), 22 eyes had branch retinal artery occlusion (BRAO), and 3 eyes had cilioretinal artery occlusion. During the acute phase, SD OCT showed the following 3 distinct patterns, related to retinal ischemia occurring at varying levels within the retina: (1) thickening and hyperreflectivity of the inner retinal layers, including the nerve fiber and ganglion cell layers owing to ischemia of the superficial capillary plexus; (2) a hyperreflective band at the level of the inner nuclear layer, termed "paracentral acute middle maculopathy," representing ischemia of the intermediate and deep retinal capillary plexuses (deep capillary ischemia); and (3) diffuse thickening and hyperreflectivity of both the inner and middle retinal layers, which represented both superficial and deep capillary ischemia. Of all eyes, 31 (78%) had both superficial and deep lesions. The remaining 9 eyes (22%) had isolated deep capillary ischemia producing paracentral acute middle maculopathy with sparing of the superficial capillary plexus and a normal fluorescein angiographic appearance. As the lesions evolved into the chronic phase over the ensuing 3 months, the resultant thinning and atrophy reflected the retinal layers affected during the acute phase. SD OCT imaging reveals the spectrum of capillary ischemia in retinal artery occlusive disease showing variable involvement of the superficial and intermediate/deep capillary plexuses. Isolated deep capillary ischemia manifested as paracentral acute middle maculopathy on SD OCT and may be seen in some eyes with retinal arterial circulation compromise despite complete absence of perfusion abnormalities on fluorescein angiography.

Paracentral acute middle maculopathy spectral-domain optical coherence tomography feature of deep capillary ischemia

To describe the novel spectral-domain optical coherence tomography (SD-OCT) finding of paracentral acute middle maculopathy (PAMM) that can be associated with acute macular neuroretinopathy (AMN) or retinal vasculopathy, and that may indicate an underlying pathogenesis related to ischemia of the retinal deep capillary plexus (DCP). With the advent of high-definition SD-OCT imaging, we are now able to detect deep capillary ischemia. Although cotton wool spots are caused by ischemia of the superficial capillary plexus, PAMM is caused by ischemia of the DCP, and appears as hyper-reflective, band-like lesions in the middle retina, extending from the inner nuclear layer (INL)/outer plexiform layer junction to involve the full-thickness INL. Over time, these lesions resolve with atrophy of the INL, resulting in persistent paracentral scotomas for the patient. Originally, PAMM was identified in isolation and thus classified into a subcategory of AMN. Subsequent reports have demonstrated PAMM lesions in the setting of coexisting retinal vascular disease, notably nonproliferative diabetic retinopathy and central retinal vein occlusion. PAMM refers to a recently described class of characteristic SD-OCT lesions involving the middle layers of the retina at the level of the INL. If a patient presenting with PAMM is young and healthy without any other vascular disease, this may represent a novel variant of AMN. However, if the patient has an underlying retinal vascular disease, such as diabetic retinopathy or retinal vascular occlusion, one would refer to the lesion as PAMM complicating the underlying retinal vascular disease.

MULTIMODAL IMAGING FINDINGS IN RETINAL DEEP CAPILLARY ISCHEMIA

To evaluate the multimodal imaging findings in retinal deep capillary ischemia (DCI). This was a retrospective review of 5 eyes of 4 patients with sudden onset of paracentral scotomas caused by DCI. Multimodal imaging techniques, including color and red-free photographs, near-infrared reflectance, fluorescein angiography, and spectral-domain optical coherence tomography, were performed in all eyes, and the findings were correlated with microperimetry in two eyes. Imaging findings in DCI were compared with those of a cotton wool spot caused by superficial capillary ischemia (SCI). Unlike SCI, the imaging findings in DCI were subtler during both the acute and chronic phase, but specific optical coherence tomographic findings could readily differentiate these entities. Acute SCI showed inner retinal whitening, edema, and increased reflectivity, whereas acute DCI showed increased reflectivity of middle retinal layers. Chronic DCI showed retinal thinning with middle layer atrophy, whereas chronic SCI showed inner layer atrophy. In one patient, microperimetry showed a paracentral dense scotoma that corresponded well to the optical coherence tomographic findings. Deep capillary ischemia may represent a nonspecific finding of retinal ischemia and produces characteristic changes within the middle retinal layers, analogous to a deep cotton wool spot, but with distinct features differing from the superficial cotton wool spot which is seen in SCI. Among the various multimodal imaging techniques, optical coherence tomography seemed to be the most sensitive and specific technique in detecting DCI in both the acute and chronic phases.

Multifocal Electroretinograms in Patients With Branch Retinal Artery Occlusion

To describe the spectrum of retinal capillary ischemia, including superficial and deep capillary ischemia, as identified with spectral-domain optical coherence tomography (SD OCT), that occurs in retinal arterial occlusive disease.Retrospective observational case series.Clinical charts, color fundus photography, red-free fundus photography, fluorescein angiography, near-infrared reflectance, and SD OCT imaging in 40 eyes of 35 patients with retinal arterial occlusive disease were studied in both the acute and chronic phases in multicenter clinical practices. SD OCT imaging analysis was employed to characterize the presence of superficial and deep capillary ischemia in each eye.Of the 40 eyes, 15 eyes had central retinal artery occlusion (CRAO), 22 eyes had branch retinal artery occlusion (BRAO), and 3 eyes had cilioretinal artery occlusion. During the acute phase, SD OCT showed the following 3 distinct patterns, related to retinal ischemia occurring at varying levels within the retina: (1) thickening and hyperreflectivity of the inner retinal layers, including the nerve fiber and ganglion cell layers owing to ischemia of the superficial capillary plexus; (2) a hyperreflective band at the level of the inner nuclear layer, termed “paracentral acute middle maculopathy,” representing ischemia of the intermediate and deep retinal capillary plexuses (deep capillary ischemia); and (3) diffuse thickening and hyperreflectivity of both the inner and middle retinal layers, which represented both superficial and deep capillary ischemia. Of all eyes, 31 (78%) had both superficial and deep lesions. The remaining 9 eyes (22%) had isolated deep capillary ischemia producing paracentral acute middle maculopathy with sparing of the superficial capillary plexus and a normal fluorescein angiographic appearance. As the lesions evolved into the chronic phase over the ensuing 3 months, the resultant thinning and atrophy reflected the retinal layers affected during the acute phase.SD OCT imaging reveals the spectrum of capillary ischemia in retinal artery occlusive disease showing variable involvement of the superficial and intermediate/deep capillary plexuses. Isolated deep capillary ischemia manifested as paracentral acute middle maculopathy on SD OCT and may be seen in some eyes with retinal arterial circulation compromise despite complete absence of perfusion abnormalities on fluorescein angiography.