IMPACT OF MACULAR INTRARETINAL HEMORRHAGE AND MACULAR HOLE ON THE VISUAL PROGNOSIS OF SUBMACULAR HEMORRHAGE DUE TO RETINAL ARTERIAL MACROANEURYSM RUPTURE.

PURPOSE: To compare the effects of macular intraretinal hemorrhage (IRH) and macular hole (MH) on best-corrected visual acuity (BCVA) after displacement of submacular hemorrhage (SMH) due to retinal arterial macroaneurysm (RAM) rupture. METHODS: This multicenter retrospective study included 48 eyes with SMH due to RAM rupture. Cases underwent vitrectomy to displace SMH and were followed up for 6 months. We classified cases according to the presence of IRH and MH and compared the postoperative BCVA among the groups. RESULTS: We classified the eyes into IRH(+)MH(+) group (10 eyes), IRH(+)MH(-) group (23 eyes), and IRH(-)MH(-) group (15 eyes). The postoperative BCVA was significantly worse in the IRH(+)MH(+) and IRH(+)MH(-) groups than in the IRH(-)MH(-) group (0.91 ± 0.41 in logarithm of the minimal angle of resolution units, Snellen equivalent 20/163, 0.87 ± 0.45, 20/148, and 0.18 ± 0.21, 20/30, respectively; P < 0.001). The postoperative central retinal thickness was significantly lower in the IRH(+) group (IRH(+)MH(+) and IRH(+)MH(-) groups combined) than in the IRH(-) group (IRH(-)MH(-) group) (121.4 ± 70.1 µ m and 174.3 ± 32.9 µ m, respectively, P = 0.008). The postoperative external limiting membrane and ellipsoid zone continuities were significantly discontinuous in the IRH(+) group ( P < 0.001, P = 0.001, respectively). The multiple linear regression analysis showed that both IRH(+)MH(+) and IRH(+)MH(-) were associated with the postoperative BCVA (regression coefficient, 0.799 and 0.711, respectively; P < 0.001 for both). CONCLUSION: Both IRH and MH were poor prognostic indicators in cases with SMH due to RAM rupture.

DETECTION AND CHARACTERISTICS OF UNRUPTURED RETINAL ARTERIAL MACROANEURYSMS.

PURPOSE: To determine the presence of unruptured retinal arterial macroaneurysms (RAMs) and to examine the characteristics of the detected lesions. METHODS: This retrospective observational study included the affected and contralateral eyes of 50 patients (100 eyes) with symptomatic, unilateral, ruptured RAMs who visited the Department of Ophthalmology at the Kyoto University Hospital (April 2014-April 2020) and were followed up for at least 6 months after the onset. The presence and characteristics of unruptured RAMs were examined by reviewing the findings of color fundus photography and infrared scanning laser ophthalmoscopy performed before the onset or during the follow-up period. RESULTS: Unruptured RAMs were detected in six of the 50 patients. Some patients had bilateral or multiple unruptured RAMs, and a total of 12 unruptured RAMs were detected in eight eyes of the six patients. Among the detected lesions, eight exhibited a longitudinal increase in their diameter during the follow-up period, whereas six exhibited ruptures. CONCLUSION: Unruptured RAM is not an uncommon retinal vascular abnormality and can enlarge and progress to ruptured RAM.

Angiotensin II is a crucial factor in retinal aneurysm formation.

Retinal arterial macroaneurysms are characterized by the acquired fusiform or saccular dilatations of the retinal artery. Angiotensin II (Ang II) is a major signal molecule of the renin-angiotensin system, which exerts a range of pathogenic actions that are relevant to retinal vascular abnormalities. We aimed to study the effect of Ang II on retinal vessels and explore its relationship with retinal aneurysmal disease. C57BL/6J male mice were administered Ang II at 1000 ng/kg/min for 28 days, and the mice given saline served as controls. The mice in the treatment group were treated once daily by gastric gavage of candesartan cilexetil (an antagonist of Ang II type 1 (AT1) receptor) at 100 mg/kg/day. The in vivo imaging of murine retinas was performed using fundus photography, optical coherence tomography, fluorescein angiography, and indocyanine green angiography at 7th, 14th, and 28th days of infusion. At the end of the infusion and treatment, the morphological changes were evaluated by histopathological examination and electron microscopy; the levels of related proteins in murine retinas were examined by antibody array and Western blot analyses. We found that Ang II infusion induced aneurysm formation in mice retina, which presented as either solitary aneurysms or retinal arterial beading. The aneurysm formation was often accompanied with vessel leakage. Moreover, Ang II infusion itself may result in increased vascular permeability and ganglion cell and inner plexiform layer thickening. The blockade of AT1 receptors by systemic administration of candesartan cilexetil alleviated the Ang II-induced retinal vasculopathy. The protein level analysis further showed that Ang II upregulated IL-1ß, PDGFR-ß, and MMP-9 expression, and the expression of IL-1ß could be inhibited by AT1 receptor antagonist. Our study provides evidence that Ang II is a crucial factor in retinal aneurysm formation and vessel leakage. It is probably the combined effect of Ang II on vessel inflammatory response, pericyte function, and extracellular matrix remodeling that predisposes the retinal arterial wall to aneurysm formation and blood-retinal barrier breakdown.

Retinal detachment following focal laser to ruptured retinal artery macro aneurysm.

A 53-year-old lady presented with inferior retinal detachment (RD) following focal laser for retinal artery macroaneurysm (RAM). She underwent focal laser with intravitreal gas injection elsewhere; however, no retinal break was localized on the examination. The patient was taken up for vitreoretinal surgery. Intraoperatively, it was noted that the retinal detachment was not extending to the retinal periphery and primary retinal break was not localized even during the scleral depression. Under high magnification, using a macular lens, a slit-like retinal break was noted at the area of previous focal laser. Focal laser for RAM probably caused this retinal break leading to RD. The clinician needs to be aware that during focal laser of ruptured RAM, haemorrhage may preclude the view of retinal structures leading to inadvertent use of excessive laser energy. Retinal breaks may form at the site of laser due to coagulative necrosis. During surgical management of RD in such cases, the area of focal laser should be thoroughly examined under high magnification to avoid missed breaks.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY IN THE DIAGNOSIS AND FOLLOW-UP OF RETINAL ARTERIAL MACROANEURYSMS.

PURPOSE: To describe optical coherence tomography angiography (OCTA) findings in retinal arterial macroaneurysm (RAM) associated with macular edema and to correlate OCTA findings with conventional multimodal imaging. METHODS: The clinical course, conventional multimodal imaging findings including fundus color photography, spectral domain optical coherence tomography (Spectralis; Heidelberg Engineering, Heidelberg, Germany), fluorescein angiography (Heidelberg Engineering), and OCTA (Optovue, Inc, Freemont, CA) findings at baseline and during the follow-up of two eyes (two patients) with symptomatic RAM associated with macular edema were documented. RESULTS: Two eyes of 2 patients, both women, aged 82 and 46 years, which presented with progressive visual decline, were included. On conventional multimodal imaging, exudative RAM with macular edema and lipid exudation were visible in both included eyes. On OCTA, the flow in the two RAM was detected at baseline. Case 1 was treated by focal laser photocoagulation. One month after treatment, fluorescein angiography showed RAM occlusion. Spectral domain optical coherence tomography showed a RAM and retinal thinning and a decreased central foveal thickness, resulting in visual acuity improvement. On OCTA, no flow was detectable in the RAM at 1-month follow-up. Case 2 was not treated at baseline. In this eye, no flow was detected on OCTA at 2-month follow-up. This suggests a spontaneous occlusion, which was confirmed by fluorescein angiography. CONCLUSION: Optical coherence tomography angiography is able to detect the presence or absence of flow signal within RAMs, which may both decrease the need for dye angiography in selected cases with exudative RAM and help in treatment decision making.

Nonexudative Perifoveal Vascular Anomalous Complex: The Subclinical Stage of Perifoveal Exudative Vascular Anomalous Complex?

PURPOSE: To describe the pre-exudative stage of exudative perifoveal vascular anomalous complex (ePVAC), referred to as nonexudative PVAC (nePVAC). DESIGN: Retrospective noncomparative case series. METHODS: Patients diagnosed with nePVAC were identified at 4 retina referral centers worldwide. Multimodal retinal imaging, including structural optical coherence tomography (OCT) and OCT-angiography (OCT-A), were performed at baseline and follow-up visits. RESULTS: Six eyes (6 patients, mean 75 ± 10 years of age) were included. Unrelated chorioretinal diseases were diagnosed in the affected eyes in 5 of 6 cases. At baseline, nePVAC is characterized by microvascular abnormalities featuring an isolated, perifoveal, large intraretinal aneurysm surrounded by capillary rarefaction at OCT-A examination, without any sign of exudation with structural OCT, and without visual impairment. Four patients were followed for a mean of 21 ± 14 months. During the follow-up, 3 of 4 eyes (75%) developed signs of exudation after a mean of 15 ± 9 months, associated with metamorphopsia and visual decline at the time of exudation. Best-corrected visual acuity decreased from 20/25 to 20/40 Snellen equivalent (P = .035) and central macular thickness increased from 268 ± 27 µm to 339 ± 65 µm (P = .145). Three patients were treated with 2.3 ± 0.6 intravitreal injections of anti-vascular endothelial growth factor without significant improvement of best-corrected visual acuity or macular edema. CONCLUSIONS: nePVAC may represent the subclinical pre-exudative stage of ePVAC, notable for an absence of exudation or visual impairment. nePVAC and ePVAC should be considered as part of the same spectrum, namely PVAC. Typically, nePVAC develops signs of exudation over time, causing metamorphopsia and visual decline and therefore these lesions warrant continued close monitoring with multimodal retinal imaging.

Ruptured retinal macroaneurysm complicated by a retro hyaloidal hematoma: about a case and review of the literature.

A 45 years old patient consulted for a sudden decrease in visual acuity in the right eye. Ophthalmological examination gave visual acuity limited to luminous perceptions with a calm anterior segment, a transparent lens and at the fundus examination a dense, massive, two-level intra-retinal and retro hyaloidal pigeon nest hemorrhage with a fusiform whitish lesion on the path of the upper temporal artery. The suspected diagnosis was a complicated ruptured retinal macroaneurysm with massive retinal and retro hyaloidal hemorrhage. Fluorescein angiography confirmed the diagnosis. Our course of action was an emergency programmed evacuation vitrectomy with gas tamponade. The evolution was marked by a clear improvement in visual acuity. The rupture of retinal macroaneurysm is a frequent and serious accident. The complications of this rupture can threat the vision. Care is still being discussed. Randomized studies on large series are necessary to decide on the best therapy.

Familial Retinal Arterial Macroaneurysms with Peripheral Retinal Ischemia: A Characterization on Ultra-Widefield Fluorescein Angiography.

Retinal arterial macroaneurysm with supravalvular pulmonic stenosis (RAMSVPS), also known as familial retinal arterial macroaneurysm syndrome, is an autosomal recessive disorder associated with many life-threatening complications. The disease is characterized by progressive multiple retinal arterial macroaneurysms, retinal arterial beading, and recurrent vitreous hemorrhage. Here, we describe the first case of RAMSVPS associated with peripheral ischemia evident on ultra-widefield fluorescein angiography. A 23-year-old otherwise healthy woman presented to our ophthalmology emergency room with a 1-week history of right eye floaters and decreased vision. Dilated fundoscopic examination showed right eye vitreous and preretinal hemorrhage with bilateral active and involuted retinal arterial macroaneurysms, arterial beading, and sclerosed vessels. Ultra-widefield fluorescein angiography demonstrated multiple macroaneurysms on the optic discs and along the major retinal arteries in addition to significant peripheral retinal ischemia in both eyes. Echocardiography showed severe supravalvular pulmonic stenosis that required urgent pulmonary valvuloplasty. Indeed, peripheral retinal ischemia is an additional finding in RAMSVPS.

ADVERSE EFFECT OF MACULAR INTRARETINAL HEMORRHAGE ON THE PROGNOSIS OF SUBMACULAR HEMORRHAGE DUE TO RETINAL ARTERIAL MACROANEURYSM RUPTURE.

PURPOSE: To investigate the clinical course of submacular hemorrhage associated with ruptured retinal arterial macroaneurysm using swept-source optical coherence tomography. METHODS: This study included 23 eyes of 23 consecutive patients diagnosed with submacular hemorrhage associated with ruptured retinal arterial macroaneurysm. Cases underwent displacement of submacular hemorrhage (vitrectomy + subretinal injection of tissue plasminogen activator + air tamponade) and were followed up for 6 months after surgery. Localization of the preoperative hemorrhage and its effect on preoperative and postoperative best-corrected visual acuity, central retinal thickness, and continuity of the ellipsoid zone were measured. RESULTS: Macular intraretinal hemorrhage (IRH) was observed in 17 eyes (73.9%, IRH [+] group) and was not observed in 6 eyes (26.1%, IRH [-] group). The IRH (+) group showed worse postoperative best-corrected visual acuity values compared with the IRH (-) group (0.89 ± 0.47 in logarithm of the minimal angle of resolution units, Snellen equivalent 20/155 and 0.16 ± 0.23, 20/29, respectively; P < 0.01), smaller central retinal thickness values (97.7 ± 53.5 µm, 173.0 ± 32.3 µm, respectively; P < 0.01), and a higher rate of ellipsoid zone disruption (100%, 33.3%, respectively; P < 0.01). CONCLUSION: Patients with preoperative macular IRH showed lower postoperative visual acuity and worse macular contour after submacular hemorrhage displacement compared with patients without macular IRH.

Intraoperative Optical Coherence Tomography Demonstrating Macular Hole Associated With Ruptured Retinal Arterial Macroaneurysm.

A 67-year-old female presented with vitreous hemorrhage and sub-internal limited membrane (ILM) hemorrhage in her right eye associated with ruptured retinal arterial macroaneurysm. During pars plana vitrectomy, intraoperative optical coherence tomography aided in the diagnosis of a small macular hole previously obscured by the sub-ILM hemorrhage. Nonexpansile gas was placed followed by postoperative prone positioning, and the hole closed successfully. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:e125-e127.].