Hemorrhagic retinal arterial macroaneurysm combined with branch retinal artery occlusion treated with intravitreal conbercept injection: A case report.

RATIONALE: Branch retinal artery occlusion (BRAO) is a rare complication of retinal arterial macroaneurysm (RAM), a low-incidence ocular disease. PATIENT CONCERNS: A 75-year-old woman presented with a chief complaint of blurred vision. DIAGNOSES: The patient for 4 days received a diagnosis of RAM combined with BRAO. INTERVENTIONS: The patient was treated with two successive intravitreal conbercept injections. OUTCOMES: The patient's best-corrected visual acuity improved, and the RAM diminished. LESSONS: Administration of conbercept injection might be an effective treatment for complex RAM with BRAO.

[Diagnostic and treatment options for retinal arterial macroaneurysms in relation to our cases]. / Retinalis artéria macroaneurysmák diagnosztikai és kezelési lehetoségei eseteink kapcsán.

In our report, we present the history of four patients diagnosed with retinal arterial macroaneurysm associated with complications. Our aim is to present the varied appearance of the disease and to present the various therapeutic options. Retinal artery macroaneurysm is a rare, but potentially vision-threatening ophthalmic condition. Macroaneurysm develops from the arteriosclerotic transformation of the artery caused by high blood pressure. Macroaneurysms can be asymptomatic, or they can be associated with exudative or hemorrhagic complication which causes visual impairment. Depending on the symptoms, they can be treated with laser photocoagulation, intravitreal injections, or with vitrectomy. Our presented cases also illustrate that each case requires individual consideration because a uniform therapeutic recommendation is still yet to be developed. In addition to the ophthalmic treatment, it is extremely important to refer the patient to internal medicine. Orv Hetil. 2023; 164(42): 1673-1677.

Visual Recovery and Prognosis in the Treatment of Submacular Hemorrhage due to Polypoidal Choroidal Vasculopathy and Retinal Arterial Macroaneurysm: A Retrospective Study.

Purpose: This study was carried out to evaluate the visual acuity (VA), complications, and prognosis of patients diagnosed with submacular hemorrhage (SMH) from polypoidal choroidal vasculopathy (PCV) and retinal arterial macroaneurysm (RAM) treated by pars plana vitrectomy (PPV), subretinal tissue plasminogen activator (tPA), and air tamponade in vitreous cavity. It facilitates the development of generic treatment methods that can be widely used to improve vision and treat potential complications in patients with SMH, regardless of the underlying pathophysiological condition, such as PCV or RAM. Methods: In this retrospective study, SMH patients were divided into two groups based on their diagnosis: (1) polypoidal choroidal vasculopathy (PCV) and (2) retinal arterial macroaneurysm (RAM). The visual recovery and complications of patients with PCV and RAM after PPV + tPA (subretinal) surgery were analyzed. Results: A total of 36 eyes of 36 patients were included: PCV (47.22%, 17/36) and RAM (52.78%, 19/36). The mean age of the patients was 64 years, and 63.89% of the patients (23/36) were female. The median VA was 1.85 logMAR before surgery, 0.93 and 0.98 logMAR at 1 and 3 months after surgery, respectively, indicating that most patients' vision improved after surgery. At the 1 and 3 months postoperative follow-up, each patient was diagnosed with rhegmatogenous retinal detachment at 1 month and 3 months postoperatively, and four patients had vitreous hemorrhage at 3 months postoperatively. Preoperatively, patients exhibited macular subretinal hemorrhage, retinal bulge, and exudation around the blood clot. Postoperatively, most patients showed dispersal of subretinal hemorrhage. Optical coherence tomography results revealed retinal hemorrhage involving the macula and hemorrhagic bulges under both the neuroepithelium and the pigment epithelium under the fovea preoperatively. After surgery, the air injected into the vitreous cavity was completely absorbed and the subretinal hemorrhage was dispersed. Conclusion: PPV combined with subretinal tPA injection and air tamponade in the vitreous cavity can facilitate modest visual recovery in patients with SMH due to PCV and RAM. However, some complications may occur, and their management remains challenging.

Triple retinal arterial macroaneurysms in a hypertensive patient with hypothyroidism.

PURPOSE: To present the unique case of numerous, recurring retinal arterial macroaneurysms (RAMs) in a hypothyroid patient with hypertension. METHODS: 67-year-old woman's clinical findings, laboratory results, fundus fluorescein angiography, optical coherence tomography (OCT), and optical coherence tomography angiography (OCTA) are given. Over a two-year period, the patient was monitored. RESULTS: A 67-year-old woman presented to the outpatient clinic with vitreous and dense subretinal hemorrhages in her right eye. RAM rupture was discovered along the suprotemporal retinal arteriole. A diagnosis of systemic arterial hypertension was made. Two months later, the vitreous hemorrhage spontaneously resolved and the patient's vision improved. After nine months of initial presentation, the patient developed another RAM. Meanwhile the patient developed ichthyosis caused by hypothyroidism. Because fundus fluorescein angiography revealed that the first RAM was still active, an intravitreal injection of anti-VEGF was administered six months afterwards. More proximal RAM with macular edema developed after another six months necessitating laser photocoagulation. However macular edema didn't resolve and a second injection of intravitreal anti-VEGF was given. CONCLUSIONS: Patients with RAMs, particularly if multiple or recurring, should be thoroughly investigated and assessed, particularly for secondary causes of hypertension. OCT and OCTA are useful tools for RAM confirmation and follow-up. It is important to look into how RAM behavior interacts with coexisting macular edema, and other variables affecting hemodynamic status.

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.

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.

Long-term resolution of chronic macular edema after a single dose of intravitreal dexamethasone in familial retinal arterial macroaneurysm.

PURPOSE: To report a favorable effect of intravitreal dexamethasone implantation in Familial Retinal Arterial Macroaneurysms (FRAM). METHODS: Retrospective Case Report. RESULTS: A 32-year-old male who presented with bilateral retinal macroaneurysms. Whole Exome Sequencing (WES) revealed a homozygous c.830-1 G > A mutation in Insulin Growth Factor Binding Protein 7 (IGFBP7) gene, confirming the diagnosis FRAM. The left eye was lost in the course of the disease, whereas the right eye developed a persistent macular edema due to multiple leaking retinal arterial macroaneurysms and responded poorly to intravitreal ranibizumab and only partially to intravitreal aflibercept. Intravitreal dexamethasone implantation in the right eye, on the other hand, resulted in marked visual and structural improvement. CONCLUSION: Intravitreal dexamethasone injections have beneficial anatomical and visual outcomes in FRAM patients with persistent macular edema poorly responsive to intravitreal injections.

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.

Management of a Macular Hole Following Retinal Arterial Macroaneurysm Rupture.

BACKGROUND We report the successful treatment of a macular hole (MH) secondary to a retinal macroaneurysm (RAMA) rupture by pars plana vitrectomy (PPV) and autologous transplantation of an internal limiting membrane (ILM) during primary surgery. CASE REPORT A 60-year-old woman presented with a sudden loss of central vision in her right eye. Fundus examination revealed a large sub-ILM hemorrhage involving the macula in her right eye. We performed 25-gauge PPV and ILM peel overlaying the hemorrhage. A ruptured RAMA next to the inferotemporal branch of the central retinal artery, MH, and subretinal hemorrhage were discovered. Part of the subretinal blood clot was removed through the MH. ILM was then further peeled off outside vascular arcades and transplanted as a free flap into the MH. At the end of surgery, 10% C3F8 gas was instilled as tamponade. Postoperatively, we observed anatomical closure of the MH, restoration of outer retinal layers, and improvement of visual acuity. During the follow-up period, the patient also had cataract surgery. Two years after the vitrectomy, the MH remained closed, with visual acuity improved to 10/20. CONCLUSIONS Autologous ILM transplantation during primary PPV can be an effective surgical approach for treatment of MH related to ruptured RAMA in selected cases.

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.

Phenotypic delineation of the retinal arterial macroaneurysms with supravalvular pulmonic stenosis syndrome.

Retinal arterial macroaneurysms with supravalvular pulmonic stenosis (RAMSVPS), also known as Familial Retinal Arterial Macroaneurysms (FRAM) syndrome, is a very rare multisystem disorder. Here, we present a case series comprising ophthalmologic and systemic evaluation of patients homozygous for RAMSVPS syndrome causative IGFBP7 variant. New clinical details on 22 previously published and 8 previously unpublished patients are described. Age at first presentation ranged from 1 to 34 years. The classical feature of macroaneurysms and vascular beading involving the retinal arteries was universal. Follow up extending up to 14 years after initial diagnosis revealed recurrent episodes of bleeding and leakage from macroaneurysms in 55% and 59% of patients, respectively. The majority of patients who underwent echocardiography (18/23) showed evidence of heart involvement, most characteristically pulmonary (valvular or supravalvular) stenosis, often requiring surgical correction (12/18). Four patients died in the course of the study from complications of pulmonary stenosis, cerebral hemorrhage, and cardiac complications. Liver involvement (usually cirrhosis) was observed in eight patients. Cerebral vascular involvement was observed in one patient, and stroke was observed in two. We conclude that RAMSVPS is a recognizable syndrome characterized by a high burden of ocular and systemic morbidity, and risk of premature death. Recommendations are proposed for early detection and management of these complications.

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.].