Retinal arterial macroaneurysm rupture secondary to white coat effect in a case with hypertension

A 65-year-old female patient presented to our department with a chief complaint of blurred vision, which was first observed at a local medical clinic (LMC) 3 days ago. The patient's medical history included chronic hypertension that had been well-controlled (blood pressure [BP] 140/90mm Hg, measured regularly at home using a digital BP set [Omron HEM-7320]) for 8 years. The patient visited the LMC 3 days before presentation for regular follow-up of hypertension, and the initial examination revealed a BP reading of 170/100mm Hg (recorded using a digital BP set [Omron HBP-9020]). Furthermore, after 10min of rest, the BP was rechecked and revealed to be 210/110mm Hg. Floaters and blurring vision were noted in the left eye. The patient was observed at LMC without any medication for 1h. Her BP decreased to 145/90mm Hg. Because the blurring vision persisted and was progressive, she went to our outpatient department (OPD) for help. Upon examination, her best-corrected visual acuity (BCVA) was 3/60 in the left eye and 6/20 in the right eye. Fundoscopic examination revealed a yellowish round lesion superotemporal in the optic nerve head. This lesion was surrounded by retinal hemorrhage [Figure 1A]. Fluorescein angiography (FA) revealed hyperfluorescence with partial blockage by intraretinal and preretinal hemorrhages. There were no diabetic or other major systemic vascular abnormalities except for chronic hypertensive vascular changes. The intermediate phase of indocyanine green angiography (ICGA) revealed a saccular hyperfluorescent lesion compatible with retinal arterial macroaneurysm (RAM) [Figure 1B]. Optical coherence tomography (OCT) revealed significant macular edema with retinal hemorrhage [Figure 1C]. The patient underwent five intravitreal injections of antivascular endothelial growth factor (anti-VEGF) with ranibizumab for macular edema. One year after treatment, the yellowish saccular lesion [Figure 1D] superotemporal to the optic disc showed shrinkage and retinal hemorrhage with macular edema resolution. The patient's BCVA improved to 6/12 in the left eye.Figure 1:: (A) Yellowish saccular lesion (arrow) superotemporal to the optic nerve head, surrounded by preretinal and subretinal hemorrhages (arrowheads). (B) Intermediate phase of indocyanine green angiography shows a corresponding saccular hyperfluorescent lesion (arrow) in the superotemporal arcade of the involved arteriole, which is compatible with RAM, mild neighboring retinal vessel visualization is blocked by preretinal hemorrhage, and moderate neighboring choroidal vessel visualization is blocked by subretinal hemorrhage. (C) Optical coherence tomography shows subfoveal fluid (asteroid) with intraretinal hemorrhage (arrowhead) and subretinal hemorrhage (arrows). (D) Yellowish saccular lesion shrinkage (arrow) and retinal hemorrhage with resolution of macular edema after treatmentRAM is a hallmark of chronic hypertension. The risk factors associated with RAM include chronic hypertension (75%), arteriosclerosis, and abnormal lipid levels.[1] RAM is an acquired focal dilation of the retinal artery approximately 100 to 250 μm in size. This aneurysmal dilatation typically occurs within the first three bifurcations of the central retinal artery and is usually located in the temporal retina. RAM is fragile and can rupture to cause hemorrhage and compromise vision. These lesions result from weakness in the muscular layer of the arteriolar wall. The rupture of RAMs usually occurs when huge fluctuations in BP hit the fragile vessel walls of RAMs. Our patient presented with transit hypertension with a systolic BP > 200mm Hg in the LMC, but home BP readings were regularly recorded at approximately 140/90mm Hg. The white coat effect (WCE)-induced BP fluctuations up to 210mm Hg hitting the fragile vessel walls of the RAM may account for this ruptured macroaneurysm. WCE is diagnosed when the office BP presents a significant elevation (>20mm Hg for systolic BP and >10mm Hg for diastolic BP) compared with out-of-office measurements of BP.[2] To the best of our knowledge, this is the first report in which RAM ruptured secondary to WCE. The diagnosis of RAM is generally clinical and is performed through imaging, such as FA and ICGA. When FA is inconclusive, ICGA may be especially useful in cases where hemorrhage blocks the visualization of RAMs because the emission wavelengths of ICG dye are in the near-infrared spectrum, allowing for better visualization through dense hemorrhage. OCT is beneficial for diagnosing and monitoring associated vision-compromised lesions from ruptured RAM, such as subretinal fluid, macular edema, and hemorrhage. Although RAM is generally spontaneously resolved, potential hemorrhage and exudation may involve the macular zone and impair vision. Laser photocoagulation or other surgical interventions may be considered to seal an aneurysm and prevent further complications. In cases with ruptured RAM without macula involvement, observation is recommended because there is minimal visual threat. In cases of macular involvement due to edema or hemorrhage, prompt treatment is recommended to prevent irreversible visual impairment. Previous studies have suggested that anti-VEGF agents are an effective therapy for macular edema and hemorrhage.[3] Our case also revealed significant improvement after anti-VEGF treatment. Patients presenting with RAM require careful evaluation for hypertension, lipid accumulation, and arteriosclerosis. In cases of vision-threatening condition, RAM should be referred to an ophthalmologist for further treatment. Accurate diagnosis and comanagement are necessary. Declaration of patient consent The authors certify that they have obtained patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed. Author contributions Conceptualization, Ming-Ling Tsai; Methodology, Ming-Ling Tsai; Software, Ping-Feng Tsai; Validation, Ming-Ling Tsai and Ping-Feng Tsai; Formal Analysis, Ming-Ling Tsai; Investigation, Ping-Feng Tsai; Resources, Ming-Ling Tsai.; Data Curation, Ming-Ling Tsai.; Writing – Original Draft Preparation, Ping-Feng Tsai; Writing – Review & Editing, Ming-Ling Tsai; Visualization, Ming-Ling Tsai; Supervision, Ming-Ling Tsai; Project Administration, Ming-Ling Tsai.; Funding Acquisition, Ming-Ling Tsai. All authors have read and agreed to the final version of the manuscript. Data availability statement The datasets generated and analyzed during the current study are available from the corresponding author on reasonable request. Financial support and sponsorship Nil Conflicts of interest All authors declared no conflicts of interest.