A rare case of acute vitreous hemorrhage secondary to ruptured retinal arterial macroaneurysm during a period of inadequate control

Joseph Kane, O.D.

Abstract: Acute vitreous hemorrhage without proliferative or trauma is discovered during inadequate BP control. Ruptured retinal arterial macroaneurysm subsequently diagnosed. Serial multimodal ophthalmic imaging and laboratory studies, medical status, and role of co-management are discussed.

Case History: • Patient Demographics: 85 y.o. African American Male • Chief Complaint: recent difficulty controlling blood pressure prompting emergency room visit; consulted for associated dramatic increase in OD and mild decrease in vision OD over the past week • Ocular History: o Last BCVA = 20/20 OD and OS o Type 2 DM w/o retinopathy or macular OU (at last dilated eye exam 11/14/13) • Medical History: o Type 2 Diabetes Mellitus o (recent dramatic fluctuation, as high as 190/100) o Congestive Heart Failure o Hyperlipidemia • Medications: o Amlodipine 2.5 mg QD o ASA 81 mg QD o Hydralazine 50 mg TID o Isosorbide 30 mg QAM o Metformin 500 mg QAM o Simvastatin 20 mg QHS o Terazosin 5 mg QHS • Other Relevant Information: o Recent h/o hospital admission due to bradycardia prompting carvedilol & chlorthalidone discontinuation à patient subsequently notes “high blood pressure readings” during self-checks at home o Denies , jaw claudication, shortness of breath, dizziness, chest pain

Pertinent Findings • Clinical: o BCVA at presentation = 20/30 OD and OS o , EOM’s, and CFC all WNL OD/OS o Slit lamp exam: moderate OU o IOP = normotensive OU o Gonioscopy OD: open anterior chamber angles without of the angle o Dilated exam OD: posterior vitreous detachment with extensive vitreous hemorrhage; partially dehemoglobinized blood and fibrin emanating from superior-temporal arcade, difficult to assess exact layer or depth clinically o Dilated fundus exam OS: flame shaped hemorrhage inferior to head, otherwise no retinopathy; posterior vitreous detachment w/o hemorrhage o Optic nerves are pink, with sharp/distinct margins OU o Maculae are flat, healthy OU • Ophthalmic Imaging: o Serial SD-OCT scans tracking the clinical course of the lesion in question o B-Scan Ultrasound during the initial presentation: reveals dense vitreal debris and hemorrhaging, no visible retinal break or detachment, sectoral hemorrhage emanating from superior-temporal arcades with spillover into • Laboratory Studies: o CRP = 1.22 mg/L o ESR = 35 mm/hr o Glucose = 111 mg/dL o Urea Nitrogen = 23 mg/dL o Creatinine = 1.52 mg/dL o EGFR = 53 mL/min o CBC w/ diff = overall WNL with mild reduction in WBC, RBC, HBG, and HCT o BNP (brain natriuretic peptide, biomarker for CHF) = 493.5 (elevated, >300 indicates at least mild heart failure) o PTT/PT/INR = WNL • Radiology Studies: o Head CT w/o contrast: no acute abnormality (no hemorrhage, infarct, intracranial abnormality) à stable to CT 2 years prior

Differential diagnosis • Primary/leading: vitreous hemorrhage OD • Secondary (etiology of vitreous hemorrhage): o Proliferative o Hypertensive retinopathy o Branch retinal vein occlusion o Retinal break/detachment o o Hemorrhagic posterior vitreous detachment o Hemorrhagic pigment epithelial detachment o Ruptured choroidal neovascular membrane / peripheral exudative hemorrhagic chorioretinopathy o Ruptured retinal arterial macroaneurysm o Valsalva retinopathy o Malignant melanoma

Diagnosis and discussion Diagnosis: vitreous hemorrhage secondary to ruptured retinal arterial macroaneurysm OD • Retinal arterial macroaneurysms (RAM) represent acquired, focal dilatations in the retinal vasculature, most commonly associated with hypertension. Many are discovered upon routine examination and do not cause symptoms, unless the macula or vitreous is involved. RAM are often misdiagnosed at initial presentation and considered by some to be “masqueraders” due to the highly variable nature of its presentation; hemorrhage can be seen at multiple layers of the , rarely with spillover into the vitreous. Prognosis is generally favorable, and observation is often a sufficient management strategy. Patients with RAM should be monitored for secondary complications such as retinal vein occlusion, chronic , secondary angle closure , and hemorrhagic . Depending on the clinical scenario, there are several proposed management strategies for RAM and its sequelae including observation, , focal laser treatment (either directly to the lesion or indirect), and anti-VEGF injections. A systemic work-up is indicated to rule out underlying medical conditions (especially cardiovascular disease) and treat as appropriate.

Unique features in this case: • RAM is a relatively rare clinical entity (only 1/9,000 eyes in the Beijing eye study) and even more uncommon as a mechanism for vitreous hemorrhage (~0.6-7%, depending on the study); many vitreous hemorrhage prevalence studies do not even cite RAM as an etiology • We have B-scan ultrasound and serial SD-OCT images capturing the acute bleeding emanating from the area of RAM into the vitreous cavity, which has only been described in a handful of case reports (Tang, et al); to the best of our knowledge, B-scan ultrasound and SD-OCT have not previously been described in conjunction during acute ruptured RAM; this represents a unique means by which to track these lesions both in the acute stage and throughout their clinical course • We have access to serial blood pressure readings and laboratory studies both before and after his retinal presentation, allowing us to track his ocular status in conjunction with his medical status • The patient was subsequently diagnosed with variable degree heart block (with periods of complete heart block) and underwent implantation of a permanent pacemaker, highlighting the importance of co- management in cases of ruptured RAM to uncover underlying medical conditions; there has not yet been a direct correlation established between heart block and RAM, though HTN and arteriosclerosis link both of these conditions

Treatment, management • Ocular = the patient is currently being monitored for clearance of his vitreous hemorrhage; the RAM is also being monitored for spontaneous involution with serial SD-OCT scans through the lesion • Medical = the patient reported to the emergency room several times after his initial eye examination complaining of difficulty controlling his blood pressure at home; he has been seen multiple times by primary care, cardiology, and electrophysiology; it was discovered that he also was having varying degrees of heart block and was given Ziopatch to monitor at home; one month after his initial eye exam, he was admitted to the hospital and underwent implantation with a permanent pacemaker; the procedure went well, but unfortunately his blood pressure control remains inadequate; per his last cardiology visit, his physicians are attempting to re-establish optimal blood pressure control medically

Conclusion & Clinical Pearls • Ruptured RAM is a rare cause of vitreous hemorrhage; clinicians must consider all possible etiologies for vitreous hemorrhage, especially in the absence of proliferative retinopathy or retinal break • B-scan ultrasound and SD-OCT imaging are of paramount importance in the acute stage of vitreous hemorrhage and may reveal a surprising etiology for the bleed; both imaging modalities provide clinically useful information for RAM cases and allow serial monitoring to determine proper management • RAM is often a sign of underlying systemic disease; co-management with other health care providers (i.e. cardiologists, primary care, endocrinologists) may result in additional medical diagnoses, even in patients with known diabetes or hypertension; proper recognition and timely intervention can be both vision and life saving

Bibliography 1. Asao, Kazunobu et al. “Vitreous hemorrhage caused by ruptured retinal macroaneurysm.” Case Reports in . 5. (2014):44-49. 2. Koinzer S, et al. “Long-term, therapy-related visual outcome of 49 cases with retinal arterial macroaneurysm: a case series and literature review.” Br J Ophthalmol. 0 (2015): 1-9. 3. Ryan, Stephen J. "Acquired Retinal Macroaneurysms." Retina. 5th ed. London: Saunders, 2013. 1026-1028. 4. Speilburg, Ashley M., and Stephanie A. Klemencic. "Ruptured Retinal Arterial Macroaneurysm: Diagnosis and Management." Journal of Optometry. 7 (2014): 131-37. 5. Spraul, Christoph W., and Hans E. Grossniklaus. "Vitreous Hemorrhage." Survey of Ophthalmology. 42.1 (1997): 3-39. 6. Tang, William M., et al. “Echographic characteristics of retinal arterial macroaneurysm.” The Journal of Retinal and Vitreous Diseases. 18.6. (1998): 559-563. 7. Xu L, et al. “Frequency of retinal Macroaneurysms in adult Chinese: the Beijing Eye Study.” Br J Ophthalmol. 91 (2007): 840-841.