History
A 66-year-old white male presented to the emergency department with a chief complaint of lost vision in his right eye. He explained that he woke up with poor vision three days ago, but couldn’t get anyone to take him to the hospital. He also said that he had consistently poor vision in his left eye since childhood because of a “lazy, crossed eye.” Finally, he called the police and asked them to take him to the emergency room when it became clear that his vision had worsened and was no longer functioning in his only “correctly working” eye.
His ocular history was remarkable for strabismic amblyopia and corrective muscle surgery in his left eye. His systemic history was positive for hypertension. He denied using any medications and reported no known allergies.
Diagnostic Data
His best-uncorrected entering visual acuity was no light perception (NLP) O.D. and 20/400 O.S. at distance and near. Pupil testing uncovered a grade IV afferent defect in his right eye. Extraocular muscle movements were full and unrestricted in both eyes, with orthophoric position. Confrontation fields were full in all fields of gaze O.S.
Slit lamp examination revealed normal and healthy anterior segment structures with no evidence of iris neovascularization in either eye. Additionally, both anterior chambers appeared deep and quiet.
His intraocular pressure measured 18mm Hg O.U. The pertinent dilated fundus findings are illustrated in the photographs.
Our patient presented to the emergency room with a chief complaint of vision loss in his right eye. What is the correct diagnosis?
Your Diagnosis
How would you approach this case? Does this patient require any additional tests? What is your diagnosis?
How would you manage this patient? What is the suspected prognosis?
Laser interferometry under patched conditions was completed to insure NLP status. We also performed bilateral fundus photodocumentation. We ordered laboratory testing in an attempt to uncover an underlying cause, such as coagulopathy, hyperviscosity, dyslipidemia, cardiac disease, valvular heart disease, infectious disease, inflammatory disease, autoimmune disease or carotid artery disease. Laboratory testing included a complete blood count with differential and platelets (CBC with Diff and platelets), prothrombin time (PT), activated partial thromboplastin time (aPTT), lipid panel and carotid Doppler.
The diagnosis in this case is ophthalmic artery occlusion in the patient’s right eye. The ophthalmologic management for retinal and ophthalmic vein occlusion must take place within 70 minutes to offer hope of visual recovery. Two approaches can be used in concert:
• Decrease the IOP in an attempt to reduce the resistance for retinal arterial perfusion using fast-acting, topical, oral and/or surgical modalities, such as topical beta blockers, topical apraclonidine, oral carbonic anhydrase inhibitors, oral hyperosmotic preparations and/or parecentisis.
• Activate retinal autoregulatory mechanisms in an attempt to increase vascular diameter, thereby allowing the embolis to pass via aggressive digital ocular massage either with or without increasing blood carbon dioxide levels via breathing into a paper bag or using Carbogen.1,2
Elements of systemic vascular disease that ultimately contribute to an increased risk of arterial occlusion include coagulopathy, hyperviscosity, dyslipidemia, cardiac disease, cardiac valvular disease and carotid artery disease.2,3 Systemic testing with the goal of aborting life-altering catastrophic outcomes is an essential management component of this ocular condition.2,3 In cases that potentially involve giant cell arteritis, temporal artery biopsy is the standard method of definitive diagnosis, with medical treatment consisting of intravenous methylprednisone administration.
There is some evidence to suggest that thrombolysis using deliverable injected compounds could offer a beneficial effect in retinal arterial occlusion.4 However, this approach carries the risk of inducing hemorrhage.4 One study indicated that retrograde cannulation of the supraorbital arteries followed by irrigation with fibrinolytic agents may have the potential to minimize the risk of major complications while offering sight saving benefits.4 According to this study, the supratrochlear artery appears to provide the most reliable local access route.4
Urokinase has also been selectively studied as an agent that can be infused into the ophthalmic artery as an emergency treatment for combined central retinal arterial occlusion (CRAO) and central retinal venous occlusion (CRVO).5 Researchers have found limited success for this otherwise unmanageable condition. And, when no treatment is proffered, poor visual outcome is certain. Urokinase, for example, has few systemic complications and may be beneficial in such cases, but can also cause intravitreal hemorrhage.5
Because the patient failed to react within a reasonable time period, the damage caused by more than 72 hours of ischemia to all structures distal to the blockage was not treatable or reversible. Instead, we focused on identifying the underlying cause with the goal of arresting or reversing potential mortal disease processes (cerebral vascular accident and myocardial infarction).
Unfortunately, this case was lost to follow-up. So, while the correct laboratory work-up was initiated, there was no report from either the patient or his primary care physician.
1. Rumelt S, Brown GC. Update on treatment of retinal arterial occlusions. Curr Opin Ophthalmol. 2003 Jun;14(3):139-41.
2. Schmidt D. Ocular massage in a case of central retinal artery occlusion the successful treatment of a hitherto undescribed type of embolism. Eur J Med Res. 2000 Apr 19;5(4):157-64.
3. Yamamoto T, Mori K, Yasuhara T, et al. Ophthalmic artery blood flow in patients with internal carotid artery occlusion. Br J Ophthalmol. 2004 Apr;88(4):505-8.
4. Schwenn OK, Wustenberg EG, Konerding MA, Hattenbach LO. Experimental percutaneous cannulation of the supraorbital arteries: implication for future therapy. Invest Ophthalmol Vis Sci. 2005 May;46(5):1557-60.
5. Vallee JN, Paques M, Aymard A. Combined central retinal arterial and venous obstruction: emergency ophthalmic arterial fibrinolysis. Radiology. 2002 May;223(2):351-9.
