Eyeworld

EW RESIDENTS June 2014 53 Not your garden-variety amaurosis Introduction from Dr. Waxman T his month we have a case from the resident clinic at the University of Pittsburgh Medical Center (UPMC) Eye Center. The patient was referred in as a third opinion by the patient's primary care doctor who simply didn't agree with the oph- thalmologists that the patient had seen previously. The diagnosis was finally made by listening carefully to the patient, understanding the anatomy of the eye, and thinking outside the box. The case has been expertly pre- pared by Jared Knickelbein, MD, PhD, currently a third-year resident and chief resident in our program. Dr. Knickelbein is a graduate of the MD/PhD program at Pittsburgh. He will be headed off to a uveitis fellow- ship at the NEI when he graduates. Discussants include Ian Conner, MD, PhD, from the glaucoma division at UPMC Eye Center, Ellen Mitchell, MD from the neuro-ophthalmology division, and Alex Mammen, MD, from the cornea division. Case presentation A 64-year-old male with a history of bilateral retinal detachments re- paired with scleral buckles, bilateral narrow anterior chamber angles treated with laser peripheral irido- tomies, glaucoma in the left eye treated with bimatoprost, and bilat- eral pseudophakia presented with intermittent blurred vision in the right eye that he described as a "waxy sensation." These episodes lasted for several minutes before returning to normal and had been going on for about 1 month. He denied any associated pain or other symptoms, including headache or lightheadedness. On further ques- tioning, the patient reported that he only noticed the blurred vision when bending over while gardening, and that the vision returned to base- line once he was upright for several minutes. He had a history of atrial fibrillation and was on warfarin therapy. His previous scleral buckle and cataract surgeries were all more than 10 years earlier. Records of his right cataract surgery from an outside ophthalmologist revealed an uneventful surgery with the use of a superior scleral tunnel that was closed with three Prolene sutures. On initial presentation, the pa- tient was asymptomatic with Snellen visual acuities of 20/20 in both eyes, normal reacting pupils without an APD, and IOP of 8 mmHg in the right eye and 10 mmHg in the left eye. Figure 1 shows his anterior segment demonstrating a clear cornea, deep and quiet anterior chamber, super iris atrophy, a peripheral iridotomy at 12:30, and three scleral sutures superiorly covered by conjunctiva. Gonioscopy of his superior angle revealed disruption of angle structures with a few peripheral anterior synechiae (Figure 2). The remainder of the angle was normal and open. Fundus exam of the right eye revealed clear media, a flat optic nerve with cup-to-disc ratio of 0.8 and superiorotemporal thinning of the neuroretinal rim, normal macula and vessels, and peripheral retinal changes consistent with previous retinal detachment repair and without any apparent residual subretinal fluid. The patient was asked to demonstrate the position that caused his blurred vision in the office. After kneeling with his head down for a few minutes, the patient noted return of waxy vision. Exam at this time showed hyphema with the source of bleeding from the superior angle on gonioscopy. Discussion The case was presented to several attending subspecialists, who shared their thoughts. Dr. Conner commented: "What a great case! I suspect that the blood is coming from a disruption in the blood-aqueous barrier allowing direct access to Schlemm's canal and the aqueous collector channels, similar to what is intentionally done in the ab interno trabeculotomy (AIT, Trabectome, NeoMedix, Tustin, Calif.) surgery. In fact, there was a paper last year in the American Journal of Ophthalmology detailing this very phenomenon—delayed onset spontaneous hyphema in approximately 5% of AIT patients. 1 This would explain why his baseline, untreated IOP in OD is lower that the treated IOP in OS. The hyphema in this case would be secondary to an increase in episcleral venous pressure when bending with the head below the waist, perhaps exacerbated by the presence of the scleral buckle." "Of course, one must also consider an UGH syndrome in the differential. In fact, the superior iris atrophy suggests that there may be a haptic out of the capsular bag at 1200. UBM should demonstrate this nicely if this is the culprit." "We still don't have any infor- mation about the status of the optic nerves (cupping?) and the IOP when the symptoms are reproduced. This info, along with the UBM, will help guide what to do next for this patient. It would also be helpful to know the extent of the hyphema— is there blood in the anterior vitre- Jared Knickelbein, MD, chief resident Evan L. Waxman, MD, director of residency training, University of Pittsburgh Medical Center (UPMC) continued on page 54 Academic grand rounds Figure 1. An image of the patient's anterior segment demonstrating a clear cornea, deep and quiet anterior chamber, super iris atrophy, a peripheral iridotomy at 12:30, and three scleral sutures superiorly covered by conjunctiva. Figure 2: Gonioscopy of the superior angle revealed disruption of angle structures with a few peripheral anterior synechiae. The remainder of the angle was normal and open. 51-54 Residents_EW June 2014-DL_Layout 1 6/3/14 12:39 PM Page 53

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