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View Returned the Following Day, Less Than OR s s CONSEQUENTIAL CASES CASES THAT CHANGED THE WAY WE PRACTICE Three retina specialists share stories of patient encounters that left lasting impressions. LET THERE BE LIGHT By Ninel Z. Gregori, MD vision requires daily practice and hard my career in the direction of other A 52-year-old patient work. The patient had an intense innovative surgeries and clinical trials who had seen nothing rehabilitation process ahead of her at Bascom Palmer. but weak light for to help her to make sense of the And it did much more: It helped me 19 years since devel- lights. She is now able to identify light truly understand and appreciate the oping end-stage retinitis pigmentosa objects against dark backgrounds, value of careful preoperative counsel- (RP) came into my care. Then, in 2013, sort light and dark clothes, see light ing and setting realistic expectations the long-awaited promise of artificial coming in the windows, identify the for patients undergoing new treat- vision became a reality because the handle on the refrigerator, and even ments. After observing the experiences US FDA had approved the first retinal identify some letters, numbers, and of my patient receiving an Argus II prosthesis for patients with RP. I dis- simple words on a computer screen. implant, I now make sure to carefully cussed the Argus II Retinal Prosthesis She cannot, however, recognize explain the risks and limitations of System (Second Sight) with the objects and people in the environ- artificial vision options to patients who patient, and we agreed that she was a ment consistently, see faces, or read. are interested in the technology. good candidate to receive the implant. At times, she is frustrated with the When speaking to the patients limitations of bionic vision and the enrolled in the choroideremia gene A BASCOM PALMER FIRST mental exhaustion she feels while therapy trials at Bascom Palmer, for After a visit to the University of using the Argus II for activities associ- example, I make sure they understand Southern California to watch Mark ated with daily living. the risks involved: that their vision Humayun, MD, PhD, and Lisa Olmos may or may not improve, that the de Koo, MD, MBA, implant an Argus ONE CASE, MANY LESSONS LEARNED goal is to preserve what vision they in a patient, I drafted my own surgical For me as a retina surgeon, it was have, and that the surgery has its own protocol with diagrams and sketches incredibly rewarding to be able to risks, which we of course minimize to and was ready to lead my surgical treat a patient with an irreversibly the best of our abilities. team on our first case at Bascom blinding condition who had previously This experience has also helped me Palmer in 2014. had no therapeutic options. It was a to better educate the patients in my The surgery lasted more than unique opportunity to develop a close daily clinical practice. No matter how 4 hours, but everything went as personal connection with the patient routine a vitrectomy or cataract sur- planned. After the patient had healed and her family through frequent com- gery may seem to the surgeon, there for several weeks, we programmed munication at the institute and via are always risks involved, and we can- the retinal prosthesis and turned it emails and telephone. In addition, this not eliminate those risks completely. on. The patient was mesmerized and groundbreaking procedure pushed my Thus, we must always carefully inform simultaneously confused by the new limits as a surgeon, opened my mind and educate patients before we take fluttering lights she saw. Artificial to continuous learning, and launched them into the OR. 54 RETINA TODAY | OCTOBER 2018 CONSEQUENTIAL CASES s TEAMWORK MAKES THE DREAM WORK It taught me to keep my mind open 31 patients as part of phase 1/2 and In 2018, I became one of the sur- when listening and to consider the phase 3 gene therapy trials, learning geons at Bascom Palmer to perform expertise of others, while at the same new subretinal injection techniques subretinal injections of the first FDA- time trusting my own abilities as a and designing safer, more controlled approved ocular gene therapy, voreti- surgeon. Participating in these trials surgical approaches. I look forward to gene neparvovec-rzyl (Luxturna, Spark has also helped me to understand that performing more innovative surgeries Therapeutics), for treatment of Leber a team approach with talented junior as my lifelong learning continues. congenital amaurosis or severe early and senior colleagues is the ultimate onset RP due to biallelic mutations of path to surgical excellence and better the RPE65 gene. Participating in gene outcomes for patients. NINEL Z. GREGORI, MD therapy and stem cell therapy trials at It is important to continue to n Associate Professor of Clinical Ophthalmology, Bascom Palmer years after completing evolve throughout our careers. Since Bascom Palmer Eye Institute, Miami, Florida my vitreoretinal fellowship opened my that first patient, I have implanted n [email protected] mind to incorporating input from oth- three more Argus devices and have n Financial disclosure: Grant Support (Nightstar ers regarding my surgical technique. participated in gene therapy for Therapeutics) AN UNUSUAL PRESENTATION OF HORV By Tarek S. Hassan, MD Later that evening, the patient called (EVS) would recommend continued I have been in clinical complaining of worsening pain and observation over vitrectomy. But the practice for more than decreased vision. He was examined significant pace and worsening severity 20 years, and I still try within an hour and was found to have of symptoms, even over the course to learn something increased anterior chamber fibrin, of 1 day, gave us pause. The patient new each time I am in the office or more vitreous opacities, a poorer view returned the following day, less than OR. However, it has been quite a of the posterior segment, and visual 48 hours after initial presentation, with while since a case has fundamentally acuity that had decreased to 20/800. light perception vision, unchanged changed how I manage patients as He was reassured that often after pain, and an anterior chamber much as the one I detail below. injection of antibiotics for endophthal- with more fibrin but no hypopyon mitis there is a short period when the (Figure 1). B-scan still demonstrated A STRAIGHTFORWARD CASE OF clinical picture appears worse because only mild to moderate vitritis. ENDOPHTHALMITIS? of increased inflammation. Topical We proceeded with vitrectomy later Two years ago, a 60-year-old obese steroids were increased. No gram stain that evening, which began with remov- and hypertensive man was referred report was yet available from the vitre- al of the prominent central fibrin clot. to me with pain and decreased vision ous tap done a few hours earlier. With our improved view, we found a (20/200) in his right eye 3 days after nearly confluent hemorrhagic retinitis uncomplicated cataract extraction. RECONSIDERING THE DIAGNOSIS with no retinal detachment and only His medical history was unremarkable. The patient returned the next day mild vitreous inflammatory debris At presentation, his eye displayed with no relief of his pain, a rise in (Figure 2). We diagnosed hemorrhagic injection and he had a mild subcon- IOP to 30 mm Hg, increased corneal occlusive retinal vasculitis (HORV). junctival hemorrhage. He had 2+ cells, edema, and vision that had dropped to We completed a simple vitrectomy trace fibrin in the anterior chamber, hand motions. B-scan ultrasonography and sent vitreous fluid for bacterial and and no hypopyon. His fundus was showed that the retina was attached fungal cultures, universal bacterial and visible, although the view was slightly and there was only mild vitritis. At fungal primer polymerase chain reac- hazy. We noted a few scattered intra- only 1 day after the tap and inject, we tions, viral polymerase chain reactions retinal hemorrhages in the posterior were concerned about his worsening for herpes simplex virus, varicella zoster pole but otherwise noted no remark- clinical picture and discussed the virus, and cytomegalovirus, and patho- able findings. We made a diagnosis of possibility of repeat antibiotic injec- logic evaluation. All of these studies endophthalmitis and proceeded with tion but more likely vitrectomy. The were negative. We initiated a uveitis a vitreous tap and injection using 1 mg patient had postcataract endophthal- workup for common inflammatory and of vancomycin and 2.25 mg of ceftazi- mitis with hand motions vision. The infectious causes of retinal vasculitis, dime per our usual protocol. Endophthalmitis Vitrectomy Study and this was also entirely negative. OCTOBER 2018 | RETINA TODAY 55 s CONSEQUENTIAL CASES vasculopathy without vasculitis, chronic nongranulomatous choroiditis, and an unusual glomeruloid prolifera- tion of endothelial cells in the choroid and elsewhere in the eye, rather than an overt retinal vasculitis, which had traditionally been expected but never actually verified pathologically.1 RETRACING OUR STEPS At presentation, this patient appeared to have routine postcataract endophthalmitis. Our standard proto- col, like that of most retina specialists, is to perform a vitreous and/or anterior chamber tap and injection of vancomy- cin and ceftazidime. We had not con- tacted the patient’s cataract surgeon Figure 1. Appearance of the patient’s right eye on day 2. initially, but did so after the vitrectomy. He informed us that he used intracam- eral vancomycin during his surgery. I am concerned that our second dose of intravitreal vancomycin may have compounded the effects of the initial vancomycin, thereby worsening the severity of the patient’s HORV. There had been no prior reports of an endophthalmitic form of HORV in the literature, and thus we did not sus- pect it at initial presentation.
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