Matthew Starr, MD
Assistant Professor of Ophthalmology
Following the recent release of the top 10 accessed papers from RETINA in 2021, we now are presenting the top 10 case reports from RETINAL CASES & BRIEF REPORTS in 2021. Authors from all over the world submitted challenging patients, including those with atypical CSR, CMV retinitis, lipemia retinalis, persistent placoid maculopathy, iron overload, POEMS, and more. These were the top 10 most accessed reports from 2021.
We hope these cases offer great insight into handling all of the tough cases that will be presented to us all in this upcoming year. The top case of 2021 comes out of Philadelphia by Drs. Anton Kolomeyer and Alexander Brucker, “PERSISTENT PLACOID MACULOPATHY IN A PATIENT WITH HIGH MYOPIA.”
1. PERSISTENT PLACOID MACULOPATHY IN A PATIENT WITH HIGH MYOPIA
Kolomeyer, Anton M. MD, PhD; Brucker, Alexander J. MD
Drs. Kolomeyer and Brucker present the case of a 59-year-old male who was previously diagnosed with exudative AMD in his left eye and was subsequently treated with a single intravitreal anti-VEGF injection before presenting to them for a second opinion. He was a high myope with a spherical equivalent of -8.25D and axial length of 26.7 mm in both eyes. On initial presentation the patient was 20/20 bilaterally and had a normal exam in his right eye and the exam was consistent with a presumed CNV in his left eye. However, there were no drusen or other signs of myopic maculopathy in either eye. The patient was maintained on anti-VEGF therapy using a treat-and-extend approach. Six months after presentation he reported visual changes in his right eye. Visual acuity was 20/25 in both eyes; however, he had a new hypopigmented lesion in the temporal foveal region of his right eye with outer retinal thickening and SRF on OCT in the right eye. OCT of the left eye revealed evolving macular changes with resolution of the previous SRF. OCTA of both eyes revealed marked hyposignal in the choriocapillaris. ICG showed persistent hypocyanesence in both eyes.
At this time, the authors made the diagnosis of persistent placoid maculopathy (after ruling out the typical infections such as TB and syphilis) due to the bilateral nature of the disease, preservation of vision, and patient’s age. The patient was promptly initiated on oral prednisone and received two more anti-VEGF injections in his left eye. The prednisone was tapered slowly, however, with each taper there was recurrent outer retinal thickening and SRF as well as flow void defects on OCTA. The patient ultimately required both mycophenolate mofetil and methotrexate for disease maintenance. Twenty-three months after presentation the patient was 20/20 in the right eye and 20/25 in the left eye with RPE mottling and resolution of SRF in both eyes.
The authors report the first case of PPM associated with high myopia. The patient, however, had a relatively thick choroid for his level of myopia and thus these structural characteristics may have led to the subsequent OCT findings. The authors conclude that cases of persistent placoid maculopathy such as this one require prompt diagnosis and often need both anti-VEGF and immunosuppressive therapy in order to preserve vision over time.
2. BILATERAL ENDOGENOUS ENDOPHTHALMITIS FROM STREPTOCOCCUS PNEUMONIAE
Brill, Daniel A. MD; Farley, Nathan D. MD; Albert, Desiree C. MD; Sassalos, Therese M. MD; Sangave, Amit A. MD; Desai, Uday R. MD
The #2 accessed report was by Brill and colleagues who presented an interesting case of bilateral endogenous endophthalmitis due to S. pneumoniae in a patient with a history of an acute ear infection as well as self-extraction of his own teeth. A certainly unique aspect to his clinical presentation. The patient unfortunately succumbed to the serious systemic sequalae of the infection. The authors note the importance of a timely systemic evaluation in patients with endogenous endophthalmitis to augment patient morbidity.
3. RANIBIZUMAB 0.5 MG TREATMENT IN ADOLESCENTS WITH CHOROIDAL NEOVASCULARIZATION: SUBGROUP ANALYSIS DATA FROM THE MINERVA STUDY
Hykin, Philip G. FRCOphth; Staurenghi, Giovanni MD; Wiedemann, Peter MD; Wolf, Sebastian MD, PhD; Liew, Shiao Hui Melissa MBBS, MRCOphth; Desset-Brethes, Sabine PhD; Staines, Harry PhD; Li, Jun MD, PhD; Lai, Timothy Y. Y. MD, FRCOphth, on behalf of the MINERVA study group
The MINERVA study group reported a post-hoc case series of 5 teenage patients with any choroidal neovascularization etiology (2 Best disease, 2 idiopathic, and 1 due to optic disc drusen) treated with ranibizumab 0.5mg at presentation and then subsequent pro re nata approach. The patients received a mean of 3 injections over the 1 year study period with significant improvements in visual acuity and central subfield thickness. The authors conclude that adolescents with CNV may see a significant improvement with anti-VEGF therapy using a PRN approach with only a few injections needed over a 1 year period.
4. RAPIDLY PROGRESSIVE NEOVASCULAR GLAUCOMA FROM CYTOMEGALOVIRUS RETINITIS IN A NON–HUMAN IMMUNODEFICIENCY VIRUS PATIENT
Cho, Jane MD; Siegel, Nicole MD; Subramanian, Manju L. MD; Ying, Howard MD, PhD; Ness, Steven MD
Cho et al describe the case of a 71-year-old male with multiple myeloma on monthly infusions of daratumumab after a stem cell transplant. He presented with 2 weeks of floaters, photophobia and worsening vision and was found to have CMV retinitis with diffuse occlusive vasculitis and despite aggressive therapy, developed neovascular glaucoma. The patient was found to be HIV negative with a CD4 count of 450/mm3. A truly devastating outcome in a very complex case. The authors note that this atypical ischemic CMV retinitis may become more prominent in the non-HIV era in the setting of increasing use of systemic immunosuppressive therapy.
5. CENTRAL SEROUS CHORIORETINOPATHY ASSOCIATED WITH STEROID ENEMA
Peiris, Timothy J. MD; El Rami, Hala E. MD; Sun, Jennifer K. MD, MPH
Peiris, El Rami, and Sun describe a case of central serous chorioretinopathy with an atypical steroid exposure. The patient is a 47-year-old male with ulcerative colitis managed with steroid enemas. The patient was able to be weaned from the steroid enemas and within 1 month his vision returned. He remained off the steroid enemas for 7 years until he again tried resuming them, but unfortunately developed recurrent subretinal fluid soon after, having to seek alternative therapy for his ulcerative colitis.
6. DOCUMENTATION OF A NEW CHOROIDAL NEVUS
Parikh, Ravi MD, MPH; Gal-Or, Orly MD; Sakurada, Yoichi MD, PhD; Leong, Belinda MD; Freund, K. Bailey MD
Parikh and colleagues report a case of a 73-year-old male with timely referral for pachychoroid neovasculopathy. Initial fundoscopic examination confirmed the diagnosis and he was also noted to have two small peripheral nevi in his right eye and a single small nevi in his left temporal macula. One year after initial presentation a new nevus in his left eye was documented and confirmed to be new after reviewing to the previous imaging one year prior. Extensive systemic work up did not reveal any evidence of occult malignancy. The patient remained stable 4 years after the new nevus was diagnosed and the authors concluded this may be perhaps the first documented case of a new choroidal nevus.
7. OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY IN THE DIAGNOSIS AND FOLLOW-UP OF RETINAL ARTERIAL MACROANEURYSMS
Astroz, Polina MD; Miere, Alexandra MD; Cohen, Salomon Yves MD, PhD; Querques, Giuseppe MD, PhD; Souied, Eric H. MD, PhD
This 2 patient case series describes two females with retinal arterial macroaneurysms with associated macular edema. Initial OCTA imaging detected flow in both RAMs at baseline. The first patient underwent focal laser therapy and the second patient was observed. In both cases, no detectable flow was seen at 1 month follow up visits on OCTA, suggesting closure by laser in the first case and spontaneous closure in the second case. The authors conclude that OCTA can be useful in guiding decision making strategies without the need for FA at follow up appointments.
8. RETINAL MICROSTRUCTURE IN POEMS SYNDROME
Tomita, Ryutaro MD; Sekiryu, Tetsuju MD; Shintake, Hiroaki MD; Saito, Kuniharu MD
This was a nice imaging report of a 67-year-old female with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome (POEMS) using adaptive optics and OCT. The patient presented with bilateral serous retinal detachments with hyperreflective foci on OCT and low-reflective dots on adaptive optics within the inner retina. Three months following systemic treatment, the lesions disappeared. The authors highlight the ability of adaptive optics to detect retinal microstructures in vivo.
Abraham reports a unique case of bull’s eye maculopathy of a 6-year-old girl with a history of thalassemia major requiring frequent blood transfusions since she was two years old. The patient presented with subacute bilateral vision loss after recently stopping her iron chelator, deferasirox, one month prior to presentation. The typical bull’s eye maculopathy had a unique purplish hue in both eyes thought possible due to iron deposition. Dr. Abraham reports that not all bull’s eye maculopathies in children will be genetic in etiology and history is of the utmost importance.
10. LIPEMIA RETINALIS DURING CHEMOTHERAPY WITH ADJUNCTIVE GLUCOCORTICOID TREATMENT IN A PATIENT WITH COLON CARCINOMA
Wetzel, Barbara MD; Mylonas, Georgios MD; Puntus, Thomas MD; Prager, Franz MD; Bernhart, Clemens MD; Amon, Michael MD
Wetzel and co-authors report aa unique case of lipemia retinalis in a 55-year-old male with previously well controlled hyperlipidemia who developed vision loss during a course of chemotherapy. He received 6 cycles of FOLFIRI/bevacizumab with dexamethasone and it was during this treatment he had acute decompensation of his hyperlipidemia with resultant cholesterol and triglyceride levels above 8,200 mg/dL. Six weeks after finishing his chemotherapy the lipemia retinalis resolved. The authors highlight the potential for patients with previously well controlled hyperlipidemia to decompensate when taking dexamethasone.