ASRS 2022 – Case Presentations Part II

Jacob Light, MD
Abtin Shahlaee, MD
Meera Sivalingam, MD
Wills Eye Hospital

We hope that you are enjoying our ASRS 2022 coverage. The case presentation session is always a popular segment of the ASRS meeting, and this year, 24 medical and surgical cases were showcased. Part I of our summary can be read here: ASRS 2022: Case Presentations Part I.

The following is a summary of the second half of the case presentation session (cases 13-24). There are many clinical pearls that can be learned from these unique cases.

Case 13: Dr. Bradley Smith, The Retina Institute

A young man presented with progressive bullous serous retinal detachments. ICG showed multifocal areas of choroidal hyperfluorescence, consistent with central serous chorioretinopathy. The right eye, which had the worse serous retinal detachment, improved nicely with full fluence PDT. Dr. Lee Jampol shared the opinion that given the data from the VICI trial, which was a randomized controlled trial demonstrating no benefit to eplerenone in CSCR, retina specialists should not continue to prescribe it for this clinical indication.

Case 14: Dr. Manju Subramanian, Boston Medical Center

An older man with history of NPDR and renal transplantation presented with decreased vision in the left eye. Exam showed intraretinal hemorrhages in both eyes and macular edema in the left eye. Anti-VEGF therapy was initiated in the left eye, but over the course of 6-7 months, he developed frank vitreous cell. PCR of aqueous humor was positive for CMV. CMV retinitis can masquerade as an indolent retinopathy, at times difficult to distinguish from other retinal vasculopathies like diabetic retinopathy. In this case, the patient’s immunocompromised status post-renal transplantation was a key contributing factor, and the development of vitreous cell (which should not occur in diabetic retinopathy) a critical exam finding, particularly in non-HIV related CME retinitis.

Case 15: Dr. Eduardo Uchiyama, Retina Group of Florida

A middle aged man with HIV and SLE on oral prednisone and plaquenil presented with new blurry vision in both eyes for 2 weeks. He had bilateral anterior chamber cell, disc edema, and outer retinal pigmentary changes. He was found to have extensive rashes over the limbs (which had not been detected despite multiple visits to rheumatology in the interim) and skin biopsy was positive for spirochetes, consistent with syphilis. RPR titers were surprisingly negative, but this was likely the result of the prozone effect, where high concentration of antigen interferes with proper function of the serologic lab testing.

Case 16: Dr. Leila Vajzovic, Duke Eye Center

An elderly man with subretinal hemorrhage underwent vitrectomy with subretinal tPA injection under intraoperative OCT guidance. Dr. Vajzovic demonstrated how volumetric segmentation of the iOCT images can be used to measure the amount of subretinal fluid delivered. Interestingly, in this presented case, the volume measured by OCT was actually 20% more than what was expected.

Case 17: Dr. Nita Valikodath, Duke Eye Center

A young boy s/p open globe repair two weeks prior in the right eye presented with new vision loss in the left eye. Findings of serous RD OS, multiple deep foci of hyperfluorescence on FA in setting of recent contralateral ocular trauma confirmed suspicion of sympathetic ophthalmia. He was treated with combination IV and oral steroids and immunomodulatory infusions and the serous RD resolved nicely.

Case 18: Dr. Taku Wakabayashi, Wills Eye Hospital

A young girl with moderate myopia had 20/50 vision and peripapillary subretinal fluid in the left eye as well as a “ridge-shaped papilla” with inward convexity of the peripapillary sclera. Fluorescein angiography showed hyperfluroescence without leakage at temporal border of the disc consistent with window defect. This is similar to a subtype of staphyloma known as “type IX” as described by Victor Curtin in the 1970s. The serous RD likely stems from choroidal thinning and resultant RPE atrophy due to the abnormal scleral contour. The investigators have termed this unique condition “dome shaped peripapilla.”

Case 19: Dr. Jay Wang, Northern California Retina Vitreous Associates

A middle aged man presented with sudden monocular vision loss and headache and left-sided chest pain. Retinal whitening in a cilioretinal arterial distribution with multiple emboli in the left eye was seen. Initial stroke work-up in the emergency department neglected to include echocardiogram, but this was prudently ordered by Dr. Wang and revealed a large cardiac atrial myxoma. Complete retinal arterial occlusion work-up, especially in the setting of retinal emboli, must include echocardiogram. This critical test may result in life-saving intervention for a patient.

Case 20: Dr. Christina Weng, Baylor College of Medicine

A middle aged woman with history of renal stones presented with deep yellow choroidal lesions in both eyes. Radiographic studies of the knee and ankle showed chondrocalcinosis, and a hard forehead lesion was seen on exam. She had persistent hypokalemia and hypomagnesemia despite oral supplementation. In this context, the ocular findings were deemed to be consistent with sclerochoroidal calcifications. These are often idiopathic but can be secondary to hyperparathyroidism and other systemic processes, thus the clinician should be prepared to evaluate for systemic abnormalities.

Case 21: Dr. David Xu, Wills Eye Hospital

A middle aged man with intraocular foreign body underwent pars plana vitrectomy, lensectomy, IOFB removal and silicone oil placement. He developed acute IOP elevation from oil prolapse into the anterior chamber. He thus required placement of oil retention sutures. Dr. Xu shared an interesting technique in which the max grip forceps (which has ample length) can be passed transocular across the entirety of the anterior segment through diametrically opposed entry and exit sclerotomies facilitate passing of the retention sutures. The panel discussion did point out that retention sutures may not be successful in hypotonous eyes and that sufficient aqueous production in the eye is essential to maintain an intact aqueous/oil interface and surface tension.

Case 22: Dr. Daniel Brill, Cincinnati Eye Institute

A young woman presented with eye redness and blurred vision, and found to have monocular vitritis and white retinal lesions. Vitreous tap and injection of antibiotics and antifungals was performed to cover for endogenous endophthalmitis. Despite treatment, the clinical picture worsened and the fellow eye became involved. Serum testing was ASO positive, and the patient was diagnosed with poststreptococcal syndrome uveitis. This is typically a bilateral nongranulomatous uveitic process in patients less than 40 years. It is immune-mediated, occurring 1-8 weeks after strep infection. Vitritis is only present in roughly a third of cases, and retinitis is rare. Take home: post-streptococcal uveitis is difficult to diagnose as it requires high clinical suspicion and specific testing. This is likely an under-recognized entity.

Case 23: Dr. Yoshihiro Yonekawa, Wills Eye Hospital

A young girl was referred for evaluation of high myopia. She had known cognitive delay, short stature, microcephaly, and iris coloboma. Retinal exam showed bilateral vessel attenuation, temporal non-perfusion, and peripheral hyloidal organization and opacification over lattice like changes. Differential included FEVR, but genetic testing was negative. Ultimately a DYRK1A (kinase important in CNS development) mutation was detected. The non-perfused periphery was treated with scatter laser photocoagulation. This is the first known reported case of a DYRK1A-associated retinopathy.

Case 24: Dr. Jaclyn Kovach, Bascom Palmer Eye Institute

A woman in her 30’s was found to have very subtle bilateral and symmetric macular pigmentary abnormalities. OCT revealed subretinal hyperreflective material in both eyes. She had been diagnosed with age-related macular degeneration, but this was deemed to be inconsistent with the patient’s young age. Thoughtful consideration of the differential diagnosis included pattern dystrophy, Stargardt disease, maternally inherited diabetes and deafness (MIDD) macular dystrophy, and pentosan toxicity. Testing was positive for MIDD and further history revealed the patient had mild hearing loss and diabetes. Take home: the MIDD maculopathy phenotype can be very subtle and should be considered, especially in cases where patient factors are inconsistent with classic AMD.