Subretinal fluid associated with pemigatinib therapy for cholangiocarcinoma; a case report and literature review Pemigatinib-associated subretinal fluid.

PURPOSE: To describe the retinal findings associated with pemigatinib, an oral competitive inhibitor of fibroblast growth factor receptor (FGFR) approved for the treatment of cholangiocarcinoma. METHODS: We present a case report and performed a literature review, revealing only two prior reported cases of pemigatinib-mediated subretinal fluid. RESULTS: A 72-year-old woman presented with transient visual symptoms while on pemigatinib for the treatment of cholangiocarcinoma. Optical coherence tomography (OCT) revealed subfoveal subretinal fluid bilaterally that resolved during a drug holiday, however fluctuated throughout her treatment course. She remained on pemigatinib for ten months without other ocular sequelae. CONCLUSIONS: This is one of the few reported cases of pemigatinib-related retinopathy, which further supports the self-limited and benign nature of subretinal fluid associated with FGFR inhibitors.

Gonioscopy-Assisted Transluminal Trabeculotomy for the Treatment of Glaucoma in Uveitic Eyes.

PURPOSE: To evaluate the outcomes of gonioscopy-assisted transluminal trabeculotomy (GATT) in adult eyes with uncontrolled uveitic glaucoma. METHODS: We reviewed 16 eyes from 13 patients. Surgical success was defined as intraocular pressure (IOP) reduction >20% from baseline or IOP between 5 and 21 mmHg by the 3-month visit while on a stable number or fewer IOP-lowering agents and no need for additional glaucoma surgery. RESULTS: At 12 months, the cumulative success rate was 81%. Mean IOP was 37.8 ± 13.0 mmHg at baseline and 12.2 ± 3.0 mmHg at 12 months (68% reduction; p < .0001). The average number of glaucoma medications was 4.6 ± 1.3 at baseline and 2.2 ± 0.7 at 12 months (52% reduction; p < .0001). Transient hyphema was seen in 44% of eyes at 1 week. CONCLUSIONS: This small retrospective study suggests that GATT is effective and safe as an initial surgical treatment for medically refractory glaucoma in uveitic adult eyes.

Retinal Detachment Present at Birth in an Infant With a Novel CTNNB1 Mutation.

A full-term neonate was diagnosed on birth with a unilateral total retinal detachment. The contralateral eye had extensive fibrovascular proliferation, temporal dragging, and peripheral nonvascularized retina. Genetic testing confirmed a mutation in the CTNNB1 gene, which has been associated with familiar exudative vitreoretinopathy and phenotypic features including intellectual disability and spastic diplegia. This novel mutation and its associated syndrome should be considered as a cause of retinal detachment presenting in the neonatal period. [Ophthalmic Surg Lasers Imaging Retina 2022; 53:403-405.].

Conjunctival squamous cell carcinoma arising in immunosuppressed patients (organ transplant, human immunodeficiency virus infection).

PURPOSE: To describe the relationship between chronic systemic immune suppression and conjunctival squamous cell carcinoma (SCC). DESIGN: Retrospective interventional case series. PARTICIPANTS: Thirteen immunosuppressed patients with conjunctival SCC. METHODS: Surgical excision in all cases plus additional topical interferon alpha-2B or mitomycin. MAIN OUTCOME MEASURES: Tumor control. RESULTS: There were 3 groups of patients with chronic immunosuppression and conjunctival SCC, including 8 patients who received an organ transplant, 4 patients with human immunodeficiency virus (HIV), and 1 patient with systemic lupus erythematosus (SLE) receiving long-term corticosteroids. The transplanted organ was kidney (n=4) (1 with additional pancreas transplant), lung (n=2), liver (n=1), and heart (n=1). The mean patient age at presentation for the organ transplant group was 60 years, and the mean interval from transplant to conjunctival SCC was 8.2 years. Management included surgical excision (n=8) plus additional topical interferon alpha-2B (n=3) and mitomycin C (n=1). Three patients showed aggressive recurrence or new tumor, and 1 patient died of brain invasion of SCC. In the HIV group, the mean patient age at presentation was 54 years and the mean interval from HIV diagnosis to conjunctival SCC was 5 years. Management included surgical excision (n=5) plus additional topical interferon alpha-2B (n=3) and mitomycin C (n=1). One patient showed aggressive extensive recurrence requiring enucleation and radiotherapy, and there were no related deaths. The patient with SLE was 49 years old, had been taking systemic corticosteroids for 18 years, and showed control with surgical resection and topical interferon alpha-2B. Of the 5 patients treated with excision and prompt topical interferon alpha-2B, none showed recurrence or new tumor. CONCLUSIONS: Conjunctival SCC can occur in immunosuppressed patients and can be more aggressive and invasive, requiring enucleation or exenteration. Surgical resection plus topical interferon alpha-2B might reduce the risk for recurrence or new tumor. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Incidence of Management Changes at the Postoperative Day One Visit After Pars Plana Vitrectomy for Retinal Detachment.

PURPOSE: To evaluate the incidence of unexpected management changes on the first day after pars plana vitrectomy (PPV) for retinal detachment repair. DESIGN: Retrospective cohort study. METHODS: The medical and billing records of a large academic private practice were electronically queried for all cases of PPV for retinal detachment performed between January 1, 2017, and December 31, 2017. All cases of PPV for rhegmatogenous or tractional retinal detachment with completed postoperative day 1 (POD1) and postoperative week 1 (POW1) visits were included. The preoperative consultation, operative report, and POD1 and POW1 (postoperative days 5-14) visits were reviewed. Main outcome measures were incidence of unexpected management changes (change in or extended positioning, additional procedure, change in drop regimen, or shortened interval follow-up) at the POD1 visit after uncomplicated PPV for retinal detachment. RESULTS: Overall, 418 surgeries from 364 eyes and 355 patients were included. Eleven cases (2.6%) had an intraocular pressure (IOP) over 30 mm Hg at POD1. IOP-lowering drops were prescribed for 30 cases (7.2%). Silicone oil tamponade was positively associated with high IOP at POD1 (relative risk = 3.23, 95% confidence interval 0.96-10.84, P = 0.06). No additional management changes were made besides treating elevated IOP. CONCLUSIONS: Management changes on POD1 after vitrectomy for retinal detachment repair are relatively uncommon and were solely IOP related in this patient group. There may be flexibility regarding the type of POD1 encounter necessary, including an IOP check with an ophthalmic technician or non-retinal eye care provider. Larger, prospective studies are needed to better determine the most efficient follow-up routine.

INVISIBLE, HONEYCOMB-LIKE, CAVITARY RETINAL ASTROCYTIC HAMARTOMA.

PURPOSE: To report a patient with clinically invisible retinal astrocytic hamartomas (RAHs) detected on optical coherence tomography (OCT) and studied with OCT angiography (OCTA). METHOD: Case report. RESULTS: An 11-year-old healthy girl was evaluated for reduced visual acuity in the right eye. On examination, best-corrected visual acuity was 20/50 in the right eye and 20/20 in the left eye. Fundus examination revealed blunted foveal reflex in the right eye and normal findings in the left eye. Optical coherence tomography disclosed an abruptly elevated, honeycomb-like macular mass with thin filamentous walls in the right eye, limited to the nerve fiber layer and measuring 756 µm in thickness. The numerous intralesional cavities (optically empty spaces) displayed no calcification. These findings were consistent with Type IV RAH. Optical coherence tomography of the left eye revealed intact foveola and focal nerve fiber layer thickening inferiorly without cavity, measuring 136 µm in thickness, consistent with Type I RAH. En face OCTA detected microvascular flow within the walls of the cavitary RAH in the right eye. Bilateral RAH in a child with no systemic evidence of tuberous sclerosis syndrome was established. CONCLUSION: Multimodal imaging is important in revealing causes of visual loss and in the detection of subclinical fundus tumors. In this case, clinically invisible RAHs were detected only by OCT and surrounding vascular flow by OCTA.

Outcomes of Eyes Lost to Follow-up with Neovascular Age-Related Macular Degeneration Receiving Intravitreal Anti-Vascular Endothelial Growth Factor.

PURPOSE: To determine outcomes of eyes with neovascular age-related macular degeneration (nAMD) receiving intravitreal anti-vascular endothelial growth factor (VEGF) injections who return after a period of being lost to follow-up (LTFU). DESIGN: Retrospective, cross-sectional study. PARTICIPANTS: Eyes that received intravitreal bevacizumab, ranibizumab, or aflibercept for nAMD and were LTFU for >6 months. METHODS: Comparison of visual outcomes and structural parameters at the visit before LTFU, return visit, and final visit. MAIN OUTCOME MEASURES: Logarithm of the minimum angle of resolution (logMAR) visual acuity (VA), presence of subretinal fluid and intraretinal fluid, and central foveal thickness (CFT) by OCT. RESULTS: A total of 93 eyes of 77 patients were included in the analysis. Mean duration from date of LTFU to return was 346 (±122) days. Overall, 53.7% of patients had worse median logMAR VA by the final visit. Median logMAR VA worsened from 0.60 (0.40-2.00) (Snellen 20/80 [20/50-20/2000]) at the visit before LTFU to 1.00 (0.48-2.00) (20/200 [20/60-20/2000]) at the return visit (P < 0.001). Median logMAR VA remained worse at 6- and 12-months after return from LTFU: 1.00 (0.48-2.00) (20/200 [20/60-20/2000]) (P = 0.001) and 0.70 (0.44-1.30) (20/100 [20/55-20/399]) (P = 0.004), respectively. Despite a mean of 383 (±270) days of follow-up after returning and 5.0 (±5.1) additional injections, the median logMAR VA remained worse at 1.00 (0.54-2.00) (20/200 [20/70-20/2000]) at the final visit compared with the visit before LTFU (P < 0.001). There was greater worsening in mean logMAR VA from the visit before LTFU to the final visit in eyes that received bevacizumab (0.32) and ranibizumab (0.28) compared with aflibercept (P = 0.003, P = 0.04, and P = 0.03, respectively). Mean CFT increased from 201 (±106) µm at the visit before LTFU to 240 (±147) µm at return (P = 0.004). By the final visit, the mean CFT had decreased to 183 (±101) µm, which was not significantly different from the visit before LTFU (P = 0.10). CONCLUSIONS: Eyes with nAMD receiving intravitreal anti-VEGF that were LTFU experience significant VA decline at the return visit that persists on final follow-up despite normalization of CFT.

Clinical Outcomes of Eyes With Diabetic Macular Edema Switched From Aflibercept to Ranibizumab Therapy.

BACKGROUND AND OBJECTIVE: In 2018, cases of inflammation were reported after intravitreal aflibercept (IVA), which resulted in switches to intravitreal ranibizumab (IVR). The authors' purpose was to evaluate outcomes after switching from IVA to IVR in diabetic macular edema (DME). PATIENTS AND METHODS: Retrospective cohort study. Eyes switched from IVA to IVR for treating DME were included. Data were gathered from three visits before to three visits post-switch. Outcome measures included central subfoveal thickness (CFT) and Snellen visual acuity (VA). RESULTS: There was a statistically significant increase in CFT at the first visit (325 µm ± 234 µm; P = .006) compared to the switch visit, but no difference later visits (268 µm ± 103 µm; P = .32; 284 µm ± 118 µm; P = .11; n = 54). There was no statistically significant change in mean logarithm of the minimum angle of resolution VA between the switch and later visits (0.43 ± 0.38, P = .95; 0.38 ± 0.30, P = .12; 0.41 ± 0.37, P = .69). CONCLUSIONS: The authors observed transient worsening of macular edema in eyes treated for DME when switched from aflibercept to ranibizumab. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:691-697.].

Combined Endoscopic and External Resection of an Inverted Papilloma of the Lacrimal Sac: A Case Report and Review of the Literature.

BACKGROUND: Inverted papillomas (IPs) are benign, locally aggressive neoplasms with a high recurrence rate that most commonly arise from the lateral nasal wall. Rarely, IP can originate from the lacrimal sac and/or nasolacrimal duct (NLD) system. A 58-year-old man presented with chronic epiphora and an enlarging mass inferior to his left medial canthal tendon (MCT) for 2 years. OBJECTIVE: The patients' clinical presentation and surgical management are described. A literature review on IP of the lacrimal sac and NLD system was conducted. METHODS: Case report. RESULTS: We present a case of an IP that arose primarily from the lacrimal sac and grew below the MCT rather than extending above it. This is one of few published case reports detailing the use of a combined endoscopic and external approach for the resection of an IP of the lacrimal sac. CONCLUSION: Combined endoscopic and external resection is a viable approach for complete removal of extensive IP of the lacrimal sac and NLD system. IP arising from the lacrimal sac may grow below the MCT or extend above it as previously reported.

Comparison of a Three-Dimensional Heads-Up Display Surgical Platform with a Standard Operating Microscope for Macular Surgery.

PURPOSE: To assess safety, efficacy, and outcomes of vitreoretinal surgery for macular pathology using a 3-dimensional heads-up display (3D HUD) surgical platform compared with a standard operating microscope (SOM). DESIGN: Prospective, single-center, unmasked, randomized study. PARTICIPANTS: Patients undergoing pars plana vitrectomy (PPV) for epiretinal membrane (ERM) or full-thickness macular hole (MH) at Wills Eye Hospital. METHODS: Patients were randomized 1:1 to undergo surgery with a 3D HUD surgical platform or SOM. Patients who had previous PPV were excluded. Surgical choices, including PPV gauge, were based on surgeon preference. Standard surgical safety parameters, Early Treatment Diabetic Retinopathy Study visual acuity (VA), minimum required endoillumination levels, operative times, and surgeon "ease of use" of the viewing platform were recorded. Patients were followed up to postoperative month 3 (POM3). MAIN OUTCOME MEASURES: The main outcome measures were total operative time, macular peel time, surgeon rating of viewing system ease of use, minimum required endoillumination, intraoperative complication rate, and postoperative VA. RESULTS: Thirty-nine eyes of 39 patients with a mean age of 67.60±8.21 SD years were enrolled. Indications included ERM (n = 26 [3D HUD = 14, SOM = 12]) and MH (n = 13 [3D HUD = 9, SOM = 4]). Minimum required endoillumination was significantly lower with 3D HUD (mean 22.70%±15.10% SD) compared with SOM (mean 39.06%±2.72%; P < 0.001). There was no significant difference in overall operative time, but macular peel time was significantly longer using 3D HUD (mean 14.76±4.79 minutes) than SOM (11.87±8.07 minutes; P = 0.004). Surgeon-reported ease of use was significantly higher (easier) using SOM compared with 3D HUD (P = 0.004). There was no statistically significant difference between the groups in POM3 logarithm of the minimum angle of resolution (logMAR) VA or change in logMAR VA from baseline (all P > 0.681). There were no clinically significant intraoperative complications in either group. CONCLUSIONS: Three-dimensional heads-up display surgical visualization is an evolving technology demonstrating comparable efficacy to the SOM for macular surgery. Although overall surgical times were similar, 3D HUD macular peel times were longer and associated with less ease of use in this study, which may partly be due to a learning curve with new technology.

Trends in Retina Specialist Imaging Utilization from 2012 to 2016 in the United States Medicare Fee-for-Service Population.

PURPOSE: To characterize temporal trends and regional variance in retinal imaging utilization in the United States Medicare fee-for-service population from 2012-2016. DESIGN: Cross-sectional, retrospective database analysis. METHODS: This study addresses office or operating-room based retinal imaging. Our study population included retina specialists, defined as ophthalmologists performing either intravitreal anti-vascular endothelial growth factor injections or posterior segment laser photocoagulation and no neodymium-doped yttrium aluminum garnet laser capsulotomy. We recorded fundus photography, optical coherence tomography (OCT), intravenous fluorescein angiography (IVFA), indocyanine-green angiography, and ophthalmic ultrasound (B-scan) billed in the Medicare fee-for-service population from 2012-2016. Imaging obtained on any platform or device was eligible for inclusion (eg, posterior pole imaging vs ultrawidefield imaging). The main outcome measure was the relative utilization of retinal imaging modalities. RESULTS: National relative utilization of OCT increased from 61.5% in 2012 to 70.5% in 2016 (P < .001), while IVFA fell from 20.9% to 15.1% over the same interval (P < .001). Fundus photography decreased from 14.6% in 2012 to 11.7% in 2016 (P < .001). By 2016, the Midwest region had the highest relative utilization of OCT (75.2%) and lowest of IVFA (12.0%), while the West region had the lowest OCT (68.4%) and highest IVFA (17.0%). CONCLUSIONS: Among retina specialists, OCT usage increased while the utilization of fundus photography and IVFA has declined. The Midwest region had the highest utilization of OCT and lowest of IVFA.

American joint committee on cancer staging of uveal melanoma.

In January 2010, the American Joint Committee on Cancer (AJCC) updated its staging criteria for uveal melanoma. Here, we discuss the staging classifications and evaluate an interesting case to demonstrate. The development of a uniform means of staging cancer, such as defined by the AJCC, is critical for information sharing among the research community.

Giant conjunctival nevus: clinical features and natural course in 32 cases.

IMPORTANCE: Conjunctival nevus is the most common tumor of the ocular surface and we selected the largest nevi to evaluate for clinical features and outcomes. OBJECTIVE: To describe the clinical features and outcomes of giant (≥10 mm diameter) conjunctival nevus. DESIGN, SETTING, AND PATIENTS: Retrospective case series of 618 patients with conjunctival nevus, 32 of which had giant conjunctival nevus, treated at an ocular oncology service between July 1, 1974, and June 30, 2012. EXPOSURES: Observation, excisional biopsy, and cryotherapy. MAIN OUTCOMES AND MEASURES: Management, nevus recurrence, and nevus transformation into melanoma. RESULTS: Of 618 patients with conjunctival nevus, 32 (5%) were classified as having giant conjunctival nevus. The mean patient age at diagnosis was 34 years. Of those with giant conjunctival nevus, a history of an increase in nevus base or thickness was noted in 15 cases (47%) and an increase in color intensity in 2 cases (6%). The giant nevus involved cornea in 11 cases (34%), limbus in 23 (72%), bulbar conjunctiva in 31 (97%), fornix in 9 (28%), tarsus in 3 (9%), semilunar fold in 10 (31%), and caruncle in 7 (22%). The nevi had mean diameter of 16 mm and mean thickness of 2 mm. On slitlamp examination, intrinsic cysts were identified in 25 cases (78%), intrinsic blood vessels in 26 (81%), and feeder vessels in 22 (69%). Management included excisional biopsy with cryotherapy in 23 cases (72%) and observation in 9 cases (28%). Amniotic membrane graft reconstruction was used following excision in 3 cases (13%). Malignant melanoma developed within the giant nevus in 1 case after 23 years of observation. Postexcisional biopsy, nevus recurrence was detected in 4 cases (17%), pseudopterygium in 1 (4%), dry eye in 1 (4%), and eyelid blepharoptosis in 1 (4%). CONCLUSIONS AND RELEVANCE: In an ocular oncology practice, giant conjunctival nevus represents 5% of conjunctival nevi. This benign tumor rarely transforms into conjunctival melanoma. Management alternatives include observation or wide excisional biopsy, cryotherapy, and reconstruction, possibly with amniotic membrane graft.

Autofluorescence of treated retinoblastoma.

PURPOSE: To describe the autofluorescent features of retinoblastomas after treatment. METHODS: Standard fundus photography and autofluorescence (AF) photography (580 nm excitation, 695 nm barrier filter) were performed on 88 tumors of 61 patients. Clinical features were correlated with autofluorescent features. RESULTS: The mean patient age at AF was 10.3 years. Of the 88 tumors, 5 (6%) were untreated, and 83 (94%) were treated. The untreated retinoblastomas showed hyperautofluorescence (hyperAF) at the site of calcification in all 5 cases (100%). The treated retinoblastomas showed intrinsic calcification in 54 cases (65%) and bright hyperAF at the site of calcification was detected in all cases. Of the 60 tumors with noncalcified remnant, the noncalcified portion was mildly hyperAF in 20 (33%), isoautofluorescence in 31 (52%), and mildly hypoautofluorescence (hypoAF) in 9 (15%). Surrounding retinal pigment epithelium hyperplasia appeared moderately hypoAF in 58 of 58 eyes (100%). Retinal pigment epithelium atrophy appeared mildly hyperAF in 29 (37%), isoautofluorescence in 33 (42%), and mildly hypoAF in 16 (21%). CONCLUSIONS: AF of retinoblastoma generally shows bright hyperAF of the calcified portion and variable AF of the noncalcified portion. The AF of calcification in retinoblastoma was confirmed by fluorescent microscopy of unstained sections of retinoblastoma after enucleation.