Shields, Carol L.

Purpose: To describe the outcome of ultra-low-dose (boom-boom) radiotherapy for choroidal lymphoma. Methods: Retrospective series of three consecutive patients with biopsy-proven choroidal lymphoma treated with ultra-low-dose radiotherapy. Results: The three patients (two male, one female) of mean age 70 years (range, 64–74 years) demonstrated presenting visual acuity in the affected eye between 20/40 and 20/50. The choroidal lymphoma was unilateral in all cases and presented with multifocal yellow patchy choroidal infiltration, located in all four quadrants and measuring mean 2.9 mm (range, 1.9–4.0 mm) in thickness by ultrasonography. Anterior epibulbar extension of 5 mm diameter was noted in one case. By enhanced depth imaging optical coherence tomography, the choroidal infiltration demonstrated classic undulating appearance (n = 3), with subretinal fluid (n = 2) and intraretinal edema (n = 1). There was no systemic lymphoma in any case. Biopsy was performed in all three cases and was diagnostic (n = 1) or suggestive (n = 2) of B-cell lymphoma. Management involved ultra-low-dose radiotherapy (4 Gy delivered in two fractions, “boom-boom”). On follow-up (mean = 14 months, range = 6–24 months), complete tumor regression on ophthalmoscopy was documented in all three cases, with enhanced depth imaging optical coherence tomography and ultrasonography demonstrating evidence of lymphoma resolution and visual acuity improvement to 20/25–20/40. There were no radiation complications. Conclusion: In this small case series, ultra-low-dose (boom-boom) radiotherapy was effective for choroidal lymphoma with favorable response and minimal side effects.

AbstractImportanceRetinoblastoma is a life‐ and sight‐threatening malignancy.BackgroundTo assess the relationship between tumour perfusion and intra‐arterial chemotherapy (IAC) requirements to achieve retinoblastoma control.DesignRetrospective case series at the Ocular Oncology Service of Wills Eye Hospital (Philadelphia, Pennsylvania).ParticipantsFifty‐nine eyes of 55 patients.MethodsReview of medical and fluorescein angiography (FA) records for retinoblastoma treated with primary or secondary IAC from 2012 to 2017. Vascular supply of the main tumour was evaluated in the pre‐treatment FA.Main Outcome MeasuresTumour fluorescence was classified as partial <67% or complete tumour perfusion >67%. Partially vs completely perfused tumours were compared for IAC cycle requirements.ResultsThere were 59 eyes of 55 patients with pre‐treatment FA managed with IAC. Partially perfused tumours (n = 20, 34%) required fewer IAC infusions than completely perfused tumours (n = 39, 66%) (2.5 vs 3.7 infusions, P = .02), even after adjustment for confounding factors (tumour diameter, thickness and drug scheme, adjusted P = .04). Tumour perfusion correlated with number of IAC cycles required for tumour control (r = 0.46, P < .001). For primary IAC (n = 18, 31%), tumour perfusion was not associated with number of IAC cycles (P = .63). For secondary IAC (n = 41, 69%), partially perfused tumours (n = 15, 37%) required fewer IAC infusions than completely perfused tumours (n = 26, 63%) (2.1 vs 3.7 infusions, P < .01).Conclusions and RelevanceFA demonstrating partial retinoblastoma tumour perfusion is associated with fewer IAC cycle requirements for secondary but not primary IAC. FA might be useful in judging anticipated treatment cycles of retinoblastoma managed with primary or secondary IAC.

Purpose: To describe a case of ciliochoroidal melanoma with massive spontaneous hemorrhagic retinal and choroidal detachment that presented a diagnostic challenge because of poor fundus visualization and atypical imaging features. Methods: Case report. Results: A 71-year-old white man on apixaban for atrial fibrillation presented with sudden onset of pain and vision loss in the left eye. The patient was found to have total hemorrhagic retinal detachment and was referred for our opinion. On examination, visual acuity was 20/25 in the right eye and light perception in the left eye. Intraocular pressures were 10 mmHg in the right eye and 21 mmHg in the left eye. The unaffected right eye demonstrated only macular retinal pigment epithelial alterations. Evaluation of the left eye revealed dilated episcleral sentinel vessels superonasally, shallow anterior chamber, and total hemorrhagic retinal and choroidal detachment to the back of the crystalline lens. There was no view of the choroid. Transillumination demonstrated diffuse blockage of light from blood and no specific shadow. B-scan ultrasonography revealed total retinal and choroidal detachment with dense echoes of blood under the retina and in the suprachoroidal space with elevation of 12 mm and no definite mass or spontaneous vascular pulsations. Magnetic resonance imaging revealed diffuse high signals within the globe on T1-weighted images with no specific mass. On gadolinium enhancement, a localized enhancing area temporally of 12-mm thickness and a localized nonenhanced area nasally of 11-mm thickness was noted. Both areas showed low signal on T2-weighted images. Despite atypical features, suspicion for underlying ciliochoroidal melanoma and poor visual prognosis lead to enucleation. Histopathology confirmed an extensively necrotic, mushroom-shaped ciliochoroidal melanoma with total hemorrhagic retinal and choroidal detachment. Conclusion: Diagnosis of necrotic posterior uveal melanoma can be challenging, because these tumors can be camouflaged by overlying hemorrhagic retinal and choroidal detachment and can present with atypical features on ultrasonography and magnetic resonance imaging. Patients with spontaneous hemorrhagic retinal and choroidal detachment should be evaluated for possible underlying malignancy.

Purpose: To evaluate the outcome of photodynamic therapy (PDT) in the management of extrafoveolar choroidal osteoma. Methods: The authors performed a retrospective chart review of all patients with choroidal osteoma that did not involve the foveola and were treated with standard-fluence PDT. Results: Nine eyes with extrafoveolar choroidal osteoma were studied. Mean logarithm of the minimum angle of resolution best-corrected visual acuity at initial examination was 0.07 (Snellen ∼20/25). The osteoma was treated with 1 (8/9) or 2 (1/9) PDT sessions using 50 J/cm 2 . After a mean follow-up of 49 months, the treated area of osteoma demonstrated complete (4/9) or partial (5/9) regression, with a mean of 73% regression in the PDT-treated areas. Tumor growth in the region of PDT was noted in 3 cases (3/9) (one tumor toward the foveola and two tumors at the margin away from the foveola), but in no case did the tumor reach the foveola. Therefore, PDT controlled tumor growth in 8 of 9 cases with only 1 of 9 cases showing growth through the PDT scar into foveola. Mean logarithm of the minimum angle of resolution visual acuity at last follow-up was 0.04 (Snellen ∼20/20) ( P = 0.59). Conclusion: Photodynamic therapy is an effective modality for the management of extrafoveolar choroidal osteoma, minimizing tumor growth toward the foveola and preserving visual acuity.

Purpose: To determine whether treatment order affects ophthalmic vascular event rates after intra‐arterial chemotherapy (IAC) for retinoblastoma. Methods: Patients who received IAC as primary or secondary treatment for retinoblastoma from January 2009 to January 2018 were included. All eyes were imaged with fundus photography and fluorescein angiography. Patient characteristics and vascular event rates were compared using t-test and Fisher's exact test. Results: There were 196 patients treated with 682 infusions of IAC, divided into primary (no previous therapy, 98 eyes of 98 patients, 328 infusions) and secondary (after other therapy, 105 eyes of 98 patients, 354 infusions) treatment. Overall, ophthalmic vascular events were found after 5% of infusions (17% eyes). A comparison of ophthalmic vascular events (primary vs. secondary IAC), with mean three infusions per eye (median 3, range 1–7), revealed no difference in overall percentage of eyes affected (18% vs. 15%, P = 0.57). Adverse vascular events per eye included retinal vasculature attenuation (1% vs. 0%, P = 0.99), peripheral retinal pruning (1% vs. 0%, P = 0.99), branch retinal artery occlusion (0% vs. 1%, P = 0.99), central retinal artery occlusion (0% vs. 1%, P = 0.99), macular ischemia (0% vs. 2%, P = 0.51), vitreous hemorrhage (2% vs. 3%, P = 0.92), subretinal hemorrhage (1% vs. 0%, P = 0.99), retinal pigment epithelium atrophy (6% vs. 3% P = 0.43), choroidal atrophy (4% vs. 2%, P = 0.92), optic disk pallor (1% vs. 0%, P = 0.99), and ophthalmic artery occlusion (9% vs. 6%, P = 0.35). Conclusion: Ophthalmic vascular events after IAC for retinoblastoma affect only 5% of eyes per infusion (17% of treated eyes). Vascular event risk per eye is similar when using IAC as primary or secondary treatment.