Faculty Bibliography

The purpose of this study was to report on a case of Terson's syndrome (TS) using a novel instrument and technique to harvest posterior pole pathology from a postmortem eye. A modified ocular clamp was used to remove the posterior pole from the postmortem enucleated eye. Gross photographs were taken and an ocular sample of the posterior pole was sent to The New York Eye and Ear Pathology Laboratory. TS was identified from gross pathology and histologic examinations. The case history was consistent with that diagnosis. The authors concluded that high-quality gross and histopathologic examination of the posterior pole can be obtained with this novel instrument and technique. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:170-174.].

PURPOSE: To describe a minimally invasive method to create multifocal full-thickness surgical iridectomy biopsies. METHODS: Seven patients suspected to have diffuse iris melanoma underwent multifocal surgical iridectomy biopsy through a single 1.0-mm clear corneal incision. A 25-G inked trocar was used to create one visible corneal portal, through which the anterior chamber was filled with sodium hyaluronate 1%. Then, a 25-G aspiration cutter probe was introduced through the corneal incision such that the aspiration portal was occluded by the iris biopsy sites. Then aspiration (600 mm Hg) cutting (300 cuts per minute) was used to create full- and partial-thickness surgical iridectomy biopsies at multiple locations. After each biopsy the probe was removed, and its contents aspirated into a separate 3-mL syringe (marked by clock hour location). The sodium hyaluronate 1% was removed and wound checked for leakage. RESULTS: Diagnostic specimens were obtained in all cases. Five were found to be diffuse iris melanomas and 2 were indeterminate iris melanocytic proliferations. No patients developed a secondary increase in intraocular pressure, hyphema, infection, cataract, or vision loss. No cases of intraocular tumor dissemination were observed. No patient complained of glare or monocular diplopia. Three of 7 corneal wounds were self-sealing. CONCLUSIONS: Multifocal surgical iridectomy biopsy can be minimally invasive and effective allowing for partial- and full-thickness iris biopsy. Use of a 25-G aspiration cutting probe allowed for small incision surgery, rapid rehabilitation, and no significant complications.

PURPOSE: To report a case of neovascular and angle closure glaucoma secondary to breast cancer metastatic to the iris that was successfully treated with injections of intravitreal bevacizumab (Avastin) 1.25 mg/0.05 mL. METHODS: Case report. PATIENTS: A 47-year-old woman with metastatic breast cancer presented to The New York Eye Cancer Center with left ocular pain, photosensitivity, vision loss, and multiple iris nodules. Her intraocular pressure was uncontrolled. Gonioscopy revealed neovascularization of the iris and angle; no choroidal neovascularization was noted. Ultrasound biomicroscopy demonstrated tumor invasion of iris stroma with marked anterior uveal thickening and narrowed angles. RESULTS: Three monthly injections of intravitreal bevacizumab resulted in nearly complete resolution of iris neovascularization, reduction of intraocular pressure, and control of tumor (although a small amount of residual tumor remained). CONCLUSION: Intravitreal anti-vascular endothelial growth factor therapy for breast cancer metastatic to the iris with secondary neovascular glaucoma provided good local control for a limited follow-up period, because the patient died because of systemic complications of her disease.

PURPOSE: To describe an unusual case of pediatric iris melanocytoma with pigment dispersion glaucoma that resolved after resection of the primary tumor. METHODS: Retrospective case review of the clinical record, ultrasonographic images, and histopathology. RESULTS: A 6-year-old Asian girl, with a dark iris tumor, pigment dispersion, and secondary glaucoma, was initially treated with topical antiglaucoma medication and observation. Rapid growth prompted biopsy, revealing melanocytoma. As the tumor continued to grow, excision of the primary tumor was performed. Surgery proved curative in that the pigment dispersion slowly reabsorbed and her glaucoma resolved. CONCLUSIONS: In this case, rapid growth did not indicate malignant transformation. Initial observation for growth and judiciously timed surgical intervention prevented progression, loss of vision, and potentially the loss of the eye.

Importance: To date, the clinical features of the various subtypes of conjunctival lymphoma (CL) have not been previously evaluated in a large cohort. Objective: To characterize subtype-specific clinical features of CL and their effect on patient outcome. Design, Setting, and Participants: A retrospective multicenter study was performed. Patient data were collected from January 1, 1980, through December 31, 2010. The dates of the analysis were May 15, 2015, to August 20, 2015. The median follow-up period was 43 months. Seven eye cancer centers were involved in the study. In total, 268 patients with CL were identified, 5 of whom were excluded because of missing clinical data. Main Outcomes and Measures: Overall survival, disease-specific survival, and progression-free survival were the primary end points. Results: Two hundred sixty-three patients with CL were included in the study. Their mean age was 61.3 years, and 55.1% (145 of 263) were female. All lymphomas were of B-cell type. The most frequent subtype was extranodal marginal zone lymphoma (EMZL) (68.4% [180 of 263]), followed by follicular lymphoma (FL) (16.3% [43 of 263]), mantle cell lymphoma (MCL) (6.8% [18 of 263]), and diffuse large B-cell lymphoma (DLBCL) (4.6% [12 of 263). Conjunctival lymphoma commonly manifested in elderly individuals (age range, 60-70 years old), with EMZL having a female predilection (57.8% [104 of 180]) and MCL having a marked male predominance (77.8% [14 of 18]). Unlike EMZL and FL, DLBCL and MCL were frequently secondary diseases (41.7% [5 of 12] and 88.9% [16 of 18], respectively), with MCL showing a frequent occurrence of stage IVE lymphoma (61.1% [11 of 18]) and bilateral manifestation (77.8% [14 of 18]). Localized disease (stage IE or IIE) was commonly treated with external beam radiation therapy (EBRT) with or without chemotherapy, while widespread lymphoma (stage IIIE or IVE) and MCL of any stage were managed with chemotherapy with or without EBRT. Diffuse large B-cell lymphoma and MCL had a poor prognosis, with 5-year disease-specific survival of 55.0% and 9.0%, respectively, in contrast to EMZL (97.0%) and FL (82.0%). Further survival predictors included age (EMZL), sex (FL), and Ann Arbor staging classification (EMZL and FL). The American Joint Committee on Cancer TNM staging showed limited prognostic usefulness, only being able to predict survival for patients with DLBCL. Conclusions and Relevance: Conjunctival lymphoma consists of mainly 4 subtypes of B-cell non-Hodgkin lymphoma: EMZL, FL, MCL, and DLBCL. Mantle cell lymphoma is characterized by a particularly high frequency of secondary disease of stage IVE and bilateral manifestation. The histological subtype is the main outcome predictor, with MCL and DLBCL having a markedly poorer prognosis than EMZL and FL.

Purpose: To assess of the effect of local recurrence of uveal melanoma on metastasis using a multicenter international tumor registry. Design: Retrospective study using an online tumor registry. Participants: Patients with uveal melanoma diagnosed between 2001 and 2011. Methods: A committee was formed to create uveal melanoma patient-specific data fields. Ten subspecialty ophthalmic oncology centers from 4 continents shared data. Patient selection criteria included diagnosis of uveal melanoma and adequate records to allow tumor staging by American Joint Committee on Cancer (AJCC) criteria and follow-up for metastatic melanoma. Main Outcome Measures: Local tumor recurrence and metastatic uveal melanoma. Results: Of 3809 total entries, 3217 patients with ciliary body and choroidal (CBC) melanoma and 160 with iris melanoma were evaluated. There was a median follow-up of 3.7 years (95% confidence interval [CI], 3.5-3.8). One hundred fifty-two patients (4.7%) with CBC melanoma experienced local recurrence, with a cumulative incidence of 11%. Kaplan-Meier point estimates for remaining free of local recurrence were 99% (95% CI, 99-99) at 1 year, 93% (95% CI, 92-94) at 5 years, and 89% (95% CI, 86-91) at 10 years. Five-and 10-year metastasis-free Kaplan-Meier estimates for the recurrence-free group were 87% (95% CI, 86-89) and 82% (95% CI, 79-84), and those for the local recurrence group were 71% (95% CI, 62-78) and 62% (95% CI, 49-72). The difference between these 2 groups was statistically significant (P < 0.001). Furthermore, local tumor recurrence increased the risk of metastasis by a hazard ratio (HR) of 6.28 (95% CI, 4.4-8.9; P < 0.001). Local recurrence was detected up to 9.8 years after treatment. Extrascleral extension also was associated with local recurrence (HR, 3.2; 95% CI, 1.5-6.7; P = 0.003), but higher AJCC T-size category was not (P = 0.63). Five patients (n = 5/161 [3.1%]) with iris melanoma demonstrated local recurrence and 1 metastasized. Conclusions: International multicenter data sharing was used to evaluate the effect of local tumor recurrence on metastatic rate. In that local tumor recurrence was associated with a significantly higher risk of systemic metastasis, effective initial treatment and long-term surveillance of treated uveal melanoma patients is necessary. (C) 2016 by the American Academy of Ophthalmology.

PURPOSE: To report long-term experience with intravitreal anti-vascular endothelial growth factor treatment for radiation maculopathy. METHODS: From 2005-2015, 120 consecutive patients underwent intravitreal anti-VEGF therapy for radiation maculopathy. Inclusion criteria included a diagnosis of uveal melanoma treated with plaque radiotherapy and subsequent macular radiation vasculopathy (exudate, retinal hemorrhage, intraretinal microangiopathy, neovascularization, edema). Anti-VEGF therapy involved continuous injections in 4- to 12-week intervals with doses of 1.25 mg/0.05 mL, 2.0 mg/0.08 mL, 2.5 mg/0.1 mL, or 3.0 mg/0.12 mL of bevacizumab as well as 0.5 mg/0.05 mL or 2.0 mg/0.05 mL of ranibizumab. Goals were maintenance of visual acuity and normative macular anatomy. Safety and tolerability (retinal detachment, hemorrhage, infection), visual acuity, central foveal thickness on optical coherence tomography imaging, and clinical features of radiation maculopathy were analyzed. RESULTS: Progressive reductions in macular edema, hemorrhages, exudates, cotton-wool spots, and microangiopathy were noted. At last follow-up, 80% remained within 2 lines of their initial visual acuity or better, with a mean treatment interval of 38 months (range 6-108 months). Kaplan-Meier analysis of the probability of remaining within 2 lines of initial visual acuity was 69% at 5 years and 38% at 8 years of anti-VEGF therapy. Discontinuation of therapy was rare. Relatively few acute or long-term side effects were noted, allowing for good long-term patient accrual. CONCLUSIONS: Continuous intravitreal anti-VEGF therapy in patients with radiation maculopathy was well-tolerated and preserved vision. In most cases, reductions or resolution of retinal hemorrhages, cotton-wool spots, and retinal edema were noted for up to 10 years.

PURPOSE: Cross-characterization of retinal astrocytomas (RA) using ocular diagnostic imaging techniques. METHODS: Fundus photography, fluorescein angiography, optical coherence tomography, fundus autofluorescence images, and ultrasonography were used to define characteristics of RA. RESULTS: Thirteen patients had sporadic RA and 12 had tuberous sclerosis. Of the latter, 1 patient had multiple (n = 14), bilateral tumors and was excluded so as not to skew the analysis. Of those patients with a solitary RA, 63% were juxtapapillary. All were white to yellow. A mulberry-shaped surface was observed in 33%. Fluorescein angiography revealed intrinsic tumor vessels and late diffuse tumor staining in 42%. Optical coherence tomography revealed cells spreading from the tumor surface into the vitreous (75%), intraretinal fluid (25%), and multiple, ovoid hypolucencies within the tumor stroma (54%). Fundus autofluorescent imaging revealed that hypolucencies were hyperautofluorescent and solid tumor stroma appeared hypoautofluorescent. B-scan imaging revealed moderate to high internal tumor reflectivity (100%) with orbital shadowing (46%). Optical coherence tomography revealed a mean tumor thickness of 0.8 mm. Ultrasound imaging showed a mean thickness of 1.4 mm (range 1.0-1.7). Of the 21 patients subsequently followed in our centers, no tumor growth was noted (mean 54 months, range 1-96). CONCLUSIONS: This study reveals unique characteristics of RA and suggests that RA were most commonly amelanotic, moderate to high echoic,