Optical Coherence Tomography of Small Retinoblastoma.

PURPOSE: To investigate hand-held optical coherence tomography (HH-OCT) characteristics of small (<1 mm thickness) retinoblastoma. DESIGN: Retrospective observational case series. METHODS: Patient and tumor data were extracted from the medical record and analyzed along with HH-OCT scans. Determination of tumor layer of origin was performed using a layer-by-layer analysis of HH-OCT data and specific HH-OCT-related features were described. RESULTS: There were 20 sub-millimeter retinoblastomas from 16 eyes of 15 patients. Mean largest tumor basal diameter by HH-OCT was 2.2 mm (median, 1.9; range, 0.7-4.1 mm), and mean tumor thickness was 468 µm (median, 441; range, 151-998 µm). In all cases, the retinoblastoma caused discontinuity or disruption of the inner nuclear (INL), outer plexiform (OPL), outer nuclear (ONL), and external limiting membrane (ELM) layers (20/20, 100%). Tumor origin was in the INL in 19/20 (95%) and equivocal (INL vs ONL) in 1/20 (5%). Intratumoral microcalcification was present in 14/20 tumors (70%). There were 2 characteristic findings (signs) on HH-OCT including the INL "fish tail" sign with splaying of the INL at the tumor margin (19/20, 95%) and the ONL "shark fin" sign with folding of the ONL and OPL, conforming to the lateral tumor margins (15/20, 75%). Both signs were concurrently present in 15 tumors (15/20, 75%). CONCLUSIONS: HH-OCT demonstrated that sub-millimeter retinoblastoma seems to originate from the INL, with tumor base and thickness growth progressing in a linear relationship. Characteristic HH-OCT findings included intratumoral microcalcification, INL "fish tail" sign, and ONL "shark fin" sign.

LARGE UVEAL MELANOMA (≥10 MM THICKNESS): Clinical Features and Millimeter-by-Millimeter Risk of Metastasis in 1311 Cases. The 2018 Albert E. Finley Lecture.

PURPOSE: To analyze the clinical features and rate of metastatic disease in eyes with large (≥10 mm thickness) uveal melanoma. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: There were 1,311 consecutive patients. METHODS: Retrospective medical chart review. MAIN OUTCOME MEASURES: Clinical features and rate of metastatic melanoma. RESULTS: Of 1,311 patients with large melanoma, the mean age was 59 years (median 60, range 6-98 years) and 95% were white. Mean tumor basal dimension was 17 mm (median 17, range 7-25 mm), and mean tumor thickness was 12 mm (median 12, range 10-24 mm). Mean distance to the foveola was 6 mm (median 6, range 0-19 mm) and to optic nerve was 6 mm (median 5, range 0-19 mm). Of all eyes, using Kaplan-Meier analysis, metastasis occurred in 11, 30, 45, and 52% at 1, 3, 5, and 7 years, respectively. According to tumor thickness (10.0-11.0, 11.1-12.0, 12.1-13.0, 13.1-14.0, 14.1-15.0, 15.1-16.0, and >16.0 mm), metastasis at 1 year was found in 7, 12, 13, 15, 18, 22, and 20%; metastasis at 3 years was 24, 27, 37, 35, 51, 69, and 57%; metastasis at 5 years was 38%, 42%, 56%, 48%, 61%, not available, and 66%; and metastasis at 7 years was 47%, 47%, 61%, 57%, 61%, not available, and 66%. Clinical features associated with fewer metastatic events included Bruch membrane rupture (7-year metastasis at 48%, P = 0.018) and macular location (7-year metastasis at 32%, P = 0.014), whereas those with worse outcome included extraocular extension (7-year metastasis at 79%, P < 0.001). There was no significant difference in rate of melanoma-related metastasis for patients treated with plaque radiotherapy versus enucleation. CONCLUSION: Large uveal melanoma demonstrates 7-year rate of metastasis at 52%, with generalized increasing risk per 1-mm or 2-mm thickness increments. Extraocular extension was associated with greater metastatic rate, whereas Bruch membrane rupture and macular location demonstrated lower rate.

CLINICALLY INVISIBLE RETINAL HEMANGIOBLASTOMAS DETECTED BY SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY AND FLUORESCEIN ANGIOGRAPHY IN TWINS.

PURPOSE: To report subclinical retinal hemangioblastoma detected by enhanced depth imaging optical coherence tomography and fluorescein angiography in at-risk twins. METHODS: Case report. RESULTS: A set of twins, age 7 years, (Twin A and Twin B) with known family history of von Hippel-Lindau disease (gene test positive) and no systemic manifestations were evaluated. Visual acuity was 20/20 in both eyes of both twins. Anterior segment examination and intraocular pressures were unremarkable in both eyes. Twin A showed no clinically visible tumor in the right eye, and a clinically evident 4-mm hemangioblastoma in the superior retina of the left eye. The enhanced depth imaging optical coherence tomography demonstrated normal fovea in both eyes. However, imaging at the inferonasal juxtapapillary region in the right eye documented an intraretinal mass from nerve fiber layer to outer plexiform layer on enhanced depth imaging optical coherence tomography and with hyperfluorescence on fluorescein angiography, consistent with retinal hemangioblastoma. Twin B demonstrated no clinically visible tumors in both eyes, but the left eye showed a small hyperreflective lesion in the parafoveal region on spectral domain optical coherence tomography from inner to outer nuclear layers, with no cystoid changes or subretinal fluid. The lesion was slightly hyperfluorescent on fluorescein angiography, consistent with hemangioblastoma. The optical coherence tomography angiography showed no vascularity within the lesion. Twin A was treated with laser photocoagulation to the larger hemangioblastoma in the left eye, and the asymptomatic juxtapapillary tumor was observed. Twin B was managed with cautious observation as treatment to the left eye could lead to vision loss. CONCLUSION: Patients at risk for retinal hemangioblastoma should have routine imaging with fundus photography, fluorescein angiography, and enhanced depth imaging optical coherence tomography for subclinical detection of asymptomatic tumors.

Optical coherence tomography angiography of iris microhemangiomatosis.

PURPOSE: To report optical coherence tomography angiography (OCTA) of iris microhemangiomatosis. OBSERVATIONS: A 75-year-old asymptomatic Caucasian man was found to have bilateral pupillary vascular lesions during cataract evaluation. Visual acuity was counting fingers in the right eye (OD) and 20/40 in the left eye (OS) with normal intraocular pressures in both eyes (OU). In each eye there were multifocal, round, dark red, pinpoint vascular tufts at the pupillary margin, randomly distributed and numbering 1 in OD and 7 in OS, each measuring 0.2-0.3 mm in diameter and without active bleeding or hyphema. Fundus examination OU was normal. By fluorescein angiography, the multifocal pupillary vascular tufts demonstrated mild staining without leakage. By OCTA, the tufts were clearly delineated and were fed by normal appearing radial iris vessels. OCT b-scan documented the optically dense vascular tufts at 0.1 mm in thickness and angio-overlay confirmed blood flow emanating from the deep iris stroma. Observation was recommended with the option of cataract surgery to improve vision. CONCLUSIONS AND IMPORTANCE: Non-invasive imaging of iris microhemangiomatosis with OCTA delineates the vascular lesion with flow arising from the posterior iris stroma.

Optical Coherence Tomography Angiography Features of Iris Racemose Hemangioma in 4 Cases.

Importance: Optical coherence tomography angiography (OCTA) allows visualization of iris racemose hemangioma course and its relation to the normal iris microvasculature. Objective: To describe OCTA features of iris racemose hemangioma. Design, Setting, and Participants: Descriptive, noncomparative case series at a tertiary referral center (Ocular Oncology Service of Wills Eye Hospital). Patients diagnosed with unilateral iris racemose hemangioma were included in the study. Main Outcomes and Measures: Features of iris racemose hemangioma on OCTA. Results: Four eyes of 4 patients with unilateral iris racemose hemangioma were included in the study. Mean patient age was 50 years, all patients were white, and Snellen visual acuity was 20/20 in each case. All eyes had sectoral iris racemose hemangioma without associated iris or ciliary body solid tumor on clinical examination and ultrasound biomicroscopy. By anterior segment OCT, the racemose hemangioma was partially visualized in all cases. By OCTA, the hemangioma was clearly visualized as a uniform large-caliber vascular tortuous loop with intense flow characteristics superimposed over small-caliber radial iris vessels against a background of low-signal iris stroma. The vascular course on OCTA resembled a light bulb filament (filament sign), arising from the peripheral iris (base of light bulb) and forming a tortuous loop on reaching its peak (midfilament) near the pupil (n = 3) or midzonal iris (n = 1), before returning to the peripheral iris (base of light bulb). Intravenous fluorescein angiography performed in 1 eye depicted the iris hemangioma; however, small-caliber radial iris vessels were more distinct on OCTA than intravenous fluorescein angiography. Conclusions and Relevance: Optical coherence tomography angiography is a noninvasive vascular imaging modality that clearly depicts the looping course of iris racemose hemangioma. Optical coherence tomography angiography depicted fine details of radial iris vessels, not distinct on intravenous fluorescein angiography.

Personalized Prognosis of Uveal Melanoma Based on Cytogenetic Profile in 1059 Patients over an 8-Year Period: The 2017 Harry S. Gradle Lecture.

PURPOSE: To determine the personalized rate of uveal melanoma-related metastasis on the basis of individual tumor cytogenetic profile. DESIGN: Retrospective case series. PARTICIPANTS: A total of 1059 patients with uveal melanoma. METHODS: Fine-needle aspiration biopsy (FNAB) for DNA amplification and whole genome array-based assay were performed for analysis of chromosomes 3, 6, and 8. MAIN OUTCOME MEASURES: Melanoma-related metastasis. RESULTS: The mean patient age was 57 years, and most were white (1026/1059, 97%). The melanoma involved the choroid (938/1059, 89%), ciliary body (85/1059, 8%), or iris (36/1059, 3%), with 19% being macular in location. The mean largest basal diameter was 11 mm (median, 12 mm; range, 3-24 mm), and mean thickness was 5 mm (median, 4 mm; range, 1-20 mm). On the basis of individual chromosomal mutations, risk for metastasis was increased for chromosome 3 partial monosomy (hazard ratio [HR], 2.84; P = 0.001), 3 complete monosomy (HR, 6.7, P < 0.001), 6q loss (HR, 3.1, P = 0.003), 8p loss (HR, 21.5, P < 0.001), and 8q gain (HR, 9.8, P < 0.001). Kaplan-Meier estimate for melanoma-related metastasis in 1, 3, 5, and 7 years for 3 partial monosomy was 1%, 5%, 14%, and 17%; for 3 complete monosomy was 3%, 19%, 28%, and 37%; for 6q loss was 8%, 23%, 49%, and 49%; for 8p loss was 8%, 29%, not estimable (NE), and NE; and for 8q gain was 6%, 21%, 35%, 48%, respectively. On the basis of personalized cytogenetic profiles, Kaplan-Meier estimates (1, 3, and 5 years) for melanoma-related metastasis for 3, 6, and 8 disomy (1%, 1%, 4% [HR, 1]) were low compared with the higher-risk combinations of 3 complete monosomy, 6p gain, and 8q gain (0%, 29%, 29% [HR, 10.6, P = 0.02]); 3 complete monosomy, 6 disomy, 8q gain, and 8p gain (14%, 14%, NE [HR, 18.3, P = 0.02]); 3 complete monosomy, 6 disomy, and 8q gain (8%, 27%, 39% [HR, 19.5, P < 0.001]); and 3 complete monosomy, 6 disomy, 8q gain, and 8p loss (3%, 28%, NE [HR, 31.6, P < 0.001]), respectively. CONCLUSIONS: Risk for melanoma-related metastasis strongly correlates with personalized cytogenetic profiles, with 5-year Kaplan-Meier estimates ranging from 4% with chromosomes 3, 6, and 8 disomy up to 39% for 3 complete monosomy, 6 disomy, and 8q gain.

Unrelenting Ocular Pain as a Masquerading Symptom of Occult Choroidal Metastasis.

PURPOSE: To report a case of chronic eye pain as a presenting feature of choroidal metastasis from lung cancer. METHODS: We report the case of a 58-year-old Caucasian woman with stage IV lung adenocarcinoma presenting with an 8-month history of left eye pain and blurred vision. RESULTS: The patient had previously consulted 14 ophthalmologists with varying diagnoses including posterior scleritis and trigeminal neuralgia. Visual acuity at presentation was 20/20 in the right eye and 20/80 in the left eye. Examination of the right eye was normal, while the left eye showed ill-defined flat yellow discoloration of the choroid with overlying shifting subretinal fluid. Ultrasonography demonstrated a dense choroidal thickening measuring 2.6 mm in size and showing subretinal fluid. Enhanced depth imaging optical coherence tomography revealed choroidal thickening with a 'lumpy bumpy' surface topography consistent with a metastatic choroidal tumor presumably from the patient's lung adenocarcinoma. Fine needle aspiration biopsy followed by treatment was recommended, but the patient declined and later succumbed to metastatic disease. CONCLUSION: We present a case of chronic eye pain associated with diffuse choroidal thickening from metastatic lung adenocarcinoma that was previously unrecognized and misdiagnosed. This case emphasizes the importance of recognizing pain as a presenting symptom of choroidal metastasis.

Stones, bones, groans, thrones, and psychiatric overtones: Systemic associations of sclerochoroidal calcification.

Sclerochoroidal calcification (SCC) is a frequent masquerader of choroidal melanoma with important systemic associations such as hyperparathyroidism and parathyroid adenoma. Herein, we describe a case of a 67-year-old male who presented with an amelanotic choroidal lesion in the right eye (OD) and a history of kidney stones. Ultrasonography showed the lesion to be flat and calcified OD. Incidentally, a subclinical calcified plaque was also found in the fellow eye. Optical coherence tomography showed an elevated suprachoroidal mass in a table mountain configuration OD and flat configuration left eye, consistent with type 4 and type 1 SCC. The patient was referred for metabolic testing to rule out the underlying electrolyte imbalance and was found to be normal.

Cytogenetic Abnormalities in Uveal Melanoma Based on Tumor Features and Size in 1059 Patients: The 2016 W. Richard Green Lecture.

PURPOSE: To determine the risks for altered cytogenetic profile based on melanoma features and size. DESIGN: Retrospective case series. PARTICIPANTS: A total of 1059 patients with uveal melanoma. METHODS: Fine-needle aspiration biopsy (FNAB) of tumor for DNA amplification and whole genome array-based assay. MAIN OUTCOME MEASURES: Risk for cytogenetic abnormalities based on features and size: small (≤3 mm thickness), medium (>3-<8 mm), and large (≥8 mm). RESULTS: Of 1059 patients with uveal melanoma sampled for status of chromosomes 3, 6, and 8, comparison (normal [disomy] chromosomes 3, 6, and 8 vs. any 3, 6, or 8 abnormality) revealed differences in mean age (55 vs. 58 years, P = 0.018), ocular melanocytosis (1% vs. 5%, P = 0.027), mean visual acuity (VA) (20/30 vs. 20/50, P = 0.011), poor VA (≤20/200) (9% vs. 15%, P = 0.041), ciliary body location (5% vs. 11%, P < 0.001), extramacular location (73% vs. 87%, P < 0.001), increased mean distance to optic disc (3.3 vs. 5.0 mm, P < 0.001) and foveola (3.1 vs. 4.7 mm, P < 0.001), and increased mean basal diameter (9.8 vs. 12.6 mm, P < 0.001) and thickness (3.8 vs. 5.9 mm, P < 0.001). Tumors classified as small, medium, and large showed abnormalities with loss of disomy of chromosomes 3 (35%/52%/65%), 6 (15%/34%/51%), and 8 (19%/41%/69%), respectively. By comparison (medium/large vs. small melanoma), the odds ratio (OR) included complete monosomy 3 (3.09, P < 0.001), partial monosomy 3 (1.44, P = 0.053), 6p gain (3.78, P < 0.001), 6q gain (1.37, P = 0.537), 6p loss (2.52, P = 0.410), 6q loss (12.61, P < 0.001), 8p gain (6.16, P < 0.001), 8p loss (6.04, P < 0.001), and 8q gain (4.87, P < 0.001). For chromosome 3 monosomy, the OR was highest for ciliary body location (8.17, P < 0.001), tumor thickness ≥8 mm (2.70, P < 0.001), tumor base ≥10 mm (2.59, P < 0.001), and age ≥60 years (1.83, P < 0.001). For chromosome 8p loss, the OR was highest for ciliary body location (53.91, P = 0.008), ocular melanocytosis (3.95, P = 0.038), and thickness ≥8 mm (5.14, P < 0.001), whereas for 8q gain, the OR was highest for ciliary body location (102.87, P = 0.001), thickness >8 mm (4.44, P < 0.001), and ocular melanocytosis (2.75, P = 0.049). CONCLUSIONS: Increasing melanoma size demonstrates greater cytogenetic alterations. Alterations in chromosome 8 show unique correlation with melanocytosis. This suggests that prompt management of small melanoma might reduce chromosomal instability and could improve overall patient survival.

Juvenile X-linked retinoschisis presenting as juxtapapillary retinal fold mimicking combined hamartoma of the retina and retinal pigment epithelium.

A 21-month-old boy presumptively diagnosed with combined hamartoma of the retina and retinal pigment epithelium was found to have juvenile X-linked retinoschisis with vitreomacular traction and prominent retinal folding.

Clinical Features Differentiating Benign From Malignant Conjunctival Tumors in Children.

IMPORTANCE: Conjunctival tumors in children are usually benign and rarely malignant. OBJECTIVE: To evaluate clinical features of conjunctival tumors in children by comparing benign tumors with their malignant counterparts. DESIGN, SETTING, AND PARTICIPANTS: This retrospective case series reviewed 806 cases of conjunctival tumor in children (aged <21 years) who were evaluated at a tertiary referral center between November 1, 1975, and July 1, 2015. This study included 262 children who were part of a published review. MAIN OUTCOMES AND MEASURES: Features of benign and malignant tumors were compared. Data were collected on patient demographics, tumor features, and specific diagnoses to determine findings related to each tumor. RESULTS: Among the 806 patients with conjunctival tumor, the top 5 diagnoses included nevus (492 [61%]), benign reactive lymphoid hyperplasia (BRLH) (38 [5%]), nodular conjunctivitis (31 [4%]), dermoid (30 [4%]), and primary acquired melanosis (27 [3%]). Overall, conjunctival tumors were benign (779 [97%]) or malignant (27 [3%]), including melanoma (18 [2.2%]) and lymphoma (9 [1.1%]). The mean age at detection was 11 years for benign tumors and 14 years for malignant tumors (P = .005), with mean difference of 3 years (95% CI, 1.2-4.6). The relative frequency of any malignancy (per all conjunctival tumors) by age bracket (0-5 years, >5-10 years, >10-15 years, and >15-<21 years) was 1%, 2%, 3%, and 7%, respectively. A comparison between nevus and melanoma found differences with melanoma in the 10 to 15 years age bracket (29% vs 61%; difference of 32% [95% CI, 10%-55%]; P = .006), mean tumor thickness (1.1 mm vs 1.5 mm; difference of 0.4 mm [95% CI, -0.29 mm to 1.12 mm]; P = .04), tumor base of 10 mm or greater (relative risk [RR] = 4.92; 95% CI, 1.73-13.97; P = .003), tumor hemorrhage (RR = 25.30; 95% CI, 11.91-53.78; P < .001), and lack of intrinsic cysts (RR = 5.06; 95% CI, 1.84-13.98; P = .002). A comparison between BRLH and lymphoma revealed lymphoma with a larger base (RR = 5.16; 95% CI, 1.19- 22.19; P = .002) and diffuse location (RR = 16.50; 95% CI, 4.31-63.22; P < .001) and inferior (RR = 12.38; 95% CI, 2.88-53.16; P < .001) or superior vs nasal (RR = 8.25; 95% CI, 1.56-43.51; P = .01). The small cohort of malignant lesions precluded determining if these features were independent of one another. CONCLUSIONS AND RELEVANCE: These data, from an ocular tertiary referral center, suggest that conjunctival tumors in children are nearly always benign. The few malignant tumors included melanoma and lymphoma. Melanoma, compared with nevus, was associated with older children (aged >10-15 years) with larger tumor, hemorrhage, and lack of cyst. Lymphoma, compared with BRLH, was associated with larger size and diffuse involvement.

VARIABILITY IN FOVEAL AVASCULAR ZONE AND CAPILLARY DENSITY USING OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY MACHINES IN HEALTHY EYES.

PURPOSE: To evaluate the variability in foveal avascular zone (FAZ) and capillary density measurements on optical coherence tomography angiography using Optovue RTVue XR Avanti (OA) (Optovue) and Zeiss Cirrus HD-OCT 5000 (ZC) (Carl Zeiss Meditec). METHODS: In this prospective, comparative case series, parafoveal (3 × 3 mm) optical coherence tomography angiography scans were obtained on healthy volunteers using both the Avanti and Cirrus. The FAZ area and capillary density at the level of both the superficial and deep capillary plexus were measured automatically using the built-in ReVue software (Optovue) with the Avanti as well as manually using ImageJ (National Institutes of Health) with both machines. RESULTS: There were 50 eyes in 25 healthy volunteers included in the analysis. Mean subject age was 33 years and there were 14 women (56%). On optical coherence tomography, mean central macular thickness was significantly greater on OA (259.1 µm) than ZC (257.6 µm, P = 0.0228). On optical coherence tomography angiography, mean superficial and deep plexus FAZ measured 0.2855 mm and 0.3465 mm on Avanti automated (A-A), 0.2739 mm and 0.3637 mm on Avanti manual (A-M), and 0.2657 mm and 0.3993 mm on Cirrus manual (C-M), respectively. There were no statistically significant differences in superficial plexus FAZ measurements between the A-A and A-M (P = 0.4019) or A-A and C-M (P = 0.1336). The A-M measured significantly larger than C-M (P = 0.0396). Deep plexus FAZ measurements were similar on A-A and A-M (P = 0.6299), but both were significantly less compared with C-M (P < 0.0001 for A-A vs. C-M, P = 0.0184 for A-M vs. C-M). Mean superficial and deep plexus capillary densities were 53.6% and 59.3% on A-A, 48.1% and 47.7% on A-M, and 52.5% and 48.1% on C-M, respectively. Superficial plexus capillary density measurements were statistically similar on A-A and C-M (P = 0.0623), but both were significantly higher than A-M (P < 0.0001 for A-A vs. A-M, P < 0.0001 for A-M vs. C-M). However, deep plexus capillary density measurements on A-A were significantly higher than A-M (P < 0.0001) and C-M (P < 0.0001), but A-M and C-M measurements were similar (P = 0.5986). There was no significant difference in all parameters measured in both eyes of one subject using any of the three measuring techniques. CONCLUSION: While measurements taken with the same machine and technique are consistent and reliable between fellow eyes, significant variability exists in FAZ and capillary density measurements among different machines and techniques. Comparison of measurements across machines and techniques should be considered with caution.

OPTIC NERVE MENINGOCELE SIMULATING EXTRAOCULAR EXTENSION OF CHOROIDAL MELANOMA.

PURPOSE: To report a case of optic nerve meningocele simulating massive, recurrent extraocular extension of choroidal melanoma. METHOD: Case report. RESULTS: A 53-year-old white man with choroidal melanoma in his left eye of 7.3-mm thickness was treated with plaque radiotherapy and transpupillary thermotherapy. On 1-year follow-up examination, visual acuity was 20/20 in the right eye and 20/30 in the left eye. The regressed choroidal melanoma scar in the left eye measured 1.5 mm in thickness with stable margins. The optic disk was normal. Ultrasonography demonstrated regressed echogenic choroidal scar, with an echolucent multilobulated retrobulbar mass, suspicious for extraocular extension. On magnetic resonance imaging, the retrobulbar mass corresponded to a distended and kinked optic nerve sheath, filled with extensive subarachnoid fluid and normal-size optic nerve with apposition against the posterior globe. There was no extraocular extension of tumor. Similar but less distended right optic nerve sheath was documented, consistent with optic nerve sheath meningocele in both eyes. Observation was advised and the findings remained stable. CONCLUSION: Optic nerve sheath meningocele is a benign dilatation of the optic nerve sheath that can simulate orbital tumor or extraocular extension of intraocular tumor. Magnetic resonance imaging can reliably differentiate these conditions.

Role of pars plana vitrectomy and membrane peel in vitreomacular traction associated with retinal vasoproliferative tumors.

To report a case of retinal vasoproliferative tumor (VPT) with secondary epiretinal membrane (ERM) formation and vitreo-macular traction managed by pars plana vitrectomy (PPV) and membrane peel. A 29-year-old male was referred for management of decreased vision in the right eye (OD) for 1 week. Presenting visual acuity was 20/50 Snellen feet (ft) OD, and fundus examination showed an ERM associated with a reddish-yellow mass in the inferotemporal quadrant with overlying exudation, hemorrhage, and subretinal fluid consistent with VPT, and cryotherapy was recommended. Two months later, there was complete tumor regression, but there was decreased vision from progressive vitreomacular traction to 20/400 ft. PPV with combined ERM and internal limiting membrane (ILM) peel were performed with resolution of vitreomacular traction and improvement of visual acuity to 20/50 ft at 6 months. PPV with combined ERM and ILM peel is effective for vision loss secondary to ERM and vitreomacular traction associated with retinal VPT.

Retinoblastoma Control With Primary Intra-arterial Chemotherapy: Outcomes Before and During the Intravitreal Chemotherapy Era.

PURPOSE: To compare outcomes of intra-arterial chemotherapy for retinoblastoma as primary therapy before (Era I) and during (Era II) the intravitreal chemotherapy era. METHODS: In this retrospective interventional case series at a tertiary referral center, 66 eyes of 66 patients with untreated unilateral retinoblastoma were used. intraarterial chemotherapy into the ophthalmic artery under fluoroscopic guidance was performed using melphalan in every case, with additional topotecan as necessary. Intravitreal chemotherapy using melphalan and/or topotecan was employed as needed for active vitreous seeding. Globe salvage was measured based on the International Classification of Retinoblastoma (ICRB) during two eras. RESULTS: The two eras encompassed 2008 to 2012 (intraarterial chemotherapy alone, Era I) and 2012 to 2015 (intraarterial chemotherapy plus intravitreal chemotherapy, Era II). Over this period, there were 66 patients with unilateral untreated retinoblastoma treated with primary intra-arterial chemotherapy. A comparison of features (Era I vs Era II) revealed no significant difference in mean patient age (24 vs 24 months), ICRB groups, mean largest tumor diameter (19 vs 17 mm), mean largest tumor thickness (10 vs 10 mm), vitreous seed presence (56% vs 59%), subretinal seed presence (67% vs 62%), retinal detachment (70% vs 66%), or vitreous hemorrhage (0% vs 5%). There was no significant difference in mean number of intra-arterial chemotherapy cycles (3 vs 3.1) or intraarterial chemotherapy dosages. Following therapy, there was a significant difference (Era I vs Era II) in the need for enucleation overall (44% vs 15%, P = .012), especially for group E eyes (75% vs 27%, P = .039). Four of the eyes that initiated therapy in Era I later required intravitreal chemotherapy during Era II. The enucleation rate was 0% for groups B and C in both eras and non-significant for group D (23% vs 13%). There were no patients with stroke, seizure, limb ischemia, extraocular tumor extension, secondary leukemia, metastasis, or death. CONCLUSIONS: The current era of retinoblastoma management using intra-arterial chemotherapy plus additional intravitreal chemotherapy (as needed for vitreous seeding) has improved globe salvage in eyes with advanced retinoblastoma. [J Pediatr Ophthalmol Strabismus. 2016;53(5):275-284.].