The role of oral steroids in the treatment of photodynamic therapy-associated exudative maculopathy, a case report and review of the literature.

BACKGROUND: Photodynamic therapy (PDT) is an effective treatment of pachychoroid spectrum disease. PDT can cause a rare complication known as PDT-associated exudative maculopathy (PAEM). Treatments including intravitreal anti-vascular endothelial growth factor (anti-VEGF) medications, local or systemic steroids, and observation have been attempted with variable success to address this complication. METHODS: A thorough literature review was performed using the PubMed database on search terms aimed at treatments of PAEM. These cases were compared with each other and a novel case of PAEM in polypoidal choroidal vasculopathy (PCV) treated with oral prednisone by the authors. RESULTS: Fifteen patients were compared; 11 were treated with anti-VEGF alone or in combination with intravitreal steroid and/or vitrectomy, one was treated with topical steroid, one was observed, one was treated with intravenous methylprednisolone, and one was treated with oral prednisone. The two cases treated with systemic steroids were given adjunctive sub-tenon's triamcinolone acetonide (STTA) after a favorable response was observed. Most cases had anatomic resolution of serous retinal detachment with stability of vision between 16 days and 2 months, with the most rapid resolution occurring in a patient with PCV treated with oral prednisone and STTA. CONCLUSIONS: Reported treatment of PAEM includes intravitreal anti-VEGF agents with or without local or systemic steroids. Oral steroids may be advantageous in cases where there is concern regarding the risk profile of periocular steroids, intravitreal steroids or anti-VEGF agents. However, data describing the various treatments of this rare complication is limited, precluding firm conclusions regarding relative safety and efficacy.

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.

Conjunctival Tumors in 5002 Cases. Comparative Analysis of Benign Versus Malignant Counterparts. The 2016 James D. Allen Lecture.

PURPOSE: To evaluate frequency of conjunctival tumors in all ages and compare benign vs malignant counterparts. DESIGN: Retrospective series. METHODS: setting: Tertiary referral center. STUDY POPULATION: Total of 5002 patients. OBSERVATION: Clinical features. MAIN OUTCOME MEASURE: Differentiation of benign from malignant counterparts. RESULTS: The tumor was benign (52%), premalignant (18%), or malignant (30%). Malignant tumors included melanoma (12%), squamous cell carcinoma (SCC) (9%), lymphoma (7%), and others. Comparison of primary acquired melanosis vs melanoma revealed melanoma with greater median patient age (54 vs 61 years, P < .0001), male sex (35% vs 49%, P < .0001), location in fornix (2% vs 6%, P = .0016) and tarsus (1% vs 4%, P = .0018), larger median basal diameter (6 vs 8 mm, P < .0001) and thickness (<1 vs 1 mm, P < .0001), and intralesional cysts (0% vs 7%, P < .0001), feeder vessels (10% vs 48%, P < .0001), intrinsic vessels (4% vs 33%, P < .0001), and hemorrhage (<1% vs 3%, P = .0001). Comparison of conjunctival intraepithelial neoplasia (CIN) vs SCC revealed SCC with greater diffuse involvement (1% vs 8%, P < .0001) and larger median basal diameter (7 vs 8 mm, P < .0001) and thickness (1 mm vs 2 mm, P < .0001). Comparison of benign reactive lymphoid hyperplasia vs lymphoma revealed lymphoma with greater median patient age (50 vs 61 years, P < .0001), fornix location (32% vs 54%, P < .0001), larger median basal diameter (10 vs 20 mm, P < .0001), and less involvement of nasal region (50% vs 23%, P < .0001). CONCLUSION: In an ocular oncology practice, conjunctival tumors are benign (52%), premalignant (18%), or malignant (30%). Malignant tumors tend to occur in older patients and demonstrate greater basal diameter and thickness, compared with benign counterparts.

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.].

Unilateral Retinoblastoma Managed With Intravenous Chemotherapy Versus Intra-Arterial Chemotherapy. Outcomes Based on the International Classification of Retinoblastoma.

PURPOSE: The objective of this study was to compare outcomes after intravenous chemotherapy (IVC) versus intra-arterial chemotherapy (IAC) for unilateral retinoblastoma. DESIGN: A retrospective comparative interventional case series. METHODS: Patients with unilateral retinoblastoma managed with either IVC using vincristine, etoposide, and carboplatin or IAC using melphalan with or without topotecan with a minimum of 1-year follow-up were compared. The primary outcome measure was globe salvage. RESULTS: Of 91 patients with unilateral retinoblastoma, IVC was employed in 42 (46%) cases and IAC in 49 (54%). By comparison (IVC vs IAC), patients in the IAC group had greater mean tumor diameter (14 vs 18 mm, P < 0.001) and thickness (7 vs 10 mm, P = 0.001), greater percentage with active vitreous seeds (29% vs 55%, P = 0.01), and greater total retinal detachment (10% vs 43%, P < 0.001). There were no cases of group A in either treatment arm. Globe salvage was not significantly different in groups B, C, or E, but there was significantly improved globe salvage with IAC for group D (48% vs 91%, P = 0.004). Control was significantly better with IAC for solid tumor (62% vs 92%, P = 0.002), subretinal seeds (31% vs 86%, P = 0.006), and vitreous seeds (25% vs 74%, P = 0.006). There were no patients with pinealoblastoma, second cancer, metastasis, or death in either group. CONCLUSIONS: For unilateral retinoblastoma, IAC provided significantly superior globe salvage compared with IVC for group D eyes. In addition, IAC provided significantly superior control for solid tumor, subretinal seeds, and vitreous seeds.