Sruthi Arepalli

Intra-arterial chemotherapy for retinoblastoma in 70 eyes: outcomes based on the international classification of retinoblastoma.

OBJECTIVE To analyze our 5-year experience of intra-arterial chemotherapy (IAC) for retinoblastoma as primary or secondary therapy. DESIGN Retrospective interventional case series. PARTICIPANTS A total of 70 eyes of 67 patients. INTERVENTION Ophthalmic artery chemotherapy infusion under fluoroscopic guidance was performed using melphalan (3, 5, or 7.5 mg) in every case, with additional topotecan (1 mg) and/or carboplatin (30 or 50 mg) as necessary. MAIN OUTCOME MEASURES Tumor control and treatment complications. RESULTS The mean patient age at IAC was 30 months. The treatment was primary in 36 eyes and secondary in 34 eyes. Those primary therapy eyes were classified according to the International Classification of Retinoblastoma (ICRB) as group A (n = 0), B (n = 1), C (n = 4), D (n = 17), or E (n = 14). The secondary therapy eyes had failed previous intravenous chemotherapy (n = 34) in every case. Each eye received a mean of 3 IAC sessions per eye (median, 3; range, 1-7 sessions). After IAC with a mean follow-up of 19 months, globe salvage was achieved in 72% of primary-treated cases and in 62% of secondary-treated cases. Specifically, primary therapy achieved globe salvage for group B (100%), group C (100%), group D (94%), and group E (36%). Of all 70 eyes, complete regression was achieved for solid tumor in 48 of 51 eyes (94%), subretinal seeds in 40 of 42 eyes (95%), and vitreous seeds in 34 of 39 eyes (87%). After each catheterization (n = 198), the main complications included transient eyelid edema (5%), blepharoptosis (5%), and forehead hyperemia (2%). More lasting complications included vitreous hemorrhage (2%), branch retinal artery obstruction (1%), ophthalmic artery spasm with reperfusion (2%), ophthalmic artery obstruction (2%), partial choroidal ischemia (2%), and optic neuropathy (<1%). Over the past 3 years, the combined incidence of ophthalmic, retinal, and choroidal vascular ischemia was reduced to 1%. There was no patient with stroke, seizure, neurologic impairment, limb ischemia, secondary leukemia, metastasis, or death. CONCLUSIONS Five-year experience with IAC indicates that this technique is remarkably effective for the management of retinoblastoma as both a primary and a secondary treatment.

Original articleIntra-arterial Chemotherapy for Retinoblastoma in 70 Eyes: Outcomes Based on the International Classification of Retinoblastoma

OBJECTIVE To analyze our 5-year experience of intra-arterial chemotherapy (IAC) for retinoblastoma as primary or secondary therapy. DESIGN Retrospective interventional case series. PARTICIPANTS A total of 70 eyes of 67 patients. INTERVENTION Ophthalmic artery chemotherapy infusion under fluoroscopic guidance was performed using melphalan (3, 5, or 7.5 mg) in every case, with additional topotecan (1 mg) and/or carboplatin (30 or 50 mg) as necessary. MAIN OUTCOME MEASURES Tumor control and treatment complications. RESULTS The mean patient age at IAC was 30 months. The treatment was primary in 36 eyes and secondary in 34 eyes. Those primary therapy eyes were classified according to the International Classification of Retinoblastoma (ICRB) as group A (n = 0), B (n = 1), C (n = 4), D (n = 17), or E (n = 14). The secondary therapy eyes had failed previous intravenous chemotherapy (n = 34) in every case. Each eye received a mean of 3 IAC sessions per eye (median, 3; range, 1-7 sessions). After IAC with a mean follow-up of 19 months, globe salvage was achieved in 72% of primary-treated cases and in 62% of secondary-treated cases. Specifically, primary therapy achieved globe salvage for group B (100%), group C (100%), group D (94%), and group E (36%). Of all 70 eyes, complete regression was achieved for solid tumor in 48 of 51 eyes (94%), subretinal seeds in 40 of 42 eyes (95%), and vitreous seeds in 34 of 39 eyes (87%). After each catheterization (n = 198), the main complications included transient eyelid edema (5%), blepharoptosis (5%), and forehead hyperemia (2%). More lasting complications included vitreous hemorrhage (2%), branch retinal artery obstruction (1%), ophthalmic artery spasm with reperfusion (2%), ophthalmic artery obstruction (2%), partial choroidal ischemia (2%), and optic neuropathy (<1%). Over the past 3 years, the combined incidence of ophthalmic, retinal, and choroidal vascular ischemia was reduced to 1%. There was no patient with stroke, seizure, neurologic impairment, limb ischemia, secondary leukemia, metastasis, or death. CONCLUSIONS Five-year experience with IAC indicates that this technique is remarkably effective for the management of retinoblastoma as both a primary and a secondary treatment.

Choroidal metastases: origin, features, and therapy.

The choroid is the most common ocular site for metastatic disease, owing to abundant vascular supply. The primary cancers that most commonly lead to choroidal metastases include breast cancer (40-47%) and lung cancer (21-29%). Bilateral, multifocal metastases are most often secondary to breast cancer, whereas unilateral, unifocal metastasis are more commonly found with lung cancer. The treatment of choroidal metastasis depends on the systemic status of the patient and number, location, and laterality of the choroidal tumors. Treatment options include observation in patients with poor systemic status or those with resolved or asymptomatic disease; systemic chemotherapy, immunotherapy, hormone therapy, or whole eye radiotherapy if the metastases are active, multifocal and bilateral; plaque radiotherapy, transpupillary radiotherapy, or photodynamic therapy for active, solitary metastasis; and enucleation for those with blind painful eye. A database search was performed on PubMed, using the terms “choroidal metastasis,” or “choroidal metastases,” in combination with terms such as “treatment,” “features,” or “diagnosis.” Relevant articles were extracted and reviewed.

Combined hamartoma of the retina and retinal pigment epithelium: findings on enhanced depth imaging optical coherence tomography in eight eyes.

Purpose: To assess combined hamartoma of the retina and retinal pigment epithelium with enhanced depth imaging optical coherence tomography. Methods: Retrospective, observational cases series in eight eyes of eight patients, with comparison between affected and unaffected eyes regarding enhanced depth imaging optical coherence tomography features of tumor, fovea, and choroid. Results: The mean age at presentation was 7 years. The tumor was macular (n = 5) or extramacular (n = 3). Enhanced depth imaging optical coherence tomography revealed irregularities in inner retina (n = 8) and/or all retinal layers (n = 3), with epiretinal membrane (n = 8), causing an inner retinal sawtooth (mini-peak) pattern (n = 2), full thickness retinal folds (maxi-peak) (n = 3), or both (n = 3). In the 5 macular tumors, foveal retinal thickness measured mean 608 &mgr;m compared with 244 &mgr;m in the unaffected eye (P = 0.0004). Mean tumor epicenter retinal thickness in 8 tumors measured 650 &mgr;m compared with 327 &mgr;m in a corresponding area in the unaffected eye (P = 0.01). In all cases, choroidal thickness beneath the tumor epicenter was decreased at mean 210 &mgr;m compared with 328 &mgr;m in the corresponding area of unaffected eye (P = 0.009). Conclusion: Enhanced depth imaging optical coherence tomography of combined hamartoma revealed epiretinal membrane with vitreoretinal traction in a sawtooth (mini-peak) or folded (maxi-peak) pattern. Combined hamartoma seems to be a thickened retinal mass secondary to focal vitreoretinal traction.

Choroidal lymphoma shows calm, rippled, or undulating topography on enhanced depth imaging optical coherence tomography in 14 eyes.

Purpose: To describe the features of choroidal lymphoma on enhanced depth imaging optical coherence tomography. Methods: This retrospective observational case series included 14 eyes of 13 patients, with choroidal lymphoma, studied by enhanced depth imaging optical coherence tomography. Results: The mean age at presentation was 63 years (median, 65 years; range, 32–87 years). Systemic lymphoproliferative disease was present in 2 cases as non-Hodgkin lymphoma (n = 1) or Waldenstrom macroglobulinemia (n = 1). On clinical examination, the choroidal infiltrate was classified as unifocal (n = 3), multifocal (n = 4), or diffuse (n = 7). Enhanced depth imaging optical coherence tomography scans through the tumor epicenter revealed infiltration of the choroid with apparent inward compression of choroidal vascular structures, creating an anterior tumor surface topography that appeared smooth (calm) (n = 7), mini-wavy (rippled) (n = 2), or maxi-wavy (undulating) (n = 5). Greater tumor thickness correlated with increasing tumor surface fluctuation as calm was mean 1.7mm, rippled was 2.8 mm, and undulating surface was 4.1 mm in ultrasonographic thickness. On enhanced depth imaging optical coherence tomography, the mean subfoveolar choroidal thickness, measurable in 9 eyes, was 484 &mgr;m (median, 423 &mgr;m; range, 156–1,002 &mgr;m) (81% greater) versus 267 &mgr;m (median, 276 &mgr;m; range, 142–501 &mgr;m) in the unaffected eye. The mean maximal tumor thickness, measurable in 8 eyes, was 117% greater at 602 &mgr;m (median, 538 &mgr;m; range, 241–966 &mgr;m) compared with the corresponding unaffected choroid in the contralateral eye at 278 &mgr;m (median, 245 &mgr;m; range, 189–511 &mgr;m) (P = 0.046). Inability to measure choroidal thickness was due to dense tumor-induced optical shadowing with inability to visualize the sclerochoroidal junction (P = 0.009). There was no visible infiltration into the overlying retina in any case. Conclusion: Enhanced depth imaging optical coherence tomography of choroidal lymphoma revealed 1 of 3 surface topographical patterns resembling an ocean as calm (n = 7), rippled (n = 2), or undulating (n = 5), correlating with increasing tumor thickness.

Iris Stromal Cyst Management With Absolute Alcohol–Induced Sclerosis in 16 Patients

IMPORTANCE The management of symptomatic iris stromal cyst is challenging using methods of aspiration with or without adjunctive cryotherapy, intracameral cautery, or photocoagulation. Failed cases often require surgical resection, with risks for epithelial downgrowth, glaucoma, blindness, and loss of the eye. OBJECTIVE To study the safety and efficacy of the management of iris stromal cysts with aspiration and alcohol irrigation to induce cyst sclerosis. DESIGN, SETTING, AND PARTICIPANTS Interventional case series at a tertiary referral center among 16 patients. EXPOSURES Microscopically monitored transcorneal aspiration of cysts was performed with a 30-gauge needle on a 3-way T-extension into a 3-mL syringe, followed by immediate infusion of absolute alcohol from a separate 1-mL syringe through the other arm of the T-extension. Subsequent alcohol aspiration and repetition of the cycle was performed until the collapsed cyst wall appeared gray. MAIN OUTCOMES AND MEASURES Cyst involution, visual acuity, and treatment complications. RESULTS The iris stromal cysts were primary congenital (n = 6), primary acquired (n = 4), or secondary (n = 6). The cysts had a median basal diameter of 12 mm and a thickness of 4 mm, occupying 50% or more of the anterior or posterior chamber in each case. Treatment was administered following failure of simple aspiration (n = 16) and additional methods (n = 8). During a median follow-up period of 5 years, treatment was successful in 14 of 15 patients (1 patient was lost to follow-up). Cyst sclerosis with stabilization (n = 1) or involution (n = 13) was achieved following 1 (n = 10), 2 (n = 2), or 3 (n = 2) procedures. The single failure occurred in a 3-year-old child with cyst recurrence and severe photophobia requiring resection. Visual acuity remained stable or had improved in 14 patients and was reduced in 1 patient because of cataract. Complications included transient corneal edema (n = 4) and transient anterior chamber inflammation (n = 1), which resolved following topical corticosteroid therapy. No evidence was seen of treatment-related glaucoma, epithelial downgrowth, tissue necrosis, cataract, posterior segment toxic effects, or need for enucleation. CONCLUSIONS AND RELEVANCE Microscopically monitored aspiration and absolute alcohol-induced sclerosis of iris stromal cysts is safe and effective, with cyst involution obtained in 93% (14 of 15) of patients.

Conjunctival Papilloma: Features and Outcomes Based on Age at Initial Examination

IMPORTANCE Conjunctival papilloma is a benign epithelial tumor occurring in both children and adults with varying clinical features and outcomes. In this article, we describe our experience regarding the difference in the clinical features and outcomes of conjunctival papilloma based on age at initial examination. OBJECTIVE To evaluate the clinical features, treatment, and outcomes in patients with conjunctival papilloma based on age at initial examination. DESIGN Retrospective study. SETTING Ocular Oncology Service, Wills Eye Hospital, Philadelphia, Pennsylvania. PARTICIPANTS Ten children and adolescents (aged ≤20 years) and 63 adults (aged >20 years) with conjunctival papilloma. INTERVENTIONS Excisional biopsy, cryotherapy, oral cimetidine, topical or injection interferon alfa-2b, and photodynamic therapy. MAIN OUTCOME MEASURE Tumor response. RESULTS A comparison of conjunctival papillomas between age groups revealed significant differences in the mean number of tumors per eye (children and adolescents vs adults, 2 vs 1; P = .05), tumor basal dimension (8 vs 6 mm; P = .05), and associated feeder vessels (20% vs 47%; P = .05). Primary treatment included sole treatment with oral cimetidine (15% vs 5%), topical interferon alfa-2b (0% vs 1%), cryotherapy (0% vs 3%), photodynamic therapy (0% vs 1%), excisional biopsy and cryotherapy (38% vs 65%), excisional biopsy and cryotherapy with adjuvant oral cimetidine (8% vs 9%), and excisional biopsy and cryotherapy with adjuvant topical or injection interferon alfa-2b (38% vs 15%). Significant differences in age groups in treatment outcome during the follow-up period (mean, 24 vs 38 months) included complete regression with single treatment (38% vs 95%; P < .01) and tumor recurrence (15% vs 1%; P = .05). CONCLUSIONS AND RELEVANCE Conjunctival papillomas are larger and more likely to be multiple in children and adolescents than in adults. Excisional biopsy and cryotherapy with or without adjuvant oral cimetidine and/or topical interferon alfa-2b provide satisfactory tumor control. Papilloma recurrence is more common in children and adolescents than in adults.

Choroidal osteoma shows bone lamella and vascular channels on enhanced depth imaging optical coherence tomography in 15 eyes.

Purpose: To describe enhanced depth imaging optical coherence tomography findings of choroidal osteoma. Methods: Retrospective, observational case series of 15 eyes with choroidal osteoma imaged with EDI-OCT. Results: The mean age at presentation was 27 years. There were 10 women and 3 men. The visual acuity ranged from 20/20 to hand motion, with reduced visual acuity secondary to photoreceptor loss in the foveola (n = 5) or subfoveal fluid (n = 1), and additional choroidal neovascular membrane (n = 3). The mean basal tumor diameter was 8.0 mm, and ultrasonographic thickness was 1.5 mm. Using enhanced depth imaging optical coherence tomography, the mean tumor thickness was 589 &mgr;m when compared with a matched choroidal region in the unaffected eye of 247 &mgr;m (138% increased thickness) (P = 0.009). The tumor surface topography was classified (ultrasonography vs. enhanced depth imaging optical coherence tomography) as flat (87 vs. 13%), dome (13 vs. 40%), or undulating (0 vs. 47%). On enhanced depth imaging optical coherence tomography, unique features included horizontal lamellar lines (presumed bone lamella) (n = 15, 100%) and hyperreflective horizontal lines (presumed cement lines) (n = 8, 53%). Other features included horizontal tubular lamella with optically empty center (presumed Haversian canals or vascular channels) (n = 9, 60%), vertical tubular lamella (presumed Volkmann canals or vascular channels) (n = 2, 13%), and speckled regions (presumed compact or small trabecular bone) (n = 6, 40%). Of the nine eyes with subfoveolar osteoma, the tumor was completely ossified (n = 4), partially deossified (n = 2), or completely deossified (n = 3). Photoreceptor thinning/atrophy was found in all five eyes with deossified osteoma, whereas intact photoreceptor appearance was noted in the four eyes with ossified subfoveal osteoma. Conclusion: Enhanced depth imaging optical coherence tomography reveals characteristic surface topography of choroidal osteoma as dome or undulating (87%) with unique intrinsic features of horizontal lamellar lines (100%), horizontal (60%) or vertical (13%) tubules, and speckled regions (40%). Photoreceptor loss was evident in every case of tumor deossification.

Ciliary body medulloepithelioma association with pleuropulmonary blastoma in a familial tumor predisposition syndrome.

Ciliary body medulloepithelioma can rarely present in association with pleuropulmonary blastoma as part of a familial tumor predisposition syndrome. This is thought to occur secondary to a germline mutation in the DICER1 gene. The authors describe a case of ciliary body medulloepithelioma in a 9-year-old girl with a known medical history of pleuropulmonary blastoma.

Optical coherence tomography characteristics of epi-iridic membrane in a child with recurrent hyphema and presumed juvenile xanthogranuloma

We report a case of spontaneous hyphema in a 6-month-old girl with no history of trauma and no visible iris mass. Subtle green-blue heterochromia was noted in the right eye. The iris crypts in the right eye appeared flattened by a thin, transparent layer on the iris surface. Anterior segment optical coherence tomography (AS-OCT) disclosed a thin homogenous membrane overlying the entire iris surface in the right eye. Fluorescein angiography revealed diffuse hyperfluorescence without neovascularization. These features were suggestive of diffuse iris juvenile xanthogranuloma. Sub-Tenons triamcinolone acetate plus topical corticosteroids eyedrops resolved the condition within 1 month.