IMPORTANCE Recurrent or persistent vitreous seeds following treatment of retinoblastoma poses difficult management and often leads to enucleation. OBJECTIVE To describe the technique and evaluate the efficacy and complications of intravitreal melphalan for vitreous seeding from retinoblastoma. DESIGN, SETTING, AND PARTICIPANTS This retrospective noncomparative analysis was conducted at a tertiary referral center. The study included 11 consecutive eyes of 11 patients with viable persistent or recurrent vitreous seeds following treatment of retinoblastoma. INTERVENTIONS All eyes received intravitreal melphalan injection (20-30 μg) by transconjunctival pars plana route with concomitant triple-freeze cryotherapy at the injection site during needle withdrawal for prevention of extraocular seeding. Each patient was offered planned 6 monthly cycles. MAIN OUTCOMES AND MEASURES Vitreous seed control and complications of therapy. RESULTS The mean patient age at vitreous injection was 37 months (median, 27 months; range, 16-82 months). Viable vitreous seeds involved 2 (n = 1), 3 (n = 4), or 4 (n = 6) quadrants. The solid intraretinal retinoblastoma and subretinal seeds showed regression in all eyes following intravenous chemotherapy (n = 6) or intra-arterial chemotherapy (n = 5). There were a total of 55 injections, with a mean number per patient of 5 (median, 6; range, 2-6). Fewer than 6 injections (n = 5) were delivered owing to complete vitreous seed control and parental desire to avoid more injections. By a mean of 9 months' follow-up (median, 9 months; range, 6-16 months), therapeutic success with complete vitreous seed regression was achieved in all 11 cases (100%). Globe salvage was attained in all cases (100%). Further vitreous seed development did not occur in any case. Complications included focal retinal pigment epithelial mottling near the site of chemotherapy injection (2 eyes) and nonaxial posterior lens opacity (2 eyes). There was no case of extraocular tumor extension, hypotony, or phthisis bulbi. CONCLUSIONS AND RELEVANCE These preliminary short-term results suggest that intravitreal melphalan injection for persistent or recurrent vitreous retinoblastoma seeding can provide tumor control with minimal toxicity and complications.
The higher prevalence of autoimmune diseases in women compared to men could be due to effects of ovarian hormones, pregnancy and/or the presence of a 2nd X chromosome. To elucidate the role of these factors, we investigated the prevalence and spectrum of autoimmune diagnoses in women with primary ovarian insufficiency associated with X chromosome monosomy (Turner syndrome, TS, n=244) and women with karyotypically normal (46,XX) primary ovarian insufficiency (POI, n=457) in a prospective study, conducted at the National Institutes of Health. We compared the study group prevalence to normative data for the U.S. population of women. Chronic lymphocytic (Hashimoto’s) thyroiditis (HT) occurred in 37% of women with TS vs. 15% with POI (P<0.0001); HT prevalence in both ovarian insufficiency groups significantly exceeded that in U.S. population of women (5.8%). Inflammatory bowel (IBD, 4%) and celiac disease (CD, 2.7%) were significantly increased in TS, but not in POI. No other autoimmune diagnosis, including Graves’ disease or Type 1 diabetes appears to be significantly increased in either group. Women with TS had higher pro-inflammatory IL6 and TGF β1 levels (p<0.0001 for both), and lower anti-inflammatory IL10 and TGF β2 levels (p<0.005 for both) compared to POI and to normal volunteers. Lifetime estrogen exposure and parity were significantly lower in TS compared to POI, which were in turn lower than the general population of women. The finding that lymphocytic thyroiditis is greatly increased in both women with TS and POI suggests that factors associated with ovarian insufficiency per se promote this form of autoimmunity. The absence of a normal second X-chromosome further contributes to increased autoimmunity in TS.
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.
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.
IMPORTANCE Conjunctival squamous cell carcinoma (SCC) is surgically managed with wide surgical excisional biopsy, superficial keratectomy, and cryotherapy. In eyes with residual tumor showing scleral invasion or intraocular involvement, enucleation is advised.OBJECTIVE To explore the role of plaque radiotherapy for residual invasive conjunctival SCC as an alternative to enucleation. DESIGN, SETTING, AND PARTICIPANTS Interventional case series involving 15 patients with histopathologically confirmed scleral and/or intraocular invasion of SCC at Wills Eye Hospital. INTERVENTION Plaque radiotherapy. MAIN OUTCOMES AND MEASURES Tumor control, treatment complications, and globe salvage. RESULTSThe primary tumor prior to excision displayed a mean basal diameter of 12.1 mm (median, 12 mm; range, 4-25 mm) and mean thickness of 2.6 mm (median, 3 mm; range, 1-5 mm). In all cases, the tumor was managed by primary surgical resection using wide excisional biopsy, limited superficial keratectomy, and cryotherapy to the remaining conjunctival margins. Histopathology confirmed conjunctival SCC in all cases with residual tumor demonstrating scleral invasion (n = 15) and/or anterior chamber invasion (n = 3). The residual tumor measured a mean basal diameter of 10.6 mm (median, 10 mm; range, 2.5-20 mm) and mean thickness of 1.5 mm (median, 1 mm; range, 1-5 mm). Plaque radiotherapy was applied in all cases for a mean apex dose of 56 Gy (median, 50 Gy; range, 50-80 Gy) over a mean of 132 hours (median, 142 hours; range, 93-170 hours). Over a mean follow-up of 41 months (median, 46 months; range, 9-96 months), local tumor control was achieved in all 15 cases (100%). However, 4 cases showed further distant conjunctival tumor recurrence remote from the site of radiotherapy with positive orbital involvement at a mean of 5 months postradiotherapy, necessitating enucleation (n = 2) or orbital exenteration (n = 2). Globe salvage was achieved in 10 cases, as 1 patient required enucleation for chronic ocular irritation. Radiation complications included cataract (n = 13), iris telangiectasia (n = 5), corneal epithelial defect (n = 4), corneal edema (n = 3), and glaucoma (n = 1). There was no evidence of metastasis.CONCLUSIONS AND RELEVANCE Plaque radiotherapy delivered over a mean of 6 days can be an effective alternative to enucleation for residual scleral-invasive conjunctival SCC following resection. Local tumor control was achieved in all cases.
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