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Purpose To analyze the results of Retinoblastoma (Rb) treatment according to the University Hospital of Siena guidelines in terms of complete ophthalmoscopic remission (COR) of disease. Methods A total of 109 eyes of 87 patients were enrolled from September 2013 to November 2016. The mean patient age at diagnosis was 22.8 months (± sd 20.5 months). Sixtysix/109 eyes (60.55% of cases) were unilateral, 42 (38.53%) bilateral. Thirtyfive/109 (32.11%) were classified as low stage (A-C of ICRB classification), 36 (33.03%) as D stage and 31 (28.44%) as E stage. Interventions: Conservative treatment with intravenous (IV) chemotherapy (CHT) and/or intrarterial (IA) CHT, both as first or second line therapies; primary or secondary enucleation. Results Fourtysix/109 eyes (42.20%) received primary IV CHT, 40 (36.70%) primary IA CHT and 22 (20.1%) were enucleated at diagnosis. Twentyfive eyes (22.9%) were treated with secondary IA CHT (for failure of IV CHT, the majority being bilateral cases), 4 (3.6%) with second line IV CHT (mainly thermo-CHT after IA CHT). Twentythree/109 eyes (21.1%) were secondary enucleated. Globe salvage with COR of disease has been obtained by 53/109 eyes (49% of all cases, 60.9% of the treated ones), in particular: by the 32.5% of the eyes treated with primary IV CHT, by the 52.5% of those treated with primary IA CHT and by the 60% of the eyes treated with secondary IA CHT. Mean follow-up is of 21.9 months (± sd 16.9 months). Conclusions These results confirm the efficacy of our guidelines for Rb treatment. Particularly we obtained a better ocular salvage rate (p > χ2 = 0.0007, Mantel Cox Comparisons) for the cases treated with secondary IA CHT than with primary IA CHT. This observation demonstrates the efficacy of IA CHT even in recurrences of Rb after primary IV CHT, if early detected with a close follow-up.

Purpose The aim of this case report is to describe a case of a 19 months old female with diffuse infiltrating retinoblastoma in her right eye misdiagnosed as corneal dystrophy in another center. Diffuse infiltrating retinoblastoma can lead to anterior segment seeding with pseudohypopion, pseudoialitis and parsplanitis, intraocular hemorrhage confusing it with uveitis, unexplained vitreous hemorrhage, hyphema or endophalmitis. Methods In October 2016 the girl was observed at our Ocular Oncology Unit at the University of Siena. At ophthalmoscopic examination in her right eye she presented hyperemia, corneal edema, endothelium alterations, flocculi in the angle, iris neovascularization, and hematic clots (3–10 h). At fundus examination there were not retinal masses, but cloudy vitreous and vitreous deposits inferiorly. The left eye was normal. B scan echography documented thickened retina and vitreous deposits. RMI excluded extraocular disease. Results The eye was enucleated and the diagnosis of diffuse infiltrating retinoblastoma was confirmed. Conclusions Diffuse infiltrating is the least common growth pattern of retinoblastoma and often the most difficult to recognize clinically. It grows diffusely within the retina .Any child with signs of uveitis, hyphema, hypopion, vitreous hemorrhage and ialitis should be evaluated for retinoblastoma.

To measure the adrenocorticotropic hormone (ACTH) and cortisol (F) cord plasma levels in preterm and term infants in relation to their mode of delivery. We studied 180 newborns appropriate for gestational age (GA) with birth weights between 365 and 4380 g and GAs between 21 and 41 weeks divided into three groups: born by vaginal delivery (VD), elective cesarean section (ECS) and emergency cesarean section (EMCS). ACTH and F levels were valued with enzyme-linked immunosorbent assay testing. Median concentrations were compared between groups by Student’s T-test for independent and paired data. Multiple regression analysis was used to investigate the effect of GA on F and ACTH concentrations. A significant positive correlation between GA and plasma concentrations of ACTH (P

The aim of the study is to establish whether healthy full-term breastfed infants require supplemental vitamin D for proper bone mineralization.Bone mineralization was studied by performing ultrasound scans of 73 healthy full-term subjects at the age of 3 months. The infants were divided into three groups. Group A: breastfed without supplemental vitamin D (BF); group B: breastfed with supplement of 400 IU/day of vitamin D (BFD); group C: fed with formula (with and without supplemental vitamin D 400 IU/day) (FF). The values of mcSOS (m/sec) and mcBTT (µsec) were assessed in all subjects.A statistically significant difference has been found between group A vs group B both in mcSOS (P=0.03) and in mcBTT (P=0.01) values and also between group A vs group C both in mcSOS (P=0.012) and in mcBTT (P=0.003) values. Significant differences between group B vs group C were not found. In 75% of subjects of group A mcSOS and mcBTT values were ≤ the 10th percentile, while in group B they were between the 10th and 50th percentile. In FF infants given supplemental vitamin D mcSOS and mcBTT values were between the 25th and 75th percentile.Human breast milk is an appropriate source of nutrition for the growth of healthy full-term newborns, but is poor in vitamin D as demonstrated by the impaired bone mineralization in the breastfed infants without supplemental vitamin D. The results presented demonstrate that exclusively breastfed infants require at least 400 IU/day of supplemental vitamin D.

Purpose Pediatric ocular granuloma is a rare condition which may result from several causes including trauma, inflammatory diseases, or infectious agents such as fungi, viruses, or mycobacteria. The purpose of this case report is to increase the knowledge about the ophthalmic clinical picture, magnetic resonance imaging findings, histological pattern of a rare case of iris tubercular granuloma occurring in a 4-year-old boy. Methods Case report Results Inflammatory granulomas are usually the expression of systemic inflammatory diseases. Clinical picture may be that of an anterior or posterior uveitis and it may occur with uveal granulomas or episcleral nodules. Our case at diagnosis simulated a medulloepithelioma, both for ophthalmogic and neuroradiologic findings. Pathology following biopsy revealed a tuberculous granulomatous lesion. Conclusion A complete systemic investigation confirmed tuberculous aetiology of this granulomatous lesion.

Purpose To report our experience with superselective ophthalmic artery infusion of Melphalan (SOAIM) for macular retinoblastoma, in order to obtain tumor control while preserving as much as possible useful vision. Methods This report concerns 5 cases with ‘naive’ unilateral retinoblastoma involving the macula, selected from a group of patients scheduled for SOAIM as the primary treatment. Follow-up ranged from a minimum of 6 months to a maximum of 30 months. Results Each eye of each patient was treated with a cycle of three SOAIM procedures, made of 3-5 mg of Melphalan per eye per treatment. SOAIM procedure was well tolerated in all 5 patients. One of the eyes was also treated with cryotherapy while all eyes underwent transpupillary thermo-therapy and/or argon laser during or after the intra-arterial treatment cycle. All patients are alive and free of metastatic spread. All the eyes achieved ophthalmoscopic remission of the tumor foci, showing types I to III regression, and no enucleation was necessary.Ultrasounds and fluorangiography were performed. Conclusion SOAIM is effective for the treatment of macular retinoblastoma, when performed in adequate settings by operators with skills in angiographic diagnosis and treatment of intracranial vascular diseases. Moreover, Melphalan selectively delivered in the ophthalmic artery may allow the salvation of eyes which should otherwise be enucleated, showing a very low rate of complications due to local and systemic toxicity.