ABSTRACT. Retinoblastoma is the most common intraocular malignancy of childhood. It may rarely present with white spots on the iris and pseudohypopyon. We report a case of an 11-month old child with polydactyly with this presentation of retinoblastoma. There was no positive family history of the disease. Investigations included anterior segment examination under anaesthesia, fundoscopy with scleral indentation, A-and B-scan ultrasound and MRI examination of the head. This was a Reese Ellsworth group 5 retinoblastoma with an indication for enucleation. Pathology reports of the enucleated globe showed choroidal and ciliary body invasion. Therefore, subsequent chemotherapy treatment was undertaken. The retinoblastoma gene is located in the long arm of chromosome 13. Almost all familial and bilateral cases carry the abnormal gene. In unilateral isolated retinoblastomas -as in our case -most patients do not have a germinal mutation, however, only DNA analysis can safely exclude that. We also discuss possible factors having a link to both polydactyly and retinoblastoma.
Purpose: The aim of our study was to compare the depth of the demarcation line developing in the cornea after the standard Dresden protocol versus the accelerated, pulsed, epithelium-off corneal collagen cross-linking (CXL). Methods: This was a nonrandomized, retrospective case series. Patients with progressive keratoconus were treated with either the standard Dresden protocol (Group 1) or accelerated, epithelium-off CXL using the Avedro (Waltham, MA, USA) device (Group 2). The accelerated CXL protocol involved 18 min of pulsed ultraviolet-A (20 mW/cm2, 7.2 J/cm2, pulsed pro-file: 1 s on, 2 s off). The depth of the demarcation line was measured about 3 months postoperatively. Results: Fifty-nine eyes of 35 subjects were included in the analysis. Group 1 consisted of 19 eyes, and Group 2 of 40 eyes. The mean age of the participants was 22.21 years in Group 1 and 26.55 years in Group 2 (p = 0.184). The mean preoperative K value was 44.89 D in Group 1 and 45.20 D in Group 2 (p = 0.768). The depth of the demarcation line was 322.50 μm in Group 1 and 319.95 μm in Group 2 (p = 0.937). Conclusions: The demarcation line depth was not statistically significantly different between the two protocols. The significance of the demarcation line depth has not been fully clarified in the literature. Our results support the contention that these two techniques may have similar structural outcomes and efficacies in the treatment of keratoconus.
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