Photodynamic therapy in von Hippel-Lindau disease in children.

Von Hippel-Lindau disease is characterized by retinal hemangiomas that often produce hemorrhages and retinal detachment. A 10-year-old boy with von Hippel-Lindau disease presenting with telangiectasias, endophytic angiomas, and exudative retinal detachment underwent treatment with retrobulbar steroid injection, laser photocoagulation, and photodynamic therapy, which led to improvement of visual acuity.

Corneal curvature and axial length values in children with congenital/infantile cataract in the first 42 months of life.

PURPOSE: To evaluate corneal curvature (K(m)) and axial length (AL) of eyes of term-born children aged <3.5 years with uncomplicated congenital cataract and provide age-specific estimates. METHODS: This was a retrospective review of patients undergoing cataract extraction from January 1994 to December 2006. Exclusion criteria were preterm birth, microphthalmia, microcornea, megalocornea, glaucoma, traumatic or complicated cataract, retinal disease. Keratometric readings were taken with an autokeratometer before surgery. Linear regression with the logarithm of the patient's age as an independent variable was used to model the relationship between the patient's age and biometric and keratometric readings. RESULTS: All unilateral cataractous eyes (n = 69) and a randomly selected eye in bilateral cases (n = 111) were included in the analysis, for a total of 180 eyes. Mean age was 15.5 +/- 11.8 months; mean AL, 20.03 +/- 2.25 mm; mean K(m), 45.07 +/- 3.00 D. K(m) was significantly greater and AL shorter in younger children (P < 0.001). No differences according to sex were found. As a group, eyes from unilateral cataract had significantly longer AL than those from bilateral cases (P = 0.029). In a small subgroup of unilateral cataract patients for which readings from the clear lens eye were available (n = 39), K(m) of the affected eye was significantly greater than that of the fellow healthy eye (P = 0.007). CONCLUSIONS: In the first 42 months of age, K(m) and AL are significantly different according to age. These findings have implications for the calculation of intraocular lens power in children.

Expression of the lipogenic enzyme fatty acid synthase (FAS) in retinoblastoma and its correlation with tumor aggressiveness.

PURPOSE: Fatty acid synthase (FAS) performs the anabolic conversion of dietary carbohydrate or protein to fatty acids. Many common human cancers express high levels of FAS, and its differential expression between normal and neoplastic tissues has led to the consideration of FAS as a target for anticancer therapy. To investigate the potential of targeting FAS in the treatment of retinoblastoma, we first determined whether FAS was activated in this human tumor. Moreover, correlation of FAS expression with tumor aggressiveness was determined. METHODS: FAS reactivity was evaluated by immunohistochemistry in 66 retinoblastoma specimens from 65 patients. Degree of tumor differentiation, choroid invasion, optic nerve infiltration, mitotic rate, and necrosis extension were estimated. FAS expression was correlated with all these tumor characteristics by means of parametric and nonparametric statistical analyses. RESULTS: Eighty-two percent of tumors were FAS positive. Stronger FAS expression correlated with more advanced choroid (P < 0.001) and optic nerve (P = 0.016) invasion, high mitotic index (P < 0.001), and less differentiated histology (P = 0.047). Correlation with extension of necrosis was not statistically significant. Unaffected retina was negative. CONCLUSIONS: The data suggest that expression of FAS and fatty acid synthesis support an essential functional aspect of retinoblastoma cells, perhaps cell growth or survival. FAS activation may serve as a novel target for systemic and local antineoplastic therapy and, because it increases with tumor aggressiveness, its inhibition could represent an alternative treatment strategy in advanced and resistant retinoblastomas.