P. Vadala

Intraocular Lens Implantation in Marfan's Syndrome

PURPOSE To describe the results of the removal of ectopic lenses from patients with Marfans syndrome using the scleral fixation method. METHODS Intraocular lenses (IOLs) were implanted in six eyes affected by lens dislocation (ectopia lentis) using either the scleral fixation method (five eyes) or a silicone IOL in the capsular bag (one eye). Mean patient age ranged from 8-11 years and follow-up ranged from 7-20 months. RESULTS Functional success was obtained in all eyes. Postoperative visual acuity was 20/20 to 20/40. One patient showed a dislocation of the IOL in the anterior chamber. In three eyes, an opacification of the posterior capsule was treated using an Nd:YAG laser. CONCLUSION Intraocular lens implantation using the scleral fixation technique is the first choice in patients with Marfans syndrome because it reduces the complications of IOL decentration.

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 patients age as an independent variable was used to model the relationship between the patients 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.

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.