M. Fortunato

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.

Anterior Ischemic Optical Neuropathy in Children on Chronic Peritoneal Dialysis: Report of 7 Cases

♦ Background: Anterior ischemic optic neuropathy (AION) is characterized by infarction of the optic nerve head due to hypoperfusion of the posterior ciliary arteries and causes sudden blindness in adults on chronic dialysis, but has rarely been described in children. Unlike adults, children do not have comorbidities related to aging. ♦ Methods: We retrospectively analyzed data of 7 children on nocturnal continuous cycling peritoneal dialysis (CCPD) who developed AION identified within the Italian Registry of Pediatric Chronic Dialysis. We also summarized data from 10 cases reported in the literature. ♦ Results: Our 7 patients suffered from acute onset bilateral blindness. Their mean age was 3.2 years and chronic hypotension had been observed prior the AION in 3 of the 7 children. Low systolic blood pressure (SBP) was associated with higher risk of developing AION according to statistical analysis. None recovered completely. In total, 11 out of 16 experienced a partial recovery and no clear evidence emerged favoring specific treatments. ♦ Conclusions: Hypotensive children treated with CCPD are at increased risk of developing AION, which often results in irreversible blindness.

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.

Optical Coherence Tomography Angiography in Children with Leber-Coats Disease

Introduction: The aim of this work was to describe the optical coherence tomography (OCT) angiography (OCTA) features in paediatric Leber-Coats disease. Methods: Three children presenting with unilateral, clinically active Leber-Coats disease were evaluated using spectral domain OCT and OCTA. OCT angiograms were analysed and compared with the current literature. Results: All 3 OCT angiograms showed that the vessels had lost most of their collateral branches and presented many loops. The capillaries were rarefied and anomalies in vessel size, vasodilatation, and macroaneurysms were evident. Conclusion: This is the first case series of OCTA performed in children with Leber-Coats disease. OCTA is a non-invasive and non-contact technique, which can be considered of fundamental importance for the diagnosis, clinical management, and follow-up of the disease.