Optical coherence tomography aspect of crystalline macular dystrophy in Sjögren-Larsson syndrome.

Sjögren-Larsson syndrome is an autosomal-recessive disease caused by a deficiency of the microsomal fatty aldehyde dehydrogenase enzyme. The syndrome is defined by congenital ichthyosis, spasticity, mental retardation and ocular features. We report the case of a 10-year-old boy presenting with bilateral visual impairment and photophobia. Fundus examination showed a mark of yellow-white refractile, perifoveal crystals in each eye. Optical coherence tomography (OCT) detected focal reflective structures corresponding to clinically visible intraretinal crystals and macular macrocystoids space. This case is presented to highlight the ocular findings and to evaluate the contribution of OCT in the study of the fovea anatomic changes.

Changes in intraocular pressure after clear corneal phacoemulsification in normal patients.

PURPOSE: To evaluate changes in intraocular pressure (IOP) after clear corneal phacoemulsification (CCP) in normal patients. MATERIALS AND METHODS: A prospective study including 273 normal patients selected for cataract extraction by CCP. Intraocular pressure was recorded on the 15(th) day, l(st), 2(nd), 3(rd) month and 6 months after surgery. STATISTICAL ANALYSIS: For statistical analysis, Epi Info was used to determine the statistical significance of changes in IOP. RESULTS: The mean age of 96 women and 177 men was 71 ± 12 years. The mean IOP before surgery was 14.18 ± 3.4 mmHg. Our patients showed a mean decrease in IOP of 2.25 mmHg (16%) compared to preoperative values. Change in IOP was not related to lens thickness (P = 0.12), but significantly correlated with change in anterior chamber depth (ACD) (P = 0.002). The postoperative IOP was inversely related to preoperative ACD (P = 0.012). Age, sex and axial length were not significantly related to IOP reduction (P = 0.2-0.5) CONCLUSION: CCP was associated with a statistically significant reduction in IOP. The exact mechanism by which cataract surgery results in IOP reduction is unclear. CCP can be performed with the intent of achieving better IOP control.