Coats plus in prematurity.

BACKGROUND: Coats plus syndrome or cerebroretinal microangiopathy with calcifications and cysts (CMCC) is an exceedingly rare autosomal recessive disorder that predominantly affects the microvasculature in the retina, brain, bones, and gastrointestinal system. Unlike Coats disease, CMCC is bilateral and affects multiple organ systems. MATERIALS AND METHODS: Case report. RESULTS: We report the case of two brothers with Coats Plus syndrome who presented with variable phenotypic expression. One sibling (Patient 1) was thought to have atypical retinopathy of prematurity and was only diagnosed with Coats plus after his older brother (Patient 2) presented with a seizure and a left upper extremity tremor at 4 years of age. The CTC1 mutation was confirmed in both patients. Aggressive treatment with laser photocoagulation and intravitreal bevacizumab dramatically improved the retinal vascular and exudative changes. CONCLUSION: Coats Plus syndrome can have a variable phenotypic presentation, including retinal vascular findings. This rare genetic disease should be in the differential diagnosis in patients who present with atypical retinal pathology, including Retinopathy of Prematurity, Familial Exudative Vitreoretinopathy, or Coats disease associated with non-specific multiorgan abnormalities.

Central corneal thickness as a predictor of visual field loss in primary open angle glaucoma for a Hispanic population.

BACKGROUND: Primary Open Angle Glaucoma is a multi-factorial disease with a devastating impact on the quality of life of the patient in the moderate and severe stages of the disease. Identifying risk factors for the development of moderate to severe visual field loss may decrease the proportion of patients that experience the severe forms of this disease. PURPOSE: To evaluate whether the central corneal thickness correlates inversely with the severity of visual field loss in Primary Open Angle Glaucoma. METHODS: Retrospective review of 308 charts of patients seen during a six-week period by a glaucoma specialist in his community practice in a large Hispanic area. Patients were classified as normal, ocular hypertensive, and those with Primary Open Angle Glaucoma. Odds ratios and 95% confidence interval were calculated to evaluate risk factors associated to ocular hypertension and Primary Open Angle Glaucoma. Finally, a multivariate polytomous regression model was used to evaluate central corneal thickness as an independent predictor of outcome after adjustment for age and hypertension. Statistical significance was set at p<0.05. RESULTS: Patients with Primary Open Angle Glaucoma show a statistically significant inverse correlation between central corneal thickness and the severity of the visual field damage. CONCLUSION: Thinner corneas could be considered a risk factor for the severity of visual field loss in Primary Open Angle Glaucoma.