Strabismus surgery at the Puerto Rico Medical Center: a brief report.

OBJECTIVE: We undertook a retrospective study to evaluate strabismus surgeries done at the University Hospital and at the University Pediatric Hospital of the Puerto Rico Medical Center. METHODS: The surgery schedules ranging from January 2003 to December 2007 were reviewed. Three types of strabismus were evaluated, including esotropia, exotropia, and hypertopia. Differences in the distribution of strabismus type according to age group and gender were assessed using the extension of the Fisher's exact test. RESULTS: A total of 120 patients were included (56 males and 64 females); the patients ranged in age from 4 months to 70 years (average: 15.1 years. Analysis of the distribution of strabismus type showed that 63 (52.5%) had esotropia; 49 (40.8%) had exotropia; 4 (3.3%) had hypertropia; 3 (2.5%) had both hypertropia and exotropia; and 1 (0.9%) had both hypertropia and esotropia. Both esotropia and exotropia were significantly (p < 0.0001) more common in children than they were in adults; however, hypertropia and combined strabismus types were more common in adults than they were in children. There was no statistical (p > 0.05) significance found upon analyzing strabismus distribution according to gender. CONCLUSION: Among patients with this eye disorder at the Puerto Rico Medical Center, the most common type of surgically treated strabismus was esotropia. Further studies will elucidate the prevalence of strabismus in Puerto Rico.

Humphrey visual fields and optical coherence tomography findings in patients with the Axenfeld-Rieger syndrome: a case series.

INTRODUCTION: Axenfeld-Rieger syndrome is characterized by clinical characteristics including: ocular, craniofacial, dental, umbilical, and other systemic abnormalities. Previous studies have reported that the Axenfeld-Rieger syndrome (ARS) occurs in one out of 200,000 patients. PURPOSE: To study patients with the ARS in a sub-urban community in Puerto Rico. PATIENTS AND METHODS: We conducted a non-concurrent study of 17,800 patients. Of these, 57 patients had the syndrome. Every patient had a comprehensive ophthalmology evaluation. RESULTS: A frequency of 320 per 100,000 was found in the studied population. Using Humphrey's visual field analyzer, we found a mean deviation of less than -2.00 dB in 56.4 and 60.5 percent of the right and left eye respectively. OCT evaluation of the optic nerve rim showed glaucomatous changes in 29.2 and 41.7 percent of the right and left eyes respectively. OCT evaluation of the retinal nerve fiber layer showed glaucomatous changes in 20.8 and 16.7 percent of the right and left eyes respectively. CONCLUSION: The frequency of patients with the syndrome in Puerto Rico is higher than in other populations reported in the literature. Also, the percent of patients with glaucoma is higher than previously reported. A relationship between hemochromatosis and ARS has not been previously described. Further studies will elucidate the genetics of Axenfeld-Rieger syndrome in Puerto Rico.

Usher syndrome in Puerto Rico: a clinical and genetic study.

PURPOSE: To evaluate patients with the Usher syn drome in Puerto Rico. METHODS: Three patients with the Usher syndrome underwent an ophthalmic and audiologic evaluation; and genetic linkage analysis. RESULTS: All patients were legally blind based on visual acuity and visual field results. Two patients had macular edema as shown on Stratus OCT. All patients had moderate hearing loss as part of the syndrome. A patient, and two family members had three mutations leading to protein changes including: p.S4588Y; p.Y4505C; and p.14474M. CONCLUSIONS: Phenotypic findings in patients with the Usher syndrome in Puerto Rico are similar to those previously reported. However, to our knowledge, neither these mutations nor OCT findings have been previously described in patients with the syndrome.