ABSTRACT
A 5-year-old boy developed profound loss of vision in his right eye and was found to have a 11778 mitochondrial point mutation consistent with Leber hereditary optic neuropathy (LHON). He maintained 20/20 vision in the left eye for 18 years until age 23, when he experienced loss of vision in that eye. This 18 year interval between eye involvement in LHON is the longest reported to date and reinforces the variability in presentation and progression seen in this disease.
Subject(s)
Optic Atrophy, Hereditary, Leber/diagnosis , Optic Nerve/pathology , Point Mutation , Child, Preschool , DNA, Mitochondrial/genetics , Humans , Magnetic Resonance Imaging , Male , Optic Atrophy, Hereditary, Leber/genetics , Time FactorsABSTRACT
PURPOSE: To report refractive and surgical outcomes in a cohort of children with accommodative esotropia. DESIGN: Retrospective population-based cohort. PARTICIPANTS: All pediatric patients (<19 years) diagnosed with accommodative esotropia in Olmsted County, Minnesota, from January 1, 1975, to December 31, 1994. METHODS: Medical records identified by the Rochester Epidemiology Project were reviewed for spectacle and surgical management. MAIN OUTCOME MEASURES: Rate of surgical intervention and discontinuation of spectacles for esotropia. RESULTS: A total of 306 children with accommodative esotropia were diagnosed during the study years; 244 (80%) had fully accommodative esotropia (FAET) and 62 (20%) had partially accommodative esotropia (PAET). The Kaplan-Meier rate of discontinuing spectacles for strabismus in this population was 8% by 5 years after diagnosis, 20% by 10 years, and 37% by 20 years. Children born prematurely (P = 0.046) or with a greater initial hyperopic refractive error (P<0.001) were significantly less likely to become spectacle-free during the follow-up period. During a median follow-up of 9.8 years (range, 0-27.9 years), 33 (13.5%) of the 244 subjects with FAET eventually underwent strabismus surgery. Male gender (P = 0.039) and an earlier age at onset (P = 0.008) and diagnosis (P<0.001) of FAET were associated with a higher likelihood of requiring surgery. CONCLUSIONS: The majority of children with accommodative esotropia in this population continued to require spectacle correction into their second decade of life. A small proportion of children with FAET required surgical intervention, which was more likely to occur among boys and in those with an earlier age at onset and diagnosis of their deviation.
