ABSTRACT
PURPOSE: To evaluate the frequency and severity of ophthalmic manifestations and associated diseases, as well as the epidemiological data in patients with Williams syndrome. METHODS: The authors prospectively studied 30 patients clinically diagnosed as having Williams syndrome as confirmed by the fluorescence in situ hybridization test. Patient history included gender, age, race, education level, previous illnesses, and surgeries. The ophthalmologic examination included best-corrected visual acuity, dynamic and static refraction, extraocular motility test, stereopsis test (Titmus and Lang), and direct and indirect funduscopy. RESULTS: Thirty patients were included in this study. The mean age was 14.5 ± 1.38 years (range: 7 to 26 years). Fifty percent of the patients were male and 50% were female. Among the children examined, 77% had a refractive error. Hyperopia and astigmatism were noted in 67% and 20% of the patients, respectively, and myopia in 7%. Only one case of amblyopia was noted. On external examination, 23% of children had epicanthus; via biomicroscopy, 3 children with stellate patterns of the irides were observed. Eleven patients (36.6%) had measurable strabismus, 9 (82%) had esotropia, and 2 (18%) had exotropia. Binocular vision was abnormal in 43% of patients. Diffuse arteriovenous tortuosity on funduscopy was observed in 27% of patients. CONCLUSIONS: Williams syndrome is rare and is associated with multiple phenotypes and diseases that are susceptible to treatment. Multidisciplinary clinical management is critical and, in some cases, surgical intervention is required.
Subject(s)
Cardiovascular Diseases/diagnosis , Developmental Disabilities/diagnosis , Refractive Errors/diagnosis , Strabismus/diagnosis , Williams Syndrome/diagnosis , Adolescent , Adult , Child , Depth Perception/physiology , Eye Movements/physiology , Female , Follow-Up Studies , Humans , In Situ Hybridization, Fluorescence , Male , Prospective Studies , Refraction, Ocular/physiology , Visual Acuity/physiology , Young AdultABSTRACT
UNLABELLED: Permanent exotropia (XT) occurs in 1 to 2% of the pediatric population. Its management involves careful assessment of patient, treatment of amblyopia, refractive errors and surgery. The aim of the surgery is to straighten the eyes in the primary gaze position, giving a better cosmetic outcome. The factors reported to affect surgical outcome after exotropia surgery vary widely in reports and success rates for strabismus surgery have been reported to range from 60% to 80%. There are few reports to determine the relation between amblyopia and surgical outcome in exotropic patients. PURPOSE: To compare the surgical outcome of permanent exotropia surgery in amblyopic and non-amblyopic patients. METHODS: This is a retrospective study of 37 clinical records from amblyopic patients (Group A) and non-amblyopic patients (Group B) who underwent recess-resect in one eye for XT. Postoperative deviation was analyzed in one month (immediate) and in six months (final) in both groups and in between. Age: group A 24.7 ± 14.2 years, group B 22.6 ± 18.6 years; Preoperative deviation: group A 29.1 ± 7.2(Δ), group B 28.4 ± 6.8(Δ). RESULTS: The success rate in the immediate postoperative period was 60% (Group A) and 100% (Group B) (p<0.05); 50% (Group A) and 82.3% (Group B) (p=0.082) in the final postoperative period. There was a statistical difference in the immediate postoperative deviation, but the final deviation and the variation of the deviation were similar in both groups. CONCLUSION: There is a better outcome in patients of group B and no diference in the surgical outcome between these amblyopic and non-amblyopic patients in the final postoperative period.
Subject(s)
Amblyopia/complications , Exotropia/surgery , Adolescent , Adult , Aged , Child , Child, Preschool , Exotropia/complications , Female , Follow-Up Studies , Humans , Infant , Male , Middle Aged , Ophthalmologic Surgical Procedures , Retrospective Studies , Treatment Outcome , Young AdultABSTRACT
A exotropia permanente (XT) acomete cerca de 1 a 2 por cento da população. Seu tratamento é clínico: antiambliogênico e correção dos erros refrativos, e cirúrgico. O objetivo do tratamento cirúrgico é alinhar os olhos na posição primária do olhar, proporcionando melhor resultado estético. Há muito tempo diversos autores estudam os fatores pré, per e pós-operatórios relacionados ao resultado cirúrgico, uma vez que a taxa de sucesso varia de 60 a 80 por cento. Ainda são poucos os estudos que comparam a presença de ambliopia como fator de influência no resultado final. OBJETIVO: Comparar o resultado cirúrgico dos pacientes amblíopes e não-amblíopes submetidos à cirurgia de correção de XT. MÉTODOS: Análise retrospectiva de 37 prontuários de pacientes amblíopes (Grupo A) e não-amblíopes (Grupo B) submetidos à correção cirúrgica de XT por retrocessoressecção monocular, sendo avaliados os registros pós-operatórios imediatos e tardios. Idade: grupo A 24,7 ± 14,2 anos, grupo B 22,6 ±18,6 anos; Desvio pré-operatório: grupo A 29,1± 7,2Δ, grupo B 28,4 ± 6,8Δ. RESULTADOS: A taxa de sucesso foi de 60 por cento e 100 por cento (p<0,05), no pós-operatório imediato e 50 por cento e 82,3 por cento (p=0,082), no pós-operatório final, nos grupos A e B, respectivamente. Não houve diferença significante quanto aos desvios pós-operatórios imediatos, tardios e variação do desvio. CONCLUSÃO: Pode-se concluir que o grupo B mostrou melhor resultado no pós-operatório imediato; porém não houve diferença no resultado cirúrgico de correção de exotropia permanente entre pacientes amblíopes e não-amblíopes no período pós-operatório de seis meses.
Permanent exotropia (XT) occurs in 1 to 2 percent of the pediatric population. Its management involves careful assessment of patient, treatment of amblyopia, refractive errors and surgery. The aim of the surgery is to straighten the eyes in the primary gaze position, giving a better cosmetic outcome. The factors reported to affect surgical outcome after exotropia surgery vary widely in reports and success rates for strabismus surgery have been reported to range from 60 percent to 80 percent. There are few reports to determine the relation between amblyopia and surgical outcome in exotropic patients. PURPOSE: To compare the surgical outcome of permanent exotropia surgery in amblyopic and non-amblyopic patients. METHODS: This is a retrospective study of 37 clinical records from amblyopic patients (Group A) and non-amblyopic patients (Group B) who underwent recess-resect in one eye for XT. Postoperative deviation was analyzed in one month (immediate) and insixmonths (final) in both groups and in between. Age: group A 24.7 ± 14.2 years, group B 22.6 ± 18.6 years; Preoperative deviation: group A 29.1 ± 7.2Δ, group B 28.4 ± 6.8Δ. RESULTS: The success rate in the imediate postoperative period was 60 percent (Group A) and 100 percent (Group B) (p<0.05); 50 percent (Group A) and 82.3 percent (Group B) (p=0.082) in the final postoperative period. There was a statistical difference in the imediate postoperative deviation, but the final deviation and the variation of the deviation were similar in both groups. CONCLUSION: There is a better outcome in patients of group B and no diference in the surgical outcome between these amblyopic and non-amblyopic patients in the final postoperative period.
