Abstract：
OBJECTIVE: To report the birth prevalence and natural history of congenital corneal opacities among a population-based cohort of children.
METHODS: The medical records of patients <5 years diagnosed with a congenital onset corneal opacity while residing in Olmsted County, Minnesota, from January 1, 1977, through December 31, 2016, were retrospectively reviewed.
RESULTS: Fourteen patients were diagnosed with a congenital corneal opacity during the 40-year study period for a birth prevalence of 1 in 5188 live births. The mean age at diagnosis was 7.5 months (range 0-48 months) and 9 (64.3%) were males. Four patients had congenital glaucoma, 4 had limbal dermoids, 2 had sclerocornea, and 1 patient each had Descemet\'s tear from birth trauma, herpes simplex virus type 1 keratitis, corneal leukoma, and an undiagnosed scar. Six (42.8%) patients required treatment for their underlying corneal opacity including the four patients with congenital glaucoma. The other 8 (57.1%) patients had a clear central axis. Four (28.6%) of 14 patients required amblyopia therapy, and 4 (28.6%) developed strabismus. Four (28.6%) patients had associated systemic conditions. During a mean follow up of 5.4 years (range 1.3-27.0 years), the median best corrected visual acuity (BCVA) was logmar 0.16 (20/25) (range 20/20-hand motion) with one patient with unilateral BCVA less than 20/60 and one patient with bilateral BCVA less than 20/60.
CONCLUSIONS: In this 40-year cohort, congenital corneal opacities were relatively rare and the result of a variety of disorders. Although amblyopia and strabismus occurred commonly, most patients had good visual outcomes.
METHODS: The medical records of patients <5 years diagnosed with a congenital onset corneal opacity while residing in Olmsted County, Minnesota, from January 1, 1977, through December 31, 2016, were retrospectively reviewed.
RESULTS: Fourteen patients were diagnosed with a congenital corneal opacity during the 40-year study period for a birth prevalence of 1 in 5188 live births. The mean age at diagnosis was 7.5 months (range 0-48 months) and 9 (64.3%) were males. Four patients had congenital glaucoma, 4 had limbal dermoids, 2 had sclerocornea, and 1 patient each had Descemet\'s tear from birth trauma, herpes simplex virus type 1 keratitis, corneal leukoma, and an undiagnosed scar. Six (42.8%) patients required treatment for their underlying corneal opacity including the four patients with congenital glaucoma. The other 8 (57.1%) patients had a clear central axis. Four (28.6%) of 14 patients required amblyopia therapy, and 4 (28.6%) developed strabismus. Four (28.6%) patients had associated systemic conditions. During a mean follow up of 5.4 years (range 1.3-27.0 years), the median best corrected visual acuity (BCVA) was logmar 0.16 (20/25) (range 20/20-hand motion) with one patient with unilateral BCVA less than 20/60 and one patient with bilateral BCVA less than 20/60.
CONCLUSIONS: In this 40-year cohort, congenital corneal opacities were relatively rare and the result of a variety of disorders. Although amblyopia and strabismus occurred commonly, most patients had good visual outcomes.
摘要：
目的：报告以人口为基础的儿童队列中先天性角膜混浊的出生患病率和自然史。
方法：居住在奥姆斯特德县时诊断为先天性角膜混浊的<5岁患者的医疗记录，明尼苏达,从1977年1月1日至2016年12月31日,我们进行了回顾性回顾.
结果：在40年的研究期间，有14例患者被诊断为先天性角膜混浊，出生患病率为5188例活产婴儿中的1例。诊断时的平均年龄为7.5个月（范围0-48个月），男性为9岁（64.3％）。四名患者患有先天性青光眼，4人患有角膜缘皮肤样病变，2有巩膜角膜，每个患者都有一个因出生创伤而撕裂的Descemet\'s，单纯疱疹病毒1型角膜炎，角膜白瘤，还有一个未确诊的伤疤.六名（42.8％）患者需要治疗其潜在的角膜混浊，其中包括四名先天性青光眼患者。其他8例（57.1%）患者的中轴清晰。14例患者中有4例（28.6%）需要弱视治疗，4例（28.6%）出现斜视。四名（28.6%）患者有相关的全身状况。在平均5.4年的随访期间（范围1.3-27.0年），中位最佳矫正视力(BCVA)为logmar0.16(20/25)(20/20-手部活动范围),其中1例单侧BCVA小于20/60,1例双侧BCVA小于20/60.
结论：在这个40年的队列中，先天性角膜混浊相对罕见，是多种疾病的结果。虽然弱视和斜视通常发生，大多数患者的视力预后良好.
方法：居住在奥姆斯特德县时诊断为先天性角膜混浊的<5岁患者的医疗记录，明尼苏达,从1977年1月1日至2016年12月31日,我们进行了回顾性回顾.
结果：在40年的研究期间，有14例患者被诊断为先天性角膜混浊，出生患病率为5188例活产婴儿中的1例。诊断时的平均年龄为7.5个月（范围0-48个月），男性为9岁（64.3％）。四名患者患有先天性青光眼，4人患有角膜缘皮肤样病变，2有巩膜角膜，每个患者都有一个因出生创伤而撕裂的Descemet\'s，单纯疱疹病毒1型角膜炎，角膜白瘤，还有一个未确诊的伤疤.六名（42.8％）患者需要治疗其潜在的角膜混浊，其中包括四名先天性青光眼患者。其他8例（57.1%）患者的中轴清晰。14例患者中有4例（28.6%）需要弱视治疗，4例（28.6%）出现斜视。四名（28.6%）患者有相关的全身状况。在平均5.4年的随访期间（范围1.3-27.0年），中位最佳矫正视力(BCVA)为logmar0.16(20/25)(20/20-手部活动范围),其中1例单侧BCVA小于20/60,1例双侧BCVA小于20/60.
结论：在这个40年的队列中，先天性角膜混浊相对罕见，是多种疾病的结果。虽然弱视和斜视通常发生，大多数患者的视力预后良好.
