Khetpal Vijay, Donahue Sean P
Department of Ophthalmology and Visual Sciences, Vanderbilt University Medical Center, Nashville, Tennessee 37232-8808, USA.
J AAPOS. 2007 Jun;11(3):235-9. doi: 10.1016/j.jaapos.2007.01.122. Epub 2007 Apr 24.
To evaluate the etiology, prognosis, and associated neurological and ophthalmologic findings of children with cortical visual impairment (CVI) at a tertiary care referral facility.
Records from patients visiting the Vanderbilt University Pediatric Ophthalmology Center during 2002 to 2005 were reviewed, and 98 patients were identified with an International Classification of Disease (9th ed.) coding of CVI (377.75). The charts were reviewed to assess presenting symptoms. The clinic and imaging notes were correlated with visual function (graded on a scale of I to VI).
The most common etiologies were perinatal hypoxia (35%), prematurity (29%), hydrocephalus (19%), structural central nervous system abnormalities (11%), and seizures (10%). Many children (69%) had multiple etiologies. Associated ophthalmic abnormalities included esotropia (19%), exotropia (40%), nystagmus (21%), and optic atrophy (42%). Significant refractive error (> +3.00 D or < -2.00 D) was common (20%). Associated neurological findings included seizures (60%), cerebral palsy (37%), periventricular leukomalacia (12%), hemiparesis (21%), and hearing loss (11%). Fifty-three percent of children initially diagnosed with CVI were followed for a period of 0.5 to 10 years. Forty percent of the patients showed no improvement in visual function; 34% had minimal improvement, and 17% had mild improvement. Only 6% of the patients had significant improvement in visual function. Eight patients had fixing and following or better acuity at last follow-up.
The major risk factors for CVI are perinatal hypoxia, premature birth, and hydrocephalus. Most patients have associated serious neurological and ophthalmologic abnormalities. While many patients have some recovery in vision acuity, most never see well. Patients with the most improvement in visual function were those having better initial acuity.
在一家三级医疗转诊机构评估皮质视觉障碍（CVI）患儿的病因、预后以及相关的神经和眼科表现。
回顾了2002年至2005年期间就诊于范德比尔特大学小儿眼科中心的患者记录，确定了98例国际疾病分类（第9版）编码为CVI（377.75）的患者。查阅病历以评估就诊时的症状。将临床和影像学记录与视觉功能（按I至VI级分级）进行关联。
最常见的病因是围产期缺氧（35%）、早产（29%）、脑积水（19%）、中枢神经系统结构异常（11%）和癫痫（10%）。许多儿童（69%）有多种病因。相关的眼科异常包括内斜视（19%）、外斜视（40%）、眼球震颤（21%）和视神经萎缩（42%）。显著屈光不正（>+3.00 D或<-2.00 D）很常见（20%）。相关的神经学表现包括癫痫（60%）、脑瘫（37%）、脑室周围白质软化（12%）、偏瘫（21%）和听力损失（11%）。最初诊断为CVI的儿童中有53%接受了0.5至10年的随访。40%的患者视觉功能无改善；34%有轻微改善，17%有轻度改善。只有6%的患者视觉功能有显著改善。8例患者在最后一次随访时有注视和追视或更好的视力。
CVI的主要危险因素是围产期缺氧、早产和脑积水。大多数患者伴有严重的神经和眼科异常。虽然许多患者视力有一定恢复，但大多数视力仍不佳。视觉功能改善最大的患者是那些初始视力较好的患者。
