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[彼得斯异常的长期预后]

[Long-term prognosis of Peters anomaly].

作者信息

Reichl S, Böhringer D, Richter O, Lagrèze W, Reinhard T

机构信息

Klinik für Augenheilkunde, Universitätsklinikum Freiburg, Medizinische Fakultät der Albert-Ludwigs-Universität Freiburg, Killianstr. 5, 79106, Freiburg, Deutschland.

出版信息

Ophthalmologe. 2018 Apr;115(4):309-313. doi: 10.1007/s00347-017-0498-7.

DOI:10.1007/s00347-017-0498-7
PMID:28484852
Abstract

BACKGROUND

Peters anomaly normally presents in early childhood. Common features are central corneal opacities and dysgeneses of the anterior eye segment. Early surgery is commonly warranted to prevent deep amblyopia or because of severe glaucoma. We herein present the clinical outcomes of all patients treated in the Eye Center of the Albert Ludwigs University of Freiburg since 2005. Emphasis is placed on the Peters subtype.

METHODS

Data were collected retrospectively by means of chart review. Kaplan-Meier analyses were used to estimate visual prognosis, the indication for keratoplasty, and the incidence of retinal detachment.

RESULTS

A total of 23 patients were identified. Subtype distribution was 40% type 1, 50% type 2, and 10% Peters plus syndrome. Ten patients were female (45%). Mean age at first presentation was 5 years; mean follow-up period censored in terms of eyeball preservation was 2 years (0 months-8 years). At mean follow-up, 40.5% of all patients had undergone at least one keratoplasty (up to six per eye); 43% had undergone glaucoma surgery (cylophotocoagulation, trabeculectomy, implants) at this time. Important complications were retinal detachment (31%) and phthisis bulbi (15%). After 4 years, visual acuity in the better eye was at most 0.05 in every second patient.

CONCLUSION

Prognosis of visual acuity in Peters anomaly is poor. It is generally not possible to restore visual function in the long run, i. e., reading-grade visual acuity is rarely achieved. Surgical interventions are associated with a high risk of severe complications. Therefore, the young patients should be connected to institutions for visually impaired persons at an early stage.

摘要

背景

彼得斯异常通常在儿童早期出现。常见特征为中央角膜混浊和眼前节发育异常。通常需要早期手术以预防严重弱视或因严重青光眼而行手术。我们在此介绍自2005年以来在弗莱堡阿尔伯特·路德维希大学眼科中心接受治疗的所有患者的临床结果。重点关注彼得斯亚型。

方法

通过病历回顾进行回顾性数据收集。采用Kaplan-Meier分析来评估视力预后、角膜移植术指征和视网膜脱离发生率。

结果

共确定23例患者。亚型分布为1型占40%,2型占50%,彼得斯综合征加其他异常占10%。10例为女性(45%)。首次就诊时的平均年龄为5岁;以眼球保留为截尾标准的平均随访期为2年(0个月至8年)。平均随访时,所有患者中有40.5%至少接受过一次角膜移植术(每只眼最多6次);此时43%的患者接受了青光眼手术(睫状体光凝术、小梁切除术、植入物)。重要并发症为视网膜脱离(31%)和眼球痨(15%)。4年后,每两名患者中就有一名较好眼的视力最多为0.05。

结论

彼得斯异常的视力预后较差。从长远来看,一般不可能恢复视觉功能,即很少能达到阅读级视力。手术干预伴有严重并发症的高风险。因此,应在早期将年轻患者转至视力障碍者机构。

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