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唐氏综合征患儿先天性鼻泪管阻塞的管理。

Management of congenital nasolacrimal duct obstruction in down syndrome.

机构信息

Division of Ophthalmology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA and the Edwin and Fannie Gray Hall Center for Human Appearance, The University of Pennsylvania, Perelman School of Medicine, Pennsylvania, USA.

Division of Ophthalmology, the Goldschleger Eye Institute, Sheba Medical Center, Tel Hashomer, Ramat Gan, Israel.

出版信息

Eye (Lond). 2023 Mar;37(4):739-743. doi: 10.1038/s41433-022-02047-w. Epub 2022 Apr 4.

DOI:10.1038/s41433-022-02047-w
PMID:35379922
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9998547/
Abstract

BACKGROUND

Congenital nasolacrimal duct obstruction (CNLDO) is common in Down Syndrome (DS), and more difficult to treat. Our purpose was to describe the management of CNLDO in paediatric patients with DS.

METHODS

Retrospective cohort study. Medical chart review of all DS patients diagnosed with CNLDO at the Division of Ophthalmology at the Children's Hospital of Philadelphia during a 12-year period (2009-2020). Main outcome measures included: Surgical interventions, primary probing outcome, rate of dacryocystorhinostomy (DCR) and/or conjunctivodacryocystorhinostomy (CDCR), and overall success.

RESULTS

126 patients (236 eyes) were included, mean age of 1.8 ± 2.1 years (range 0.1-11.3 y), 110 (87%) had bilateral CNLDO. Mean follow-up time was 41 months. 84 patients (67%) underwent at least one surgical intervention; the mean number of surgical interventions in this group was 1.8 ± 1.4 per patient (range, 1-6). The most common primary intervention was probing (n = 74, 88%), mostly (n = 57, 68%) with monocanalicular silicone intubation. Probing with silicone intubation had a higher success rate compared to probing alone (P = 0.002). Twenty (24% of 84) patients underwent DCR/CDCR during the follow-up period. Complete resolution was achieved in 123 patients (98%).

CONCLUSIONS

CNLDO in Down syndrome is associated with high rates of bilateral obstructions and with less favourable surgical outcomes. Many patients ultimately require a more robust surgical intervention such as DCR or CDCR. The use of monocanalicular stent in initial probing was associated with a higher success rate, and would appear to be appropriate in all CNLDO-DS cases.

摘要

背景

先天性鼻泪管阻塞(CNLDO)在唐氏综合征(DS)中较为常见,且治疗更为困难。我们的目的是描述在费城儿童医院眼科分部对患有 DS 的小儿 CNLDO 的管理。

方法

回顾性队列研究。对在费城儿童医院眼科分部诊断为 CNLDO 的所有 DS 患者的病历进行回顾性分析,时间跨度为 12 年(2009-2020 年)。主要观察指标包括:手术干预、初次探通术的结果、泪囊鼻腔吻合术(DCR)和/或结膜囊鼻腔吻合术(CDCR)的比率以及整体成功率。

结果

共纳入 126 例患者(236 只眼),平均年龄为 1.8±2.1 岁(范围 0.1-11.3 岁),110 例(87%)为双侧 CNLDO。平均随访时间为 41 个月。84 例(67%)患者至少接受了一次手术干预;该组患者的平均手术次数为 1.8±1.4 次/人(范围,1-6 次)。最常见的初次干预是探通术(n=74,88%),其中大多数(n=57,68%)采用单通道硅胶插管。与单纯探通术相比,探通术联合硅胶插管的成功率更高(P=0.002)。在随访期间,20 例(84 例中的 24%)患者接受了 DCR/CDCR。123 例(98%)患者完全缓解。

结论

唐氏综合征相关的先天性鼻泪管阻塞双侧阻塞发生率高,手术结果不佳。许多患者最终需要更有效的手术干预,如 DCR 或 CDCR。初次探通术中使用单通道支架与更高的成功率相关,且似乎适用于所有 CNLDO-DS 病例。

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本文引用的文献

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Down Syndrome.唐氏综合征
N Engl J Med. 2020 Jun 11;382(24):2344-2352. doi: 10.1056/NEJMra1706537.
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Prevalence and Treatment Outcome of Nasolacrimal Duct Obstruction in Saudi Children with Down Syndrome.沙特唐氏综合征患儿鼻泪管阻塞的患病率及治疗结果
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Visual acuity deficits in children with nystagmus and Down syndrome.眼球震颤合并唐氏综合征儿童的视力缺陷。
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