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《Loeys-Dietz 综合征的矫形外科管理：系统评价》。

Orthopaedic Management of Loeys-Dietz Syndrome: A Systematic Review.

机构信息

From the Department of Orthopaedic Surgery (Mr. Lynch, Ms. Patel, Dr. M.A. Seeley) and the Department of Pediatrics (Dr. A.H. Seeley), Geisinger Medical Center, Danville, PA.

出版信息

J Am Acad Orthop Surg Glob Res Rev. 2021 Nov 15;5(11):e21.00087. doi: 10.5435/JAAOSGlobal-D-21-00087.

DOI:10.5435/JAAOSGlobal-D-21-00087
PMID:34779796
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8594655/
Abstract

INTRODUCTION

Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder associated with aortic aneurysm/dissection in children. However, LDS may also present with a host of orthopaedic conditions. This study aimed to elucidate the management of orthopaedic conditions and associated outcomes in patients with LDS.

METHODS

PubMed, Ovid MEDLINE, and Cochrane Library were systematically searched for primary articles regarding the management of orthopaedic conditions in patients with LDS. The goals and findings of each included study were described. Data regarding demographics, conditions studied, treatment modalities, and outcomes were extracted and analyzed.

RESULTS

Three hundred sixty-two unique articles were retrieved, 13 of which were included, with 4 retrospective cohort studies and 9 case reports representing 435 patients. In total, 19.8% of patients presenting with orthopaedic conditions received surgical treatment;54.3% of them experienced adverse outcomes, and 44.4% required revision surgery. The mean age at surgery was 9.0 ± 2.1 years.

CONCLUSION

Patients with LDS may require early surgical intervention for a variety of orthopaedic conditions and may be at an increased risk for surgical complications. The current LDS literature is primarily focused on spinal conditions with a relative paucity of data on the management of hip deformity, joint subluxation, clubfoot, and trauma. Additional research is required regarding orthopaedic management for this unique population.

摘要

简介

Loeys-Dietz 综合征（LDS）是一种常染色体显性结缔组织疾病，与儿童的主动脉瘤/夹层有关。然而，LDS 也可能表现出多种骨科疾病。本研究旨在阐明 LDS 患者骨科疾病的管理及相关结局。

方法

系统检索了 PubMed、Ovid MEDLINE 和 Cochrane Library 中关于 LDS 患者骨科疾病管理的原始文章。描述了每个纳入研究的目标和发现。提取并分析了有关人口统计学、研究的疾病、治疗方式和结局的数据。

结果

共检索到 362 篇独特的文章，其中 13 篇被纳入，包括 4 项回顾性队列研究和 9 项病例报告，共涉及 435 名患者。共有 19.8%的骨科疾病患者接受了手术治疗；其中 54.3%的患者出现不良结局，44.4%的患者需要再次手术。手术时的平均年龄为 9.0±2.1 岁。

结论

LDS 患者可能需要早期手术干预多种骨科疾病，并且手术并发症的风险可能增加。目前 LDS 的文献主要集中在脊柱疾病上，而髋部畸形、关节半脱位、马蹄内翻足和创伤的管理数据相对较少。对于这一特殊人群，需要进一步开展骨科管理方面的研究。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2a8f/8594655/bcb95d00d513/jagrr-5-e21.00087-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2a8f/8594655/15f8b44ae624/jagrr-5-e21.00087-g001.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2a8f/8594655/a9aa63e45e94/jagrr-5-e21.00087-g002.jpg
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