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延迟单侧头颈部淋巴管畸形的侵入性治疗可改善预后。

Delaying Invasive Treatment in Unilateral Head and Neck Lymphatic Malformation Improves Outcomes.

作者信息

Bonilla-Velez Juliana, Whitlock Kathryn B, Ganti Sheila, Shivaram Giri M, Bly Randall A, Dahl John P, Manning Scott C, Perkins Jonathan A

机构信息

Division of Pediatric Otolaryngology-Head and Neck Surgery, Seattle Children's Hospital, Seattle, Washington, U.S.A.

Department of Otolaryngology-Head and Neck Surgery, University of Washington School of Medicine, Seattle, Washington, U.S.A.

出版信息

Laryngoscope. 2023 Apr;133(4):956-962. doi: 10.1002/lary.30237. Epub 2022 Jun 3.

Abstract

OBJECTIVES

Large (De Serres stage [IV-V]) head and neck lymphatic malformations (HNLMs) often have multiple, high-risk, invasive treatments (ITs) to address functional compromise. Logically reducing HNLM ITs should reduce treatment risk. We tested whether delaying HNLM ITs reduces total IT number.

MATERIALS

Consecutive HNLM patients (n = 199) between 2010 and 2017, aged 0-18 years.

METHODS

ITs (surgery or sclerotherapy) were offered for persistent or dysfunction causing HNLMs. Treatment effectiveness categorized by IT number: optimal (0-1), acceptable (2-5), or suboptimal (>5). Clinical data were summarized, and outcome associations tested (χ ). Relative risk (RR) with a Poisson working model tested whether HNLM observation or IT delay (>6 months post-diagnosis) predicts treatment success (i.e., ≤1 IT).

RESULTS

Median age at HNLM diagnosis was 1.3 months (interquartile range [IQR] 0-45 m) with 107/199(54%) male. HNLM were stage I-III (174 [88%]), IV-V (25 [13%]). Initial treatment was observation (70 [35%]), invasive (129 [65%]). Treatment outcomes were optimal (137 [69%]), acceptable (36 [18%]), and suboptimal (26 [13%]). Suboptimal outcome associations: EXIT procedure, stage IV-V, oral location, and tracheotomy (p < 0.001). Stage I-III HNLMs were initially observed compared with stage I-III having ITs within 6 months of HNLM diagnosis, had a 82% lower relative treatment failure risk ([i.e., >1 IT], RR = 0.09, 95% CI 0.02-0.36, p < 0.001). Stage I-III HNLMs with non-delayed ITs had reduced treatment failure risk compared with IV-V (RR = 0.47, 95% CI 0.33-0.66, p < 0.001).

CONCLUSION

Observation and delayed IT in stage I-III HNLM ("Grade 1") is safe and reduces IT (i.e., ≤1 IT). Stage IV-V HNLMs ("Grade 2") with early IT have a greater risk of multiple ITs.

LEVEL OF EVIDENCE

4 Laryngoscope, 133:956-962, 2023.

摘要

目的

大型(德塞尔阶段[IV - V])头颈部淋巴管畸形(HNLMs)通常需要多种高风险的侵入性治疗(ITs)来解决功能受损问题。从逻辑上讲,减少HNLMs的ITs应能降低治疗风险。我们测试了延迟HNLMs的ITs是否会减少总的IT数量。

材料

2010年至2017年间连续纳入的199例年龄在0至18岁的HNLMs患者。

方法

针对导致HNLMs持续存在或功能障碍的情况提供ITs(手术或硬化治疗)。根据IT数量对治疗效果进行分类:最佳(0 - 1次)、可接受(2 - 5次)或次优(>5次)。总结临床数据并测试结果关联(χ检验)。使用泊松工作模型的相对风险(RR)测试HNLMs观察或IT延迟(诊断后>6个月)是否能预测治疗成功(即≤1次IT)。

结果

HNLMs诊断时的中位年龄为1.3个月(四分位间距[IQR] 0 - 45个月),107/199(54%)为男性。HNLMs为I - III期(174例[88%]),IV - V期(25例[13%])。初始治疗为观察(70例[35%]),侵入性治疗(129例[65%])。治疗结果为最佳(137例[69%])、可接受(36例[18%])和次优(26例[13%])。次优结果关联因素:EXIT手术、IV - V期、口腔部位和气管切开术(p < 0.001)。与在HNLMs诊断后6个月内接受ITs的I - III期患者相比,I - III期HNLMs最初接受观察的患者相对治疗失败风险降低了82%(即>1次IT,RR = 0.09,95% CI 0.02 - 0.36,p < 0.001)。与IV - V期相比,I - III期HNLMs未延迟ITs的患者治疗失败风险降低(RR = 0.47,95% CI 0.33 - 0.66,p < 0.001)。

结论

I - III期HNLMs(“1级”)的观察和延迟IT是安全的,并能减少IT次数(即≤1次IT)。早期接受ITs的IV - V期HNLMs(“2级”)有更高的多次ITs风险。

证据水平

4 喉镜,133:956 - 962,2023年

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