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小儿头颈部淋巴管畸形的治疗与预后:一项20年单机构经验

Treatments and Outcomes of Pediatric Head and Neck Lymphatic Malformations: A 20-Year Single Institution Experience.

作者信息

Zhao Jared, Thompson Elaine, Weiss Clifford Raabe, Walsh Jonathan

机构信息

Johns Hopkins University School of Medicine, Johns Hopkins University, Baltimore, Maryland, USA.

Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins University, Baltimore, Maryland, USA.

出版信息

Otolaryngol Head Neck Surg. 2025 Mar;172(3):1026-1035. doi: 10.1002/ohn.1077. Epub 2024 Dec 15.

Abstract

OBJECTIVE

Examine the management and outcomes of pediatric head and neck lymphatic malformations (HNLMs).

STUDY DESIGN

Retrospective case series.

SETTING

Tertiary academic hospital.

METHODS

Patients aged 18 years or younger treated for HNLMs at Johns Hopkins Hospital from 2000 to 2023 were identified. Demographics, clinical characteristics, and treatment courses were recorded from charts. Outcome measures included estimated clinical post-treatment size reduction and complications. Fisher's exact test was performed for comparisons between treatments.

RESULTS

We identified 101 pediatric patients (50.5% female) with HNLMs. Fifty-two received unimodal treatment, 11 received multimodal treatment, and 38 underwent active observation or no treatment. Of those who received treatment, 44% had their first treatment before 2 years of age. 54.5% of all patients received sclerotherapy. Sclerotherapy caused nearly complete or complete resolution in 52.2% (n = 24) of low-stage (de Serres Stages 1-3) HNLMs and complete resolution in 26.1%. Most (71.4%) high-stage (de Serres Stages 4-5) malformations demonstrated limited size reduction response to all treatments. Surgery had a higher all-inclusive complication rate (25%) compared to sclerotherapy (17%). Multimodal treatment strategies were most often used to treat microcystic malformations of all stages, achieving a 42.9% nearly complete or complete resolution rate.

CONCLUSION

Low-stage and macrocystic pediatric HNLMs respond well to treatment. Sclerotherapy had the lowest complication rate. High-stage malformations had limited size reduction response to multimodal treatment. As new medical therapies continue to develop, opportunities for improved treatment algorithms will emerge.

摘要

目的

研究小儿头颈部淋巴管畸形(HNLMs)的管理及治疗结果。

研究设计

回顾性病例系列研究。

研究地点

三级学术医院。

方法

确定2000年至2023年在约翰霍普金斯医院接受HNLMs治疗的18岁及以下患者。从病历中记录人口统计学、临床特征和治疗过程。结局指标包括治疗后估计的临床尺寸缩小情况及并发症。采用Fisher精确检验对不同治疗方法进行比较。

结果

我们确定了101例患有HNLMs的儿科患者(50.5%为女性)。52例接受单模式治疗,11例接受多模式治疗,38例接受积极观察或未接受治疗。在接受治疗的患者中,44%在2岁前接受了首次治疗。所有患者中有54.5%接受了硬化治疗。硬化治疗使52.2%(n = 24)的低分期(de Serres 1 - 3期)HNLMs几乎完全或完全消退,完全消退率为26.1%。大多数(71.4%)高分期(de Serres 4 -至5期)畸形对所有治疗的尺寸缩小反应有限。与硬化治疗(17%)相比,手术的总体并发症发生率更高(25%)。多模式治疗策略最常用于治疗各阶段的微囊性畸形,实现了42.9%的几乎完全或完全消退率。

结论

低分期和大囊型小儿HNLMs对治疗反应良好。硬化治疗的并发症发生率最低。高分期畸形对多模式治疗的尺寸缩小反应有限。随着新的医学疗法不断发展,将出现改进治疗方案的机会。

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