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淋巴管畸形结构:对溶链菌制剂OK-432治疗的意义

Lymphatic Malformation Architecture: Implications for Treatment With OK-432.

作者信息

Malic Claudia C, Guilfoyle Regan, Courtemanche Rebecca J M, Arneja Jugpal S, Heran Manraj K S, Courtemanche Douglas J

机构信息

*Children's Hospital of Eastern Ontario, Ottawa †University of Alberta Hospital, Edmonton ‡University of British Columbia §BC Children's Hospital, Vancouver, Canada.

出版信息

J Craniofac Surg. 2017 Oct;28(7):1721-1724. doi: 10.1097/SCS.0000000000003789.

DOI:10.1097/SCS.0000000000003789
PMID:28834841
Abstract

PURPOSE

Herein, the authors aim to describe their findings of novel architectural types of lymphatic malformations (LM) and explain the relationship between these architectures and OK-432 treatment outcomes.

METHODS

A retrospective review was conducted of all patients diagnosed with a LM treated with OK-432 at the Vascular Anomalies Clinic at BC Children's Hospital from December 2002 to January 2012.

RESULTS

Twenty-seven patients were included in the study. Sixty percent of lesions were present by 2 years of age with the majority located in the head and neck (59%). The average number of sclerotherapy procedures was 1.4 per patient. Treatment under fluoroscopic guidance revealed 3 new LM architectures: open-cell microcystic, closed-cell microcystic, and lymphatic channel. Response to treatment was complete or good for 14/19 macrocystic and for 1/2 mixed lesions. Open-cell microcystic LMs gave a complete or good response for 3/3, which was attributed to OK-432 freely communicating between cysts. Closed-cell microcystic LM had localized cysts that did not allow OK-432 to freely communicate and were associated with partial responses, 2/2. The lymphatic channel had a partial response. There were 2 minor complications and 1 instance of recurrence.

CONCLUSIONS

The identification of 3 new LM architectures expands the current accepted classification to include: open-cell microcystic, closed-cell microcystic, and lymphatic channels. The majority of complete responses to OK-432 were found with macrocystic lesions. Open-cell microcystic lesions respond better to OK-432 than closed-cell microcystic lesions, and lymphatic channels may respond to OK-432. These key architecture-response relationships have direct clinical implications for treatment with OK-432 sclerotherapy.

摘要

目的

在此,作者旨在描述他们对新型淋巴管畸形(LM)结构类型的发现,并解释这些结构与OK-432治疗结果之间的关系。

方法

对2002年12月至2012年1月在不列颠哥伦比亚省儿童医院血管异常诊所接受OK-432治疗的所有诊断为LM的患者进行回顾性研究。

结果

27名患者纳入研究。60%的病变在2岁时出现,大多数位于头颈部(59%)。每位患者硬化治疗的平均次数为1.4次。透视引导下的治疗发现了3种新的LM结构类型:开放细胞微囊型、封闭细胞微囊型和淋巴管型。19例大囊型病变中的14例以及2例混合性病变中的1例对治疗反应完全或良好。开放细胞微囊型LM的3例中有3例反应完全或良好,这归因于OK-432可在囊肿间自由连通。封闭细胞微囊型LM有局部囊肿,不允许OK-432自由连通,与部分反应相关,2例中有2例。淋巴管型有部分反应。有2例轻微并发症和1例复发情况。

结论

3种新的LM结构类型的发现扩展了当前公认的分类,包括:开放细胞微囊型、封闭细胞微囊型和淋巴管型。OK-432治疗的大多数完全反应见于大囊型病变。开放细胞微囊型病变对OK-432的反应优于封闭细胞微囊型病变,淋巴管型可能对OK-432有反应。这些关键的结构-反应关系对OK-432硬化治疗具有直接的临床意义。

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