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儿童巨大头颈部淋巴管畸形的管理

The Management of Giant Cerviofacial Lymphatic Malformations in Children.

作者信息

Li Wanpeng, Xu Hongming, Zhao Liming, Li Xiaoyan

机构信息

Department of Otolaryngology-Head and Neck Surgery, Shanghai Children's Hospital, Shanghai Jiao Tong University.

Department of Otorhinolaryngology, EYE and ENT Hospital of Fudan University, Shanghai, China.

出版信息

J Craniofac Surg. 2019 Sep;30(6):e553-e555. doi: 10.1097/SCS.0000000000005520.

Abstract

OBJECTIVE

Lymphatic malformations (LMs) are rare and benign diseases. This article aimed to present a series of surgically treated giant cervicofacial LMs in children.

METHODS

A retrospective analysis of giant cervicofacial LMs (tumor diameter greater than 10 cm) in children over the past 8 years was performed in our department.

RESULTS

The 10 patients included 4 males and 6 females. Macrocystic lesion presented in 4 patients, mixed cystic lesion presented in 4 patients, and microcystic lesion presented in 2 patients. All the patients underwent surgical resection combined with bleomycin sclerotherapy. Despite the wide range of lesions, most patients (7/10, 70%) received only a single treatment, 3 patients had a recurrent lesion. Seven patients experienced complete or almost complete resolution of the disease with an average follow-up time of 26.4 months.

CONCLUSIONS

Surgical resection is the preferred method for the treatment of giant LMs, combination of bleomycin sclerotherapy can reduce the recurrence rate. The curative effect of macrocystic disease is relatively enhanced compared with microcystic and mixed cystic diseases.

摘要

目的

淋巴管畸形(LMs)是一种罕见的良性疾病。本文旨在介绍一系列经手术治疗的儿童巨大头颈部淋巴管畸形。

方法

对我科过去8年收治的儿童巨大头颈部淋巴管畸形(肿瘤直径大于10厘米)进行回顾性分析。

结果

10例患者中,男性4例,女性6例。4例为大囊型病变,4例为混合囊型病变,2例为微囊型病变。所有患者均接受手术切除联合博来霉素硬化治疗。尽管病变范围广泛,但大多数患者(7/10,70%)仅接受了一次治疗,3例患者出现病变复发。7例患者疾病完全或几乎完全缓解,平均随访时间为26.4个月。

结论

手术切除是治疗巨大淋巴管畸形的首选方法,联合博来霉素硬化治疗可降低复发率。与微囊型和混合囊型疾病相比,大囊型疾病的疗效相对更好。

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