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淋巴管畸形的管理

Management of lymphatic malformations.

作者信息

Bloom David C, Perkins Jonathan A, Manning Scott C

机构信息

Department of Otolaryngology/Head and Neck Surgery University of Washington, Division of Pediatric Otolaryngology Children's Hospital and Regional Medical Center, Seattle, Washington 98105, USA.

出版信息

Curr Opin Otolaryngol Head Neck Surg. 2004 Dec;12(6):500-4. doi: 10.1097/01.moo.0000143971.19992.2d.

Abstract

PURPOSE OF REVIEW

Innovative otolaryngologists, plastic surgeons, craniofacial surgeons, pediatric surgeons, radiologists, anesthesiologists, neonatologists, obstetricians, and scientists have continued to advance our understanding of the etiology, diagnosis, and treatment of lymphatic malformations. This article reviews the publications over the past 2 years with respect to these advances.

RECENT FINDINGS

Fast-sequence MRI limits motion artifacts and allows prenatal MR to be used as a complementary study to ultrasound in the evaluation of large congenital neck masses. Three-dimensional ultrasonography may also be helpful in evaluating prenatal lymphatic malformations. Fluorescence in situ hybridization techniques can be used to evaluate lymphatic malformations for prenatal chromosomal analysis with emphasis on chromosomes 13, 18, 21, X, and Y. The sclerosing agent OK-432 is effective for macrocystic lymphatic malformations but showed less promise for microcystic lesions, mixed lesions, and lesions outside the head and neck region. Somnoplasty shows promise for reduction of tongue lymphatic malformations. Surgical excision, staged when necessary, continues to be integral to management in many cases.

SUMMARY

Basic science research has furthered understanding of lymphatic malformations. Clinical research has expanded and refined our diagnostic and therapeutic options for patients with these lesions. Further identification of genes selectively expressed by lymphatic endothelium should facilitate identification of usable vascular markers that can enable analysis of the underlying biology, physiology, pathology, and treatment of the lymphatic system and its malformations.

摘要

综述目的

创新型耳鼻喉科医生、整形外科医生、颅面外科医生、儿科外科医生、放射科医生、麻醉科医生、新生儿科医生、产科医生和科学家们不断推动我们对淋巴管畸形的病因、诊断和治疗的理解。本文回顾了过去两年中关于这些进展的出版物。

最新发现

快速序列磁共振成像(MRI)可减少运动伪影,使产前MRI能够作为超声的补充检查,用于评估先天性颈部大肿块。三维超声检查在评估产前淋巴管畸形方面也可能有帮助。荧光原位杂交技术可用于评估淋巴管畸形的产前染色体分析,重点是13、18、21、X和Y染色体。硬化剂OK-432对大囊型淋巴管畸形有效,但对微囊型病变、混合型病变以及头颈部以外区域的病变效果欠佳。睡眠成形术有望减少舌部淋巴管畸形。在许多情况下,必要时分阶段进行手术切除仍是治疗的重要组成部分。

总结

基础科学研究加深了对淋巴管畸形的理解。临床研究扩展并完善了我们对这些病变患者的诊断和治疗选择。进一步鉴定淋巴管内皮细胞选择性表达的基因,应有助于识别可用的血管标志物,从而能够分析淋巴系统及其畸形的基础生物学、生理学、病理学和治疗方法。

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