Lymphology Center of Department of Plastic & Reconstructive Surgery, Shanghai 9th People's Hospital, Shanghai Jiao Tong University School of Medicine, 639 Zhi Zao Ju Road, Shanghai 200011, China.
Eur J Vasc Endovasc Surg. 2012 Sep;44(3):345-9. doi: 10.1016/j.ejvs.2012.06.019. Epub 2012 Jul 23.
The study aims to investigate lymphatic-system malformations and proposes a classification of primary lymphoedema based on comprehensive imaging data of both lymph vessel- and lymph-node abnormalities.
A total of 378 patients with primary lymphoedema of the lower extremity were examined with magnetic resonance lymphangiography (MRL) using gadobenate dimeglumine as contrast agent. Lymph vessels and drainage lymph nodes were evaluated, leading to the proposal of the classification of primary lymphoedema and the relative proportions.
A total of 63 (17%) patients exhibited defects of the inguinal lymph nodes with mild or moderate dilatation of afferent lymph vessels. A total of 123 (32%) patients exhibited lymphatic anomalies as lymphatic aplasia, hypoplasia or hyperplasia with no obvious defect of the drainage lymph nodes. The involvement of both lymph vessel- and lymph-node abnormalities in the affected limb was found in 192 (51%) patients. The primary lymphoedema was classified as three major types as: (1) lymph nodes affected only; (2) lymph vessel affected only with three subtypes and (3) both lymph vessel and lymph node affected with subgroups.
A comprehensive classification of lymphatic-system malformation in primary lymphoedema is proposed, which clearly defines the location and pathologic characteristics of both lymphatics and lymph node and may lead to further study of the aetiology as well as rational treatment of the disease.
本研究旨在探讨淋巴系统畸形，并基于淋巴管和淋巴结异常的综合影像学数据提出原发性淋巴水肿的分类。
对 378 例下肢原发性淋巴水肿患者进行磁共振淋巴造影术（MRL）检查，使用钆贝葡胺作为对比剂。评估淋巴管和引流淋巴结，提出原发性淋巴水肿的分类及其相对比例。
共有 63 例（17%）患者腹股沟淋巴结存在缺陷，伴输入淋巴管轻度或中度扩张。共有 123 例（32%）患者存在淋巴管异常，表现为淋巴管发育不全、发育不良或增生，无明显引流淋巴结缺陷。192 例（51%）患者受累肢体同时存在淋巴管和淋巴结异常。将原发性淋巴水肿分为三种主要类型：（1）仅淋巴结受累；（2）仅淋巴管受累，分为三个亚型；（3）淋巴管和淋巴结均受累，分为亚组。
本研究提出了一种全面的原发性淋巴水肿淋巴系统畸形分类，明确了淋巴管和淋巴结的位置和病理特征，可能有助于进一步研究病因，并为疾病的合理治疗提供依据。
