Microsurgical treatment of primary lymphedema with interstitial lung disease: a report of 13 cases
10.3760/cma.j.cn441206-20240131-00035
- VernacularTitle:原发性淋巴水肿伴肺间质病变显微外科治疗13例
- Author:
Chen LIANG
1
;
Song XIA
;
Kun CHANG
;
Zhong LIU
;
Xin LIU
;
Ran AN
;
Jianfeng XIN
;
Yuguang SUN
;
Wenbin SHEN
Author Information
1. 首都医科大学附属北京世纪坛医院淋巴外科,北京 100038
- Keywords:
Primary lymphedema;
Interstitial lung disease;
Pulmonary lymphatic flow disorder;
Direct lymphangiography;
Lymphatic duct plasty
- From:
Chinese Journal of Microsurgery
2024;47(4):375-381
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the characteristics and diagnostic methods for the patients with primary lymphedema and interstitial lung disease, as well as the efficacy of thoracic duct or right lymphatic duct plasty.Methods:A retrospective study was performed on 13 patients who were treated for primary lymphedema in the Department of Lymphatic Surgery, Beijing Shijitan Hospital, Capital Medical University, from January 2018 to December 2022. All patients were confirmed with interstitial lung disease by high-resolution CT (HRCT) and then underwent thoracic duct or right lymphatic duct plasty surgery. The 13 patients in this study were 7 males and 6 females aged 7.3 (0.7-30.0) years old. Primary lymphedema was the first manifestation in all patients, with an average morbidity age at 1.0 (0-11.0) years old. The average time from the onset of lymphedema to the confirmed interstitial lung disease was 6.3 (0.3-19.0) years. All patients underwent thoracic duct or right lymphatic duct plasty for improvement of the lymphatic flow. In addition, 9 cases (69.2%) received a direct lymphangiography (DLG). In which, 4 cases (44.4%) received further treatment of ligation of the reflux branches, and 1 (11.1%) had the treatment plans changed, because a chylous reflux into the lung through a bronchomediastinal trunk was found from DLG, therefore a ligation of the abnormal reflux branches was performed and a life-long strict low-fat diet was required. Postoperative follow-ups for evaluation of the improvements of lymphedema and pulmonary lymphatic flow were carried out by visit of outpatient clinic or via telephone interviews.Results:All patients recovered well after surgery with an average follow-up time of 47.5 (19.0-68.0) months. Lymphedema was stable in 2 cases (15.4%) and relieved in 11 cases (84.6%). Ten cases (76.9%) had completed the postoperative HRCT reviews and 9 (90.0%) were found with significant improvement in the interstitial lung disease.Conclusion:The interstitial lung disease found during the examination of patients with primary lymphedema can be explained with a lymphatic flow disorder. DLG can identify the cause and make to guide the treatment. Thoracic duct or right lymphatic duct plasty can effectively improve both of the symptom of lymphedema and interstitial lung disease.
