Analysis of clinical diagnosis and treatment of complex lymphatic anomalies
10.3760/cma.j.cn114453-20220624-00191
- VernacularTitle:复杂性淋巴管畸形临床诊断分析及综合治疗
- Author:
Tong QIU
1
;
Jiangyuan ZHOU
;
Xue GONG
;
Kaiying YANG
;
Zixin ZHANG
;
Yuru LAN
;
Xuepeng ZHANG
;
Zilong ZHOU
;
Geng ZHANG
;
Jianlei FU
;
Siyuan CHEN
;
Yi JI
Author Information
1. 四川大学华西医院小儿外科，成都 610041
- Keywords:
Lymphatic abnormalities;
Clinical characteristics;
Differential diagnosis;
Treatment;
Disease prognosis
- From:
Chinese Journal of Plastic Surgery
2023;39(11):1167-1174
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To analyze the clinical features, differential diagnosis, treatment and prognosis of complex lymphatic malformations.Methods:The clinical data of patients with complex lymphatic malformation were retrospectively analyzed from April 2010 to April 2022 in the Multidisciplinary Outpatient Department of the Vascular Disease Team of West China Hospital, Sichuan University. All patients were diagnosed with complex lymphatic malformation after consultation with multidisciplinary experts in pediatric surgery, radiology, plastic surgery, pathology, rehabilitation and other departments. The clinical manifestations, blood routine, coagulation function, magnetic resonance imaging and treatment methods of the patients were analyzed. According to the follow-up and disease results, the patients were divided into improvement, stability, progress and death.Results:A total of 18 patients with complex lymphatic malformations were included in the study, including 6 males and 12 females. The age of first diagnosis ranged from 1 month to 29 years old, and the median age was 2.5 years old. Patients were followed up and treated for 0.4 to 12.0 years, with an average follow-up of 3.5 years. Ten patients had pleural and pericardial effusion; 15 patients had visceral involvement which showed multifocal changes in imaging examinations; 9 cases were accompanied by bone destruction, which in Gorham-Stout disease patients broke through the cortex while in generalized lymphatic anomalies it did not; 14 patients had various degrees of coagulation abnormalities, of which 8 patients with severe coagulation dysfunction were all diagnosed as kaposiform lymphangiomatosis. Of the 18 patients, one kaposiform lymphangiomatosis patient died; six patients progressed; eight patients were stable; and three patients improved.Conclusion:The clinical characteristics of patients with complex lymphatic malformations are systemic, diverse and complex. The clinical symptoms of patients with diffuse lymphatic malformation accompanied by involvement of bone and multiple internal organs, chest and abdominal effusion, and coagulation dysfunction should be considered as complex lymphatic malformation. However, due to overlapping clinical characteristics of each subtypes, it is difficult to distinguish patients with complex lymphatic malformation, and the curative effect and prognosis are poor. Precision targeted drugs are the future research direction for the treatment of such diseases.
