Diffuse pulmonary lymphangiomatosis in a child
10.3760/cma.j.cn114453-20210106-00002
- VernacularTitle:儿童弥漫性肺淋巴管瘤病1例
- Author:
Buhao SUN
1
;
Tao HAN
;
Jun YAN
;
Sheng CHEN
;
Yuan WANG
;
Jie CUI
;
Weimin SHEN
Author Information
1. 南京医科大学附属儿童医院烧伤整形外科，南京 210008
- Keywords:
Lymphangiomatosis;
Operation;
Sirolimus;
Chylothorax
- From:
Chinese Journal of Plastic Surgery
2022;38(3):291-294
- CountryChina
- Language:Chinese
-
Abstract:
An 8-year-old female child was admitted to Children’s Hospital of Nanjing Medical University due to paroxysmal dyspnea on July 19, 2019. After thorough examination, she was considered to suffer from pulmonary lymphangioma and underwent an exploratory thoracotomy. The diffuse distribution of lymphangioma was seen during surgery, and part of the lymphangioma tissues was excised for histopathology. The patient was diagnosed as diffuse pulmonary lymphangiomatosis according to the history, CT imaging and postoperative pathology. She was treated with sirolimus at a starting dose of 0.9 mg every 12 h. Through monitoring the blood drug concentration regularly and adjusting the drug dose, the blood drug concentration was maintained at 10-15 ng/ml. Two weeks later, the child’s dyspnea improved but she developed refractory chylothorax, which was considered related to surgical wound. Considering that the child had thoracic drainage tubes for a long time, 10 mg of betamethasone, which was dissolved in 20 ml of physiological saline, was injected into each drainage tube to reduce the effusion of pleural effusion, and the drainage tubes were successfully removed after 3 injections. Unfortunately, the child’s symptoms recurred two months later, and she eventually died of respiratory failure despite active treatment, with a duration of nearly one year. The earlier the onset of diffuse pulmonary lymphangiomatosis occurs, the worse the prognosis is. Surgical resection may not be appropriate for such diffuse lesions, and there is a risk of refractory chylothorax, further research should explore the pathogenesis of the disease and targeted drug therapy.
