Kaposiform lymphangiomatosis in a newborn
10.3760/cma.j.cn113903-20230920-00217
- VernacularTitle:新生儿卡波西样淋巴管瘤病1例
- Author:
Chao HUANG
1
;
Yanfen PENG
;
Junjian LYU
;
Huilin NIU
;
Qiuming HE
;
Wei ZHONG
Author Information
1. 广州市妇女儿童医疗中心新生儿外科监护室，广州 510623
- Keywords:
Lymphatic abnormalities;
Lymphangiectasis;
Infant, newborn
- From:
Chinese Journal of Perinatal Medicine
2023;26(10):865-868
- CountryChina
- Language:Chinese
-
Abstract:
This article reported a case of kaposiform lymphangiomatosis (KLA) identified in the fetal stage and diagnosed at the neonatal stage. A routine ultrasound examination at 19 weeks of gestation showed multiple masses in the whole body of the fetus (involving neck, chest wall and armpit) complicated by pleural and peritoneal effusion. Shunting was performed to drain pleural effusion from the right chest in another hospital at 26 +5 weeks of gestation. The patient was born at 34 +3 weeks of gestation by cesarean section due to "intrauterine distress" and required invasive ventilator assisted ventilation support after birth because of respiratory distress. A large amount of hemorrhagic effusion was drained out during the shunting. Coagulation dysfunction and thrombocytopenia occurred on the 3rd day after birth and KLA was suspected. Empirical treatment with sirolimus turned out to be ineffective. Biopsy was taken on postnatal day 7. However, the patient died on the 12th day after birth due to respiratory and circulatory failure. Pathological findings obtained the day after death were consistent with the features of KLA. The diagnosis of KLA was confirmed based on the clinical manifestations and pathological results.
