Department of Neonatal and Developmental Medicine, Singapore General Hospital, Singapore
Haematology/Oncology Service, Department of Paediatric Subspecialties, KK Women's and Children's Hospital, Singapore.
BMJ Case Rep. 2021 Apr 16;14(4):e240688. doi: 10.1136/bcr-2020-240688.
A Chinese male infant was born at 35 weeks weighing 2935 g to a mother with polyhydramnios and prenatal hydrops fetalis. He developed marked respiratory distress secondary to bilateral congenital chylothorax and required pleural drainage, high frequency oscillation and inhaled nitric oxide therapy. He was extubated to non-invasive ventilation by day 14. There was no bacterial or intrauterine infection, haematologic, chromosomal or cardiac disorder. He was exclusively fed medium-chain triglyceride formula. High-resolution CT showed diffuse interstitial lung disease. He received a dexamethasone course for chronic lung disease to facilitate supplemental oxygen weaning. A multidisciplinary team comprising neonatology, pulmonology, haematology, interventional radiology and thoracic surgery considered congenital pulmonary lymphangiectasia as the most likely diagnosis and advised open lung biopsy, lymphangiography or scintigraphy for diagnostic confirmation should symptoms of chylothorax recur. Fortunately, he was weaned off oxygen at 5 months of life, and tolerated human milk challenge at 6 months of life and grew well.
一名中国男婴，胎龄 35 周，母亲羊水过多且胎儿有心包积液，出生体重 2935 克。因双侧先天性乳糜胸导致明显呼吸窘迫，需要进行胸腔引流、高频振荡和吸入一氧化氮治疗。出生后第 14 天，患儿拔管改为无创通气。患儿无细菌或宫内感染、血液学、染色体或心脏异常。患儿完全经口喂养中链甘油三酯配方奶。高分辨率 CT 显示弥漫性间质性肺病。为了促进吸氧撤离，患儿接受了地塞米松治疗慢性肺病。一个由新生儿科、呼吸科、血液科、介入放射科和胸外科组成的多学科团队认为最可能的诊断是先天性肺淋巴管扩张症，并建议在乳糜胸症状再次出现时，进行开胸肺活检、淋巴管造影或闪烁扫描以明确诊断。幸运的是，患儿在 5 个月大时成功脱氧，6 个月大时耐受人乳喂养，且生长良好。
