Respiratory and Sleep Department, Children's Hospital at Westmead, Westmead, New South Wales, Australia
The University of Sydney Discipline of Child and Adolescent Health, Westmead, New South Wales, Australia.
BMJ Case Rep. 2022 Jul 27;15(7):e245543. doi: 10.1136/bcr-2021-245543.
We present a case of a 7-day-old male infant with severe respiratory disease requiring venoarterial extracorporeal membrane oxygenation therapy with evidence of lymphangiectasia on lung biopsy. Differentiating primary versus secondary lymphangiectasis in this patient remains a riddle despite extensive investigations including an infective screen, lung biopsy and whole-genome sequencing. In addition to the standard therapies used in paediatric acute respiratory distress syndrome, such as lung-protective ventilation, permissive hypoxaemia and hypercarbia, nursing in the prone position, early use of muscle relaxants, rescue intravenous corticosteroids and broad-spectrum antibiotics, the patient was also given octreotide despite the absence of a chylothorax based on the theoretical benefit of altering the lymphatic flow. His case raises an interesting discussion around the role of lymphatics in the pathophysiology of paediatric and adult respiratory distress syndrome and prompts the exploration of novel agents which may affect lymphatic vessels used as an adjunctive therapy.
我们报告了一例 7 天大的男性婴儿，患有严重的呼吸道疾病，需要进行静脉动脉体外膜肺氧合治疗，肺活检显示有淋巴管扩张。尽管进行了广泛的检查，包括感染筛查、肺活检和全基因组测序，但仍难以区分该患者的原发性淋巴管扩张和继发性淋巴管扩张。除了儿科急性呼吸窘迫综合征的标准治疗方法，如肺保护性通气、允许性低氧血症和高碳酸血症、俯卧位护理、早期使用肌肉松弛剂、抢救性静脉皮质类固醇和广谱抗生素外，尽管根据改变淋巴液流动的理论益处，该患者没有乳糜胸，但仍给予奥曲肽。他的病例引发了关于淋巴系统在儿科和成人呼吸窘迫综合征病理生理学中的作用的有趣讨论，并促使探索可能影响用作辅助治疗的淋巴管的新型药物。
