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乳糜胸和乳糜性腹水:管理与陷阱

Chylothorax and chylous ascites: management and pitfalls.

作者信息

Lopez-Gutierrez Juan C, Tovar Juan A

机构信息

Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain; Department of Pediatrics, Universidad Autonoma de Madrid, Madrid, Spain.

Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain; Department of Pediatrics, Universidad Autonoma de Madrid, Madrid, Spain.

出版信息

Semin Pediatr Surg. 2014 Oct;23(5):298-302. doi: 10.1053/j.sempedsurg.2014.09.011. Epub 2014 Sep 4.

Abstract

Leakage of lymph from the lymphatic ducts causes chylothorax (CT) or chylous ascitis (CA). This may happen for unknown reasons during fetal life or after birth and may also be caused by trauma after thoracic surgery or by other conditions. Fetal CT and CA may be lethal particularly in cases with fetal hydrops that sometimes benefit of intra-uterine instrumentation. After birth, symptoms are related to the amount of accumulated fluid. Sometimes, severe cardio-respiratory compromise prompts active therapy. Most patients with CT or CA benefit from observation, rest, and supportive measures alone. Drainage of the fluid may be necessary, but then loss of protein, fat, and lymphoid cells introduce new risks and require careful replacement. Low-fat diets with MCT and parenteral nutrition decrease fluid production while allowing adequate nutritional input. If lymph leakage does not stop, secretion inhibitors like somatostatin or octreotide are prescribed, although there is only weak evidence of their benefits. Imaging of the lymphatic system is indicated when the leaks persist, but this is technically demanding in children. Shunting of the lymph from one body space to another by means of valved catheters, embolization of the thoracic duct, and/or ligation of the major lymphatics may occasionally be indicated in cases refractory to all other treatments.

摘要

淋巴管的淋巴漏可导致乳糜胸(CT)或乳糜性腹水(CA)。这可能在胎儿期或出生后不明原因地发生,也可能由胸外科手术后的创伤或其他情况引起。胎儿CT和CA可能是致命的,尤其是在伴有胎儿水肿的情况下,有时宫内操作可能有益。出生后,症状与积液量有关。有时,严重的心肺功能不全需要积极治疗。大多数CT或CA患者仅通过观察、休息和支持措施即可获益。可能需要引流液体,但蛋白质、脂肪和淋巴细胞的流失会带来新的风险,需要仔细补充。含中链甘油三酯(MCT)的低脂饮食和肠外营养可减少液体生成,同时保证足够的营养摄入。如果淋巴漏不止,可使用生长抑素或奥曲肽等分泌抑制剂,尽管其益处的证据不足。当漏液持续存在时,需要对淋巴系统进行成像检查,但这对儿童来说技术要求较高。在所有其他治疗均无效的情况下,偶尔可能需要通过带瓣膜导管将淋巴从一个体腔分流到另一个体腔、胸导管栓塞和/或结扎主要淋巴管。

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