Vobruba Vaclav, Votruba Jiri, Bělohlávek Jan, Grus Tomas, Leden Pavel, Zaoral Tomas, Hřídel Jan
1 Department of Paediatrics and Adolescent Medicine, General University Hospital, Prague, Czech Republic.
2 Department of Tuberculosis and Respiratory Disorders, General University Hospital, Prague, Czech Republic.
Perfusion. 2018 Oct;33(7):599-601. doi: 10.1177/0267659118768153. Epub 2018 Apr 15.
Lung agenesis is a rare disorder with a variable, but potentially very bad clinical course. It necessitates complex clinical management, especially in life-threatening situations.
We describe a case of a 6-month-old girl with right lung agenesis who required venovenous extracorporeal membrane oxygenation (VV-ECMO) due to pneumonia complicated by exacerbated previously diagnosed left main bronchus stenosis. The stenosis was resolved by endobronchial intervention and X-ray-guided stent insertion, which enabled weaning from ECMO and was aimed at preventing such a life-threatening respiratory failure in the future. Unfortunately, even with the functional stent, the baby died 2 months post-procedure due to unresolvable bronchial spasms.
Despite high endobronchial stenting-related mortality in children, in cases where no effective pharmacological or surgical alternatives exist, stenting may be safely performed during VV-ECMO support and be a viable option to overcome critical respiratory failure caused by bronchial stenosis.
肺不发育是一种罕见的疾病，其临床病程多变，但可能非常凶险。它需要复杂的临床管理，尤其是在危及生命的情况下。
我们描述了一例6个月大的右肺不发育女童，因肺炎并发先前诊断的左主支气管狭窄加重而需要静脉-静脉体外膜肺氧合（VV-ECMO）治疗。通过支气管内介入和X线引导下支架置入解决了狭窄问题，这使得能够撤离ECMO，并旨在预防未来出现这种危及生命的呼吸衰竭。不幸的是，即使有了功能性支架，婴儿在术后2个月仍因无法缓解的支气管痉挛而死亡。
尽管儿童支气管内支架置入相关死亡率较高，但在没有有效的药物或手术替代方案的情况下，在VV-ECMO支持期间可以安全地进行支架置入，并且是克服由支气管狭窄引起的严重呼吸衰竭的可行选择。
