Filbrich Maike, Brisbois Denis, Lebrun Yves, Godin Pierre-Arnaud, Verscheure Sara
Cliniques Universitaires Saint Luc, Bruxelles, Belgium.
Clinique MontLégia CHC, Liege, Belgium.
Obstet Med. 2024 Jun;17(2):112-115. doi: 10.1177/1753495X221145809. Epub 2022 Dec 15.
We report our experience of managing a massive haemothorax caused by a ruptured, previously unknown, pulmonary arteriovenous malformation (pAVM) at 34 + 5 weeks of gestation, which proved to be a manifestation of hereditary haemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome. The patient underwent an emergency caesarean section under general anaesthesia after placement of a chest tube and gave birth to a healthy infant. A postoperative thoracic computed tomography angiography highlighted the presence of the large pAVM. Transcatheter embolization was performed right after the delivery. Subsequent patient's anamnesis, family history and genetic analysis finally revealed the presence of the syndrome. The aim of our report is to create awareness of this serious condition with potential life-threatening complications, especially in pregnancy. Simple criteria have been published and allow to easily consider HHT and the presence of potential AVM during anamnesis, ideally even before pregnancy.
我们报告了在妊娠34 + 5周时处理一例由破裂的、此前未知的肺动静脉畸形(pAVM)引起的大量血胸的经验,该肺动静脉畸形被证明是遗传性出血性毛细血管扩张症(HHT)的一种表现,HHT也被称为奥斯勒 - 韦伯 - 伦杜综合征。患者在放置胸管后接受全身麻醉下的急诊剖宫产,产下一名健康婴儿。术后胸部计算机断层扫描血管造影突出显示了大的pAVM的存在。分娩后立即进行了经导管栓塞术。随后患者的既往史、家族史和基因分析最终揭示了该综合征的存在。我们报告的目的是提高对这种具有潜在危及生命并发症的严重疾病的认识,尤其是在妊娠期间。已经公布了简单的标准,便于在问诊时,理想情况下甚至在怀孕前,轻松考虑HHT和潜在AVM的存在。