Department of Intensive care, Flinders Medical Centre, Bedford Park, 5042 South Australia, Australia.
BMC Pulm Med. 2014 Mar 10;14:41. doi: 10.1186/1471-2466-14-41.
Lobular Capillary Hemangioma (LCH) is a benign tumour that is known to be hormone responsive and have a relatively high incidence during pregnancy, the most common site being the gingival surfaces. A tracheal origin for this tumour is extremely rare, with no case reported so far in this patient population, and the only reported clinical presentation of tracheal LCH in the literature is with haemoptysis.
We describe a case of a 23-year-old known asthmatic who presented at 32 weeks gestation with life-threatening respiratory failure resembling acute severe asthma, requiring invasive ventilation which was extremely difficult. This was subsequently found to be due to a large tracheal LCH producing a ball-valve phenomenon and predominantly expiratory airflow limitation similar to acute asthma. The endotracheal tube was advanced past the lesion under bronchoscopic guidance, and urgent Caesarean section performed due to foetal distress. The tumour was subsequently debulked and the trachea stented, facilitated by bi-femoral veno-venous extra-corporeal membrane oxygenation with relatively low dose of heparin.
To our knowledge, this is the first report of a unique presentation and management of largest tracheal LCH so far occurring during pregnancy. Pulmonary and critical care physicians should be aware of this unique differential of refractory asthma, the aggressive nature of this benign tumour due to hormonal influences during pregnancy, and feasibility of using bi-femoral veno-venous extra-corporeal membrane oxygenation with low dose heparin as a rescue, given the high risk of bleeding.
乳腺叶状毛细血管瘤（lobular capillary hemangioma，LCH）是一种良性肿瘤，已知对激素有反应，在怀孕期间发病率相对较高，最常见的部位是牙龈表面。这种肿瘤起源于气管的情况极为罕见，到目前为止，在该患者群体中尚未报道过这种病例，而文献中唯一报道的气管 LCH 的临床表现是咯血。
我们描述了一例 23 岁已知患有哮喘的患者，在妊娠 32 周时因危及生命的呼吸衰竭而就诊，类似于急性严重哮喘，需要进行侵入性通气，这极其困难。后来发现这是由于一个大的气管 LCH 导致球瓣现象和主要为呼气气流受限，类似于急性哮喘。在支气管镜引导下将气管内导管推进病变部位，并因胎儿窘迫紧急行剖宫产术。随后，在双股股静脉体外膜肺氧合的支持下，使用相对低剂量肝素，迅速切除肿瘤并对气管进行支架置入。
据我们所知，这是迄今为止首例报道的妊娠期发生的最大气管 LCH 的独特表现和治疗方法。肺部和危重病医生应该意识到这种独特的难治性哮喘的鉴别诊断，由于妊娠期间激素的影响，这种良性肿瘤具有侵袭性，以及在高出血风险的情况下，使用双股股静脉体外膜肺氧合联合低剂量肝素作为抢救治疗的可行性。
