哮喘联盟

   摘要
   背景：小叶毛细血管瘤（LCH）是一种与孕激素反应相关、发生率较高的良性肿瘤，最常见于齿龈表面。以气管为源点的这类肿瘤极其罕见，到目前为止还没有任何报道，仅文献中有气管LCH伴咯血的临床表现描述。
   病例介绍：一例23岁哮喘患者，怀孕32周出现哮喘急性发作呼吸衰竭，需要侵入性通气但难操作非常困难。后来发现这是由于出现大量气管LCH产生ball-valve现象，呼气气流受阻表现为急性哮喘发作。因胎儿窘迫，在支气管镜引导下行气管内导管，使其通过病变部位行紧急剖腹产。随后肿瘤被切除，行气管支架术，低剂量肝素双侧股静脉-静脉体外膜肺支持治疗。
   总结：据我们所知，这是目前为针对孕期巨大LCH独立特征与治疗的首次报道。肺科和重症监护科医生应该注意这一罕见的有别于难治性哮喘，受孕期激素影响的进展性良性肿瘤，以及使用双侧股静脉-静脉体外膜肺氧合伴低剂量肝素治疗，避免高出血风险的可行性。
 

（苏楠 审校）
BMC Pulm Med. 2014 Mar 10;14(1):41. [Epub ahead of print]
 

A rare case of rapidly enlarging tracheal lobular capillary hemangioma presenting as difficult to ventilate acute asthma during pregnancy.
 

Prakash S, Bihari S, Wiersema U.
 

Abstract
BACKGROUND: 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.
CASE PRESENTATION: 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.
CONCLUSION: 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.

BMC Pulm Med. 2014 Mar 10;14(1):41. [Epub ahead of print]