Xie Yewei
Department of Pediatric Cardiothoracic Surgery, Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai, China.
Transl Pediatr. 2021 Apr;10(4):1034-1038. doi: 10.21037/tp-20-356.
Hepatic pulmonary fusion (HPF) is a very rare congenital disease which is characterized by a fibrous connection between the liver and lung tissues. It is commonly associated with congenital diaphragmatic hernia (CDH), pulmonary sequestration, congenital heart disease and other diseases. Surgical operation has been reported to be the only option for the treatment of this disease. The most sophisticated point lies in how to define the dividing line between liver and lung fusion tissues. And the postoperative mortality is high. At present, the etiology and pathogenesis of HPF are not completely clear. In this study, we reported a case of a 4-month-old male infant presented with cough and shortness of breath and intraoperatively found to have HPF associated with atrial septal defect and scimitar syndrome. Staged surgery was performed to avoid the simultaneous involvement of multiple organs such as heart, lung and liver, and shorten the operation and anesthesia time to a certain extent, improving the success rate of the operation. We only separated the fusion tissues and repaired the diaphragmatic hernia in the first operation, and in the second surgery, we conducted intra-cardiac repair of cardiac malformations. The follow-up results showed that the right lung gradually developed and there were no significant abnormalities in liver. This experience can provide a useful reference for future cases.
肝肺融合(HPF)是一种非常罕见的先天性疾病,其特征是肝组织与肺组织之间存在纤维连接。它通常与先天性膈疝(CDH)、肺隔离症、先天性心脏病等疾病相关。据报道,手术是治疗这种疾病的唯一选择。最复杂的点在于如何界定肝肺融合组织之间的分界线。而且术后死亡率很高。目前,HPF的病因和发病机制尚不完全清楚。在本研究中,我们报告了一例4个月大的男婴,出现咳嗽和呼吸急促症状,术中发现患有与房间隔缺损和弯刀综合征相关的HPF。进行了分期手术,以避免心脏、肺和肝等多个器官同时受累,并在一定程度上缩短手术和麻醉时间,提高手术成功率。我们在第一次手术中仅分离了融合组织并修复了膈疝,在第二次手术中,对心脏畸形进行了心内修复。随访结果显示右肺逐渐发育,肝脏无明显异常。这一经验可为未来的病例提供有益参考。