先天性肺气道畸形的诊治现状和问题

Diagnosis and treatment of congenital pulmonary airway malformation:Recent progress and ongoing questions

先天性肺气道畸形(CPAM)作为最常见的先天性肺发育畸形,是一组可累及气道和肺实质的异质性罕见病。随着医学影像学技术的发展和人们健康体检意识的增强,在胎儿期CPAM被越来越多地发现,给临床医师在产前和产后患儿管理方面带来了新的挑战。部分CPAM患者由产前超声和常规体检发现,还有一部分患者因出现结构性或感染性并发症于就诊时发现。对于CPAM的流行病学、无症状患者的治疗方式和随访时间等存在争议,国内外尚无统一的CPAM诊疗指南。2021年,中国多位小儿外科专家联合发表《先天性肺气道畸形诊疗中国专家共识(2021版)》指导临床医师工作。本文着重介绍CPAM的诊治现状,并讨论疾病自然史、CPAM发展为恶性肿瘤等尚存争议的问题。

As the most frequent congenital lung malformation,congenital pulmonary airway malformation(CPAM)is a rare and heterogeneous disorder involving the airways and lung parenchyma.With the continuous progress of medical imaging technology and the increase of health awareness,CPAM is increasingly found in the fetal period,presenting clinicians with a unique set of challenges to prenatal and postpartum management.Some CPAM patients are found by prenatal ultrasound and routine physical examination,while others are diagnosed when they develop structural or infectious complications.Due to many aspects of the epidemiology,treatment of asymptomatic patients,and follow-up time remaining mired in controversy,there is no unified management guideline of CPAM at home and abroad.In 2021,a number of pediatric surgeons in China jointly published the"National Expert Consensus on Diagnosis&Treatment of Congenital Pulmonary Airway Malformations in China(2021)".Our article offered a clinically focused review of recent advances in the diagnosis and treatment of CPAM and discussed the remaining controversial issues like the natural history and malignant transformation.