Pederiva Federica, Dalena Paolo, Pasqua Noemi, Bresesti Ilia, Testa Valeria, Zirpoli Salvatore, Gentilino Valerio
Pediatric Surgery, "F. Del Ponte" Hospital, ASST Settelaghi, Varese, Italy
Institute for Maternal and Child Health, IRCCS "Burlo Garofolo", Trieste, Italy.
Eur Respir Rev. 2025 Apr 2;34(176). doi: 10.1183/16000617.0254-2024. Print 2025 Apr.
Although there is agreement on surgically removing symptomatic congenital lung malformations (CLMs), the management of asymptomatic cases remains controversial. Some paediatric surgeons suggest conservative management for asymptomatic cases due to the low perceived risk of developing symptoms or malignancy. This study aimed to investigate the characteristics and management of adults with CLMs.
Following PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines, a systematic review was conducted to find studies reporting on adult patients (aged ≥15 years) with CLMs.
Out of 12 908 studies reviewed from 1947 to 2024, 653 studies met the inclusion criteria, covering 980 patients. None of the patients had a prenatal diagnosis. The mean±sd age at surgery was 39.4±15.7 years (range 15-86). A total of 74.4% of adults with CLMs presented with symptoms, leading to the detection of CLM. Pneumonia was the most common symptom in congenital pulmonary airway malformation (CPAM) and intralobar sequestration, with over 30% of these patients experiencing recurrent respiratory infections. In 11.7% of adults, CLMs harboured tumours at the time of surgical removal, with CPAM associated with lung tumours in over 20% of cases.
Thoracic surgeons recommend surgical resection for all adult CLM patients, including asymptomatic cases, due to risks of infections and malignancy. Conservative management was considered only when surgery was not feasible.
虽然对于手术切除有症状的先天性肺发育异常(CLM)已达成共识,但无症状病例的管理仍存在争议。一些小儿外科医生建议对无症状病例采取保守管理,因为出现症状或恶变的风险较低。本研究旨在调查成年CLM患者的特征及管理情况。
遵循PRISMA(系统评价和Meta分析的首选报告项目)指南,进行系统评价以查找报告成年患者(年龄≥15岁)CLM的研究。
在1947年至2024年检索的12908项研究中,653项研究符合纳入标准,涵盖980例患者。所有患者均未进行产前诊断。手术时的平均年龄±标准差为39.4±15.7岁(范围15 - 86岁)。共有74.4%的成年CLM患者出现症状,从而得以发现CLM。肺炎是先天性肺气道畸形(CPAM)和叶内型肺隔离症最常见的症状,超过30%的此类患者有反复呼吸道感染。11.7%的成年患者在手术切除时CLM伴有肿瘤,超过20%的CPAM病例与肺部肿瘤相关。
胸外科医生建议对所有成年CLM患者,包括无症状病例,进行手术切除,因为存在感染和恶变风险。仅在手术不可行时才考虑保守管理。