Congenital pulmonary airway malformations: state-of-the-art review for pediatrician’s use

Abstract

Surgery remains the cornerstone treatment of symptomatic lesions but the postnatal management of asymptomatic CPAM remains controversial. There are pros and cons of surgical resection, as increasing rate of infections over time renders the surgery more difficult after months or years of evolution, as well as risk of malignancy, though exact incidence is still unknown. What is known: • Congenital pulmonary airway malformations (CPAM) are rare developmental lung malformations mainly antenatally diagnosed. • While the neonatal management of symptomatic CPAM is clear and includes prompt surgery, controversies remain for asymptomatic CPAM due to risk of infections and malignancies. What is new: • Increased rate of infection over time renders the surgery more difficult after months or years of evolution and pushes for recommendation of early elective surgery. • New molecular or pathological pathways may help in the distinction of type 4 CPAM from type I pleuropulmonary blastoma.

Further delineation of the phenotypic spectrum of nevus comedonicus syndrome to include congenital pulmonary airway malformation of the lung and aneurysm

“…Congenital pulmonary airway malformations are developmental abnormalities of the lung that consist of a spectrum of cystic and noncystic lung lesions. The incidence of CPAMs is estimated at 1 in 11,000 to 1 in 35,000 live births (Gornall, Budd, Draper, Konje, & Kurinczuk, ; Laberge et al, ; Leblanc et al, ). CPAMs are usually sporadic and do not recur in families.…”

“…Congenital pulmonary airway malformations are developmental abnormalities of the lung that consist of a spectrum of cystic and noncystic lung lesions. The incidence of CPAMs is estimated at 1 in 11,000 to 1 in 35,000 live births (Gornall, Budd, Draper, Konje, & Kurinczuk, 2003;Laberge et al, 2001;Leblanc et al, 2017) (Kaliyadan, Nampoothiri, Sunitha, & Kuruvilla, 2010;Patrizi, Neri, Fiorentini, & Marzaduri, 1998;Pavithra, Pai, Mallya, & Pai, 2011;Ferrari et al, 2015;Suite and Mahabir, 1994;Seo, Piao, Suhr, Lee, & Park, 2001;Yadav, Mendiratta, Rana, & Chander, 2015;Qian, Liu, Zhou, & Zhang, 2015;Engber, 1978Engber, , 1982Whyte, 1968;Ghelbazouri et al, 2007 (Baker & Agim, 2014) and Alagille syndrome (Woods, Larcher, & Harper, 1994). Abbreviation: Unk, unknown.…”

A Missed Late Presentation of a Congenital Pulmonary Airway Malformation as a Large Infected Bulla

“…Nevertheless, most CPAM patients remain asymptomatic and otherwise healthy. 16 Previous research has shown post-lobectomy lung volumes in asymptomatic CPAM patients to be 90% that of predicted normal values. 19…”

“…Among symptomatic infants with CPAMs, urgent surgical excision is usually advocated because segmental resection may be incomplete, resulting in recurrent infections or persistent pneumothorax. [15][16][17][18] Among 19 surgically-treated infants and children, Pinter et al reported respiratory infections and chest deformities occurring in seven and eight patients, respectively, over an average follow-up period of 7.7 years. 18 However, in the current case, the patient underwent VATS dissection of the left upper lobe bulla.…”

Uniportal Thoracoscopic Lobectomy for Congenital Lung Malformations in Children

“…The standard treatment for congenital lung malformation is anatomical resection [1,2]. Since the first case in 1993 and the first large series of Video-Assisted Thoracoscopic Surgery (VATS) lobectomy reported by Dr. Rothenburg in 2000 [3], minimal invasive surgeries for paediatric congenital lung lesions have become increasingly popular worldwide [4][5][6].…”