Current Management of Congenital Pulmonary Airway Malformations: A “European Pediatric Surgeons' Association” Survey

Aim To define current management of congenital pulmonary airway malformation (CPAM). Methods A total of 181 European Pediatric Surgeons' Association members (91% senior) from 48 countries completed an online questionnaire. Main Results Prenatal: 93% respondents work in centers with prenatal diagnosis facilities, and 27% in centers offering in utero surgery. Prenatal counseling is performed by 86% respondents, 22% of whom see >10 cases per year. Risk of single pre-/postnatal complications is deemed low (<5%) by more than 60% of respondents. Eighty-six percent respondents do not offer pregnancy termination for prenatally diagnosed CPAM. Fetal hydrops is the most frequent indication for termination (87%), followed by parental willingness (52%). Prenatal surgery is an option for 44% respondents, preferring thoracoamniotic shunt (82%).Postnatal: 75% respondents operate on asymptomatic patients, 18% before 6 months of age, 62% between 6 and 12 months of age, and 20% after 12 months of age. Risk of infection (86%), cancer (63%), and symptoms development (62%) are indications for surgery in asymptomatic CPAM. Sixty-three percent prefer a thoracotomy. Lobectomy is the preferred procedure (58% respondents). Motivations against surgery include lesion <1 cm (64%), risk of postoperative complications (37%), and lack of evidence favoring surgery (27%). Seventeen percent respondents have seen at least one patient with CPAM with lung cancer, in 89% of the cases within the CPAM. Of all the respondents, 83% and 22% offered dedicated follow-up and genetic screening, respectively. Conclusion Current pre- and postnatal management of CPAM lacks uniformity, particularly for surgical indication, timing, and approach. Efforts should be made toward standardization. Risk of CPAM-associated cancer is not clear.

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Thoracoscopic Lobectomy for Congenital Pulmonary Airway Malformation: Where Are We in 2019?

European Journal of Pediatric Surgery ◽

10.1055/s-0040-1702221 ◽

2020 ◽

Vol 30 (02) ◽

pp. 146-149

Author(s):

Arnaud Bonnard

Keyword(s):

Fast Track ◽

Thoracoscopic Surgery ◽

Pulmonary Function Tests ◽

Congenital Pulmonary Airway Malformation ◽

Adequate Treatment ◽

The Past ◽

Track Management ◽

Pulmonary Airway ◽

Tomography Scan

AbstractThoracoscopic surgery for congenital pulmonary airway malformation (CPAM) is still a matter of debate and used by approximately 50% of the surgeons in Europe. Several questions need to be addressed about CPAM. The adequate treatment, the surgical approach, and the follow-up are few of them. A review of recent articles published in the literature over the past 5 years is done in trying to respond to these questions. A multidisciplinary team is required to follow these patients since approximately 10 to 15% will develop a chronic lung disease and asthma. In the case of conservative management, computed tomography scan should be perform to monitor the evolution of the CPAM. Minimally invasive surgery should be used whenever possible, although the advantages of pulmonary function tests are not clearly defined, allowing a postoperative fast-track management.

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Refractory tension pneumothorax as a result of an internally displaced thoracoamniotic shunt in an infant with a congenital pulmonary airway malformation

BMJ Case Reports ◽

10.1136/bcr-2016-216324 ◽

2016 ◽

pp. bcr2016216324 ◽

Cited By ~ 1

Author(s):

Brenda Hiu Yan Law ◽

Ioana Bratu ◽

Venu Jain ◽

Marc-Antoine Landry

Keyword(s):

Tension Pneumothorax ◽

Congenital Pulmonary Airway Malformation ◽

Internally Displaced ◽

Pulmonary Airway ◽

Thoracoamniotic Shunt

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It is not pneumothorax: Multicystic lung lesion in a six-year-old child

Proceedings of Singapore Healthcare ◽

10.1177/2010105816664357 ◽

2016 ◽

Vol 26 (2) ◽

pp. 121-124

Author(s):

Eman Sobh ◽

Amira Bahour ◽

Shady Elia Anis

Keyword(s):

Surgical Resection ◽

Lung Development ◽

Lung Lesion ◽

Congenital Cystic Adenomatoid Malformation ◽

Congenital Pulmonary Airway Malformation ◽

Cystic Lung ◽

Cystic Adenomatoid Malformation ◽

Pulmonary Airway ◽

Cystic Lung Lesion

Congenital cystic adenomatoid malformation (CCAM) – recently reported as congenital pulmonary airway malformation – is a rare abnormality of lung development. We present a case of a six-year-old child having a cystic lung lesion that proved to be CCAM after surgical resection. The child is performing well on follow-up.

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Squamous metaplasia in the cyst epithelium of type 1 congenital pulmonary airway malformation after thoracoamniotic shunt placement

Human Pathology ◽

10.1016/j.humpath.2011.10.017 ◽

2012 ◽

Vol 43 (9) ◽

pp. 1413-1417

Author(s):

Kentaro Matsuoka ◽

Satoshi Hayashi ◽

Fumihiko Urano ◽

Lihua J. Zhu ◽

Hajime Okita ◽

...

Keyword(s):

Squamous Metaplasia ◽

Congenital Pulmonary Airway Malformation ◽

Shunt Placement ◽

Pulmonary Airway ◽

Thoracoamniotic Shunt

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Lung Ultrasound: A New Tool in the Management of Congenital Lung Malformation

American Journal of Perinatology ◽

10.1055/s-0039-1692131 ◽

2019 ◽

Vol 36 (S 02) ◽

pp. S99-S105 ◽

Cited By ~ 3

Author(s):

Michele Quercia ◽

Raffaella Panza ◽

Grazia Calderoni ◽

Antonio Di Mauro ◽

Nicola Laforgia

Keyword(s):

Ct Scan ◽

Lung Ultrasound ◽

Case Series ◽

Pulmonary Sequestration ◽

Type I ◽

Congenital Pulmonary Airway Malformation ◽

Congenital Lung Malformation ◽

First Case ◽

Pulmonary Airway

Objective This study was aimed to evaluate effectiveness of lung ultrasound (LUS) in the management of congenital pulmonary airway malformation and pulmonary sequestration in NICUs. Study Design This is a nonconsecutive case series of neonates admitted to the academic NICU of Policlinico of Bari, Italy, from 2010 to 2018, for suspected lung malformations and examined by LUS. Results Seven neonates were admitted for suspected pulmonary malformations, four neonates were diagnosed with pulmonary sequestration and three with congenital pulmonary airway malformation either type I (two cases) or type II (one case) according to Adzick classification. Prenatal scans had described lung malformations in six patients. Two underwent surgical intervention during the 1st month of life. All were successfully discharged home and their follow-up has been uneventful thereafter. In all the seven neonates, LUS easily detected the lesion showing a significantly high correspondence with computed tomography (CT) scan findings. Conclusion We described the first case series of neonates affected by complex pulmonary malformations, assessed by LUS. In our experience, LUS was safe and effective for the diagnosis with high degree of consistency with CT scan findings. We suggest that LUS might be an important diagnostic method for lung malformations in newborns and a useful technique for their follow-up and late management, avoiding multiple exposures to radiations.

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