Evidence-Based Resource

What is the ideal timing of elective resection of asymptomatic CPAM lesions?

Evidence Level III

The treatment of patients with asymptomatic congenital pulmonary airway malformation (CPAM) is controversial, with questions regarding not only the necessity of elective resection, but what age is optimal for surgery. While most surgeons agree that surgical resection currently represents the best treatment for these patients, recommendations as to when surgery should occur vary widely.

We identified 14 studies. Five contained relevant data in the published report (Aziz 2004, Calvert 2007, Conforti 2009, Pelizzo 2009, Waszak 1999); for the other 9, we obtained useable data from the authors on CPAM patients who underwent elective surgery (Aspirot 2008, Beres 2011, Boubnova 2011, Chen 2010, Furukawa 2015, Kim 2005, Kongstad 2012, Laje 2015, Naito 2012).

Seven comparative studies examined the effect of operative age on patient complications. Meta-analysis results generally indicated that the odds of experiencing complications were not significantly different between younger and older patients undergoing elective resection of CPAM lesions, although older patients were favoured (Aspirot 2008, Aziz 2004, Boubnova 2011, Conforti 2009, Furukawa 2015, Kim 2005, Naito 2012). Data from 3 additional studies allowed for non-comparative assessment of complications following elective surgery, finding complications following surgery in 31% of neonates (Waszak  1999), 7% of patients younger than 4 months of age (Laje 2015), and 8% of patients undergoing surgery at an average of 12 months of age (Kongstad 2012). Older patients had shorter length of hospital stay and chest tube duration, but greater evidence of infection on pathology. All other outcomes were similar between age comparisons (need for ventilation, length of ventilation, blood loss, pulmonary function, and mortality).

We also identified 15 literature reviews on this topic (Andrade 2011, Chuang 2009, Di Prima 2012, Durkin 2009, Eber 2007, Fitzgerald 2007, Guidry 2012, Kotecha 2012, Laberge 2005, Lakhoo 2007, Lakhoo 2009, Puligandla 2012, Shanti 2008, Singh 2015, Wall 2014). Though varied in their recommendations, it was generally advised that surgery occur sometime between 3 and 12 months of age.

The majority of the literature available on this topic, however, is opinion-based. We identified 42 studies that provided recommendations based on personal experience, rather than an evaluation of data. While the most popular recommendation appears to be that resection should occur between 3 and 6 months of age, values range from the neonatal period to 3 years of age. You can view this table to see a summary of recommendations from opinion-based articles. In addition, two surveys of pediatric surgeons confirmed the lack of standardization in practice, with the majority of respondents preferring to resect after the neonatal period but before 12 months of age (Lo 2008, Peters 2013).

In conclusion, the literature published concerning the optimal age of elective resection of asymptomatic CPAM is largely opinion-based in nature. Even where evidence does exist, it is difficult to get an overall picture of how age influences clinical outcomes, as papers tend to limit their data to a single age evaluation/comparison. Based on the evidence, however, it would appear as though resection between 3 and 6 months is most commonly practiced, and that resection at this age is safe and effective.

Future studies that seek to evaluate the effect of age on clinical outcomes should be comparative and focus only on: a) CPAM and not combine data from different thoracic lesions, b) asymptomatic patients and not combine data from emergency and elective surgery, and c) evaluating multiple age comparisons rather than just one.

Acknowledgement: We thank Katrina J. Sullivan, Michelle Li, Sarah Haworth, Elizabeth Chernetsova, Jessica Kapralik, and Emily Chan for their work on this review.

The full systematic review can be found here.

Non-Randomized Trials: Comparison Studies

Aspirot A, Puligandla PS, Bouchard S, Su W, Flageole H, Laberge JM. A contemporary evaluation of surgical outcome in neonates and infants undergoing lung resection. Journal of Pediatric Surgery 2008;43(3):508-12.

Aziz D, Langer JC, Tuuha SE, Ryan G, Ein SH, Kim PC. Perinatally diagnosed asymptomatic congenital cystic adenomatoid malformation: to resect or not? Journal of Pediatric Surgery 2004;39(3):329-34; discussion 329-34.

Boubnova J, Peycelon M, Garbi O, David M, Bonnard A. Thoracoscopy in the management of congenital lung disease in infancy. Surgical Endoscopy 2011;25:593-596.

Calvert JK, Lakhoo K. Antenatally suspected congenital cystic adenomatoid malformation of the lung: postnatal investigation and timing of surgery. Journal of Pediatric Surgery 2007;42(2):411-4.

Chen HW, Hsu WM, Lu FL, Chen PC, Jeng SF, Peng SS, Chen CY, Chou HC, Tsao PN, Hsieh WS. Management of congenital cystic adenomatoid malformation and bronchopulmonary sequestration in newborns. Pediatric Neonatology 2010;51(3):172-7.

Conforti A, Aloi I, Trucchi A, Morini F, Nahom A, Inserra A, Bagolan P. Asymptomatic congenital cystic adenomatoid malformation of the lung: is it time to operate? Journal of Thoracic and Cardiovascular Surgery 2009;138(4):826-30.

Furukawa T, Kimura O, Sakai K, Higashi M, Fumino S, Aoi S, Tajiri T. Surgical intervention strategies for pediatric congenital cystic lesions of the lungs: A 20-years single-institution experience. Journal of Pediatric Surgery 2015;50:2025-2027.

Laje P, Pearson EG, Simpao AF, Rehman MA, Sinclair T, Hedrick HL, Adzick NS, Flake AW. The first 100 infant thoracoscopic lobectomies: Observations through the learning curve and comparison to open lobectomy. Journal of Pediatric Surgery 2015;50:1811-1816.

Naito Y, Beres A, Lapidus-Krol E, Ratjen F, Langer JC. Does earlier lobectomy result in better long-term pulmonary function in children with congenital lung anomalies? A prospective study. Journal of Pediatric Surgery 2012;47(5):852-6.

Pelizzo G, Barbi E, Codrich D, Lembo MA, Zennaro F, Bussani R, Schleef J. Chronic inflammation in congenital cystic adenomatoid malformations. An underestimated risk factor? Journal of Pediatric Surgery 2009;44: 616-619.

Other Study Designs

Andrade CF, Ferreira HP, Fischer GB. Congenital lung malformations. Jornal Brasileiro de Pneumologia 2011;37(2):259-71.

Chuang S, Sugo E, Jaffe A. A review of postnatal management of congenital pulmonary airway malformations. Fetal and Maternal Medicine Review 2009;20(3):179-204.

Di Prima FA, Bellia A, Inclimona G, Grasso F, Teresa M, Cassaro MN. Antenatally diagnosed congenital cystic adenomatoid malformations (CCAM): Research Review. Journal of Prenatal Medicine 2012;6(2):22-30.

Durkin EF, Shaaban A. Commonly encountered surgical problems in the fetus and neonate. Pediatric Clinics of North America 2009; 56: 647-669.

Eber E. Antenatal diagnosis of congenital thoracic malformations: early surgery, late surgery, or no surgery? Seminars in Respiratory and Critical Care Medicine 2007;28(3):355-66.

Fitzgerald DA. Congenital cyst adenomatoid malformations: resect some and observe all? Paediatric Respiratory Reviews 2007;8(1):67-76.

Guidry C, McGahren ED. Pediatric chest I: Developmental and physiologic conditions for the surgeon. Surgical Clinics of North America 2012; 92(3): 615-643.

Kotecha S, Barbato A, Bush A, Claus F, Davenport M, Delacourt C, Deprest J, Eber E, Frenckner B, Greenough A, Nicholson AG, Antón-Pacheco JL, Midulla F. Antenatal and postnatal management of congenital cystic adenomatoid malformation. Paediatric Respiratory Reviews 2012;13(3):162-70.

Laberge JM, Puligandla P, Flageole H. Asymptomatic congenital lung malformations. Seminars in Pediatric Surgery 2005;14(1):16-33.

Lakhoo K. Fetal counselling for congenital malformations. Pediatric Surgery International 2007; 23: 509-519.

Lakhoo K. Management of congenital cystic adenomatous malformations of the lung. Archives of Disease in Childhood - Fetal and Neonatal Edition 2009;94(1):F73-6.

Lo AY, Jones S. Lack of consensus among Canadian pediatric surgeons regarding the management of congenital cystic adenomatoid malformations of the lung. Journal of Pediatric Surgery 2008; 43: 797-799.

Peters RT, Burge DM, Marven SS. Congenital lung malformations: an ongoing controversy. Annals of The Royal College of Surgeons of England 2013; 95: 144-147.

Puligandla PS, Laberge JM. Congenital lung lesions. Clinical Perinatology 2012;39(2):331-47.

Shanti CM, Klein MD. Cystic lung disease. Seminars in Pediatric Surgery 2008; 17: 2-8.

Singh R, Davenport M. The argument for operative approach to asymptomatic lung lesions. Seminars in Pediatric Surgery 2015; 24: 187-195.

Wall J, Coates A. Prenatal imaging and postnatal presentation, diagnosis and management of congenital lung malformations. Current Opinions in Pediatrics 2014; 26(3): 315-319.

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