Evidence-Based Resource

What is the ideal management of asymptomatic congenital pulmonary airway malformation (CPAM)?

Evidence Level III

The clinical presentation of congenital pulmonary airway malformation (CPAM) ranges from severe respiratory distress at birth to lesions which remain asymptomatic for years. An increasing proportion of neonates are presenting asymptomatically as many are now identified on antenatal ultrasound. Clinical presentation typically dictates treatment, however when cases of known cystic lung disease are asymptomatic, ideal management is less clear.

A few reports describe postnatal resolution of around 4%-13% of CPAM lesions, lending support to conservative management (Stanton 2009, Calvert 2007, Sauvat 2003, Kays 2006). However, whether this is a great enough proportion to justify observation is undetermined. It has been suggested that this phenomenon may not be true regression, but growth of healthy lung tissue around the lesion reducing its visibility on imaging (Wallis 2000).

While the natural evolution of CPAM is largely uncharacterized, it is generally assumed that persistent CPAM will eventually become symptomatic (Di Prima 2012, Fitzgerald 2007), leading to more complicated surgery and worse surgical outcomes. Therefore many authors suggest that asymptomatic patients undergo elective surgery before symptoms develop (Aspirot 2008, Conforti 2009, dell’Agnola 1996, Marshall 2000, Sueyoshi 2008, Laje 2008). In contrast, rates of symptom development have been reported as low as 3.2% indicating that surgery is unnecessary in the majority of asymptomatic patients, although this low prevalence may be attributable to inadequate follow-up length (Stanton 2009). Nevertheless, some studies have reported that there is no significant difference in post-operative outcomes between those who were symptomatic versus asymptomatic at the time of surgery, adding further support for conservative management as long as symptoms are absent (Aziz 2004, Nasr 2010).

However, the risk of malignancy, although low (1-4%; Chuang 2009), has come to play an important role in decision-making around CPAM management. Although some authors argue that the association is largely overstated (Di Prima 2012, Fitzgerald 2007), other argue that the lethal potential of malignancy may justify surgical intervention in all patients (Priest 2009, Puligandla 2012). Additionally, over 40 cases exist detailing malignancies arising in, or being indistinguishable from, CPAM lesions lending further support to prophylactic resection of all lesions (Laberge 2005). There exist reports, however, of malignancy arising in CPAM patients after resection or in an unassociated area of the lung, suggesting that these patients may have an overall increased malignant potential which no current therapy can adequately address (Bush 2009, Chuang 2009).

The greatest argument against surgical intervention for asymptomatic CPAM lesions is the issue of unnecessarily exposing these pediatric patients to surgical risk. However, low morbidity rates and no mortalities have been associated with surgery (Tsai 2008, Eber 2007, Laje 2008), and lobectomy – the standard treatment – is well tolerated without any long term functional limitations (Beres 2011, Keijzer 2009, Naito, 2012, Nakajima 1998, Parikh 2005). It should also be noted, though, that similar functional outcomes have been reported in nonsurgically managed patients (Eber 2007), indicating that conservative management may be comparable in the long-term. Be that as it may, conservative management is associated with significantly greater exposure to radiation and anesthesia due to the use of serial CT scans for monitoring (Di Prima 2012, Eber 2007, Laje 2008). While the impact of these exposures has not been investigated, they are of concern in a pediatric population.

We identified 9 non-randomized studies from which we could extract poolable data (Aziz 2004, Chow 2007, dell’Agnola 1996, Evrard 1999, Keidar 2001, Marshall 2000, Raychaudhuri 2011, Sueyoshi 2008, Wong 2009); only one was prospective in design (Keidar 2001). One hundred sixty-eight patients were asymptomatic at birth with sufficient data available for inclusion in our analyses. Just under half of these patients (n = 70, 41.7%) underwent elective surgery with 7 (10.0 %) cases of postoperative complications. Of the remainder (n = 98, 58.3%), 63 eventually developing symptoms between one month and seven years of age and consequently required surgery (64.3%); 20 experienced postoperative complications (31.8%). The remaining 35 patients continued to be managed non-surgically, with a follow-up of between three months and nine years (35.7%). There were no deaths related to the condition or surgical complications of lung resection in any of our included patients. Meta-analysis results revealed that post-operative complications were significantly more likely when surgery was performed after symptom development compared to resection when patients were asymptomatic (OR 4.59, 95% CI 1.40 to 15.11, P = 0.01; Aziz 2004, Chow 2007, dell’Agnola 1996, Evrard 1999, Marshall 2000, Wong 2009; low heterogeneity (I2 = 0%)) and that  there was no significant difference in length of hospitalization after surgery (MD 4.96, 95% CI -1.75 to 11.67, P = 0.15; Marshall 2000, Sueyoshi 2008, Evrard 1999; high heterogeneity (I2 = 63%)), although the trend favoured asymptomatic patients.

Based on these findings, we recommend elective resection of asymptomatic CPAM lesions, rather than expectant management. Lung resection in an asymptomatic patient is quite safe, prevents the risk of symptom development and complicated surgery later in life, and may help prevent the development of malignancy.

Results and recommendations from non-systematic literature reviews discussing this comparison are available in this table and a summary of these results are available here.

Acknowledgement: We thank 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.

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.

Colon N, Schlegel C, Pietsch J, Chung DH, Jackson GP. Congenital lung anomalies: can we postpone resection? Journal of Pediatric Surgery 2012;47(1):87-92.

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.

Evrard V, Ceulemans J, Coosemans W, De Baere T, De Leyn P, Deneffe G, Devlieger H, De Boeck C, Van Raemdonck D, Lerut T. Congenital parenchymatous malformations of the lung. World J Surg 1999;23(11):1123-32.

Keijzer R, Chiu PP, Ratjen F, Langer JC. Pulmonary function after early vs late lobectomy during childhood: a preliminary study. Journal of Pediatric Surgery 2009;44(5):893-5.

Liao SL, Lai SH, Hsueh C, Wong KS. Comparing late-onset and neonatally-diagnosed congenital cystic adenomatoid malformation of the lung. Chang Gung Medical Journal 2010;33(1):36-43.

Marshall KW, Blane CE, Teitelbaum DH, van Leeuwen K. Congenital cystic adenomatoid malformation: impact of prenatal diagnosis and changing strategies in the treatment of the asymptomatic patient. American Journal of Roentgenology 2000;175(6):1551-4.

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.

Nakajima C, Kijimoto C, Yokoyama Y, Miyakawa T, Tsuchiya Y, Kuroda T, Nakano M, Saeki M. Longitudinal follow-up of pulmonary function after lobectomy in childhood - factors affecting lung growth. Pediatric Surgery International 1998;13(5-6):341-5.

Sueyoshi R, Okazaki T, Urushihara N, Fujiwara T, Tobayama S, Fukumoto K, Horigome F, Tei E, Lane GJ, Hasegawa S, Yamataka A. Managing prenatally diagnosed asymptomatic congenital cystic adenomatoid malformation. Pediatric Surgery International 2008;24(10):1111-5.

Tsai YC, Wu CC, Yang SS. Open versus minilaparoscopic herniorrhaphy for children: a prospective comparative trial with midterm follow-up evaluation. Surgical Endoscopy 2010;24(1):21-4.

Non-Randomized Trials: Non-Comparison Studies

Bagolan P, Nahom A, Giorlandino C, Trucchi A, Bilancioni E, Inserra A, Gambuzza G, Spina V. Cystic adenomatoid malformation of the lung: clinical evolution and management. European Journal of Pediatrics 1999;158(11):879-82.

Beres A, Aspirot A, Paris C, Berube D, Bouchard S, Laberge JM, Lands LC, Puligandla P. A contemporary evaluation of pulmonary function in children undergoing lung resection in infancy. Journal of Pediatric Surgery 2011;46(5):829-32.

Calvert JK, Boyd PA, Chamberlain PC, Syed S, Lakhoo K. Outcome of antenatally suspected congenital cystic adenomatoid malformation of the lung: 10 years' experience 1991-2001. Archives of Diseases in Childhood – Fetal and Neonatal Edition 2006;91(1):F26-8.

Chow PC, Lee SL, Tang MH, Chan KL, Lee CP, Lam BC, Tsoi NS. Management and outcome of antenatally diagnosed congenital cystic adenomatoid malformation of the lung. Hong Kong Medical Journal 2007;13(1):31-9.

dell'Agnola C, Tadini B, Mosca F, Colnaghi M, Wesley J. Advantages of prenatal diagnosis and early surgery for congenital cystic disease of the lung. Journal of Perinatal Medicine 1996;24(6):621-31.

Hammond PJ, Devdas JM, Ray B, Ward-Platt M, Barrett AM, McKean M. The outcome of expectant management of congenital cystic adenomatoid malformations (CCAM) of the lung. European Journal of Pediatric Surgery 2010;20(3):145-9.

Keidar S, Ben-Sira L, Weinberg M, Jaffa AJ, Silbiger A, Vinograd I. The postnatal management of congenital cystic adenomatoid malformation. Israel Medical Association Journal 2001;3(4):258-61.

Kim YT, Kim JS, Park JD, Kang CH, Sung SW, Kim JH. Treatment of congenital cystic adenomatoid malformation-does resection in the early postnatal period increase surgical risk? European Journal of Cardiothoracic Surgery 2005;27(4):658-61.

Laberge JM, Bratu I, Flageole H. The management of asymptomatic congenital lung malformations. Paediatric Respiratory Reviews 2004;5 Suppl A:S305-12.

Nagata K, Masumoto K, Tesiba R, Esumi G, Tsukimori K, Norio W, Taguchi T. Outcome and treatment in an antenatally diagnosed congenital cystic adenomatoid malformation of the lung. Pediatr Surg Int. 2009 Sep;25(9):753-7.

Nasr A, Himidan S, Pastor AC, Taylor G, Kim PC. Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma? Journal of Pediatric Surgery 2010 Jun;45(6):1086-9.

Parikh D, Samuel M. Congenital cystic lung lesions: is surgical resection essential? Pediatric Pulmonology 2005;40(6):533-7.

Raychaudhuri P, Pasupati A, James A, Whitehead B, Kumar R. Prospective study of antenatally diagnosed congenital cystic adenomatoid malformations. Pediatric Surgery International 2011;27(11):1159-64.

Rocha G, Fernandes PC, Proença E, Quintas C, Martins T, Azevedo I, Guimarães H. Congenital cystic adenomatoid malformation of the lung--the experience of five medical centres. Rev Port Pneumol 2007;13(4):511-23.

Sauvat F, Michel JL, Benachi A, Emond S, Revillon Y. Management of asymptomatic neonatal cystic adenomatoid malformations. Journal of Pediatric Surgery 2003;38(4):548-52.

Truitt AK, Carr SR, Cassese J, Kurkchubasche AG, Tracy TF Jr, Luks FI. Perinatal management of congenital cystic lung lesions in the age of minimally invasive surgery. J Pediatric Surgery 2006;41(5):893-6.

van Leeuwen K, Teitelbaum DH, Hirschl RB, Austin E, Adelman SH, Polley TZ, Marshall KW, Coran AG, Nugent C. Prenatal diagnosis of congenital cystic adenomatoid malformation and its postnatal presentation, surgical indications, and natural history. Journal of Pediatric Surgery 1999;34(5):794-8; discussion 798-9.

Wong A, Vieten D, Singh S, Harvey JG, Holland AJ. Long-term outcome of asymptomatic patients with congenital cystic adenomatoid malformation. Pediatric Surgery International 2009;25(6):479-85.

Other Study Designs

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

Azizkhan RG, Crombleholme TM. Congenital cystic lung disease: contemporary antenatal and postnatal management. Pediatric Surgery International 2008;24(6):643-57.

Bush A. Prenatal presentation and postnatal management of congenital thoracic malformations. Early Human Development 2009;85(11):679-84.

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.

d'Agostino S, Bonoldi E, Dante S, Meli S, Cappellari F, Musi L. Embryonal rhabdomyosarcoma of the lung arising in cystic adenomatoid malformation: case report and review of the literature. Journal of Pediatric Surgery 1997;32(9):1381-3.

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.

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.

Goldstein RB. A practical approach to fetal chest masses. Ultrasound Quarterly 2006;22(3):177-94.

Kays DW. Congenital diaphragmatic hernia and neonatal lung lesions. Surgical Clinics of North America 2006;86(2):329-52, ix.

Klein JD, Barnewolt CE, Jennings RW. Current and future treatment of fetal thoracic masses. Expert Review of Obstetrics & Gynecology 2009;4(2):201.

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.

Laje P, Liechty KW. Postnatal management and outcome of prenatally diagnosed lung lesions. Prenatal Diagnosis 2008;28(7):612-8.

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.

Priest JR, Williams GM, Hill DA, Dehner LP, Jaffé A. Pulmonary cysts in early childhood and the risk of malignancy. Pediatric Pulmonology 2009;44(1):14-30.

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

Reiss I, van de Ven CP, Tibboel D. Congenital lung malformations – Diagnostic and therapeutic approaches. Intensivmed 2008;45:12-18.

Tagge EP, Mulvihill D, Chandler JC, Richardson M, Uflacker R, Othersen HD. Childhood pleuropulmonary blastoma: caution against nonoperative management of congenital lung cysts. Journal of Pediatric Surgery 1996;31(1):187-9.

Wallis C. Clinical outcomes of congenital lung abnormalities. Paediatric Respiratory Reviews 2000;1(4):328-35.

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