Review of Early Postoperative Metrics for Children Undergoing Resection of Congenital Pulmonary Airway Malformations and Report of Pleuropulmonary Blastoma at a Single Institution

Abstract

Purpose The purpose of this study is to describe a single institution's 11-year experience treating children with congenital pulmonary airway malformations (CPAMs) and pleuropulmonary blastoma (PPB).

Materials and Methods An institutional database was sampled for all patients aged 0 to 18 years from January 1, 2005, to December 31, 2015. Patients with a pathologic diagnosis of CPAM or PPB during this period were reviewed.

Results A total of 51 patients with a pathologic diagnosis of CPAM (n = 45; 88.2%) or PPB (n = 6; 11.8%) underwent surgical resection. Among patients treated for PPB, one death occurred approximately 13 months after diagnosis. Although four patients with PPB (four out of six; 66.7%) had radiographic indicators highly suggestive of malignancy prior to surgery, two had a preoperative diagnosis of CPAM (two out of six; 33.3%). Twenty-four patients (24 out of 45; 53.3%) with CPAM underwent resection after developing symptoms and 21 (21 out of 45; 46.7%) were symptomatic at the time of surgery. Mann–Whitney's tests revealed a statistically significant difference in postoperative length of stay (median: 6 vs. 3 days; p < 0.001) and days with thoracostomy tube in place (median 3 vs. 2 days; p = 0.003) for symptomatic versus asymptomatic patients, respectively.

Conclusion CPAM patients appear to recover faster from surgery, if performed before the onset of symptoms. There may be a benefit to waiting until at least 3 months of age to complete resection in the asymptomatic patient. A low threshold for resection should be maintained in patients where delineating CPAM from PPB is difficult.

Keywords

congenital pulmonary airway malformation - pleuropulmonary blastoma - surgery

• References

• 1 Gornall AS, Budd JL, Draper ES, Konje JC, Kurinczuk JJ. Congenital cystic adenomatoid malformation: accuracy of prenatal diagnosis, prevalence and outcome in a general population. Prenat Diagn 2003; 23 (12) 997-1002
• 2 Laberge JM, Flageole H, Pugash D. , et al. Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation: a Canadian experience. Fetal Diagn Ther 2001; 16 (03) 178-186
• 3 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. Arch Dis Child Fetal Neonatal Ed 2006; 91 (01) F26-F28
• 4 Roggin KK, Breuer CK, Carr SR. , et al. The unpredictable character of congenital cystic lung lesions. J Pediatr Surg 2000; 35 (05) 801-805
• 5 Sauvat F, Michel JL, Benachi A, Emond S, Revillon Y. Management of asymptomatic neonatal cystic adenomatoid malformations. J Pediatr Surg 2003; 38 (04) 548-552
• 6 van Leeuwen K, Teitelbaum DH, Hirschl RB. , et al. Prenatal diagnosis of congenital cystic adenomatoid malformation and its postnatal presentation, surgical indications, and natural history. J Pediatr Surg 1999; 34 (05) 794-798
• 7 Aziz D, Langer JC, Tuuha SE, Ryan G, Ein SH, Kim PC. Perinatally diagnosed asymptomatic congenital cystic adenomatoid malformation: to resect or not?. J Pediatr Surg 2004; 39 (03) 329-334
• 8 Kapralik J, Wayne C, Chan E, Nasr A. Surgical versus conservative management of congenital pulmonary airway malformation in children: a systematic review and meta-analysis. J Pediatr Surg 2016; 51 (03) 508-512
• 9 Calvert JK, Lakhoo K. Antenatally suspected congenital cystic adenomatoid malformation of the lung: postnatal investigation and timing of surgery. J Pediatr Surg 2007; 42 (02) 411-414
• 10 Laberge JM, Puligandla P, Flageole H. Asymptomatic congenital lung malformations. Semin Pediatr Surg 2005; 14 (01) 16-33
• 11 Beres A, Aspirot A, Paris C. , et al. A contemporary evaluation of pulmonary function in children undergoing lung resection in infancy. J Pediatr Surg 2011; 46 (05) 829-832
• 12 Keijzer R, Chiu PP, Ratjen F, Langer JC. Pulmonary function after early vs late lobectomy during childhood: a preliminary study. J Pediatr Surg 2009; 44 (05) 893-895
• 13 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. J Pediatr Surg 2012; 47 (05) 852-856
• 14 Nakajima C, Kijimoto C, Yokoyama Y. , et al. Longitudinal follow-up of pulmonary function after lobectomy in childhood-factors affecting lung growth. Pediatr Surg Int 1998; 13 (5-6): 341-345
• 15 Tocchioni F, Lombardi E, Ghionzoli M, Ciardini E, Noccioli B, Messineo A. Long-term lung function in children following lobectomy for congenital lung malformation. J Pediatr Surg 2017; 52 (12) 1891-1897
• 16 Feinberg A, Hall NJ, Williams GM. , et al. Can congenital pulmonary airway malformation be distinguished from Type I pleuropulmonary blastoma based on clinical and radiological features?. J Pediatr Surg 2016; 51 (01) 33-37
• 17 Costanzo S, Filisetti C, Vella C. , et al. Pulmonary malformations: predictors of neonatal respiratory distress and early surgery. J Neonatal Surg 2016; 5 (03) 27
• 18 Crombleholme TM, Coleman B, Hedrick H. , et al. Cystic adenomatoid malformation volume ratio predicts outcome in prenatally diagnosed cystic adenomatoid malformation of the lung. J Pediatr Surg 2002; 37 (03) 331-338
• 19 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. J Pediatr Surg 2008; 43 (03) 508-512
• 20 Conforti A, Aloi I, Trucchi A. , et al. Asymptomatic congenital cystic adenomatoid malformation of the lung: is it time to operate?. J Thorac Cardiovasc Surg 2009; 138 (04) 826-830
• 21 Hall NJ, Chiu PP, Langer JC. Morbidity after elective resection of prenatally diagnosed asymptomatic congenital pulmonary airway malformations. Pediatr Pulmonol 2016; 51 (05) 525-530
• 22 Adams S, Jobson M, Sangnawakij P. , et al. Does thoracoscopy have advantages over open surgery for asymptomatic congenital lung malformations? An analysis of 1626 resections. J Pediatr Surg 2017; 52 (02) 247-251
• 23 Kulaylat AN, Engbrecht BW, Hollenbeak CS, Safford SD, Cilley RE, Dillon PW. Comparing 30-day outcomes between thoracoscopic and open approaches for resection of pediatric congenital lung malformations: evidence from NSQIP. J Pediatr Surg 2015; 50 (10) 1716-1721
• 24 Nasr A, Bass J. Thoracoscopic vs open resection of congenital lung lesions: a meta-analysis. J Pediatr Surg 2012; 47 (05) 857-861
• 25 Vu LT, Farmer DL, Nobuhara KK, Miniati D, Lee H. Thoracoscopic versus open resection for congenital cystic adenomatoid malformations of the lung. J Pediatr Surg 2008; 43 (01) 35-39
• 26 Delestrain C, Khen-Dunlop N, Hadchouel A. , et al. Respiratory morbidity in infants born with a congenital lung malformation. Pediatrics 2017; 139 (03) e20162988
• 27 Gulack BC, Leraas HJ, Ezekian B. , et al. Outcomes following elective resection of congenital pulmonary airway malformations are equivalent after 3 months of age and a weight of 5 kg. J Pediatr Surg 2017; DOI: 10.1016/j.jpedsurg.2017.10.017.
• 28 Nasr A, Himidan S, Pastor AC, Taylor G, Kim PC. Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma?. J Pediatr Surg 2010; 45 (06) 1086-1089