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

Robert J. Vandewalle, Joseph C. Easton, R. Cartland Burns, Brian W. Gray, Frederick J. Rescorla

Research output: Contribution to journalArticle

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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.

Original languageEnglish (US)
Pages (from-to)417-424
Number of pages8
JournalEuropean Journal of Pediatric Surgery
Issue number5
StatePublished - Jun 19 2019



  • Congenital pulmonary airway malformation
  • pleuropulmonary blastoma
  • surgery

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery

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