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, Cartland C. Burns, Brian W. Gray, Frederick Rescorla

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1 Citation (Scopus)

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). 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)
JournalEuropean Journal of Pediatric Surgery
DOIs
StateAccepted/In press - Jun 19 2018

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Lung
Pleuropulmonary blastoma
Thoracostomy
Length of Stay
Databases
Neoplasms

Keywords

  • congenital pulmonary airway malformation
  • pleuropulmonary blastoma
  • surgery

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery

Cite this

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title = "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). 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",
author = "Vandewalle, {Robert J.} and Easton, {Joseph C.} and Burns, {Cartland C.} and Gray, {Brian W.} and Frederick Rescorla",
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T1 - Review of Early Postoperative Metrics for Children Undergoing Resection of Congenital Pulmonary Airway Malformations and Report of Pleuropulmonary Blastoma at a Single Institution

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AU - Easton, Joseph C.

AU - Burns, Cartland C.

AU - Gray, Brian W.

AU - Rescorla, Frederick

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N2 - 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). 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.

AB - 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). 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.

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