Conservative approach in lobar and segmental congenital emphysema: Lung function and clinical outcome

Aim of the study: To evaluate clinical outcome and respiratory function in patients with congenital lobar/segmental emphysema (CLE/CSE) treated conservatively. Methods: In this single-centre retrospective study we included patients ≥5-year-old with a diagnosis of CLE/CSE, treated conservatively (dia...

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Main Authors: Alba Ganarin, Valentina Agnese Ferraro, Alessandra Rancan, Silvia Carraro, Stefania Zanconato, Piergiorgio Gamba
Format: Article
Language:English
Published: Elsevier 2023-04-01
Series:Journal of Pediatric Surgery Open
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2949711623000060
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author Alba Ganarin
Valentina Agnese Ferraro
Alessandra Rancan
Silvia Carraro
Stefania Zanconato
Piergiorgio Gamba
author_facet Alba Ganarin
Valentina Agnese Ferraro
Alessandra Rancan
Silvia Carraro
Stefania Zanconato
Piergiorgio Gamba
author_sort Alba Ganarin
collection DOAJ
description Aim of the study: To evaluate clinical outcome and respiratory function in patients with congenital lobar/segmental emphysema (CLE/CSE) treated conservatively. Methods: In this single-centre retrospective study we included patients ≥5-year-old with a diagnosis of CLE/CSE, treated conservatively (diagnosis from 1990). We assessed demographic/anthropometric data, clinical outcome, pulmonary function tests (PFT) analyzed according to Global Lung Initiative. GraphPadPrism® (California, USA) was used for statistical analysis. Main results: Sixteen patients were enrolled (median age 9.3y; range 5-17): 13 had a prenatal diagnosis, 3 showed symptoms at birth. On chest computed tomography (CT), performed in the first 6 months of life, 9 children had lobar emphysema and 7 segmental emphysema. All patients showed a good growth, 14 were asymptomatic, 1 had recurrent respiratory infections and wheezing, 1 had allergic asthma. In 14 patients we performed PFT (median value): FVC 93.87%pred (IQR: 85.24; 107.69), Z-Score -0.52 (IQR: -1.39; 0.77); FEV1 93%pred (IQR: 78.75; 102.23), Z-Score -0.58 (IQR: -1.92; 0.34); RV 112.48%pred (IQR: 102.26; 137.70), Z-Score 0.25 (IQR: 0.05; 0.79); FRC 106.55%pred (IQR: 94.20; 111.04), Z-Score 0.31 (IQR: -0.29; 0.67); TLC 101.44%pred (IQR: 86.98; 113.25), Z-Score 0.11 (IQR: -0.96; 0.97), RV/TLC 122.94%pred (IQR: 113.52; 144.16), Z-Score 0.54 (IQR: 0.34; 1.01). Conclusions: Our data show that patients with CLE/CSE treated conservatively have a good clinical and functional outcome. Nonetheless, due to the short follow-up, further larger studies are needed to evaluate outcome into adulthood.
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spelling doaj.art-61db864ade944c238efaf81bac13dead2023-07-04T05:12:28ZengElsevierJournal of Pediatric Surgery Open2949-71162023-04-011100008Conservative approach in lobar and segmental congenital emphysema: Lung function and clinical outcomeAlba Ganarin0Valentina Agnese Ferraro1Alessandra Rancan2Silvia Carraro3Stefania Zanconato4Piergiorgio Gamba5Unit of Pediatric Surgery, Department of Women's and Children's Health, University of Padova, 35128 Padova, Italy; Corresponding author at: Unit of Pediatric Surgery, Department of Women's and Children's Health, University of Padova, 35128 Padova, Italy.Unit of Pediatric Allergy and Respiratory Medicine, Department of Women's and Children's Health, University of Padova, 35128 Padova, ItalyUnit of Pediatric Surgery, Department of Women's and Children's Health, University of Padova, 35128 Padova, ItalyUnit of Pediatric Allergy and Respiratory Medicine, Department of Women's and Children's Health, University of Padova, 35128 Padova, ItalyUnit of Pediatric Allergy and Respiratory Medicine, Department of Women's and Children's Health, University of Padova, 35128 Padova, ItalyUnit of Pediatric Surgery, Department of Women's and Children's Health, University of Padova, 35128 Padova, ItalyAim of the study: To evaluate clinical outcome and respiratory function in patients with congenital lobar/segmental emphysema (CLE/CSE) treated conservatively. Methods: In this single-centre retrospective study we included patients ≥5-year-old with a diagnosis of CLE/CSE, treated conservatively (diagnosis from 1990). We assessed demographic/anthropometric data, clinical outcome, pulmonary function tests (PFT) analyzed according to Global Lung Initiative. GraphPadPrism® (California, USA) was used for statistical analysis. Main results: Sixteen patients were enrolled (median age 9.3y; range 5-17): 13 had a prenatal diagnosis, 3 showed symptoms at birth. On chest computed tomography (CT), performed in the first 6 months of life, 9 children had lobar emphysema and 7 segmental emphysema. All patients showed a good growth, 14 were asymptomatic, 1 had recurrent respiratory infections and wheezing, 1 had allergic asthma. In 14 patients we performed PFT (median value): FVC 93.87%pred (IQR: 85.24; 107.69), Z-Score -0.52 (IQR: -1.39; 0.77); FEV1 93%pred (IQR: 78.75; 102.23), Z-Score -0.58 (IQR: -1.92; 0.34); RV 112.48%pred (IQR: 102.26; 137.70), Z-Score 0.25 (IQR: 0.05; 0.79); FRC 106.55%pred (IQR: 94.20; 111.04), Z-Score 0.31 (IQR: -0.29; 0.67); TLC 101.44%pred (IQR: 86.98; 113.25), Z-Score 0.11 (IQR: -0.96; 0.97), RV/TLC 122.94%pred (IQR: 113.52; 144.16), Z-Score 0.54 (IQR: 0.34; 1.01). Conclusions: Our data show that patients with CLE/CSE treated conservatively have a good clinical and functional outcome. Nonetheless, due to the short follow-up, further larger studies are needed to evaluate outcome into adulthood.http://www.sciencedirect.com/science/article/pii/S2949711623000060Congenital lobar emphysemaCongenital segmental emphysemaChildrenConservative treatmentRespiratory function tests
spellingShingle Alba Ganarin
Valentina Agnese Ferraro
Alessandra Rancan
Silvia Carraro
Stefania Zanconato
Piergiorgio Gamba
Conservative approach in lobar and segmental congenital emphysema: Lung function and clinical outcome
Journal of Pediatric Surgery Open
Congenital lobar emphysema
Congenital segmental emphysema
Children
Conservative treatment
Respiratory function tests
title Conservative approach in lobar and segmental congenital emphysema: Lung function and clinical outcome
title_full Conservative approach in lobar and segmental congenital emphysema: Lung function and clinical outcome
title_fullStr Conservative approach in lobar and segmental congenital emphysema: Lung function and clinical outcome
title_full_unstemmed Conservative approach in lobar and segmental congenital emphysema: Lung function and clinical outcome
title_short Conservative approach in lobar and segmental congenital emphysema: Lung function and clinical outcome
title_sort conservative approach in lobar and segmental congenital emphysema lung function and clinical outcome
topic Congenital lobar emphysema
Congenital segmental emphysema
Children
Conservative treatment
Respiratory function tests
url http://www.sciencedirect.com/science/article/pii/S2949711623000060
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AT alessandrarancan conservativeapproachinlobarandsegmentalcongenitalemphysemalungfunctionandclinicaloutcome
AT silviacarraro conservativeapproachinlobarandsegmentalcongenitalemphysemalungfunctionandclinicaloutcome
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