The Management of Asymptomatic Congenital Pulmonary Airway Malformation: Results of a European Delphi Survey
Abstract
:1. Introduction
2. Materials and Methods
Data Analysis
3. Results
3.1. Round 1
3.2. Round 2
3.3. Round 3
3.4. Final Core Outcome Set
3.5. Attrition Rate and Attrition Bias
4. Discussion
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
References
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Outcome Category | Outcome Parameter | Age at Measurement | Median Scoring during Round 3 | ||||
---|---|---|---|---|---|---|---|
Surgical Group | Non-Surgical Group | p-Value | |||||
Respiratory Symptoms | Wheezing defined as ≥3 episodes per year | All follow-up visits | 4 | 9 | <0.000 | † | |
Respiratory insufficiency * | All follow-up visits | 9 | 7.5 | 0.006 | |||
Liverpool Respiratory Symptom Questionnaire (LRSQ) | All follow-up visits | 6 | 5 | 0.22 | |||
Respiratory rate | All follow-up visits | ‡ | 6 | 6 | 0.039 | ||
Reduced breath sounds on chest auscultation | All follow-up visits | ‡ | 6 | 6.5 | 0.037 | ||
Infection | Infection at lesion site § | All follow-up visits | 9 | 5 | <0.001 | ||
Parent reported hospital admission with IV antibiotics due to LRTI | All follow-up visits | 7 | 5.5 | <0.001 | |||
Parent reported oral antibiotics due to LRTI | All follow-up visits | 6 | 5.5 | 0.2 | |||
Chronic inflammation in resected specimens | Not applicable | ‡ | 6 | 5 | 0.569 | ||
Parental Anxiety | Amsterdam Preoperative Anxiety and Information Scale (APAIS) | All follow-up visits | 5 | 7 | <0.001 | ||
Visual Analog Scale (VAS) scoring | All follow-up visits | 5 | 7 | <0.001 | |||
Quality of Life | Pediatric Quality of Life Inventory (PedsQL) | All follow-up visits | 6 | 6 | 0.747 | ||
Anthropometric Measurements | Height for age measurements (WHO standards) | All follow-up visits | 5 | 6 | 0.004 | † | |
Weight for height measurements (WHO standards) | All follow-up visits | 5 | 6 | 0.025 | † | ||
Lesion Characteristics | Lesion size on first postnatal chest X-ray | <1month | 6 | 6 | 0.401 | ||
Lesion location on CT-scan | 3–9 months | ‡ | 6 | 5 | 0.458 | ||
Mass effect/mediastinal shift on chest x-ray | <1 month | ‡ | 8 | 8 | 0.757 | ||
Mass effect/mediastinal shift on CT-scan | 3–9 months | ‡ | 7 | 7 | 0.844 | ||
Mass effect/mediastinal shift on CT-scan | 2.5 years | ‡ | 7 | 7 | 0.933 | ||
CPAM type as seen on CT | 3–9 months | ‡ | 7 | 7 | 0.607 | ||
Systemic blood supply on CT-scan | 3–9 months | ‡ | 7 | 7 | 0.227 | ||
Multifocal disease on CT scan | 3–9 months | ‡ | 7 | 7 | 0.805 | ||
Multifocal disease on CT scan | 2.5 years | ‡ | 7 | 7 | 0.576 | ||
Quantitative lung volume measurement of first CT-scan | 3–9 months | 6 | 5 | 0.017 | |||
Quantitative lung volume measurement of follow-up CT-scan | 2.5 years | 6 | 5 | 0.004 | |||
Spirometry | FEV1 | 5 years | 5 | 7 | <0.001 | ||
FEV1 | 10 years | ‡ | 5 | 6 | 0.868 | ||
FEF25–75 | 5 years | 5 | 9 | <0.001 | |||
FEF25–75 | 10 years | ‡ | 5 | 4.5 | 0.246 | ||
FVC | 5 years | 5 | 7 | <0.001 | |||
FVC | 10 years | ‡ | 5 | 6 | 0.273 | ||
Lung clearance index | 5 years | ‡ | 4 | 4 | 0.327 | ||
FRC | 5 years | ‡ | 4 | 4 | 0.576 | ||
Exercise Tolerance | Bruce treadmill test | 5 years | 6 | 6 | 0.157 | ||
VO2 max measurements | 8 years | ‡ | 5 | 5 | 0.771 | ||
VO2 max measurements | 10 years | ‡ | 5 | 5 | 0.804 | ||
Surgical Complications | Surgical complications ¶ | Not applicable | 7 | 9 | 0.018 | ||
Malignancy | Proven malignancy on histology by experienced pathologist | Not applicable | 9 | 6 | <0.001 | ||
Skeletal Deformity | Physical examination | All follow-up visits | ‡ | 6 | 6 | 0.205 | |
Chest/Spine X-ray | 2.5 years | ‡ | 5 | 6 | 0.222 | ||
Chest/Spine X-ray | 5 years | ‡ | 6 | 6 | 0.544 | ||
CT-imaging | 2.5 years | ‡ | 5 | 6 | 0.299 | ||
CT-imaging | 5 years | ‡ | 5 | 6 | 0.392 |
Outcome Category | Outcome Parameter | Age at Measurement | Percentage Scoring during Round 3 | |
---|---|---|---|---|
7–9 “Critical” | 1–3 “Not Important” | |||
Respiratory Symptoms | Respiratory insufficiency * | All follow-up visits | 98 | 0 |
Surgical Complications | Surgical complications ¶ | Not applicable | 95 | 2 |
Lesion Characteristics | Mass effect/mediastinal shift ‡ | <1 month | 91 | 3 |
3–9 months | 89 | 0 | ||
2.5 years | 89 | 2 | ||
Multifocal disease on CT scan | 3–9 months | 73 | 2 | |
2.5 years | 71 | 4 |
Consensus | Outcome Category | Outcome Parameter | Age at Measurement | Percentage Scoring 7–9 “Critical” during Round 3 | |
---|---|---|---|---|---|
Surgical Group | Non-Surgical Group | ||||
Both Stakeholder Groups | Respiratory symptoms | Respiratory insufficiency * | All follow-up visits | 97 | 100 |
Surgical | Surgical complications ¶ | Not applicable | 91 | 100 | |
Lesion characteristics | Mass effect/mediastinal shift ‡ | <1 month | 94 | 86 | |
3–9 months | 91 | 86 | |||
2.5 years | 91 | 86 | |||
Multifocal disease on CT scan | 3–9 months | 70 | 77 | ||
2.5 years | 70 | 73 | |||
Surgical Stakeholder Group only | Malignancy | Proven malignancy on histology by experienced pathologist | Not applicable | 97 | 14 |
Infection | Infection at lesion site § | All follow-up visits | 94 | 0 | |
Parent reported hospital admission with IV antibiotics due to LRTI | All follow-up visits | 82 | 27 | ||
Non-Surgical Stakeholder Group only | Respiratory symptoms | Wheezing, defined as ≥3 episodes per year | All follow-up visits | 9 | 100 |
Spirometry | FVC | 5 years | 21 | 100 | |
FEF25–75 | 5 years | 21 | 91 | ||
FEV1 | 5 years | 21 | 82 | ||
Lesion characteristics | Systemic blood supply on CT-scan | 3–9 months | 64 | 77 |
Outcome Category | Outcome Parameter | Percentage Scored | |
---|---|---|---|
Total | |||
Respiratory Symptoms | Respiratory insufficiency * | 6 | |
Infection | LRTI | 16 | 32 |
Infection at lesion site § | 8 | ||
Frequency of antibiotics usage due to LRTI | 6 | ||
Recurrent infection ¶ | 2 | ||
Quality of Life | Quality of life | 4 | |
Lesion Characteristics | CT-scan evaluation, dimensions of lesion | 16 | 30 |
Mass effect/mediastinal shift on CT-scan | 8 | ||
Radiological imaging | 2 | ||
Evaluation with expert radiologist of first CT-scan | 2 | ||
Change in lesion size over time | 2 | ||
Spirometry | Lung function | 2 | 4 |
FEV1 | 2 | ||
Exercise Tolerance | Symptoms or mediastinal shift on CT/x ray at 6 months of age | 2 | |
Malignancy | Malignancy risk/suspicion | 6 | 14 |
Malignancy histologically proven | 4 | ||
Malignancy | 4 | ||
Other | Age | 2 | 6 |
History | 2 | ||
Oxygen supply | 2 | ||
Combinations | Symptoms or mediastinal shift on CT/x ray at 6 months of age | 2 |
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Kersten, C.M.; Hermelijn, S.M.; Mullassery, D.; Muthialu, N.; Cobanoglu, N.; Gartner, S.; Bagolan, P.; Mesas Burgos, C.; Sgrò, A.; Heyman, S.; et al. The Management of Asymptomatic Congenital Pulmonary Airway Malformation: Results of a European Delphi Survey. Children 2022, 9, 1153. https://doi.org/10.3390/children9081153
Kersten CM, Hermelijn SM, Mullassery D, Muthialu N, Cobanoglu N, Gartner S, Bagolan P, Mesas Burgos C, Sgrò A, Heyman S, et al. The Management of Asymptomatic Congenital Pulmonary Airway Malformation: Results of a European Delphi Survey. Children. 2022; 9(8):1153. https://doi.org/10.3390/children9081153
Chicago/Turabian StyleKersten, Casper M., Sergei M. Hermelijn, Dhanya Mullassery, Nagarajan Muthialu, Nazan Cobanoglu, Silvia Gartner, Pietro Bagolan, Carmen Mesas Burgos, Alberto Sgrò, Stijn Heyman, and et al. 2022. "The Management of Asymptomatic Congenital Pulmonary Airway Malformation: Results of a European Delphi Survey" Children 9, no. 8: 1153. https://doi.org/10.3390/children9081153