Congenital Lung Anomalies (CLA) Swiss Database

Study Overview
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Brief Summary
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Congenital lung anomalies include different pathologies such as congenital cystic adenomatoid malformation, pulmonary sequestration, bronchial atresia, emphysema, bronchogenic cyst. They concern less than 1/10000 births and their physiopathological origin is still poorly understood. The main goal of this project is to pool the cases from different swiss centers on a prospective cohort study, first to increase knowledge of clinical and radiological evolution and their correlation with histological data, and second to analyse the pathological embryological mechanism underlying these malformations.

Detailed Description
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To register prospectively clinical, biological, radiological and histological datas in a multicentric database (secured internet link, via Secutrial® software). for children with prenatal diagnosis of this kind of malformation. This study is conducted by a multidisciplinary team, involving obstetricians, neonatologists, pneumologists, pediatric surgeons, radiologists and anatomopathologists. To create a tissue biobank Outcomes: Contribute to the definition of a standardized procedure at the Swiss level for the treatment of patients suffering from these malformations, Improve the assessment of lesion evolution related to CLA, and Possibly validate some biomarkers, which could help to identify individuals at risk. On the long term, these results could also support the development of innovative therapies targeting the factors involved in lung development.

Official Title
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Follow up of Congenital Lung Anomalies (CLA) With Antenatal Diagnosis - a Swiss Multicentric Database

Conditions
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Congenital Lung Malformation, Congenital Lung Cyst, Sequestrum, Bronchogenic Cancer, Pleuropulmonary Blastoma, Acinar Dysplasia

Intervention / Treatment
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* Procedure: Malformations resection


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: All patients diagnosed with congenital lung anomalies Exclusion Criteria: None

Ages Eligible for Study
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Maximum Age: 16 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Patient with CLA with surgery<br> | Procedure: Malformations resection<br>* Resection of the malformation lung sections with a healthy adjacent part<br>|
| Patient with CLA without surgery<br> | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change in clinical of patients with CLA between different time point | Clinical measurements: size (cm), weight (kg), saturation (%) | Birth, 1 month of life, 4 months of life, 9-18 months of life, 6 months post surgery, 1 year post surgery, 7 years old, 10 years old, 12 years old | 
| Analysis of CLA physiopathology | Analysis of growth factors, transcription factors and extracellular components implicated in CLA genesis by immunohistochemistry, transcriptomic and proteomic methods | samples collected during surgery | 
| Change in lung function | FEV1 (l/min), FEV1/FVC (%)TLC (L) DLCO (ml/min/mmHg) | 7 years old,10 years old, 12 years old, 16 years old | 
| Change in Scar aspect and thoracic deformation of patients with CLA between different time point | Description | 6 months post surgery, 1 year post surgery, 7 years old, 10 years old, 12 years old | 
| Change in lung radiological images of patients with CLA between different time point | Chest X-ray and Thoracic CT Scan lesion description | 1 month of life, 4 months of life, 9-18 months of life, 6 months post surgery, 1 year post surgery, 7 years old, 10 years old, 12 years old | 
| Change in lesion size described by antenatal ultrasound of patients with CLA between different time point | lesion size (mm) CVR, estimated wight (gr), Head circumferences (cm) | 22, 28, 32 and 37 weeks of gestation | 
| Change in lesion description by antenatal ultrasound of patients with CLA between different time point | Lesion description (micro cysts, macrocysts),lesion localisation | 22, 28, 32 and 37 weeks of gestation | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Report biomarkers implicated in CLA with a potential role in lesion oncogenic transformation | Comparison using exome analysis : CLA lesion, healthy adjacent part and blood DNA | 1 year post surgery | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Congenital Lung Anomalies