Diaphragmatic Eventration in Children : Evaluation of Care Strategies and Results in the French Cohort.

Study Overview
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Brief Summary
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A diaphragmatic eventration is an abnormal and permanent elevation of a portion or an entire intact hemidiaphragm. This rare pathology, found in 0.2 - 1 for every 1000 patients in large radiological series, is either congenital or acquired due to phrenic nerve palsy. Most diaphragmatic eventrations are asymptomatic and discovered thanks to chest x-rays, where the diaphragmatic dome is elevated and visualized above the 4th intercostal space and sometimes up to the clavicle. Computed tomography or magnetic resonance imaging confirms the eventration by visualizing the diaphragmatic muscle distended and intact, unlike a diaphragmatic rupture or hernia. Surgical indications are usually due to respiratory disorders or visceral repercussions, such as gastric emptying disorders or acute accidents like gastric volvulus. Surgical treatment is a phrenic plication, which can be performed via a lateral thoracotomy (classical approach), thoracoscopy or laparoscopy. When surgery is not indicated, follow up consists of regular clinical and radiological monitoring. There is, however, no consensus when it comes to their medical and surgical management due to the very low number of patients per center and per year, and the fact that very few studies specifically address this subject in the literature.

Detailed Description
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This is a retrospective multicenter descriptive cohort study based on French national healthcare data. The aim of this study is to describe the current trends in management of diaphragmatic eventrations in France in order to indentify potential risk factors for complications and to improve and homogenize practices.

Official Title
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Paediatric Treatment of Diaphragmatic Eventrations in France: Management Strategies and Long-term Results : an Observational Study.

Conditions
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Diaphragmatic Eventration

Intervention / Treatment
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* Other: clinical surveillance
* Procedure: diaphragmatic plication


Participation Criteria
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Eligibility Criteria
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Inclusion Criterion: Patients of 16 years of age and under at diagnosis with diaphragmatic eventration, treated since 2010 in a pediatric surgery care unit in France. Exclusion Criterion: Patients with a diaphragmatic hernia Patient's parents or legual guardian opposed to being included in the study.

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 |
| --- | --- |
| diaphragmatic eventation with medical surveillance<br>Patients with a diagnosis of diaphragmatic eventration without respiratory or digestive consequences, thus not requiring surgical repair. | Other: clinical surveillance<br>* regular clinical follow up including chest X-rays, pulmonary investigations, etc...<br>|
| diaphragmatic eventration treated with plication<br>Patients with a diagnosis of diaphragmatic eventration with respiratory or digestive repercussion, requiring surgical repair. | Procedure: diaphragmatic plication<br>* plication of a hemidiaphragm, performed via laparoscopy, thoracoscopy (including robot-assisted surgery), or classical thoracotomy<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| To assess the level of asymptomatic patients diagnosed with diaphragmatic eventration depending on the type of treatment (surgical or not). | Asymptomatic patient rate defined as patients presenting without any digestive, respiratory or orthopaedic symptom. | Up to 16 years of age. From the date of diagnosis until the date of last check-up, assessed up to 16 years of age | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| To assess the occurrence of lung disease in patients with non treated diaphragmatic eventrations | Objectified by pulmonary function tests (PFTs), carried out according to the ATS/ERS-GLI (American Thoracic Society and European Respiratory Society-Global Lung Initiative) recommendations. | Up to 16 years of age. From the date of diagnosis until the date of last check-up, assessed up to 16 years of age | 
| To assess the rate of digestive symptoms in patients with diaphragmatic eventrations. | Clinical assessment of digestive symptoms linked to diaphragmatic eventration and/or to diaphragmatic plication, such à GERD, dumping syndrom, digestive occlusion or pain (scored by visual analogic scale). | Up to 16 years of age. From the date of diagnosis until the date of last check-up, assessed up to 16 years of age | 
| To assess the occurrence of orthopaedic impact of non treated diaphragmatic eventrations | objectified by the deformation of the spine on chest x-ray measured by the Cobb angle. | Up to 16 years of age. From the date of diagnosis until the date of last check-up, assessed up to 16 years of age | 
| To assess the severity rate of postoperative complications | Description of postoperative complications encountered after diaphragmatic plication, scored with the Clavien-Dindo classification. | Up to 16 years of age. From the date of diagnosis until the date of last check-up, assessed up to 16 years of age | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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thoracoscopy, laparoscopy, digestive disorders, restrictive syndrom