Evaluation of the Motor Activity, Cardiopulmonary Performance Capacity and Quality of Life in Patients Born With a Congenital Abdominal Wall Defect

Study Overview
=================
Brief Summary
-----------------
The two most common congenital abdominal wall defects (AWD) are gastroschisis and omphalocele. Prenatal detection is often possible and the defects are differentiated by the presence or absence of a sac around the eviscerated organs. A omphalocele occurs in 0.6-4.8 in 10,000 live births compared to 4.5 in 10,000 live births with gastroschisis. In the last years a rising incidence of gastroschisis has been shown worldwide. Both forms of AWDs necessitate early surgical intervention, mostly in one or two stages, and support at an intensive care unit in the first days of life. Additionally, patients need parenteral feeding in the first weeks of life. The outcome depends on the size of the defect and on the associated malformations. The literature about long-term outcome of these malformations is scarce. Some publications have reported long-term complications like redo-surgical procedures because of fascial gaps or umbilical or incisional hernias. Furthermore, stool irregularities, abdominal pain and several admission to the hospital due to ileus or sub-ileus have been described. Additionally, half of the patients are unsatisfied with the cosmetic result. Some other studies have shown that children born with an AWD have the same quality of life (QoL) compared with the healthy community. Nevertheless, patients with AWDs need a standardized, structured and multimodal long-time follow-up program to be able to detect any problems early and give advice to understand their illness in order to achieve the same QoL as healthy children. Therefore, the aim of this dissertation will be: to prospectively assess the motor activity, cardiopulmonary performance capacity and QoL of patients treated with AWDs in our Department to suggest a new standardized follow-up protocol for patients born with an AWD

Official Title
-----------------
Evaluation of the Motor Activity, Cardiopulmonary Performance Capacity and Quality of Life in Patients Born With a Congenital Abdominal Wall Defect

Conditions
-----------------
Abdominal Wall Defect

Intervention / Treatment
-----------------
* Diagnostic Test: CombynTM Function & Spaces ECG
* Diagnostic Test: Blood Taking
* Diagnostic Test: Spiroergometry
* Diagnostic Test: Dordel Koch Test (DKT)
* Diagnostic Test: Ultrasound
* Diagnostic Test: Stance and gait analyses


Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: All patients born with an abdominal wall defect Exclusion Criteria: mental disease

Ages Eligible for Study
-----------------
Minimum Age: 6 Years
Maximum Age: 18 Years

Sexes Eligible for Study
-----------------
All

Study Plan
=================
How is the study designed?
-----------------

Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Abdominal Wall Defect<br>patients born with an abdominal wall defect | Diagnostic Test: CombynTM Function & Spaces ECG<br>* Multi-frequency impedance measurement to assess the muscle and fat mass<br>Diagnostic Test: Blood Taking<br>* Sampling of blood of the finger pad to assess liver function<br>Diagnostic Test: Spiroergometry<br>* Spiroergometry to assess cardiopulmonary capacity. The intensity will be raised in steps until total exhaustion. In between each step we will take blood of the ear lobe to determine the lactate level<br>Diagnostic Test: Dordel Koch Test (DKT)<br>* Dordel Koch Test (DKT) to evaluate the motor activity. The DKT is a heterogeneous test battery for children and adolescents and consists of seven parts: lateral jumping, sit and reach, situps, long stand jump, one-legged stand, push-ups and 6-min-run<br>Diagnostic Test: Ultrasound<br>* Ultrasound for abdominal wall muscles<br>Diagnostic Test: Stance and gait analyses<br>* Stance and gait analyses for measuring the core stability<br>|
| Control Group<br>age and sex matched Control Group | Diagnostic Test: CombynTM Function & Spaces ECG<br>* Multi-frequency impedance measurement to assess the muscle and fat mass<br>Diagnostic Test: Blood Taking<br>* Sampling of blood of the finger pad to assess liver function<br>Diagnostic Test: Spiroergometry<br>* Spiroergometry to assess cardiopulmonary capacity. The intensity will be raised in steps until total exhaustion. In between each step we will take blood of the ear lobe to determine the lactate level<br>Diagnostic Test: Dordel Koch Test (DKT)<br>* Dordel Koch Test (DKT) to evaluate the motor activity. The DKT is a heterogeneous test battery for children and adolescents and consists of seven parts: lateral jumping, sit and reach, situps, long stand jump, one-legged stand, push-ups and 6-min-run<br>Diagnostic Test: Ultrasound<br>* Ultrasound for abdominal wall muscles<br>Diagnostic Test: Stance and gait analyses<br>* Stance and gait analyses for measuring the core stability<br>|

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Motor Activity | Comparison of the Dordel Koch Test between the two groups. (T-Test or Mann-Withney-U) | 30 minutes | 
| Cardiopulmonary Performance Capacity - lung function | Comparing relative lung function [%] between the two groups.(T-Test or Mann-Withney-U) | 30 minutes | 
| Cardiopulmonary Performance Capacity - peak VO2 | Comparing peak VO2 between the two groups.(T-Test or Mann-Withney-U) | 30 minutes | 
| Gastrointestinal Quality of Life | Gastrointestinal Quality of Life Index (GIQLI): most desirable option: 4 points, least desirable option: 0 points GIQLI score: sum of the points - Score Range: 0-148 Compare the mean between the two groups (T-Test or Mann-Withney-U) | 15 minutes |