National Study on the Quality of Life of Patients With Anorectal Malformation

Study Overview
=================
Brief Summary
-----------------
Anorectal malformations, occurring approximately 1 in 5000 live births, mainly involve the distal anus and rectum, but also sometimes the urinary and genital tracts. Defects range from the minor and easily treated with an excellent functional prognosis, to those that are complex and often associated with a poor functional prognosis. Despite the better knowledge of the anatomy and physiology and the improvement of surgical management after birth, fecal and urinary incontinence can occur, due mainly to deficient nerve supply. The quality of life of such patients is largely unknown in this country. The aim of the investigators study is to propose specific and generic questionnaires to the patients registered in the national database, correlated to their anatomical and functional status. A better understanding of such correlations should allow improvements in their medical and social management.

Detailed Description
-----------------
Anorectal malformations (ARM), occurring approximately 1 in 5000 live births, mainly involve the distal anus and rectum, but also sometimes the urinary and genital tracts. Defects range from the minor and easily treated with an excellent functional prognosis, to those that are complex and often associated with a poor functional prognosis. Despite the better knowledge of the anatomy and physiology and the improvement of surgical management after birth, fecal and urinary incontinence can occur, due mainly to deficient nerve supply. A few studies and clinical practice have shown that disease-specific problems of ARM have an effect on somatic function, mental health and psychosocial functioning. The assessment of quality of life requires a self-report questionnaire composed of items related to physical, emotional and social functioning, and disease-related symptoms. A specific ARM questionnaire, the HAQL, was developed by a Dutch team and published in 2001. Associated with generic questionnaire, it allowed quality of life assessment in Dutch population. The quality of life of such patients is largely unknown in this larger country. The aim of the investigators study is to propose specific (HAQL translated from Dutch to French) and generic questionnaires to the patients registered in the national database, correlated to their anatomical and functional status. A better understanding of such correlations should allow improvements in their medical and social management.

Official Title
-----------------
National Study on the Quality of Life of Patients With Anorectal Malformation (MARQOL)

Conditions
-----------------
Anorectal Malformation

Intervention / Treatment
-----------------
* Other: Quality of life questionnaire


Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Patients who are between 6 and 30 years old Patients initially treated in a french pediatric surgery service for anorectal malformation Exclusion Criteria: Patients lost from sight or dead Patients with concomitant malformations that can modify sphincter functions besides anorectal malformation (spina bifida, ) Patients whose brain functions do not allow to reply to a questionnaire (mental retardation)

Ages Eligible for Study
-----------------
Minimum Age: 6 Years
Maximum Age: 30 Years

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

Accepts Healthy Volunteers
-----------------
No

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

Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Children 6-7<br>Patients who are between 6 and 7 years old | Other: Quality of life questionnaire<br> <br> |
| Children 8-11<br>Patients who are between 8 and 11 years old | Other: Quality of life questionnaire<br> <br> |
| Children 12-16<br>Patients who are between 12 and 16 years old | Other: Quality of life questionnaire<br> <br> |
| Patients 17-30<br>Patients who are between 17 and 30 years old | Other: Quality of life questionnaire<br> <br> |

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

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Quality of life measured by HAQL specific questionnaire and two generic questionnaires: VSPA for children or WHOQOL-BREF for adults; functional status measured by Krickenbeck score | HAQL: between 35 and 48 items (depending on the age of the patients) composing 8 dimensions; VSPA: 39 items composing 8 dimensions; WHOQOL-BREF: 26 items composing 4 dimensions; Krickenbeck: 3 items. | At the time of the inclusion | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Kind of malformation, associated malformation, sequelas, handicap degree, social integration | Clinical questionnaire | At the time of the inclusion | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
Anorectal malformation, fecal incontinence, quality of life, child