NEW INSIGHTS IN THE DIAGNOSIS AND MANAGEMENT OF HIRSCHSPRUNG’S DISEASE

Hirschsprung’s disease is a common pathology in pediatric surgery. Besides, long-term outcome of surgically-treated patients remains a crucial issue. The management of Hirschsprung’s disease has remarkably advanced over the years, but difficulties persist particularly in the developing countries (such as Vietnam), where essential diagnostic procedures, such as preoperative histopathological exploration techniques/ facilities (mainly for acetylcholinesterase staining), or adequate postoperative management and follow-up requirements are unavailable.We, therefore, contemplated to work-out a relevant histo-diagnostic approach to overcome these constraints that limit our diagnostic approaches, namely, in Vietnam, and we introduced a “less-demanding” diagnostic approach, namely calretinin immunohistochemical staining which is known to be adequate for formalin-fixed tissues (and thus not necessitating frozen section equipment). We thus used calretinin immunohistochemistry in a prospective study on a large cohort of Vietnamese HD cases. Results showed that rectal suction biopsy using calretinin immunohistochemistry provides an effective histopathological diagnostic tool that can replace AChE and provides a valuable evaluating approach for both preoperative and postoperative management.In addition, we also studied long-term outcome in operated patients and impact of postoperative morbidities on their quality of life. Indeed, a long-term multidisciplinary management with dedicated procedures such as anorectal manometry is essentially required for patients with severe defecation disorders.Doctorat en Sciences biomédicales et pharmaceutiques (Médecine)