The Need and Scope for Stem Cell Based Therapy in Hirschsprung Disease

The role of enteric nervous system in gastrointestinal motility disorders in children has been better understood in recent years. One of the well-researched among these disorders is Hirschsprung disease (HSCR), also known as congenital intestinal aganglionosis. Even with current advances in the surgical management of HSCR significant postoperative morbidity such as defecation problems and enterocolitis occur despite correctly performed surgery. The need for adopting alternate forms of therapy in HSCR disease is well realized and stem cell therapy may provide this path in future. The understanding of embryology of neural crest stem cells and enteric nervous system progenitor cells and their migration to locations in the gut provided the early clues for stem cell therapy in HSCR. Identification of these cells in postnatal tissues at various anatomical locations including the intestines of infants, children and even patients with HSCR has provided the impetus for more intense research in this area. The autologous source of neural crest stem cells from the gut of HSCR patients is a major development in not being immunogenic. Despite these advances, several issues still remain to be solved before adoption of stem cell therapy for HSCR in humans. This mini-review highlights the current status and future directions of the role of stem cell therapy in therapy of HSCR.