[Clinical study of multiple zonal aganglionosis in long segment Hirschsprung's disease].

Objective: To discuss pathogeny and diagnosis and management of multiple zonal aganglionosis in Hirschsprung's disease.

Methods: Records of 3 children, aging 5 days, 29 days and 18 months, 3 boys, with multiple zonal aganglionosis in long segment Hirschsprung's disease between 1987-2005 were reviewed retrospectively. Total colectomy and Soave's operations were performed.

Results: 3 children were diagnosed HD before surgery, but the convulsive stenosis in distal ileum and proximal ascending colon were detected during surgery. The aganglionic cells in the stenosis gut were confirmed by pathologic diagnosis.

Conclusions: The total colon and ileum should be detected carefully during surgery in children with long segment Hirschsprung's disease. It is believed not sound that the neuroblastic cells stop moving from neuroectoderm to gut in early gestation in HD, but it is also believed that some other causes in the course of gestation might interfere the normal growth of the ganglionic cells.