Clinicopathologic features of allied disorders of Hirschsprung disease and status update.

Abstract

Allied disorders of Hirschsprung diseases (ADHDs) have abnormal enteric ganglia and symptoms that are similar to congenital megacolon. The classification of ADHDs includes neuronal dysplasia, enteric gangliocytomatosis, isolated ganglion dysfunction, immature ganglion, and internal anal sphincter achalasia. The diagnosis, treatment and prognosis of ADHDs are distinct from congenital megacolon. This review summarizes the classification, epidemiology, pathogenesis, gene basis, pathology, diagnosis, treatment, and prognosis of ADHDs based on existing diagnostic criteria.