Ultrashort segment Hirschsprung's disease: A case report

Hirschsprung's disease (HD) or congenital aganglionosis usually presents with involvement of the rectosigmoid, except for a small percentage of the ultrashort segment cases in which dysfunction is limited to the area of the anal sphincter. However, the diagnostic criteria for ultrashort segment HD are the subject of some controversy. The reported case illustrates the gross and microscopic anatomy of a documented case of ultrashort segment HD and helps define the proper biopsy technique and confirm previously established criteria for accurate diagnosis. It is suggested that modification of criteria to allow up to a 4-cm segment of aganglionosis for the diagnosis of ultrashort segment HD would eliminate some of the current ambiguity. Functional disorders of the anal sphincter should be considered separately.