ULTRASHORT SEGMENT HIRSCHSPRUNGS-DISEASE - A CASE-REPORT

Hirschsprung's disease (HD) or congenital aganglionosis usually presen ts with involvement of the rectosigmoid, except for a small percentage of the ultrashort segment cases in which dysfunction is limited to th e area of the anal sphincter. However, the diagnostic criteria for ult rashort segment HD are the subject of some controversy. The reported c ase 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. I t is suggested that modification of criteria to allow up to a 4-cm seg ment 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.