INTRODUCTION: Ultrashort-segment Hirschsprung's disease as a cause of obstr
ucted defecation is controversial because of a lack of knowledge regarding
the normal aganglionic zone of the distal rectum. The intent of this study
was to define the normal aganglionic zone of the distal rectum through hist
ologic review of cadaveric dissections. METHODS. Cadavers were obtained fro
m the anatomic pathology laboratory at the University of Massachusetts Medi
cal School. Strip myectomy with overlying mucosa was performed from distal
to the dentate line to at least 3 cm into the anal canal and rectum after t
he pelvis was hemisected. Specimens were fixed in 10 percent buffered forma
lin. Specimens were sectioned serially every 3 mm and embedded in paraffin.
Four-micron slices were then stained with hematoxylin and eosin. Each sect
ion was examined by a gastrointestinal pathologist. The presence or absence
of nerves and ganglion cells was recorded. RESULTS: Thiry cadavers were ob
tained for analysis. Mean age at time of death was 79 (range, 65-97) years.
There were 13 men. The mean distance of aganglionic bowel from the dentate
line was 6.6 (range, 0-21) mm in Meissner's plexus and 5.1 (range, 0-15) m
m in Auerbach's plexus. CONCLUSION: The normal distance of aganglionic bowe
l wall is 2 cm or less from the dentate line. The absence of ganglion cells
proximal to this normal aganglionic zone in the patient with clinical find
ings of Lifelong obstructed defecation defines ultrashort-segment Hirschspr
ung's disease.