Hypoganglionosis of the myenteric plexus with normal Meissner's plexus: a new variant of colonic ganglion cell disorders.

A 16-year-old girl with a history of achalasia of the lower esophagus was investigated for progressive constipation since the age of five years. Normal ganglion cells of the Meissner's plexus were found in a mucosal rectal biopsy. Manometric studies showed absence of the normal rectosphincteric relaxation reflex. A long posterior rectal myectomy was performed in which no ganglion cells were found between the muscular layers. The patient underwent a rectosigmoid resection with a low anastomosis according to Duhamel. In the resected bowel, a normal Meissner's plexus was again found while marked hypoganglionosis of the myenteric plexus was evident. Intranuclear inclusion bodies in Schwann cells and scattered inflammatory cells were noteworthy. This case may be an example of an acquired hypoganglionosis localized to the myenteric plexus.