Dr. Schuffler spoke on the Pathologic Basis of Chronic Intestinal Pseudo-Obstruction Syndromes at the July 2009 AGMD Digestive Motility Symposium in Bedford, Massachusetts. The Association of Gastrointestinal Motility Disorders is composed of health professionals and lay people dedicated to educating about severe gastrointestinal motility disorders, such as chronic intestinal pseudo-obstruction and severe enteric dysmotility, and providing support to affected patients and their families. It publishes a monthly newsletter and holds a biannual conference which brings together health care providers who have a clinical and research in these disorders in order to share information and report new findings. The recent meeting, held on July 24-26th, was attended by several hundred people from around the country, including adult and pediatric gastroenterologists, surgeons, nutritionists, psychologists, patients and family members.

My presentation dealt with a review of the pathologic basis of chronic intestinal pseudo-obsruction syndromes. These syndromes are often primary gut disorders, but may also be part of systemic disorders, such as progressive systemic sclerosis, polymyositis, and myotonic dystrophy. They are caused by disorders of the smooth muscles or enteric nervous system, which, by specialized techniques, can be idenified under the microscope. They are mainly degenerative disorders of the smooth muscles or myenteric plexus. In some cases they are familial, whereas in most, they are sporadic in occurrence. They may occasionally be accomapanied by inflammatory infiltration of the smooth muscles or myenteric plexus. Infants and children may also be affected, in which case, the most frequent finding is arrested development of the myenteric plexus.

The best technique to view the pathology of the myenteric plexus is the Smith's silver stain technique. This shows beautiful morphology of neurons, dendrites, and axons. Degenerative changes are easily identified. Because of the complexity of this technique and its lack of availability, immunohistrochemistry is being increasingly used to identify neural and glial cell markers, and the interstitial cells of Cajal, which are often deficient in these disorders. If a full thickeness small intestinal biopsy is done to ascertain the underlying pathology, close coordination is requried between surgeon, gastroenterologist, and pathologist to make certain the tissue is handled correctly for these studies. In my talk, I pointed out that further studies are needed to define the normal neural innervation and supporting structures, as visualized by immunohistochemistry, and that studies should by done of mucosal innervation to detemine whether immunohistochemisty of mucosal biopsies might be sufficient to diagnose underlying diseases of the myenteric plexus.