Disseminated mixed intestinal dysmotility (DMID): a new intestinal ganglion cell disorder?
We experienced two cases with disseminated HP and IND occurring with normal bowel in between (disseminated mixed intestinal dysmotility--DMID) and postulate whether it could be classified as a new intestinal motility disorder. Our cases, both boys, died at 3 and 7 months, respectively. Both had irregular stool passage, and abdominal distention with bilious vomiting since birth. On barium enema, both had rigid distal ileum and colon with narrow lumens, with dilated and atonic proximal ileum and jejunum. An ileostomy was created on days 3 and 2 of life, respectively, however, they did not function and jejunostomies were created, which also did not function well. Both boys died after repeated episodes of severe enterocolitis. In each case, three 10 cm specimens were obtained randomly from the jejunum and ileum, and two 5 cm specimens were obtained randomly from each of the ascending colon, transverse colon, descending colon, and rectum and treated with hematoxylin and eosin (H & E) staining, acetylcholine esterase (AchE) histochemistry, and protein gene product 9.5 (PGP9.5) and neural cell adhesion molecule (NCAM) immunohistochemistry for histopathologic assessment. All specimens showed a mixture of disseminated IND and HP, with normal intestine in between. There was abnormal expression of NCAM activity in the intestinal smooth muscle layers in small and large intestine. This is the first report about disseminated IND and HP occurring with normal bowel in between (DMID) and we suggest it should be classified as a new intestinal motility disorder. The present findings demonstrate that patients with DMID have a complicated abnormality of NMJ that may directly influence bowel motility and prognosis according to the severity of the abnormality.
Kobayashi
Hiroyuki
H
Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan. koba@med.juntendo.ac.jp
Yamataka
Atsuyuki
A
Lane
Geoffrey J
GJ
Miyano
Takeshi
T
D004065:Digestive System Abnormalities / Q000145:classification / Q000175:diagnosis / Q000473:pathology* / Q000503:physiopathology; D005725:Ganglia, Autonomic / Q000166:cytology*; D005769:Gastrointestinal Motility; D006627:Hirschsprung Disease / Q000145:classification / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D007231:Infant, Newborn; D008297:Male; D009130:Muscle, Smooth / Q000378:metabolism; D009197:Myenteric Plexus / Q000166:cytology; D019006:Neural Cell Adhesion Molecules / Q000378:metabolism
D019006:Neural Cell Adhesion Molecules