Chronic intestinal pseudo-obstruction (CIPO) is a syndrome defined by the p
resence of chronic intestinal dilation and dysmotility in the absence of me
chanical obstruction or gross inflammatory disease. Specific diseases may a
ffect any level of the brain-gut axis. For most patients, the diagnosis rel
ies upon a combination of historical, laboratory, manometric and histologic
al features. Recent advances into the autoimmune nature of etiologies such
as Chagas' disease and paraneoplastic dysmotility and into the genetic basi
s of mitochondrial neurogastrointestinal encephalomyopathy, multiple endocr
ine neoplasia IIB and Hirschsprung's disease have greatly refined our under
standing and diagnosis of these disorders. At present, medical therapy of C
IPO remains limited. Current and future developments in pharmacologic agent
s targeting specific enteric neurotransmitters and motility patterns hold m
uch promise for improving the care of the patients afflicted with this comp
lex and often debilitating syndrome. Copyright (C) 2000 S. Karger AG, Basel
.