Chronic intestinal pseudo-obstruction in childhood: progress in diagnosis and treatment

Chronic intestinal pseudo-obstruction is a clinical diagnosis based on signs and symptoms of bowel obstruction in the absence of a physical obstruction demonstrable by X-ray or surgery. The phenotype may be caused by gastrointestinal motor, sensory, or psychological disturbances, or even by Munchausen's syndrome-by-proxy. Interactions between these pathophysiologic mechanisms may result in changes in the clinical presentation during development, including an increase in the number of affected children complaining of chronic abdominal pain. Chronic pain or the fear of pain may cause children to limit their voluntary oral intake, necessitating tube or parenteral supplements. A multidisciplinary team approach which includes input from the pediatric gastroenterologist, pain management specialist, and psychologist is most likely to effect positive change. In considering a diagnosis of chronic intestinal pseudo-obstruction, the clinician is obligated to evaluate gastrointestinal motility and sensory function, as well as the behavior of the child and family. This review aims to provide a biopsychosocial conceptual framework for understanding the heterogeneous group of gastrointestinal neuromuscular conditions that are diagnosed as chronic intestinal pseudo-obstruction. It will also provide a review and critique of the utility of new diagnostic methods, including antroduodenal and colonic manometry, and new therapeutic options, including the prokinetic agents such as cisapride which increase motility, and intestinal transplantation.