Novel concepts in the diagnosis, pathophysiology and management of idiopathic megabowel.

A proportion of patients with intractable constipation have persistent dilatation of the bowel, which in the absence of an organic cause is termed idiopathic megabowel (IMB). Whilst uncommon, this condition results in considerable morbidity. Traditional methods of identifying such patients are associated with inherent methodological limitations with anorectal manometry and contrast studies overestimating and underestimating the prevalence of the condition, respectively. Recently, controlled, pressure-based distension during fluoroscopic imaging has allowed more accurate identification of patients on the basis of a rectal diameter > 6.3 cm at the minimum distension pressure. Histopathological abnormalities of all three final effectors of sensorimotor function have been reported, although it remains unclear whether these changes are primary, secondary or epiphenomic. Physiological abnormalities of sensorimotor function, namely impaired perception of rectal distension and delayed colonic transit are well documented in patients with IMB. Further, the recent demonstration of two subgroups of patients, defined on the basis of rectal compliance, suggests the possibility that they differ pathophysiologically, although the clinical relevance of this distinction is uncertain. Surgery is performed when conservative therapy is ineffective or poorly tolerated. Numerous procedures have been attempted with variable success rates and significant mortality and morbidity. Surgery should preferably be performed in specialist centres given the relative infrequency with which such patients are encountered, and that they require comprehensive clinical, psychological and physiological evaluation preoperatively.