Idiopathic proximal hemimegacolon: radiologic findings and analyses of clinical and physiological characteristics.

BACKGROUND

Idiopathic megacolon with bowel dilatation only proximal to the splenic flexure is an uncommon and poorly characterized disease. This study aimed to identify the characteristics of idiopathic proximal hemimegacolon.

METHODS

Five patients (2 males and 3 females) were diagnosed to have idiopathic proximal hemimegacolon and their radiologic, clinical, and physiological characteristics were reviewed. Hirschsprung's disease and other known causes of bowel dilatation were excluded by the presence of rectoanal inhibitory reflex and reviewing medical records.

RESULTS

Mean age at diagnosis and symptom onset were 50.4 years and 47.2 years, respectively. Four patients presented with constipation, and all five patients showed abdominal distention and abdominal pain. Four patients were successfully treated and maintained only with laxatives and prokinetics without enema. Only one patient underwent surgery for intractable constipation. Mean maximal diameters of each segment of colons measured by computed tomography were 57.5 mm, 69.3 mm, 73.0 mm, 33.3 mm, 24.0 mm, and 27.3 mm for cecum, ascending colon, transverse colon, descending colon, sigmoid colon, and rectum, respectively. Colon transit time was delayed in four patients with mean value of 90.0 h.

CONCLUSIONS

These results indicated that patients with idiopathic proximal hemimegacolon may be treated successfully only with medical therapy in most cases.