Intractable constipation and fecal incontinence are socially embarrassing and are commonly associated with spinal cord anomalies, anorectal malformations, Hirschsprung's disease and sacral agenesis. Initial conservative management of chronic constipation and fecal incontinence involves daily enemas, diet modification, stool softeners, suppositories, laxatives, and biofeedback. However, these programs are more unpleasant and difficult to manage as children get older. In the pre-MACE era when conservative management failed, children either faced the future wearing pads for fecal soiling or were doomed to permanent diverting colostomy as a last resort. The MACE procedure has allowed patients to become continent and independent with improved self-esteem.

Approximately 50% of patients with a neuropathic bladder secondary to myelomeningocele, who require bladder reconstructive surgery to establish continence or create a safe bladder, will also suffer from a neuropathic bowel with resultant chronic constipation and/or fecal incontinence, that does not respond to conservative measures. Conversely, up to 50% of patients born with an anorectal malformation will have long-term fecal incontinence, and as many as 50% of these will also have a neuropathic bladder, many of whom will require bladder reconstructive surgery. It is logical that these coexisting lower urinary tract and bowel problems should be managed simultaneously and not in isolation; in the majority of cases definitive bowel management usually rests with the reconstructive urologist. It is within this setting of holistic care that the MACE procedure was conceived and developed.

Principles of the MACE procedure

The MACE procedure simply combines three well-established surgical principles:

1. (a) the Mitrofanoff principle of a continent catheterizable abdominal stoma and conduit

2. […]