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22 Antegrade continence enemas Antegrade continence enemas (ACE) can be achieved by surgically creating an external entrance into the intestinal lumen, usually the cecum, to enable flushing fluids into the colon. There are different ways to establish this. A common surgical procedure to achieve ACE is the Malone appendicostomy. This procedure involves connecting the appendix to the abdominal wall and creating a valve, allowing catheterization for fluid administration and at the same time preventing leakage of stools. Another technique to achieve ACE is via percutaneous cecostomy, a minimally invasive procedure that involves the percutaneous introduction of a cecostomy tube. Outcomes of ACE are estimated to be favorable in 82% of cases, although complete resolution of constipation and fecal incontinence with reversal of the ACE stoma is achieved only in 9.5% of cases. 90 Sacral nerve stimulation (SNS) Sacral nerve stimulation (SNS) aims at stimulating the anterior ramus of sacral spinal nerves S3 and S4 via surgically positioned electrodes connected to a pulse generator that is implanted subcutaneously in the lateral buttock and can be switched on and off at will. The implantation of the device is often preceded by a period of peripheral nerve evaluation, which involves the placement of a temporary lead in an attempt to predict the outcome of SNS. Although the exact mechanism of SNS is incompletely understood, promising results have been published in adults and children with FC. 91–96 However, study designs, outcome measures and follow up durations vary widely between studies and sample sizes are generally small. 91,97 Larger randomized-controlled studies with long-term follow-up are required to gain more insights into the efficacy of SNS in the treatment of childhood FC. In chapter 15 , we evaluate long-term efficacy of SNS in children with constipation and describe patient benefit and parent satisfaction. Ostomies and resections In severe cases of intractable FC, surgery may prove to be beneficial. 90 Besides operations to achieve ACE, which are discussed above, surgical management of FC may involve botox injections, bowel resection or formation of a temporary or permanent diverting stoma. 98 In children with a megarectum or megasigmoid, which can be demonstrated by contrast enemas, a rectosigmoid resection may alleviate symptoms. 98 Surgery is generally performed in a step-up approach, beginning with the least invasive procedure and proceeding to more invasive treatment modalities only after failure of the previous step. In chapter 16 , outcomes of surgical management in children with intractable FC at a single tertiary children’s hospital are presented.

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