Surgical treatment and prognosis of chronic intestinal pseudo-obstruction in children

Chronic intestinal pseudo-obstruction (CIPO) is characterized by symptoms of intestinal obstruction in the absence mechanical blockage. The main objectives in the treatment of CIPO include maintenance of adequate nutrition and restoration of intestinal propulsion. Our aim was to document the results of surgical treatment and the long-term results of CIPO in children.

The cases of 22 children (14 boys and 8 girls) who underwent surgical treatment for CIPO between January 1985 and April 2004 were reviewed. Patients at initial presentation ranged in age from 1 day to 11 years (median, 3.5 months). The diagnosis of CIPO was based on clinical signs and symptoms, coupled with radiological and histological findings. Patients' outcomes fell into 4 groups, according to evaluations of oral intake, weight gain, and parenteral nutrition: expired, poor, fair, or good.

In 3 cases of localized disease, both total gastrectomy and total colectomy were performed in combination. Of 19 cases of generalized disease, 7 patients received enterostomy and/or intestinal biopsy, followed by total colectomy and Duhamel operation. Another 4 of those 19 patients underwent enterostomy repair, and 2 were treated by small bowel resection. In terms of outcome, 5 patients were expired, 5 were classified as poor, 4 were described as fair, and 8 had outcomes that fell into the good category. In expired patients, the causes of death were either line-associated sepsis or hypovolemic shock associated with intestinal obstruction. The only cases in which the outcomes were good were cases in which there was evidence of intestinal neuronal dysplasias.

We suggest that operative treatment should be considered, because of a high probability of both difficult diagnosis and intractable symptoms, which are unresponsive to medical therapy.