Chronic intestinal pseudo-obstruction

Chronic intestinal pseudo-obstruction (CIPO) is a rare disorder characterized by a partial or even total absence of gastrointestinal propulsion. Before assuming this diagnosis it is imperative to exclude the existence of a mechanical obstruction.

This disease may be idiopathic or even secondary when promoted by connectivities, endocrinological diseases, amyloidosis, radiation, toxic exposure, infections or malignancies.

Regarding paraneoplastic syndromes, there is a disorder called anti-Hu syndrome mediated by autoantibodies with the same name, sensitized by antigen presented by cancer cells reacting against neuronal proteins.

In the case of CIPO it is thought that autoantibodies attack the myenteric and submucosal plexus of the digestive tract which may cause a secondary aganglionosis.

Anti-Hu syndrome has a bleak prognosis, with 47% survival rate at the end of the 1st year, and 12% at 5 years

Mortality is often associated with neurological manifestations (60%) rather than with tumor progression (40%). On a count performed in 2008 there were only 69 cases of POIC by anti-Hu syndrome described in medical literature.

We briefly mention a case reported in the literature of a 45 years old woman, with POIC caused by anti-Hu syndrome, followed for 13 years, with multiple episodes of “controlled” occlusion through the use of oral erythromycin. The last described case of this kind was approached with a radical regimen that involved a subtotal resection of the small bowel. Analysis of the surgical removed tissue showed a diffuse aganglionosis of the intestinmal nerve plexus.

We believe that in this case several conditions that normally do not occur simultaneously coexist. Common genesis can occur as an immune disorder that affects the intestinal nerve plexus and disrupts the transmission of the endplate and subsequently aganglionosis.

This a clinical case with very rare occurrence, difficult to treat and that requires a multidisciplinary approach. A prognosis is difficult to establish in such cases.

Thus far, Erythromycin appears to be one of the best “weapons” to treat POIC.

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