Chronic Intestinal Pseudo Obstruction (CIPO)

Chronic intestinal pseudo obstruction (CIPO) is a rare condition that can affect all segments of the gastrointestinal tract and is characterized by failure of the propulsion of intraluminal contents due to the inability to generate suitable motor patterns, either due to deficit in the neuropathic or myopathic forces.

Commonly, in adults, it is secondary to a neuromuscular disease such as scleroderma, systemic sclerosis, bronchial carcinoma, or amyloidosis. In other cases, it may be a primary disease which selectively effects the smooth muscle or enteric nerve plexuses in which it may be a degenerative or inflammatory process.

Rarely, pseudo-obstruction arises acutely in a condition known as Ogilvie’s syndrome, and is concurrent with non-gastrointestinal disorders such as myocardial infarction, infection, or non-operative trauma. This syndrome is characterized by acute colonic dilation, pain, and abdominal distention. Ogilvie’s syndrome is thought to be caused by an imbalance of extrinsic autonomic nerves innervating the gut, with excessive inhibitory activity or diminished prokinetic cholinergic drive.

Adults with CIPO and irreversible intestinal failure are at high risk for:

• Major fluid and electrolyte imbalances associated with proximal gastrointestinal stomas or fistulas.

• Extreme bowel dilation with bacterial overgrowth.

• Intractable abdominal pain.

• Central venous line infections with resistant bacteria with high risk for metastatic abscesses.

• Infective myocarditis.

• Multiorgan failure.

Diagnosis:

• Manometry: Gold standard for diagnosis as it measures the contractions of the intestinal tract and can help to localize the functioning areas of the gastrointestinal tract. Manometry can help evaluate functional areas prior to any surgical intervention or creation of a stoma.

• Gastric emptying studies: Direct, noninvasive measurements of liquid or solid gastric emptying to assess function of the stomach.

• Full thickness biopsy: Full thickness biopsies are useful in diagnosis autoimmune or mitochondrial etiology for chronic intestinal pseudo-obstruction and can get a better understanding of abnormalities of the enteric nervous system.

Treatment:

• Nutritional Support: Majority of patients will require nutritional support, often requiring total parenteral nutrition (TPN).

• Prokinetics, such as erythromycin, octreotide, or tegaserod may be recommended.

• Antibiotics may be used to treat small intestinal bacterial overgrowth.

• Surgical: A decompressing gastrostomy or jejunostomy is reported to be a beneficial intervention in patients with chronic intestinal pseudo-obstruction.

• Small Bowel Transplant: May be indicated in patients who have TPN dependency and have TPN failure. Important to evaluate the presence or absence of gastric or colonic dysfunction, so the decision can be made whether to proceed with isolated or multivisceral transplant. It is also important to consider that the presence of bladder dysfunction in a CIPO patient may lead to recurrent urinary tract infections in the immunosuppressed patient.