Chronic intestinal pseudo-obstruction (CIP)
What is it?
Chronic pseudo-intestinal obstruction is a rare disease characterized by a decrease in the ability of the intestinal muscles to push intestinal contents and gas (peristalsis) forward, which occurs in the absence of a real organic obstruction of the gastrointestinal tract. It affects both children and adults and is one of the most important causes of chronic intestinal insufficiency. Pseudo-intestinal obstruction can have an acute or chronic cause; it can be familial (i.e. hereditary) or sporadic; depending on its cause, it can be classified as secondary or idiopathic (when the cause is not distinguished).
Which are the symptoms?
Chronic pseudo-intestinal obstruction is manifested by a clinical and radiological picture of intestinal obstruction in the absence of real obstruction due to organic lesions that narrow the intestinal lumen. The most common symptoms are recurrent episodes of pain and/or bloating with stretching, constipation with inability to empty, nausea, vomiting, growth retardation, involuntary weight loss, despite a change in diet. Motor disorders of the esophagus with difficulty swallowing, reflux and non-cardiac pain may also be the cause.
How is it diagnosed?
The diagnosis of pseudo-intestinal obstruction is made only after the exclusion of organic causes of intestinal obstruction by means of endoscopic examinations, such as esophagogastroduodenoscopy and colonoscopy, Rx transit of the first digestive tract, Rx opaque enema or CT-enterography.
Therefore, the diagnosis can be confirmed already in childhood if there are at least two of the following diagnostic criteria present:
- Disorder of the neuromuscular system of the small intestine confirmed by manometry, intestinal scintigraphy or histological examination;
- Recurrent or persistent dilation of the loops of the small intestine with the presence of hydroaerodynamic levels on the X-ray (a characteristic sign of intestinal obstruction);
- Presence of genetic or metabolic abnormalities with an established association with childhood pseudo-intestinal obstruction;
- Inability to maintain adequate nutritional status and satisfactory growth by means of oral food, with the need to resort to enteral or parenteral artificial nutrition.
Laboratory tests (in particular, immunological, endocrinological and infection tests), radiological studies, endoscopic studies, urodynamic tests, tests of the function of the vegetal nervous system or neurological examinations are useful for identifying possible causes of secondary forms.
How is it treated?
Treatment options include prokinetics (promoting bowel emptying), antiemetics (reducing nausea and vomiting), spasmolytics or painkillers to relieve pain, non-absorbable antibiotics or systemic antibiotics (suppressing the growth of intestinal bacteria) or lactose-free probiotics to relieve symptoms and treat complications.
Enteral (intestinal) and parenteral (intravenous) nutrition play a key role in the treatment of this disease.
In some cases, the possibility of surgical intervention may be considered. Available interventions include digestive ostomy (for example, decompressive gastrostomy or caecostomy, diunostomy for internal nutrition, ileostomy, colostomy), which provide direct access to the affected gastrointestinal tract.
Intestinal tract transplantation is currently the only definitive method of treating pseudo-intestinal obstruction. However, due to the risk of complications and transplant failure this strategy is reserved for patients with massive dilation of the gastrointestinal tract or who are unable to continue parenteral nutrition for one of the following reasons: severe liver failure, lack of venous access, recurrent sepsis or metabolic disorders, or life-threatening disease leading to severe loss of water and electrolytes.
Where do we treat it?
Are you interested in receiving the treatment?