Chronic Intestinal Pseudo-Obstruction (CIPO) or Paediatric Intestinal Obstruction (PIPO)

By Cristiano Antonino On Jan 20, 2023

Chronic Pseudo-Intestinal Obstruction (CIPO) is a rare disease. It involves intestinal motor activity and is characterised by slowed intestinal transit and a reduced ability to pass food through the intestine

Chronic Pseudo-Intestinal Obstruction (CIPO) is a rare disease

It can affect adults and children, characterised by a severe disorder of intestinal motility with alteration of the propulsive capacity of the intestine, i.e. the movements of the intestinal muscles that propel intestinal contents and gas forward (peristalsis).

According to the most recent international guidelines, compiled in 2018 by the European Society of Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN), it is appropriate to distinguish the forms that begin in paediatric age as Pediatric Intestinal Pseudo-Obstruction (PIPO).

Included in the definition of Chronic Intestinal Pseudo-Obstruction (POIC) or Paediatric Intestinal Obstruction (PIPO) are a group of conditions with variable manifestation, in which the digestive system is incapable of advancing its contents, resulting in symptoms of intestinal obstruction, even in the absence of true mechanical obstruction.

The result is a partial or total inability to feed by mouth.

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The cause of Paediatric Pseudo-Intestinal Obstruction is not known

There are forms due to diseases of the intestinal muscles or nerves (myopathies or neuropathies or mitochondrial diseases).

Sometimes the disease occurs as a result of metabolic, endocrine, rheumatological, muscular or neurological disorders.

An alteration of the neuromuscular structures of the intestine is present, which is the basis for the alterations in movement, up to and including the total absence of peristalsis.

The disease can be generalised, i.e. involving the entire digestive system, or localised, with involvement of the small intestine in most cases.

Sometimes the musculature of other internal organs, such as the bladder, is also affected.

Paediatric pseudo-intestinal obstruction manifests itself with obstructive bowel symptoms, even in the absence of a real mechanical obstruction

Common symptoms are:

Abdominal bloating and distension;
Vomiting;
Constipation;
Feeding intolerance;
Growth retardation.

In fact, in addition to motility, digestive function is often impaired, with reduced ability to absorb the different foods we eat.

In the case of bladder involvement, there are symptoms of urinary retention, with difficulty or inability to urinate.

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The diagnosis of paediatric pseudo-intestinal obstruction, according to current international guidelines, is based on the presence of at least two of the following diagnostic criteria:

Compromise of the neuromuscular system of the small intestine, documented by manometry, intestinal scintigraphy or histological test;
Recurrent or persistent dilatation of loops of the small intestine with the presence on radiography of hydro-aerial levels (characteristic sign of intestinal obstruction);
Presence of genetic or metabolic abnormalities with established association with Paediatric Pseudo-Intestinal Obstruction;
Inability to maintain an adequate nutritional status and satisfactory growth with oral nutrition, with the need to resort to enteral or parenteral artificial nutrition.

Some tests allow the diagnosis of Chronic Intestinal Pseudo-Obstruction (CIPO) or Paediatric Intestinal Obstruction (PIPO) to be confirmed:

Plain abdominal X-ray: allows identification of typical signs of intestinal obstruction, such as dilated loops and water levels;
Contrast-enhanced abdominal radiography, or gastrointestinal transit: with the use of contrast medium, allows us to exclude the presence of intestinal malrotation or other mechanical obstructions;
Scintigraphy of gastrointestinal transit: allows the evaluation of transit and emptying times of the various tracts of the digestive tract, typically slowed down in Paediatric Intestinal Pseudo-Obstruction;
Manometry: allows the contractile capacity of the intestine to be measured, providing indications of the strength (muscular capacity) and coordination (neuronal capacity) of the peristaltic waves; in suspected Paediatric Intestinal Pseudo-Obstruction, the study of the small intestine (anthro-duodenal manometry) plays a crucial diagnostic and prognostic role, however, it is also indicated to study the other intestinal tracts (oesophagus, colon, anorectal region);
Full-thickness intestinal biopsy: histological analysis of the intestinal wall makes it possible to highlight any structural, muscular or nervous abnormalities; it is possible to take a biopsy for diagnostic purposes or to analyse an operative specimen following surgery (e.g. intestinal resection or ostomy placement);
Ultrasound of the kidneys and urinary tract: allows the assessment of possible involvement of the urinary tract;
Computed tomography (CT) and magnetic resonance imaging (MRI): allow for a more detailed study of anatomical structures and, using contrast medium, allow for the evaluation of the progression of intestinal contents and the possible presence of mechanical obstructions;
Blood tests: there are no diagnostic laboratory tests for paediatric pseudo-intestinal obstruction; in forms caused by other diseases, specific abnormalities can be detected;
Genetic analysis: there are very few forms of Paediatric Intestinal Pseudo-Obstruction associated with known genetic mutations, however genetic counselling and possible molecular analysis should be considered in patients with other congenital anomalies or forms that may be part of a syndrome;
Digestive endoscopy: allows Pediatric Pseudo-Intestinal Obstruction to be distinguished from other diseases that may manifest similar symptoms (e.g. other causes of malabsorption or mechanical obstruction).

The therapeutic management of Paediatric Intestinal Pseudo-Obstruction involves numerous specialists in a multidisciplinary team, including paediatricians, gastroenterologists with expertise in gastrointestinal motility, digestive surgeons, urologists, specialists in metabolic and genetic diseases, nutritionists and psychologists.

The primary goals are the treatment of obstructive symptoms and the maintenance of good nutritional status and growth.

Enteral (intestinal) and parenteral (intravenous) nutrition play a key role in the management of the disease.

There are no specific drugs for the treatment of paediatric pseudo-intestinal obstruction, however some therapies can be used to alleviate symptoms and manage complications, such as prokinetics (which promote bowel movements), antiemetics (which reduce nausea and vomiting) and antibiotics (which inhibit the growth of intestinal bacteria).

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Surgery plays a crucial role in the management of patients with Paediatric Intestinal Pseudo-Obstruction

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Digestive ostomies (e.g. gastrostomy, dijunostomy, ileostomy, colostomy) provide direct access to the affected gastrointestinal tract.
They can be used for enteral nutrition or drug administration and simultaneously represent a route of decompression from the gastrointestinal contents.
Intestinal transplantation represents to date the only definitive treatment for paediatric pseudo-intestinal obstruction.

However, in view of the high risk of complications and transplant failure, this strategy is reserved for patients with severe disease, intestinal insufficiency and severe complications from prolonged parenteral nutrition.

Paediatric pseudo-intestinal obstruction is a complex disease, which requires individualised management according to the needs of each patient.

A multidisciplinary and highly specialised team is required, given the complexity of diagnostic methods and care programmes.

The sharing of experience and expertise between national and international experts is the basis for improving daily clinical care and future research perspectives.

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