Advancement in the clinical management of intestinal pseudo-obstruction

Abstract

Intestinal pseudo-obstruction is more commonly known in its chronic form (CIPO), a cluster of rare diseases characterized by gastrointestinal muscle and nerve impairment, so severe to result in a markedly compromised peristalsis mimicking an intestinal occlusion. The management of CIPO requires the cooperation of a group of specialists: the disease has to be confirmed by a number of tests to avoid mistakes in the differential diagnosis. The treatment should be aimed at relieving symptoms arising from gut dysmotility (ideally using prokinetic agents), controlling abdominal pain (possibly with non-opioid antinociceptive drugs) and optimizing nutritional support. Furthermore, a thorough diagnostic work-up is mandatory to avoid unnecessary (potentially harmful) surgery and to select patients with clear indication to intestinal or multivisceral transplantation.

Keywords:

• chronic intestinal pseudo-obstruction
• enteral nutrition
• intestinal diseases
• intestinal motility
• jejunostomy
• manometry
• parenteral nutrition
• prokinetics
• transplantation

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending or royalties.

No writing assistance was utilized in the production of this manuscript.

Key issues

• Chronic intestinal pseudo-obstruction (CIPO) is the tip of the iceberg of severe gut functional disorders.

• Based on its rarity in the clinical arena, CIPO is usually a challenge, often misdiagnosed.

• CIPO can be either idiopathic in origin or secondary to a wide array of diseases; the distinction between idiopathic and secondary cases is of crucial importance for treatment purposes.

• Gastrointestinal neuro-interstitial cells of Cajal-myopathies can represent a histopathological correlate of patients with CIPO.

• Diagnosis is essentially clinical (i.e., recurrent episodes of sub-occlusion) and confirmed by tests ruling out mechanical causes. Radiological techniques and now modern endoscopic approaches (providing full-thickness tissue biopsies) are crucial steps in the diagnostic work-up.

• Other emerging, non-invasive techniques are extremely interesting and provide optimism on future management of this rare condition.

• Nutritional support is the mainstay of CIPO clinical management.

• Medical treatment is still limited to few ‘ready-on-the market’ prokinetics agents, but the future is promising with many different types of molecules in the pipeline for clinical trials.

• Antinociceptive treatment as well as antibiotics to control abdominal pain and SIBO, respectively, is also mandatory in most cases.

• Futile, harmful laparotomies should be avoided, while elective surgery should be indicated in well-characterized cases; recurrent complications of total parenteral nutrition represent an indication to intestinal/multivisceral transplantation in pediatric and adult CIPO.

Notes