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Review

Pharmacological and nutritional therapy of children and adults with chronic intestinal pseudo-obstruction

, , , , , , & show all
Pages 325-341 | Received 24 Oct 2022, Accepted 18 Mar 2023, Published online: 26 Mar 2023
 

ABSTRACT

Introduction

Chronic intestinal pseudoobstruction (CIPO) is a rare, heterogenous, and severe form of gastrointestinal dysmotility.

Areas covered

Pertinent literature on pediatric and adult CIPO management has been assessed via PubMed, Scopus, and EMBASE from inception to June 2022. Prokinetics, aimed at restoring intestinal propulsion (e.g. orthopramides and substituted benzamides, acetyl cholinesterase inhibitors, serotonergic agents, and others), have been poorly tested and the available data showed only partial efficacy. Moreover, some prokinetic agents (e.g. orthopramides and substituted benzamides) can cause major side effects. CIPO-related small intestinal bacterial overgrowth requires treatment preferably via poorly absorbable antibiotics to avoid bacterial resistance. Apart from opioids, which worsen gut motility, analgesics should be considered to manage visceral pain, which might dominate the clinical manifestations. Nutritional support, via modified oral feeding, enteral, or parenteral nutrition, is key to halting CIPO-related malnutrition.

Expert opinion

There have been significant roadblocks preventing the development of CIPO treatment. Nonetheless, the considerable advancement in neurogastroenterology and pharmacological agents cast hopes to test the actual efficacy of new prokinetics via well-designed clinical trials. Adequate dietary strategies and supplementation remain of crucial importance. Taken together, novel pharmacological and nutritional options are expected to provide adequate treatments forthese patients.

Article highlights

  • The pharmacological and nutritional treatments of pediatric and adult chronic intestinal pseudo-obstruction (PIPO/CIPO, respectively) remain challenging.

  • Prokinetics, e.g. orthopramides and substituted benzamides, acetyl cholinesterase inhibitors, and serotonergic agents, yielded only partial results in improving intestinal propulsion and controlling dysmotility-related symptoms.

  • Abdominal pain and distension (along with bloating) are usually severe symptoms/signs in PIPO/CIPO patients and require pharmacological (avoiding opioids)/non-pharmacological (endoscopic decompressive measures) anti-nociceptive strategies.

  • Small intestinal bacterial overgrowth (SIBO) should be managed by using poorly absorbable antibiotics and prokinetic drugs. In line with this, in patients with mild forms of PIPO/CIPO dietary indications should be advised (i.e. low-sugar, -fibre and -fat diet).

  • Each patient should be evaluated by a multi-disciplinary team (including nutritionists and dieticians) to establish an individualized step-wise dietary/nutritional strategy (from oral to enteral/parenteral nutrition).

  • Significant changes are awaited in the treatment strategies hopefully improving quality and life expectations of PIPO/CIPO patients.

Abbreviations

ACIs=

acetyl cholinesterase inhibitors

ACPO=

acute colonic pseudo-obstruction

ANNA=

anti-nuclear neuronal antibodies

CIPO=

chronic intestinal pseudo-obstruction

EMA=

European Medicines Agency

FDA=

Food and Drug Administration

FMT=

fecal microbiota transplantation

FODMAP=

fermentable oligosaccharide, disaccharide, and monosaccharide and polyols

GI=

gastrointestinal

hERG=

human ether-à-go-go

5-HT=

5-hydroxytryptamine

IBS=

irritable bowel syndrome

ICC=

interstitial cells of Cajal

IFALD=

intestinal failure associated liver disease

MMC=

motor migrating complex

NSAID=

non-steroidal anti-inflammatory drug

PEG-J=

percutaneous endoscopic gastro-jejunostomy

PIPO=

pediatric intestinal pseudo-obstruction

PN=

parenteral nutrition

SIBO=

small intestinal bacterial overgrowth

TCA=

tricyclic antidepressant

Declaration of interest

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.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Data availability statement

There is no data set available for this paper.

Authors’ contribution

Conceptualization: GDN, LZ, GB, VS, RDG. Methodology: GDN, LZ, MG, AM, PP. Project administration: GDN, LZ, VS, RDG. Supervision: GDN, LZ, MG, AM, PP, GB, VS, RDG. Writing the original draft: GDN, LZ, AM, PP. Writing/review & editing: MG, GB, VS, RDG. All authors have seen and approved the manuscript and its contents.

Additional information

Funding

This paper was funded by ‘Fondi Ateneo per la Ricerca’ (FAR) and ‘Fondi Incentivazione alla Ricerca’ (FIR) research funds from the University of Ferrara, Ferrara, Italy.

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