Introduction

Chronic intestinal pseudo-obstruction (CIPO) is a rare, heterogeneous, and challenging gastrointestinal motility disorder lacking clinical guidelines in adults.

Aims & Methods

We aimed to develop an international consensus among international experts in gut motility on the diagnosis and management of CIPO based on existing literature and expert opinion. Two experts from each continental motility society and experts on pathology, surgery, and nutritional aspects of CIPO were selected for participation. A list of >130 statements was drafted, subdivided into 11 topics. After revisions and validation by the experts of the list of statements, a systematic literature review was performed to summarize available data on every statement. Using a Delphi process of 3 voting rounds, consensus statements were identified (defined as >80% agreement).

Results

Consensus was reached for 77/139 statements. The panel defined CIPO as symptoms caused by severe small bowel dysmotility without mechanical obstruction, and with documented dilated intestine or air/fluid levels, mimicking mechanical obstruction. Pseudo-obstructive episodes or continuous pseudo-obstructive symptoms were considered a cardinal feature of CIPO. Abdominal pain, bloating, and distension were also considered cardinal symptoms, while nausea/vomiting, diarrhea, or constipation were considered associated symptoms.
Radiographic imaging was considered mandatory in the diagnostic work-up, to confirm intestinal dilation or air/fluid levels and to rule out mechanical obstruction.. No other diagnostic test was considered indispensable for the diagnosis, including endoscopy, manometry, or laparoscopy. Screening for secondary causes of CIPO, including systemic sclerosis, paraneoplastic disease, or infectious disease, was supported, with a possible role for transmural histology. Differential diagnoses, including drug adverse effects, adhesions, or enteric radiation, should be ruled out. The experts also recommended screening for complications such as nutritional deficiencies and osteoporosis.
Management requires dietary adjustment, with supplementation for deficiencies, and setting up home parenteral nutrition if enteral nutrition fails. Small intestinal bacterial overgrowth was recognized as a main contributor to symptom aggravation and should be treated by intermittent antibiotics without the need for breath testing confirmation in patients with CIPO. There was no consensus regarding choice or efficacy of drug therapy, including prokinetics (e.g. pyridostigmine, prucalopride), laxatives, or somatostatin analogs. Opioids and surgical resections should be avoided; although endoscopic or surgical decompression procedures may be helpful in acute or persisting pseudo-obstructive symptoms.

Conclusion

This expert consensus developed key statements for the definition, diagnosis, and treatment of CIPO in adult patients. It provides the opportunity to harmonize the management of these patients and to highlight the gaps in the current literature.

Disclosure

Jan Tack has given Scientific advice to Aclipse, Adare, AlfaSigma, Clasado, Danone, Falk, FitForMe, Ironwood, Kyowa Kirin, Menarini, Promed, Ricordati, Takeda, Truvion, Tsumura, Zealand Pharmaceuticals, has received research support from Biohit, Kiowa Kirin, ProMed, Sofar and Takeda, and has served on the Speaker bureau for Abbott, Bio-Codex, Mayoly, Menarini, ProMed, Reckitt-Benckiser, Schwabe, Takeda, Thai Meiji and Truvion Pharmaceuticals.