Chronic intestinal pseudo-obstruction (CIPO) is a rare disorder of intestinal dysmotility that leads to the disruption of enteric flow without a true mechanical obstruction.¹ This is the most severe form of intestinal dysmotility and is associated with a poor prognosis.² Diagnosis can be difficult due to the lack of specific symptoms and the ambiguity of diagnostic criteria. Diagnosis may require a combination of imaging, endoscopy, motility studies, histopathologic examination of biopsy specimens, and laboratory tests.² There is no curative therapy at present, with patients palliated by parenteral nutrition (PN) and several surgical or endoscopic interventions to identify, manage, and relieve symptoms.² CIPO can have a significant negative impact on a patient’s quality of life, causing persistent pain, bloating, inability to eat normally, and negative psychological effects.¹ Care of these complex patients often necessitates a multidisciplinary approach, involving nutrition, medicine, pain management, psychological support, patient education, and surgical interventions.^1,2

Many medical treatments for CIPO are advocated, including somatostatin analogues, cholinesterase inhibitors, serotonergic agents, and prostaglandins. Nevertheless, surgical interventions may become necessary in cases refractory to medical therapy.³ Patients who experience complications or poor quality of life while on long-term PN may also necessitate intestinal transplantation, whether isolated or multivisceral.³ However, surgical interventions for CIPO can be complicated by post-operative adhesions, which worsen intestinal dysmotility symptoms.⁴ Therefore, surgery should be approached with trepidation, especially since literature on such is limited.⁴ Importantly, as data is sparse, recommendations for interventions are based on surgical judgement for individual patients.

This scoping review was conducted to appraise and collate the extant research on surgical interventions for CIPO in adults, as well as identify any gaps in knowledge. A scoping review is utilized for several purposes, including identifying the available evidence in a given field, clarifying key concepts in the literature, examining how research is conducted on a certain topic, and identifying and analyzing knowledge gaps.⁵ We aim to assess the current state of surgical interventions and treatment options for these patients.

Eligibility Criteria

Studies were included if they (1) focused on intestinal dysmotility, (2) included surgical interventions, and (3) assessed adult patients. Studies were excluded if they focused on pediatric patients, did not discuss surgical interventions for intestinal dysmotility diseases, were not available in English, or were unable to be retrieved as full manuscripts.

Information Sources

For the initial list of sources, the following bibliographic databases were queried for articles published from January 1985 to March 2023: PubMed, Embase, and Scopus. The search strategies were drafted by an experienced librarian, and further refined through team discussion. Search terms were developed by the senior authors.

Search Terms

PubMed:

1. (surgical[tiab] OR Laparoscopy[tiab] OR Laparoscopic[tiab] OR surgery[tiab] OR operation[tiab] OR Transplant[tiab] OR resection[tiab] or surgical[tiab]) AND (“Intestinal dysmotility”[tiab] OR “intestinal pseudoobstruction”[tiab] OR “Global intestinal dysmotility”[tiab] OR “Chronic intestinal pseudoobstruction”[tiab] OR “Gastrointestinal paresis”[tiab] OR “neurogastrointestinal motility disorder”[tiab] OR “chronic small intestinal motility disorder”[tiab])

2. (surgical[tiab] OR Laparoscopy[tiab] OR Laparoscopic[tiab] OR surgery[tiab] OR operation[tiab]) AND (“Intestinal dysmotility”[tiab] OR “intestinal pseudoobstruction”[tiab] OR “Chronic intestinal pseudoobstruction”[tiab] OR “motility disorder”[tiab])

3. (surgical[tiab] OR surgery[tiab] OR operation[tiab]) AND (“dysmotility”[tiab] OR “CIPO”[tiab] OR “Chronic intestinal pseudoobstruction”[tiab] OR “neurogastrointestinal motility disorder”[tiab] OR “small bowel motility”[tiab])

Embase:

1. (transplant:ti,ab OR resection:ti,ab OR surgical:ti,ab OR laparoscopy:ti,ab OR laparoscopic:ti,ab OR surgery:ti,ab OR operation:ti,ab) AND (‘intestinal dysmotility’:ti,ab OR ‘intestinal pseudoobstruction’:ti,ab OR ‘global intestinal dysmotility’:ti,ab OR ‘chronic intestinal pseudoobstruction’:ti,ab OR ‘gastrointestinal paresis’:ti,ab OR ‘neurogastrointestinal motility disorder’:ti,ab OR ‘chronic small intestinal motility disorder’:ti,ab)

Scopus:

1. (transplant OR resection OR surgical OR laparoscopy OR laparoscopic OR surgery OR operation) AND (“intestinal dysmotility” OR “intestinal pseudoobstruction” OR “global intestinal dysmotility” OR “chronic intestinal pseudoobstruction” OR “gastrointestinal paresis” OR “neurogastrointestinal motility disorder” OR “chronic small intestinal motility disorder”)

Selection of Sources of Evidence

We sequentially evaluated the titles, abstracts, and then full text of all publications identified by our searches for potentially relevant publications. The team then discussed and finalized the preliminary list of sources that met the eligibility criteria as outlined above. We restricted our articles to those that focused on adult populations and surgical options for CIPO. We included select case reports that provided new information or perspectives on this topic. We then screened each selected publication’s references for relevant publications to broaden our reach. The final search results were exported into an Excel file, ported to EndNote for citations, and duplicates were removed by EndNote. We resolved disagreements on study selection and data extraction by consensus and discussion with other reviewers.

Data Charting Process

We jointly developed an Excel spreadsheet to determine pertinent variables. We independently charted the data, discussed the results, and iteratively updated the spreadsheet.

Data Extraction

For each included study, we identified the first author, publication year, country of origin, sample size, number of males and females in the sample, age range, whether the study was retrospective or prospective if applicable, study type, and results of each study related to surgical interventions for CIPO.

Literature Search Results

The initial search identified 4763 records: 371 from PubMed, 719 from Embase, and 3673 from Scopus. 4722 were deemed irrelevant after screening and were excluded. The remaining 41 reports were retrieved and assessed for eligibility. An additional 21 studies were excluded after in-depth assessment. The remaining 20 reports were included in this scoping review (Figure.

Figure 1. Process of Study Identification. Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) flow diagram illustrating the process of study identification for our scoping review. Includes the identification, screening, and inclusion stages.

Characteristics of Sources of Evidence

Included studies were either cohort studies (9 studies), case reports (7 studies), or reviews (4 studies). Studies’ characteristics, baseline demographics of patients, and results are summarized in Table.

Table. Characteristics and Results of Individual Sources of Evidence

Topic	Title	First author	Year of publication	Country of origin	n = ?	Sex distribution	Age range	Retrospective or prospective	Study type	Results
Percutaneous endoscopic enterostomy	Efficacy of percutaneous endoscopic gastro-jejunostomy (PEG-J) decompression therapy for patients with chronic intestinal pseudo-obstruction (CIPO)	Ohkubo H	2017	Japan	7	2 M, 5 F	24-66	Prospective	Pilot cohort study	PEG-J decompression therapy resulted in improved abdominal symptoms and nutritional status in 7 patients.
	Percutaneous endoscopic cecostomy (Introducer Method) in chronic intestinal pseudo-obstruction: report of 2 cases and literature review	Kullmer A	2016	Germany	2	1 M, 1 F	50-84	Retrospective	Case reports and literature review	Percutaneous endoscopic cecostomy resulted in reduced abdominal distention in 2 patients, and decreased abdominal pain in 1.
	Percutaneous endoscopic colostomy in patients with chronic intestinal pseudo-obstruction	Thompson A	2004	United Kingdom	3	2 M, 1 F	23-83	Retrospective	Case reports	Percutaneous endoscopic colostomy lead to improvement in symptoms in all 3 patients.
Decompression	Repetitive colonoscopic decompression as a bridge therapy before surgery in a pregnant patient with chronic intestinal pseudo-obstruction	Kim J S	2013	Korea	1	F	31	Retrospective	Case report	In a pregnant patient, repetitive colonoscopic decompression can be performed as a bridge therapy until elective surgery can be performed.
	The Surgeon’s role in the treatment of chronic intestinal pseudoobstruction	Murr M M	1995	United States	21	11 M, 10 F	3.5-80	Retrospective	Cohort study	12 patients treated with decompressive tube jejunostomy (7 patients), tube gastrostomy (4 patients), or both (1 patient), had amelioration of their symptoms.
	Chronic intestinal pseudo-obstruction in children and adults: diagnosis and therapeutic options	Di Nardo G	2017	Italy	N/A	N/A	N/A	N/A	Review	Decompression of the gastrointestinal tract via endoscopy, rectal tubes, or nasogastric suctioning can be therapeutic.
Small bowel resection or bypass	Chronic idiopathic intestinal pseudo-obstruction treated by near total small bowel resection: a 20-year experience	Lapointe R	2010	Canada	8	3 M, 5 F	15-65	Retrospective	Cohort study	Eight patients who underwent near-total enterectomy with continued parenteral nutrition had symptom improvement. All but 1 resumed oral feeding, although they continued to be PN-dependent.
	Intestinal pseudoobstruction caused by a new form of visceral neuropathy: palliation by radical small bowel resection	Schuffler M	1985	United States	1	M	22	Retrospective	Case report	Radical resection of the small intestine allowed for symptom improvement in a patient with intractable CIPO.
	Treatment of end-stage chronic intestinal pseudo-obstruction by subtotal enterectomy and home parenteral nutrition	Mughal M M	1988	United Kingdom	3	3 M	21, 55, 41	Retrospective	Case reports	Subtotal enterectomy with home parenteral nutrition improved symptoms for 3 patients with end stage CIPO.
	The surgeon’s role in the treatment of chronic intestinal pseudoobstruction	Murr M M	1995	United States	21	11 M, 10 F	3.5-80	Retrospective	Cohort study	Nine patients underwent intestinal resection or bypass with modest results.
Ileostomy, jejunostomy and colostomy as palliative surgical procedures	Surgical Treatment of chronic intestinal pseudo-obstruction: report of 3 cases	Shibata C	2003	Japan	3	2 M, 1 F	16-48	Retrospective	Case reports	Abdominal distention was improved in 3 patients treated with a loop ileostomy, ascending colostomy, and loop jejunostomy. Postoperative oral intake was improved in 1 patient, slightly improved in 1 patient, and not improved in 1 patient.
Intestinal transplantation	Intestinal transplantation for chronic intestinal pseudo-obstruction in adult patients	Masetti M	2004	Italy	6	2 M, 4 F	21-37	Retrospective	Cohort study	Five patients received isolated small bowel transplantation and 1 received multi-visceral transplantation. Over-all 1-year patient and graft survival was 83.3% and 66.6%, respectively.
	Isolated intestinal transplant for chronic intestinal pseudo-obstruction in adults: long-term outcome	Lauro A	2013	Italy	11	3 M, 8 F	17-50	Prospective	Cohort study	1-, 3-, and 5- year patient survival rates were 90%, 70%, and 70%, respectively. Graft 1-, 3-, and 5- year survival rates were 80%, 60%, and 60%, respectively.
	Multimodal surgical approach for adult patients with chronic intestinal pseudo-obstruction: clinical and psychosocial long-term outcomes	Lauro A	2018	Italy	24	7 M, 17 F	17-61	Retrospective	Cohort study	At the end of 2-year follow-up, 45.5% of transplant patients were still alive. Over 80% recovered oral intake. Patient perception of mental health improved after intestinal transplantation. However, patients still viewed their physical health as compromised.
	Chronic Intestinal pseudo-obstruction in children and adults: diagnosis and therapeutic options	Di Nardo G	2017	Italy	N/A	N/A	N/A	N/A	Review	Only about 9% of all intestinal transplantations are for CIPO, with patients suffering from acute graft rejection, infection, and intestinal perforation, similar to that of intestinal transplantation for other causes.
Effectiveness of surgical interventions	Nationwide survey on adult type chronic intestinal pseudo-obstruction in surgical institutions in japan	Masaki T	2012	Japan	103	46 M, 56 F, 1 Unknown	Not reported	Retrospective	Cohort study	Surgical treatment was most effective for symptomatic relief in the large bowel type, less effective in the small bowel type, and least effective in the large and small bowel type. The authors found that treatment with gastrostomy or enterostomy was effective, while resection or colostomy was not effective.
	Natural history of chronic idiopathic intestinal pseudo-obstruction in adults: a single center study	Stanghellini V	2005	Italy	59	40 F, 19 M	18-67	Prospective	Cohort study	88% of patients underwent surgical procedures, averaging 2.96 procedures per patient. Despite the large number of surgeries, one-third required long-term parenteral nutrition and two-thirds had nutritional limitations.
	Non-transplantation surgical approach for chronic intestinal pseudo-obstruction: analysis of 63 adult consecutive cases	Sabbagh C	2013	France	63	30 M, 33 F	15-79	Retrospective	Cohort study	Surgical management was associated with high postoperative morbidity and mortality rates as well as frequent re-operation.
Etiology, pathology, and diagnosis	Small intestine motility disorders: chronic intestinal pseudo-obstruction	Billiauws L	2022	France	N/A	N/A	N/A	N/A	Review	Management is based on symptomatic treatment and nutritional support. Surgery should be limited to select cases.
	Pathophysiology, diagnosis, and management of chronic intestinal pseudo-obstruction	Downes TJ	2018	United Kingdom	N/A	N/A	N/A	N/A	Review	Surgery may provide symptomatic relief, although the risk of developing postoperative adhesions should be considered. Intestinal transplantation may be considered for patients with complications of parenteral nutrition.
	Chronic intestinal pseudo-obstruction in children and adults: diagnosis and therapeutic options	Di Nardo G	2017	Italy	N/A	N/A	N/A	N/A	Review	Surgical interventions may become necessary in emergencies or refractory cases. Patients who experience complications or poor quality of life while on parenteral nutrition may necessitate intestinal transplantation.
	Chronic intestinal pseudo-obstruction	Gabbard S	2013	United States	1	F	18	Retrospective	Case report	For patients who fail symptomatic treatment or develop severe side effects from PN, small intestinal transplantation may be considered.
	Chronic intestinal pseudoobstruction	Lyford G	2004	United States	N/A	N/A	N/A	N/A	Review	Resection of involved segments should be performed with caution because the initial process may manifest in other segments following surgery. Small intestinal transplantation is a high-risk surgery performed in patients unable to tolerate parenteral nutrition.

M, male; F, female; N/A, not applicable; PEG-J, Percutaneous endoscopic gastro-jejunostomy; CIPO, chronic intestinal pseudo-obstruction.

Synthesis of Results

Twenty studies examined surgical interventions for adult patients with CIPO. The majority were from Italy (n = 5), the United States (n = 4), the United Kingdom (n = 3), or Japan (n = 3). Other countries with less than 3 studies included: France, Korea, Germany, and Canada. Ten studies had a study population of < 10 patients, while 6 had ≥ 10 patients. The remaining 4 studies were reviews without their own study population.

Four studies focused on the etiology, pathology, and diagnosis of CIPO in adults. Three focused on percutaneous endoscopic enterostomy for adults with CIPO. One discussed surgical decompression for adults with CIPO. Three focused on small bowel resection or bypass. One paper focused on ileostomy, jejunostomy and colostomy as palliative surgical procedures. Three papers discussed effectiveness of surgical interventions for adults with CIPO. Three focused on intestinal transplantation for CIPO. One paper, by Di Nardo et al,³ discussed etiology, pathology, and diagnosis, decompression, and intestinal transplantation. One paper, by Murr et al,⁶ discussed both decompression and small bowel resection.

What we have shown in this scoping review is the lack of studies with large sample sizes, leading to the limited ability to draw strong conclusions regarding surgical interventions for adults with CIPO. While the reviewed data suggests that surgical options may be safe and effective in relieving symptoms for adults with CIPO refractory to other non-invasive treatments or experiencing severe complications from PN, it is important to note that this conclusion is drawn from data from only a handful of patients. It should be emphasized that most surgical procedures are palliative, with the intent to “vent” or “decompress” the alimentary tract and are not curative. The sole exception is intestinal transplantation. This situation is exacerbated by the relatively rarity of CIPO, making large, multicenter studies on any intervention difficult to perform. This has resulted in a lack of consensus on what type of operations should be performed and when. As the old surgical aphorism goes, “when there are a lot of different ways to do something, they all work equally well or equally poorly.” Until studies with larger patient populations can be conducted, the true effectiveness of surgical interventions for this disorder remains to be seen.

Primary CIPO is due to intrinsic damage to the muscles (myopathy), nerves (neuropathy), or interstitial cells of Cajal (mesenchymopathy).¹ Secondary CIPO is a result of other conditions, including but not limited to connective tissue diseases, muscular dystrophy, central or peripheral nervous system pathologies, or autoimmune conditions.¹ CIPO’s diverse etiologies in adults translates to protean clinical manifestations. The most common gastrointestinal (GI) features are abdominal pain, nausea, vomiting, bloating, constipation, and diarrhea.⁷ Other symptoms may be present based on the location and extent of involvement. The multitude of conditions associated with CIPO and the varying presentations make rapid diagnosis a challenge, especially given its overall rarity. One study found the diagnosis of CIPO was made a median of 8 years after symptom onset.⁸ CIPO is a rare disorder, with 1 study estimating the prevalence and incidence of CIPO in Japan to be 0.9 per 100 000 and 0.225 per 100 000, respectively.⁹ These proportions are low as compared to other motility disorders, and this rarity likely contributes to the increased time to diagnosis as well as the lack of high-quality data for CIPO.⁹ In fact, one can consider this disease an “orphan” disease because of its rarity, yet causing immense suffering in those who live with it.

The sine qua non of CIPO is a history of GI symptoms of intestinal blockage without evidence of mechanical obstruction.¹⁰ Therefore, mechanical bowel obstruction must be definitively ruled out. Examining the specific segments of the involved GI tract with esophageal or colonic manometry¹⁰ may be indicated. Histopathologic analyses of biopsies of intestinal wall tissue can distinguish CIPO from other secondary causes.⁷ There is no surgical cure for CIPO. Management has been centered around oral nutrition, prokinetic medications, antibiotics to curb gut flora overgrowth, and chronic pain management, with surgery as the last resort.¹

Scope of Research on Surgical Interventions for Chronic Intestinal Pseudo-obstruction in the Adult Population

Percutaneous endoscopic gastrojejunostomy, colostomy, or cecostomy

This scoping review suggests that percutaneous endoscopic gastro-jejunostomy, colostomy or cecostomy result in effective palliation for adult patients with CIPO. Percutaneous endoscopic gastro-jejunostomy decompression therapy resulted in improved abdominal symptoms and nutritional status in 7 patients.¹¹ Percutaneous endoscopic cecostomy resulted in reduced abdominal distention in 2 patients, and decreased abdominal pain in 1.¹² These authors report 20 patients in the literature treated with percutaneous endoscopic cecostomy reporting improved symptoms.¹² Percutaneous endoscopic colostomy lead to improvement in symptoms in 3 patients.¹³ Note that this combined experience is of only 33 patients.

Decompression

Decompressive procedures are often used for adults with CIPO. Colonoscopic decompression can temporize a patient until more permanent decompression can be achieved.^3,14 Murr et al⁶ reported 12 patients treated with decompressive tube jejunostomy (7 patients), tube gastrostomy (4 patients), or both (1 patient), with patient-reported amelioration of their symptoms. Together, these papers suggest that procedures to achieve intestinal decompression can improved symptoms, but this is based on the outcomes of 16 patients.

Small bowel resection or bypass

Small bowel resection is another therapeutic option. This includes a near-total enterectomy with continued PN, with a study of 8 patients documenting symptom improvement.¹⁵ The authors recommend that in most cases, resection should remove practically all the small bowel.¹⁵ Others report radical resection improved the symptoms 1 patient¹⁶ and in 3 patients.¹⁷ Nine patients underwent intestinal resection or bypass⁶ with modest results. The recommendation for this very aggressive treatment is based on the outcomes of only 21 patients.

Ileostomy, jejunostomy and colostomy as palliative surgical procedures

Abdominal distention was improved in 3 patients treated with a loop ileostomy, ascending colostomy, and loop jejunostomy.¹⁸ Once again, treatments based on the results in a very small number of patients.

Scope of research on effectiveness of surgical interventions

One concern with any treatment modality is the effectiveness, sequelae, and adverse effects. A cohort study evaluating surgical treatment of CIPO found surgical procedures were most effective in the large bowel type, less effective in the small bowel type, and least effective in the combined large and small bowel type.¹⁹ The study also found that treatment with gastrostomy or enterostomy was effective, while resection or colostomy was not effective.¹⁹ The authors note that while resection may be beneficial in rare cases of localized involvement, these cases are often revealed to be diffuse disease after resection.¹⁹ A rather large study of 59 adult patients who underwent a variety of surgical procedures found that one-third required long-term PN and two-thirds had nutritional limitations.⁸ The authors therefore argue surgical procedures should be minimized. Another study of postoperative complications in 63 found unacceptably high morbidity, mortality, and re-operation rates.⁴ The authors recommended avoidance of surgery when possible and to instead optimize nutritional status.

Scope of the outcomes of intestinal transplantation

The only potentially curative operation is intestinal transplantation. Studies of 6 patients (retrospective),²⁰ 11 patients (prospective),²¹ and 11 patients (retrospective)²² demonstrated patient survival rates between 45% to 83% and graft survival rates between 60% and 80%. Symptom improvement occurred in over 80% of patients.²² Nevertheless, only about 9% of all intestinal transplantations are for CIPO,³ with patients suffering from acute graft rejection, infection, and intestinal perforation,³ similar to that of intestinal transplantation for other causes. However, like with other treatments, this is based on a relatively small number of patients.

Knowledge gaps

While this scoping review identified several surgical interventions for adult patients with CIPO, it also revealed gaps in our current knowledge on this topic. We could not identify prospective or randomized control trials in this patient population, perhaps due to the limited number of adult patients with CIPO, the severity of the disorder, and ambiguities in diagnostic criteria. Additionally, many patients who undergo surgical interventions is as a last resort, leading to a sicker, more complicated baseline study population than the average adult patient with CIPO. While our scoping review identified small studies from multiple countries, it also illustrated a lack of large, multi-center studies. Large multi-center randomized controlled trials of adult patients with CIPO who undergo surgical interventions are necessary to further evaluate the surgical techniques detailed in this review.

Limitations

A definitive diagnosis of CIPO in an adult patient can be difficult to make, as symptoms are often non-specific and insidious, and there are no specific biomarkers. CIPO can also present similarly to other GI dysmotilities. Additionally, as many patients experience onset of CIPO symptoms in childhood, and only a minority develop sporadic onset of CIPO later in life, the number of adult patients with this disorder is limited. CIPO is also very rare⁹, leading to a small number of patients with this disorder in whom to evaluate treatment options. Due to this limited number of adult patients diagnosed with CIPO and the use of surgery as the last resort, many studies had small sample sizes. As a result of the ambiguity of diagnostic criteria for this disorder, inclusion criteria varied among the studies included in this scoping review. These factors limit the reliability of study findings for this population.

CIPO in adult patients can present with a wide range of symptoms and varying degrees of severity, often leading to prolonged time to diagnosis and treatment. While medical therapies and PN are the mainstay of treatment, surgical interventions may be necessary for refractory cases or those who experience complications from PN. This scoping review illustrates that a variety of surgical interventions have been tried, with varying success, but based on observational studies of few patients. Palliative procedures such as percutaneous endoscopic enterostomy, decompression, ileostomy, jejunostomy, or colostomy may be considered for symptom relief in patients who do not respond well to medical and nutritional therapy. Small bowel resection or bypass may be considered in select patients with refractory CIPO of localized involvement, however this surgical option is not frequently recommended as the disease may later be found to be diffuse. Intestinal transplantation is potentially curative for patients with refractory CIPO or serious complications from PN, but comes with higher mortality rates than other treatment options. Therefore, clinicians should consider surgical intervention for select patients experiencing refractory CIPO or complications from PN judiciously. Future research in the field of CIPO management should focus on large multi-center studies of surgical interventions for adult patients with CIPO. While the diverse causes, presentations, and rarity of CIPO present challenges to initiating a large multi-center study, this is the necessary next step to better evaluate surgical interventions for this population.