|
1
|
Dalby C, Shen T, Thélin C, Ganam S, Velanovich V, Sujka J. Surgical and Therapeutic Interventions for Chronic Intestinal Pseudo-obstruction: A Scoping Review. J Neurogastroenterol Motil 2025; 31:8-17. [PMID: 39779199 PMCID: PMC11735203 DOI: 10.5056/jnm241031] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/27/2024] [Revised: 04/13/2024] [Accepted: 06/27/2024] [Indexed: 01/16/2025] Open
Abstract
Background/Aims Chronic intestinal pseudo-obstruction (CIPO) is a rare cause of intestinal dysmotility. First-line treatment in adult patients is medical and nutritional therapy. For patients who fail these treatment options, surgical interventions may be an option. In this scoping review, we aim to investigate the current research on surgical interventions for CIPO in adults. Methods PubMed, Embase, and Scopus were queried for articles related to surgical interventions for adults with CIPO. Search terms included: intestinal dysmotility, intestinal pseudo-obstruction, global intestinal dysmotility, chronic intestinal pseudo-obstruction, gastrointestinal paresis, neurogastrointestinal motility disorder, and chronic small intestinal motility disorder. Results Initial search identified 4763 records; 4722 were deemed irrelevant after screening and were excluded. The remaining 41 reports were retrieved and assessed for eligibility. Twenty-one additional studies were excluded after in-depth assessment. The remaining 20 reports were: 9 cohort studies, 7 case reports, and 4 reviews. Of these, 10 studies had study populations of < 10 patients, while 6 had ≥ 10 patients. The remaining 4 were reviews. Results of these papers described the safety and effectiveness of various surgical interventions for adults with CIPO, including percutaneous endoscopic procedures, surgical decompression, small bowel resection, and intestinal transplantation. Conclusions Data pertaining to surgical therapy for CIPO is limited. Although this review suggests that surgical interventions for CIPO may be safe and effective for select patients, strong conclusions cannot be made due to limited number of relevant studies and small sample sizes. Concerted efforts to produce data from large studies on adults with CIPO are necessary.
Collapse
Affiliation(s)
- Claire Dalby
- University of South Florida Morsani College of Medicine, Tampa, Florida, USA
| | - Thomas Shen
- University of South Florida Morsani College of Medicine, Tampa, Florida, USA
| | - Camille Thélin
- Departments of Gastroenterology, University of South Florida Morsani College of Medicine, Tampa, Florida, USA
| | - Samer Ganam
- Departments of Surgery, University of South Florida Morsani College of Medicine, Tampa, Florida, USA
| | - Vic Velanovich
- Departments of Surgery, University of South Florida Morsani College of Medicine, Tampa, Florida, USA
| | - Joseph Sujka
- Departments of Surgery, University of South Florida Morsani College of Medicine, Tampa, Florida, USA
| |
Collapse
|
|
2
|
Tayama S, Furuta Y, Morito M, Naoe H, Yoshii D, Uchida Y, Yoshimaru K, Miyamoto Y, Taguchi T, Tanaka Y. Chronic intestinal pseudo-obstruction due to adult-onset acquired isolated hypoganglionosis with muscular atrophy in the small intestine: a case report and review of literature. Clin J Gastroenterol 2024; 17:244-252. [PMID: 38193986 DOI: 10.1007/s12328-023-01902-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/31/2023] [Accepted: 11/28/2023] [Indexed: 01/10/2024]
Abstract
BACKGROUND Chronic intestinal pseudo-obstruction (CIPO) is a rare intestinal disorder characterized by impaired propulsion of the digestive tract and associated with symptoms of intestinal obstruction, despite the absence of obstructive lesions. CIPO includes several diseases. However, definitive diagnosis of its etiology is difficult only with symptoms or imaging findings. CASE PRESENTATION A 56-year-old man was referred to our hospital due to a 3-year history of continuous abdominal distention. Imaging, including computed tomography of the abdomen, and endoscopy revealed marked dilatation of the entire small intestine without any obstruction point. Therefore, he was diagnosed with CIPO. Since medical therapy didn't improve his symptoms, enterostomy and percutaneous endoscopic gastro-jejunostomy were performed. These procedures improved abdominal symptoms. However, he required home central venous nutrition due to dehydration. The pathological findings of full-thickness biopsies of the small intestine taken during surgery revealed decreased number and degeneration of ganglion cells in the normal plexus. These findings led to a final diagnosis of CIPO due to acquired isolated hypoganglionosis (AIHG). CONCLUSIONS Here, we report the case of a patient with CIPO secondary to adult-onset AIHG of the small intestine. Since AIHG cannot be solely diagnosed using clinical findings, biopsy is important for its diagnosis.
Collapse
Affiliation(s)
- Sayoko Tayama
- Department of Gastroenterology and Hepatology, Kumamoto University Hospital, 1-1-1, Honjo, Chuo-ku, Kumamoto-City, Kumamoto, 860-8556, Japan
| | - Yoki Furuta
- Department of Gastroenterology and Hepatology, Kumamoto University Hospital, 1-1-1, Honjo, Chuo-ku, Kumamoto-City, Kumamoto, 860-8556, Japan
| | - Miyuki Morito
- Department of Gastroenterology and Hepatology, Kumamoto University Hospital, 1-1-1, Honjo, Chuo-ku, Kumamoto-City, Kumamoto, 860-8556, Japan
| | - Hideaki Naoe
- Department of Gastroenterology and Hepatology, Kumamoto University Hospital, 1-1-1, Honjo, Chuo-ku, Kumamoto-City, Kumamoto, 860-8556, Japan
| | - Daiki Yoshii
- Department of Diagnostic Pathology, Kumamoto University Hospital, 1-1-1, Honjo, Chuo-ku, Kumamoto-City, Kumamoto, 860-8556, Japan
| | - Yasuyuki Uchida
- Department of Pediatric Surgery, Reproductive and Developmental Medicine, Graduate School of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka-City, Fukuoka, 812-8582, Japan
| | - Koichiro Yoshimaru
- Department of Pediatric Surgery, Reproductive and Developmental Medicine, Graduate School of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka-City, Fukuoka, 812-8582, Japan
| | - Yuji Miyamoto
- Department of Gastroenterological Surgery, Kumamoto University Hospital, 1-1-1, Honjo, Chuo-ku, Kumamoto-City, Kumamoto, 860-8556, Japan
| | - Tomoaki Taguchi
- Department of Pediatric Surgery, Reproductive and Developmental Medicine, Graduate School of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka-City, Fukuoka, 812-8582, Japan
- Fukuoka College of Health Sciences, 2-15-1 Tamura, Sawara-ku, Fukuoka, 814-0193, Japan
| | - Yasuhito Tanaka
- Department of Gastroenterology and Hepatology, Kumamoto University Hospital, 1-1-1, Honjo, Chuo-ku, Kumamoto-City, Kumamoto, 860-8556, Japan.
| |
Collapse
|
|
3
|
Suzon B, Louis-Sidney F, Abel A, Moinet F, Bagoée C, Henry K, Coco-Viloin I, Cougnaud R, Wolff S, Guilpain P, Rivière S, Flori N, Deligny C, Maria A. [Severe small bowel involvement and chronic intestinal pseudo-obstruction in systemic sclerosis (scleroderma): Pathophysiological, diagnostic and therapeutic basis, including parenteral nutrition]. Rev Med Interne 2024; 45:147-155. [PMID: 38388303 DOI: 10.1016/j.revmed.2024.02.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/04/2023] [Revised: 01/15/2024] [Accepted: 02/03/2024] [Indexed: 02/24/2024]
Abstract
Gastrointestinal involvement in systemic sclerosis can be severe, reaching the critical point of chronic intestinal pseudo-obstruction, secondary to major disorders of small bowel motility. It is associated with some clinical and biological characteristics, in particular the positivity of anti-fibrillarin/U3RNP antibodies. Chronic intestinal pseudo-obstruction (CIPO) is complicated by a small intestinal bacterial overgrowth that requires cyclic antibiotic therapy. CIPO leads to a reduction of the food intake, due to painful symptoms, nausea and vomiting caused by meals, and ultimately to severe malnutrition. Meal splitting is often transiently effective and patients require exogenous nutritional support, mostly parenteral. Systemic sclerosis is not an obstacle to initiation and long-term continuation of parenteral nutrition and central venous catheter implantation is not associated with an increased risk of cutaneous or infectious complications. However, continuation of long-term parenteral nutrition requires monitoring in an expert nutrition center in order to adapt nutritional volumes and intakes and to limit potentially fatal cardiac and hepatobiliary complications. In addition to nutrition, prokinetic treatments, whose side effects must be known, can be associated. Invasive procedures, whose risk-benefit ratio must be carefully assessed, can also be used to treat symptoms exclusively.
Collapse
Affiliation(s)
- B Suzon
- Médecine Interne, CHU de Martinique, Fort-de-France, Martinique; Unité EpiCliV, Université des Antilles, Fort-de-France, Martinique.
| | - F Louis-Sidney
- Unité EpiCliV, Université des Antilles, Fort-de-France, Martinique; Rhumatologie, CHU de Martinique, Fort-de-France, Martinique
| | - A Abel
- Médecine Interne, CHU de Martinique, Fort-de-France, Martinique
| | - F Moinet
- Médecine Interne, CHU de Martinique, Fort-de-France, Martinique
| | - C Bagoée
- Médecine interne et polyvalente, Centre hospitalier territorial Gaston-Bourret, Nouméa, Nouvelle-Calédonie
| | - K Henry
- Maladies infectieuses et tropicales, Centre hospitalier de Cayenne, Cayenne, Guyane
| | - I Coco-Viloin
- Médecine Interne, CHU de Martinique, Fort-de-France, Martinique
| | - R Cougnaud
- Médecine Interne, CHU de Martinique, Fort-de-France, Martinique
| | - S Wolff
- Médecine Interne, CHU de Martinique, Fort-de-France, Martinique
| | - P Guilpain
- Médecine interne et maladies multi-organiques, Hôpital Saint Eloi, CHU de Montpellier, Montpellier, France; Institut de médecine régénérative et biothérapies, Inserm U1183, Montpellier, France; Faculté de médecine, Université de Montpellier, Montpellier, France
| | - S Rivière
- Médecine interne et maladies multi-organiques, Hôpital Saint Eloi, CHU de Montpellier, Montpellier, France
| | - N Flori
- Centre expert régional de nutrition, ICM, Montpellier, France
| | - C Deligny
- Médecine Interne, CHU de Martinique, Fort-de-France, Martinique; Unité EpiCliV, Université des Antilles, Fort-de-France, Martinique
| | - A Maria
- Institut de médecine régénérative et biothérapies, Inserm U1183, Montpellier, France; Faculté de médecine, Université de Montpellier, Montpellier, France; Médecine interne et immuno-oncologie (MedI2O), Hôpital Saint-Eloi, CHU de Montpellier, Montpellier, France
| |
Collapse
|
|
4
|
Basilisco G, Marchi M, Coletta M. Chronic intestinal pseudo-obstruction in adults: A practical guide to identify patient subgroups that are suitable for more specific treatments. Neurogastroenterol Motil 2024; 36:e14715. [PMID: 37994282 DOI: 10.1111/nmo.14715] [Citation(s) in RCA: 2] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/30/2023] [Revised: 11/06/2023] [Accepted: 11/10/2023] [Indexed: 11/24/2023]
Abstract
Chronic intestinal pseudo-obstruction is a rare and heterogeneous syndrome characterized by recurrent symptoms of intestinal obstruction with radiological features of dilated small or large intestine with air/fluid levels in the absence of any mechanical occlusive lesion. Several diseases may be associated with chronic intestinal pseudo-obstruction and in these cases, the prognosis and treatment are related to the underlying disease. Also, in its "primary or idiopathic" form, two subgroups of patients should be determined as they require a more specific therapeutic approach: patients whose chronic intestinal pseudo-obstruction is due to sporadic autoimmune/inflammatory mechanisms and patients whose neuromuscular changes are genetically determined. In a context of a widely heterogeneous adult population presenting chronic intestinal pseudo-obstruction, this review aims to summarize a practical diagnostic workup for identifying definite subgroups of patients who might benefit from more specific treatments, based on the etiology of their underlying condition.
Collapse
Affiliation(s)
- Guido Basilisco
- Gastroenterology and Endoscopic Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
| | - Margherita Marchi
- Neuroalgology Unit, Fondazione IRCCS Istituto Neurologico "Carlo Besta", Milan, Italy
| | - Marina Coletta
- Gastroenterology and Endoscopic Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
| |
Collapse
|
|
5
|
Keller J, Wedel T, Seidl H, Kreis ME, van der Voort I, Gebhard M, Langhorst J, Lynen Jansen P, Schwandner O, Storr M, van Leeuwen P, Andresen V, Preiß JC, Layer P, Allescher H, Andus T, Bischoff SC, Buderus S, Claßen M, Ehlert U, Elsenbruch S, Engel M, Enninger A, Fischbach W, Freitag M, Frieling T, Gillessen A, Goebel-Stengel M, Gschossmann J, Gundling F, Haag S, Häuser W, Helwig U, Hollerbach S, Holtmann G, Karaus M, Katschinski M, Krammer H, Kruis W, Kuhlbusch-Zicklam R, Lynen Jansen P, Madisch A, Matthes H, Miehlke S, Mönnikes H, Müller-Lissner S, Niesler B, Pehl C, Pohl D, Posovszky C, Raithel M, Röhrig-Herzog G, Schäfert R, Schemann M, Schmidt-Choudhury A, Schmiedel S, Schweinlin A, Schwille-Kiuntke J, Stengel A, Tesarz J, Voderholzer W, von Boyen G, von Schönfeld J. Update S3-Leitlinie Intestinale Motilitätsstörungen: Definition, Pathophysiologie, Diagnostik und Therapie. Gemeinsame Leitlinie der Deutschen Gesellschaft für Gastroenterologie, Verdauungs- und Stoffwechselkrankheiten (DGVS) und der Deutschen Gesellschaft für Neurogastroenterologie und Motilität (DGNM). ZEITSCHRIFT FUR GASTROENTEROLOGIE 2022; 60:192-218. [PMID: 35148561 DOI: 10.1055/a-1646-1279] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 10/19/2022]
Affiliation(s)
- Jutta Keller
- Medizinische Klinik, Israelitisches Krankenhaus in Hamburg, Hamburg, Deutschland
| | - Thilo Wedel
- Institut für Anatomie, Christian-Albrechts-Universität Kiel, Kiel, Deutschland
| | - Holger Seidl
- Klinik für Gastroenterologie, Hepatologie und Gastroenterologische Onkologie, Isarklinikum München, München, Deutschland
| | - Martin E Kreis
- Klinik für Allgemein-, Viszeral- und Gefäßchirurgie, Charité, Campus Benjamin Franklin, Berlin, Deutschland
| | - Ivo van der Voort
- Klinik für Innere Medizin - Gastroenterologie und Diabetologie, Jüdisches Krankenhaus Berlin, Deutschland
| | | | - Jost Langhorst
- Klinik für Integrative Medizin und Naturheilkunde, Klinikum Bamberg, Bamberg, Deutschland
| | - Petra Lynen Jansen
- Deutsche Gesellschaft für Gastroenterologie, Verdauungs- und Stoffwechselkrankheiten, Berlin, Deutschland
| | - Oliver Schwandner
- Abteilung für Proktologie, Krankenhaus Barmherzige Brüder, Regensburg
| | - Martin Storr
- Zentrum für Endoskopie, Gesundheitszentrum Starnberger See, Starnberg
| | - Pia van Leeuwen
- Deutsche Gesellschaft für Gastroenterologie, Verdauungs- und Stoffwechselkrankheiten, Berlin, Deutschland
| | - Viola Andresen
- Medizinische Klinik, Israelitisches Krankenhaus in Hamburg, Hamburg, Deutschland
| | - Jan C Preiß
- Klinik für Innere Medizin - Gastroenterologie, Diabetologie und Hepatologie, Klinikum Neukölln, Berlin
| | - Peter Layer
- Medizinische Klinik, Israelitisches Krankenhaus in Hamburg, Hamburg, Deutschland
| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |
Collapse
|
|
6
|
Billiauws L, Cohen M, Cazals-Hatem D, Joly F. Small intestine motility disorders: Chronic intestinal pseudo-obstruction. J Visc Surg 2022; 159:S22-S27. [DOI: 10.1016/j.jviscsurg.2022.01.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
|
|
7
|
Chronic intestinal pseudo-obstruction: a case report with review of the literature and practical guidance for the clinician. Acta Gastroenterol Belg 2022; 85:85-93. [PMID: 35304998 DOI: 10.51821/85.1.9704] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/21/2022]
Abstract
Chronic Intestinal Pseudo-obstruction (CIPO) is a rare but debilitating and severe form of gastrointestinal dysmotility. The diagnosis is often made very late in the disease course due to its rarity and complexity. Treatment is mainly supportive, as there is no definitive cure. Pharmacologic therapy comprises prokinetics, antibiotics for bacterial overgrowth and pain management. Pain can also be alleviated with intestinal decompression in selected cases. Beside the pharmacologic therapy, nutrition and fluid replacement play a key role. Rarely, intestinal transplantation is necessary in patients with CIPO and intestinal failure. In this review, we describe an advanced CIPO case and provide an update of the clinical and diagnostic features and current management strategies. The goal of our review is to raise awareness around CIPO and to give practical guidance for the clinician.
Collapse
|
|
8
|
Arai Y, Ogawa M, Arimoto R, Ando Y, Endo D, Nakada T, Sugawara I, Yokoyama H, Shimoyama K, Inomata H, Kawahara Y, Kato M, Arihiro S, Hokari A, Saruta M. Megaesophagus and Megaduodenum Found Incidentally on a Routine Chest Radiograph During a Health Examination. Intern Med 2021; 60:2039-2046. [PMID: 33518569 PMCID: PMC8313925 DOI: 10.2169/internalmedicine.6324-20] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/12/2022] Open
Abstract
Chronic idiopathic intestinal pseudo-obstruction (CIIP) caused by impaired intestinal peristalsis leads to intestinal obstructive symptoms. A 20-year-old man had marked esophageal dilatation that was found incidentally on chest radiography during a health examination. Chest/abdominal contrast-enhanced computed tomography and endoscopy showed marked esophageal and duodenal dilatation without mechanical obstruction. Upper gastrointestinal series and high-resolution esophageal manometry revealed absent peristalsis in the dilated part. CIIP was suspected in the patient's father, suggesting familial CIIP. The patient likely had signs of pre-onset CIIP. This is the first case of suspected CIIP in which detailed gastrointestinal tract examinations were performed before symptoms appeared.
Collapse
Affiliation(s)
- Yoshinori Arai
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, The Jikei University Katsushika Medical Center, Japan
| | - Maiko Ogawa
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, The Jikei University Katsushika Medical Center, Japan
| | - Rikako Arimoto
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, The Jikei University Katsushika Medical Center, Japan
| | - Yoshitaka Ando
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, The Jikei University Katsushika Medical Center, Japan
| | - Daisuke Endo
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, The Jikei University Katsushika Medical Center, Japan
| | - Tatsuya Nakada
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, The Jikei University Katsushika Medical Center, Japan
| | - Ichiro Sugawara
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, The Jikei University Katsushika Medical Center, Japan
| | - Hiroshi Yokoyama
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, The Jikei University Katsushika Medical Center, Japan
| | - Keiko Shimoyama
- Department of Endoscopy, The Jikei University Katsushika Medical Center, Japan
| | - Hiroko Inomata
- Department of Endoscopy, The Jikei University Katsushika Medical Center, Japan
| | - Yosuke Kawahara
- Department of Endoscopy, The Jikei University Katsushika Medical Center, Japan
| | - Masayuki Kato
- Department of Endoscopy, The Jikei University Katsushika Medical Center, Japan
| | - Seiji Arihiro
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, The Jikei University Katsushika Medical Center, Japan
| | - Atsushi Hokari
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, The Jikei University Katsushika Medical Center, Japan
| | - Masayuki Saruta
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, The Jikei University School of Medicine, Japan
| |
Collapse
|
|
9
|
Di Nardo G, Manca MB, Iannicelli E, D'Ambra G, Laviano A, Guarino M, Parisi P, Pontieri FE, Rosati E, De Giorgio R. Percutaneous endoscopic gastrojejunostomy in pediatric intestinal pseudo-obstruction. Nutrition 2021; 86:111174. [PMID: 33601120 DOI: 10.1016/j.nut.2021.111174] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/03/2021] [Accepted: 01/23/2021] [Indexed: 11/30/2022]
Abstract
Pediatric intestinal pseudoobstruction (PIPO) is the "tip of the iceberg" of the most severe gut motility disorders. In patients with PIPO, the impairment of gastrointestinal propulsive patterns is such as to result in progressive obstructive symptoms without evidence of mechanical causes. PIPO is an important cause of intestinal failure and affects growth and pubertal development. Bowel loop and abdominal distension represent one of the main features of intestinal pseudo-obstruction syndromes, hence intestinal decompression is a mainstay in the management of PIPO. So far, pharmacologic, endoscopic, and surgical treatments failed to achieve long-term relief of bowel distension and related symptoms, including pain. Recent data, however, indicated that percutaneous endoscopic gastrojejunostomy (PEG-J) might be a minimally invasive approach for intestinal decompression, thereby improving abdominal symptoms and nutritional status in adult patients with chronic intestinal pseudo-obstruction. Based on these promising results, we treated for the first time a 12-y-old patient affected by PIPO refractory to any therapeutic options to obtain intestinal decompression by PEG-J. We showed that PEG-J yielded sustained small bowel decompression in the reported PIPO patient with considerable improvement of both abdominal symptoms and nutritional status. The positive outcome of the present case provides a basis to test the actual efficacy PEG-J versus other therapeutic approaches to intestinal decompression in patients with PIPO.
Collapse
Affiliation(s)
- Giovanni Di Nardo
- NESMOS Department, Pediatric Unit, Faculty of Medicine & Psychology, Sapienza - University of Rome, Sant'Andrea University Hospital, Rome, Italy.
| | - Maria Beatrice Manca
- Department of Medical-Surgical and Translational Medicine, Anesthesia and Intensive Care Unit, Sant'Andrea University Hospital, Sapienza University of Rome, Rome, Italy
| | - Elsa Iannicelli
- Department of Medical-Surgical and Translational Medicine, Radiology Unit, "Sapienza" University of Rome, Sant'Andrea Hospital, Rome, Italy
| | - Giancarlo D'Ambra
- Department of Medical-Surgical Sciences and Translational Medicine, Sant'Andrea Hospital, Sapienza University of Rome, Rome, Italy
| | - Alessandro Laviano
- Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy
| | - Matteo Guarino
- Department of Translational Medicine, University of Ferrara, Ferrara, Italy
| | - Pasquale Parisi
- NESMOS Department, Pediatric Unit, Faculty of Medicine & Psychology, Sapienza - University of Rome, Sant'Andrea University Hospital, Rome, Italy
| | - Francesco Ernesto Pontieri
- NESMOS Department, Neurology Unit, Faculty of Medicine & Psychology, Sapienza - University of Rome, Sant'Andrea University Hospital, Rome, Italy &Department of Clinical and Behavioral Neurology, IRCCS - Fondazione Santa Lucia, Rome, Italy
| | - Elisa Rosati
- Department of Medical-Surgical and Translational Medicine, Radiology Unit, "Sapienza" University of Rome, Sant'Andrea Hospital, Rome, Italy
| | - Roberto De Giorgio
- Department of Translational Medicine, University of Ferrara, Ferrara, Italy
| |
Collapse
|
|
10
|
Zhu CZ, Zhao HW, Lin HW, Wang F, Li YX. Latest developments in chronic intestinal pseudo-obstruction. World J Clin Cases 2020. [DOI: 10.12998/wjcc.v8.i23.5850] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023] Open
|
|
11
|
Zhu CZ, Zhao HW, Lin HW, Wang F, Li YX. Latest developments in chronic intestinal pseudo-obstruction. World J Clin Cases 2020; 8:5852-5865. [PMID: 33344584 PMCID: PMC7723695 DOI: 10.12998/wjcc.v8.i23.5852] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/06/2020] [Revised: 10/02/2020] [Accepted: 10/19/2020] [Indexed: 02/05/2023] Open
Abstract
Chronic intestinal pseudo-obstruction (CIPO) is a type of intestinal dysfunction presenting as symptoms of intestinal obstruction but without actual mechanical obstruction. An extremely low incidence, non-specific clinical symptoms, strong heterogeneity, and no definitive cause in some patients make CIPO very difficult to diagnose correctly. Imaging and gastrointestinal manometry are commonly used. Most patients have progressive worsening of their symptoms and require intervention, and nutritional assessment and treatment are very important to determine the prognosis. With improvements in surgical techniques, small bowel transplantation is a feasible treatment option for patients with advanced CIPO; however, the long-term prognosis for CIPO patients remains unsatisfactory. Generally, the disease is rare and difficult to diagnose, which leads to clinicians' lack of understanding of the disease and results in a high rate of misdiagnosis. This review describes the characteristics of CIPO and the latest developments in diagnosis and treatment, in detail. The goal of our review is to improve clinicians' understanding of CIPO so that the disease is identified quickly and accurately, and treated as early as possible to improve patients' quality of life.
Collapse
Affiliation(s)
- Chang-Zhen Zhu
- Department of Gastrointestinal Surgery, Beijing Tsinghua Changgung Hospital, School of Clinical Medicine, Tsinghua University, Beijing 102218, China
| | - Hong-Wei Zhao
- Department of Gastrointestinal Surgery, Beijing Tsinghua Changgung Hospital, School of Clinical Medicine, Tsinghua University, Beijing 102218, China
| | - Hong-Wei Lin
- Department of Gastrointestinal Surgery, Beijing Tsinghua Changgung Hospital, School of Clinical Medicine, Tsinghua University, Beijing 102218, China
| | - Feng Wang
- Department of Gastrointestinal Surgery, Beijing Tsinghua Changgung Hospital, School of Clinical Medicine, Tsinghua University, Beijing 102218, China
| | - Yuan-Xin Li
- Department of Gastrointestinal Surgery, Beijing Tsinghua Changgung Hospital, School of Clinical Medicine, Tsinghua University, Beijing 102218, China
| |
Collapse
|
|
12
|
Abstract
Chronic small bowel pseudo-obstruction is rare, and the disease process is poorly understood. Its clinical picture and radiographic findings can resemble mechanical small bowel obstruction and may lead to unnecessary surgery. We report a case of a 68-year-old man who presented acutely with severe abdominal distension and pain after a recent laparoscopic adhesiolysis. His abdominal CT scan revealed grossly distended small bowel with pneumatosis intestinalis and free intraperitoneal air, which led to an exploratory laparotomy. He had a history of having undergone numerous radiological and endoscopic investigations and multiple laparotomies/laparoscopic procedures but without a definitive diagnosis. Subsequent episodes of small bowel pseudo-obstruction occurred, and he developed intestinal failure. His care required the input of multiple healthcare professionals. He was ultimately referred to the National Intestinal Failure Unit for further assessment and management.
Collapse
Affiliation(s)
- Joyce Lim
- General Surgery, University of Sheffield, Sheffield, GBR
| | - Daniel Ashmore
- General Surgery, Hull and East Yorkshire Hospitals National Health Service Trust, Hull, GBR
| | - Chitakattil Oommen
- General Surgery, Rotherham National Health Service Foundation Trust, Rotherham, GBR
| |
Collapse
|
|
13
|
Abstract
PURPOSE OF REVIEW Chronic intestinal pseudo-obstruction (CIPO) is the most severe and disabling form of gastrointestinal dysmotility characterized by an impairment of coordinated propulsive activity in the gastrointestinal tract mimicking mechanical intestinal obstruction. Over the last few years, major advances have been made in the diagnostic and therapeutic management of this rare disorder. RECENT FINDINGS The present narrative review aims to summarize the current literature about the management of CIPO focusing on significant novelties about definition, epidemiology, diagnosis, and therapeutic options. The most significant advancement is a consensus on classification and dedicated diagnostic criteria for CIPO in children highlighting the distinctive features between adult and pediatric forms of CIPO (hence pediatric intestinal pseudo-obstruction). Despite no single diagnostic test is pathognomonic of CIPO and no recommended drug treatment is advised to improve gastrointestinal motility, recent reports suggest promising results in both diagnostic testing and therapy that might assist the diagnosis and help the management of patients with CIPO. SUMMARY The articles referenced in this review will help in optimizing the clinical management of this rare and severe disease in adult population.
Collapse
|
|
14
|
Gonzalez Z, McCallum R. Small Bowel Dysmotility, Pseudoobstruction, and Functional Correlation with Histopathology: Lessons Learned. Curr Gastroenterol Rep 2020; 22:14. [PMID: 32078071 DOI: 10.1007/s11894-020-0748-8] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
PURPOSE OF REVIEW Small bowel dysmotility is a broad heterogeneous term that encompasses a wide range of gastrointestinal disorders resulting from abnormal gut motility. Chronic intestinal pseudo-obstruction (CIPO) is a severe, rare, and complex small bowel motility disorder at the extreme end of this spectrum. It is characterized by failure of the intestinal tract to propel contents, which results in signs and symptoms of bowel obstruction albeit in the absence of any obstructive lesion(s). In this article, we discuss up-to-date diagnostic techniques, management options, and histopathological findings in CIPO. RECENT FINDINGS We will emphasize the latest diagnostic methodologies and therapeutic options as well as enteric histopathologic abnormalities in patients with CIPO. CIPO continues to be a clinical challenge. Several novel pharmacological agents hold promise including gastrointestinal hormone agonists and prokinetics. Furthermore, histopathologic findings may help guide therapy and provide further prognostic significance. At present, nutritional support, symptom management, and avoidance of long-term complications are the mainstay of treatment in CIPO.
Collapse
Affiliation(s)
- Zorisadday Gonzalez
- Department of Gastroenterology, GI Motility Training and Research, Texas Tech University Health Sciences Center El Paso, 4800 Alberta, MSC 41007, El Paso, TX, 79905, USA.
| | - Richard McCallum
- Division of Gastroenterology, Center for Neurogastroenterology and GI Motility, Paul L. Foster School of Medicine, Texas Tech University Health Sciences Center El Paso, 4800 Alberta, MSC 41007, El Paso, TX, 79905, USA
| |
Collapse
|
|
15
|
Barros LL, Farias AQ, Rezaie A. Gastrointestinal motility and absorptive disorders in patients with inflammatory bowel diseases: Prevalence, diagnosis and treatment. World J Gastroenterol 2019; 25:4414-4426. [PMID: 31496621 PMCID: PMC6710178 DOI: 10.3748/wjg.v25.i31.4414] [Citation(s) in RCA: 37] [Impact Index Per Article: 6.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/01/2019] [Revised: 07/04/2019] [Accepted: 07/19/2019] [Indexed: 02/06/2023] Open
Abstract
Inflammatory bowel diseases (IBD), Crohn`s disease and ulcerative colitis, are chronic conditions associated with high morbidity and healthcare costs. The natural history of IBD is variable and marked by alternating periods of flare and remission. Even though the use of newer therapeutic targets has been associated with higher rates of mucosal healing, a great proportion of IBD patients remain symptomatic despite effective control of inflammation. These symptoms may include but not limited to abdominal pain, dyspepsia, diarrhea, urgency, fecal incontinence, constipation or bloating. In this setting, commonly there is an overlap with gastrointestinal (GI) motility and absorptive disorders. Early recognition of these conditions greatly improves patient care and may decrease the risk of mistreatment. Therefore, in this review we describe the prevalence, diagnosis and treatment of GI motility and absorptive disorders that commonly affect patients with IBD.
Collapse
Affiliation(s)
- Luísa Leite Barros
- Department of Gastroenterology, University of São Paulo School of Medicine, São Paulo 05403-000, Brazil
| | - Alberto Queiroz Farias
- Department of Gastroenterology, University of São Paulo School of Medicine, São Paulo 05403-000, Brazil
| | - Ali Rezaie
- Division of Gastroenterology, Department of Medicine, Cedars Sinai Medical Center, Los Angeles, CA 90048, United States
| |
Collapse
|
|
16
|
Lindberg G. Pseudo-obstruction, enteric dysmotility and irritable bowel syndrome. Best Pract Res Clin Gastroenterol 2019; 40-41:101635. [PMID: 31594655 DOI: 10.1016/j.bpg.2019.101635] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/25/2019] [Accepted: 07/18/2019] [Indexed: 02/06/2023]
Abstract
New diagnostic techniques have advanced our knowledge about the irritable bowel syndrome. The majority of patients that we believed to have a psychosomatic disorder have received other diagnoses explaining their symptoms. Endoscopy makes it possible to diagnose celiac disease before it leads to malnutrition and allows the detection of microscopic colitis as a cause of watery diarrhea. At the severe end of the symptom spectrum enteric dysmotility marks the border at which IBS ceases to be a functional disorder and becomes a genuine motility disorder. Joint hypermobility or Ehlers-Danlos syndrome is present in a substantial proportion of patients with enteric dysmotility. Chronic intestinal pseudo-obstruction is the end-stage of a large number of very rare disorders in which failed peristalsis is the common denominator. Nutritional needs and symptom control are essential in the management of pseudo-obstruction. Home parenteral nutrition is life saving in more than half of patients with chronic intestinal pseudo-obstruction.
Collapse
Affiliation(s)
- Greger Lindberg
- Karolinska Institutet, Department of Medicine, Huddinge and Karolinska University Hospital Huddinge, Patient Area Gastroenterology, Dermatology, and Rheumatology, SE-14186, Stockholm, Sweden.
| |
Collapse
|
|
17
|
Anti-Hu-Mediated Paraneoplastic Chronic Intestinal Pseudo-Obstruction Arising From Small Cell Prostate Cancer. ACG Case Rep J 2019; 6:e00105. [PMID: 31616772 DOI: 10.14309/crj.0000000000000105] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/19/2018] [Accepted: 03/27/2019] [Indexed: 12/28/2022] Open
Abstract
Chronic intestinal pseudo-obstruction is a rare syndrome with high morbidity and mortality. The pathophysiology is not well understood, although it is postulated that it involves some sort of neuropathic and/or myopathic dysfunction resulting in intestinal dysmotility. We present the first case of chronic intestinal pseudo-obstruction secondary to a paraneoplastic syndrome associated with a primary small cell prostate cancer.
Collapse
|
|
18
|
Mitsuyoshi Y, Takakura K, Kobayashi T, Ogawa N, Sakurai T, Nakano M, Ukichi T, Ishiuji Y, Torisu Y, Saruta M. Chronic intestinal pseudo-obstruction with pneumatosis cystoides intestinalis in a patient with systemic sclerosis: A case report. Medicine (Baltimore) 2019; 98:e15480. [PMID: 31045831 PMCID: PMC6504328 DOI: 10.1097/md.0000000000015480] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/10/2023] Open
Abstract
RATIONALE Chronic intestinal pseudo-obstruction (CIPO) and pneumatosis cystoides intestinalis (PCI) are rare abdominal diseases and the pathological mechanisms have not been fully elucidated. Systemic sclerosis (SSc), which is characterized by the progressive sclerotic changes of skin and internal organs, is a refractory collagen disease and is frequently associated with digestive disorders including CIPO. PATIENT CONCERNS A 68-year-old woman who has been well managed for SSc over the long term, who presented with abdominal fullness for the first time. DIAGNOSES Abdominal X-ray and computed tomography (CT) images showed PCI with pneumoperitoneum findings. Based on the diagnosis of CIPO, we evaluated the intestinal peristalsis of the patient by using cine magnetic resonance imaging (MRI). INTERVENTIONS Oral medications of 15 g/d of Daikenchuto, 750 mg/d of Metronidazole and Sodium Picosulfate were started for improving the bowel peristaltic movement and decreasing intestinal gas production. OUTCOMES A great improvement of CIPO and PCI by multidrug therapy without any surgical treatments for such an unusual case. LESSONS This case indicates that SSc can be accompanied with not only CIPO but also PCI as digestive disorders and that cine MRI, which is a definitely beneficial imaging modality, can intelligibly visualize the peristalsis of the intestines and lead to successful medical control by noninvasive treatment.
Collapse
Affiliation(s)
| | | | | | | | | | | | - Taro Ukichi
- Division of Rheumatology, Department of Internal Medicine
| | - Yozo Ishiuji
- Department of Dermatology, The Jikei University School of Medicine, Tokyo, Japan
| | | | | |
Collapse
|
|
19
|
Iida T, Hirayama D, Sudo G, Mitsuhashi K, Igarashi H, Yamashita K, Yamano H, Nakase H. Chronic intestinal pseudo-obstruction due to al amyloidosis: a case report and literature review. Clin J Gastroenterol 2018; 12:176-181. [PMID: 30232705 DOI: 10.1007/s12328-018-0909-6] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/10/2018] [Accepted: 09/13/2018] [Indexed: 02/07/2023]
Abstract
A 59-year-old woman presented to our hospital with a 6-month history of nausea, weight loss, and abdominal distension. Physical examination revealed abdominal distension without tenderness, and edema, numbness, and multiple peripheral neuropathy in the limbs. Blood test results showed anemia, hypoproteinemia, and hypoalbuminemia. Immunoelectrophoresis detected kappa-type Bence-Jones protein in both the serum and urine. Bone marrow examination did not reveal an increase of plasma cells. Computed tomography showed intestinal distension and retention of intestinal contents. No obstructive intestinal lesions were observed. Lower gastrointestinal endoscopy showed a decrease in the vascular visibility of the rectal mucosa. Histological findings showed amyloid deposition, which was positive for amyloid light-chain (AL) κ. Thus, she was diagnosed with chronic intestinal pseudo-obstruction (CIPO) due to gastrointestinal and neurological involvement of AL amyloidosis. Her abdominal symptoms were gradually improved by the insertion of an ileus tube and medication. Although we recommended chemotherapy for stopping her disease progression, she did not want to receive it. She died 1 year later because of her pneumonia. We should keep in mind that amyloidosis is an important cause of CIPO. Histopathological examination by endoscopic biopsy is required for exact diagnosis and appropriate treatment for CIPO due to amyloidosis.
Collapse
Affiliation(s)
- Tomoya Iida
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan.
| | - Daisuke Hirayama
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan
| | - Gota Sudo
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan
| | - Kei Mitsuhashi
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan
| | - Hisayoshi Igarashi
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan
| | - Kentaro Yamashita
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan
| | - Hiroo Yamano
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan
| | - Hiroshi Nakase
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan
| |
Collapse
|
|
20
|
Bianco F, Eisenman ST, Colmenares Aguilar MG, Bonora E, Clavenzani P, Linden DR, De Giorgio R, Farrugia G, Gibbons SJ. Expression of RAD21 immunoreactivity in myenteric neurons of the human and mouse small intestine. Neurogastroenterol Motil 2018; 30:e13429. [PMID: 30069982 PMCID: PMC6150808 DOI: 10.1111/nmo.13429] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/28/2018] [Revised: 06/20/2018] [Accepted: 06/22/2018] [Indexed: 12/23/2022]
Abstract
BACKGROUND RAD21 is a double-strand-break repair protein and component of the cohesin complex with key roles in cellular functions. A RAD21 loss-of-function mutation was found in cases of chronic intestinal pseudo-obstruction (CIPO) with associated enteric neuronal loss. Analysis of RAD21 expression in the enteric nervous system is lacking, thus we aimed to characterize RAD21 immunoreactivity (IR) in myenteric ganglia. METHODS Double labeling immunofluorescence in mouse and human jejunum was used to determine colocalization of RAD21 with HuC/D, PGP9.5, neuronal nitric oxide synthase (nNOS), neuropeptide Y (NPY), choline acetyl transferase (ChAT), Kit, platelet-derived growth factor receptor-α (PDGFRα), and glial fibrillary acid protein (GFAP) IRs. RESULTS A subset of PGP9.5- and HuC/D-IR neuronal cell bodies and nerve fibers in the myenteric plexus of human and mouse small intestine also displayed cytoplasmic RAD21-IR Cytoplasmic RAD21-IR was found in 43% of HuC/D-IR neurons in adult and neonatal mice but did not colocalize with nNOS. A subset of ChAT-positive neurons had cytoplasmic RAD21-IR Punctate RAD21-IR was restricted to the nucleus in most cell types consistent with labeling of the cohesin complex. Cytoplasmic RAD21-IR was not detected in interstitial cells of Cajal, fibroblast-like cells or glia. Subsets of neurons in primary culture exhibited cytoplasmic RAD21-IR Suppression of RAD21 expression by shRNA knockdown abolished RAD21-IR in cultured neurons. CONCLUSIONS Our data showing cytoplasmic RAD21 expression in enteric neurons provide a basis toward understanding how mutations of this gene may contribute to altered neuronal function/survival thus leading to gut-motor abnormalities.
Collapse
Affiliation(s)
- F Bianco
- Department of Medical and Surgical Sciences (DIMEC), University of Bologna, Bologna, Italy
- Department of Veterinary Medical Sciences (DIMEVET), University of Bologna, Bologna, Italy
| | - S T Eisenman
- Enteric NeuroScience Program, Mayo Clinic, Rochester, MN, USA
- Department of Physiology and Biomedical Engineering, Mayo Clinic, Rochester, MN, USA
| | - M G Colmenares Aguilar
- Enteric NeuroScience Program, Mayo Clinic, Rochester, MN, USA
- Department of Physiology and Biomedical Engineering, Mayo Clinic, Rochester, MN, USA
| | - E Bonora
- Department of Medical and Surgical Sciences (DIMEC), University of Bologna, Bologna, Italy
| | - P Clavenzani
- Department of Veterinary Medical Sciences (DIMEVET), University of Bologna, Bologna, Italy
| | - D R Linden
- Enteric NeuroScience Program, Mayo Clinic, Rochester, MN, USA
- Department of Physiology and Biomedical Engineering, Mayo Clinic, Rochester, MN, USA
| | - R De Giorgio
- Department of Medical Sciences, Nuovo Arcispedale S.Anna, University of Ferrara, Ferrara, Italy
| | - G Farrugia
- Enteric NeuroScience Program, Mayo Clinic, Rochester, MN, USA
- Department of Physiology and Biomedical Engineering, Mayo Clinic, Rochester, MN, USA
| | - S J Gibbons
- Enteric NeuroScience Program, Mayo Clinic, Rochester, MN, USA
- Department of Physiology and Biomedical Engineering, Mayo Clinic, Rochester, MN, USA
| |
Collapse
|
|
21
|
Downes TJ, Cheruvu MS, Karunaratne TB, De Giorgio R, Farmer AD. Pathophysiology, Diagnosis, and Management of Chronic Intestinal Pseudo-Obstruction. J Clin Gastroenterol 2018; 52:477-489. [PMID: 29877952 DOI: 10.1097/mcg.0000000000001047] [Citation(s) in RCA: 44] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Chronic intestinal pseudo-obstruction (CIPO) is a rare disorder characterized by an impairment of coordinated propulsive activity in the gastrointestinal (GI) tract, which clinically mimics mechanical intestinal obstruction. CIPO is the most severe and debilitating form of GI dysmotility. CIPO may be primary or be secondary to pathology at any level of the brain-gut axis as well as systemic disease. The clinical features of CIPO are pleomorphic and largely depend on the site and extent of the segment of the GI tract involved. The diagnostic approach includes the need for investigations to exclude mechanical GI obstruction, screening for causes of secondary CIPO and the identification of the disease phenotype as well as the prompt recognition and treatment of complications such as malnutrition and small intestinal bacterial overgrowth. In managing this disorder, a holistic, multidisciplinary approach is needed with judicious use of pharmacotherapeutic agents. While currently there are no specific therapeutic modalities for CIPO, treatment is largely directed at maintaining adequate nutrition and electrolyte balance and enhancing coordinated GI motility. Surgery should be avoided unless advisable for carefully selected patients and may include stoma formation. This narrative review provides a concise overview of the literature on this rare, severe and complex disorder, and highlights the need and areas for further research to improve both diagnostics and therapeutics.
Collapse
Affiliation(s)
- Thomas J Downes
- Department of Gastroenterology, University Hospitals of the North Midlands, Stoke-on-Trent, Staffordshire
| | - Manikandar S Cheruvu
- Department of Gastroenterology, University Hospitals of the North Midlands, Stoke-on-Trent, Staffordshire
| | - Tennekoon B Karunaratne
- Department of Medical and Surgical Sciences, St.Orsola-Malpighi Hospital, University of Bologna, Bologna
| | - Roberto De Giorgio
- Department of Medical Sciences, Nuovo Arcispedale S. Anna, University of Ferrara, Ferrara, Italy
| | - Adam D Farmer
- Department of Gastroenterology, University Hospitals of the North Midlands, Stoke-on-Trent, Staffordshire.,Centre for Trauma and Neuroscience, Blizard Institute, Wingate Institute of Neurogastroenterology, Barts and the London School of Medicine & Dentistry, Queen Mary University of London, London.,Institute of Applied Clinical Science, University of Keele, Keele, UK
| |
Collapse
|
|
22
|
Di Nardo G, Karunaratne TB, Frediani S, De Giorgio R. Chronic intestinal pseudo-obstruction: Progress in management? Neurogastroenterol Motil 2017; 29. [PMID: 29143474 DOI: 10.1111/nmo.13231] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/08/2017] [Accepted: 09/17/2017] [Indexed: 12/13/2022]
Abstract
Chronic intestinal pseudo-obstruction (CIPO) is a severe form of gastrointestinal dysmotility (often due to derangement of the innervation/smooth muscle/interstitial cells of Cajal) with recurrent episodes of intestinal subocclusion mimicking a mechanical obstruction. Because of its complexity and heterogeneity, CIPO is often misdiagnosed or remains unrecognized until advanced stages. Management is a critical aspect in CIPO patient care. So far, most prokinetic drugs have not proven efficacy in restoring intestinal propulsion, thus nutritional support, fluid/electrolyte replacement, and antibiotics are the mainstay of treatment. In this issue of the journal, Ohkubo et al showed promising data indicating that percutaneous endoscopic gastro-jejunostomy (PEG-J) can be proposed as a measure for intestinal decompression, thereby improving CIPO-associated abdominal symptoms, including pain. In addition to a concise update of clinical and diagnostic features, the present minireview tackles management options, with a major emphasis on PEG-J, for CIPO patients.
Collapse
Affiliation(s)
- G Di Nardo
- Pediatric Gastroenterology Unit, Santobono-Pausilipon Children's Hospital, Naples, Italy.,Pediatric Gastroenterology Unit, International Hospital Salvator Mundi, Rome, Italy
| | - T B Karunaratne
- Department of Medical and Surgical Sciences, University of Bologna, Italy
| | - S Frediani
- Pediatric Surgery Unit, Sapienza - University of Rome, Italy
| | - R De Giorgio
- Department of Clinical Science, University of Ferrara, Italy
| |
Collapse
|