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1
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Renaud A, Jirka A, Durant C, Connault J, Espitia O, Takoudju C, Agard C. [Gastrointestinal tract involvement in systemic sclerosis]. Rev Med Interne 2023; 44:410-422. [PMID: 37270380 DOI: 10.1016/j.revmed.2023.05.003] [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: 09/08/2022] [Revised: 04/17/2023] [Accepted: 05/08/2023] [Indexed: 06/05/2023]
Abstract
Gastrointestinal tract involvement in systemic sclerosis concerns more than 90% of patients but is of heterogeneous clinical expression. It can involve the entire intestinal tract and be responsible for multifactorial malnutrition, which is frequent in this disease. It is a major source of deterioration in the quality of life and can even be life-threatening. Management is complex and multidisciplinary, ranging from simple hygienic and dietary measures, to specialized endoscopic or surgical interventional procedures, also including medical treatments, particularly proton pump inhibitors and prokinetics, with potential side effects. Ongoing research for new diagnostic and therapeutic tools promises to improve the management and prognosis of these patients.
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Affiliation(s)
- A Renaud
- Service de médecine interne, Nantes université, CHU de Nantes, 1, place Alexis-Ricordeau, 44000 Nantes, France.
| | - A Jirka
- Service d'hépato-gastro-entérologie, Nantes université, CHU de Nantes, 44000 Nantes, France
| | - C Durant
- Service de médecine interne, Nantes université, CHU de Nantes, 1, place Alexis-Ricordeau, 44000 Nantes, France
| | - J Connault
- Service de médecine interne, Nantes université, CHU de Nantes, 1, place Alexis-Ricordeau, 44000 Nantes, France
| | - O Espitia
- Service de médecine interne, Nantes université, CHU de Nantes, 1, place Alexis-Ricordeau, 44000 Nantes, France
| | - C Takoudju
- Service d'hépato-gastro-entérologie, Nantes université, CHU de Nantes, 44000 Nantes, France
| | - C Agard
- Service de médecine interne, Nantes université, CHU de Nantes, 1, place Alexis-Ricordeau, 44000 Nantes, France
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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]
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Turcotte MC, Faure C. Pediatric Intestinal Pseudo-Obstruction: Progress and Challenges. Front Pediatr 2022; 10:837462. [PMID: 35498768 PMCID: PMC9045367 DOI: 10.3389/fped.2022.837462] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/16/2021] [Accepted: 03/15/2022] [Indexed: 11/29/2022] Open
Abstract
BACKGROUND Chronic intestinal pseudo-obstruction is a rare disorder and represents the most severe form of gastrointestinal dysmotility with significant morbidity and mortality. Emerging research shows considerable differences between the adult and pediatric population with intestinal pseudo-obstruction and the term Pediatric Intestinal Pseudo-Obstruction (PIPO) was recently proposed. PURPOSE The aim of this article is to provide pediatric gastroenterologists and pediatricians with an up to date review of the etiology and underlining pathophysiology, clinical features, diagnostic and management approaches currently available for PIPO and to discuss future perspectives for the diagnosis and management of this rare disease.
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Affiliation(s)
- Marie-Catherine Turcotte
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Sainte-Justine University Health Centre, Université de Montréal, Montreal, QC, Canada
| | - Christophe Faure
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Sainte-Justine University Health Centre, Université de Montréal, Montreal, QC, Canada
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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.
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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
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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.
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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
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Assessment of the Clinical Usefulness of Preoperative Computed Tomography in Colorectal Cancer Patients Who Received Unplanned Reoperation. Gastroenterol Res Pract 2020; 2020:6062414. [PMID: 32676105 PMCID: PMC7336223 DOI: 10.1155/2020/6062414] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/04/2020] [Revised: 05/31/2020] [Accepted: 06/16/2020] [Indexed: 11/17/2022] Open
Abstract
Background In the unplanned reoperation of colorectal cancer patients, computed tomography (CT) is increasingly utilized to locate postoperative complications and previously unlocalized lesions. The purpose of this study is to explore the application of CT in the mortality and complications of the reoperation of colorectal cancer. Patients and Methods. We performed a retrospective review of collected data from the colorectal surgeries of 90 identified colorectal cancer patients who received an unplanned reoperation from 2010 to 2018. Patients were stratified according to those with preoperative CT imaging (CT group, n = 36) and those without preoperative CT imaging (NCT group, n = 54). Twenty-four statistical indicators of each patient were studied, including their preoperative risk, surgical characteristics, and postoperative outcomes, and satisfaction was evaluated. All data were statistically analysed for predicting postoperative complications by univariate and multivariate logistic regression analyses. Results Ninety patients received an unplanned reoperation in the study, and 40% (36/90) of these patients underwent preoperative CT examination. Patients' risk factors were similar between CT and NCT groups. Preoperative imaging was more commonly performed for reoperative new anastomosis + ileostomy but less common for reoperative Dixon's procedure. The operative duration of the NCT group was longer (139 vs. 104 min, respectively, P = 0.01). Preoperative NCT examination (OR 1.24; 95% CI = 1.09-1.42; P = 0.01) was an independent predictor of postoperative complications. Importantly, three patients died after an unplanned reoperation for colorectal cancer, which occurred only in the NCT group (5.6% vs. 0.0%, P = 0.01). Conclusion The use of conventional preoperative CT optimizes the choice of the surgical site and the strategy of laparotomy, so as to reduce the length of operation. Preoperative imaging evaluation should be performed for patients undergoing repeat abdominal surgery.
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Zenzeri L, Tambucci R, Quitadamo P, Giorgio V, De Giorgio R, Di Nardo G. Update on chronic intestinal pseudo-obstruction. Curr Opin Gastroenterol 2020; 36:230-237. [PMID: 32073506 DOI: 10.1097/mog.0000000000000630] [Citation(s) in RCA: 25] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
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.
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Affiliation(s)
- Letizia Zenzeri
- Emergency Pediatric Department, Santobono-Pausilipon Children's Hospital, Naples
| | - Renato Tambucci
- Digestive Endoscopy and Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome
- Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila
| | - Paolo Quitadamo
- Department of Pediatrics, Santobono-Pausilipon Children's Hospital, Naples
| | | | | | - Giovanni Di Nardo
- Chair of Pediatrics, NESMOS Department, Faculty of Medicine and Psychology, Sapienza University of Rome, Sant'Andrea University Hospital, Rome, Italy
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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.
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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.
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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
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Paediatric Intestinal Pseudo-obstruction: Evidence and Consensus-based Recommendations From an ESPGHAN-Led Expert Group. J Pediatr Gastroenterol Nutr 2018; 66:991-1019. [PMID: 29570554 DOI: 10.1097/mpg.0000000000001982] [Citation(s) in RCA: 100] [Impact Index Per Article: 14.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
OBJECTIVES Chronic intestinal pseudo-obstructive (CIPO) conditions are considered the most severe disorders of gut motility. They continue to present significant challenges in clinical care despite considerable recent progress in our understanding of pathophysiology, resulting in unacceptable levels of morbidity and mortality. Major contributors to the disappointing lack of progress in paediatric CIPO include a dearth of clarity and uniformity across all aspects of clinical care from definition and diagnosis to management. In order to assist medical care providers in identifying, evaluating, and managing children with CIPO, experts in this condition within the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition as well as selected external experts, were charged with the task of developing a uniform document of evidence- and consensus-based recommendations. METHODS Ten clinically relevant questions addressing terminology, diagnostic, therapeutic, and prognostic topics were formulated. A systematic literature search was performed from inception to June 2017 using a number of established electronic databases as well as repositories. The approach of the Grading of Recommendations Assessment, Development and Evaluation (GRADE) was applied to evaluate outcome measures for the research questions. Levels of evidence and quality of evidence were assessed using the classification system of the Oxford Centre for Evidence-Based Medicine (diagnosis) and the GRADE system (treatment). Each of the recommendations were discussed, finalized, and voted upon using the nominal voting technique to obtain consensus. RESULTS This evidence- and consensus-based position paper provides recommendations specifically for chronic intestinal pseudo-obstruction in infants and children. It proposes these be termed paediatric intestinal pseudo-obstructive (PIPO) disorders to distinguish them from adult onset CIPO. The manuscript provides guidance on the diagnosis, evaluation, and treatment of children with PIPO in an effort to standardise the quality of clinical care and improve short- and long-term outcomes. Key recommendations include the development of specific diagnostic criteria for PIPO, red flags to alert clinicians to the diagnosis and guidance on the use of available investigative modalities. The group advocates early collaboration with expert centres where structured diagnosis and management is guided by a multi-disciplinary team, and include targeted nutritional, medical, and surgical interventions as well as transition to adult services. CONCLUSIONS This document is intended to be used in daily practice from the time of first presentation and definitive diagnosis PIPO through to the complex management and treatment interventions such as intestinal transplantation. Significant challenges remain to be addressed through collaborative clinical and research interactions.
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12
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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] [MESH Headings] [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.
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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
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Di Nardo G, Di Lorenzo C, Lauro A, Stanghellini V, Thapar N, Karunaratne TB, Volta U, De Giorgio R. Chronic intestinal pseudo-obstruction in children and adults: diagnosis and therapeutic options. Neurogastroenterol Motil 2017; 29. [PMID: 27683196 DOI: 10.1111/nmo.12945] [Citation(s) in RCA: 78] [Impact Index Per Article: 9.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/20/2016] [Accepted: 08/21/2016] [Indexed: 12/12/2022]
Abstract
BACKGROUND Chronic intestinal pseudo-obstruction (CIPO) represents the most severe form of gastrointestinal dysmotility with debilitating and potentially lethal consequences. Symptoms can be non-specific, and result in this condition being diagnosed incorrectly or too late with consequences for morbidity and even mortality. PURPOSE The present article aims to provide pediatric and adult gastroenterologists with an up to date review about clinical features, diagnosis and therapeutic options for CIPO. Although pediatric and adult CIPO share many clinical aspects distinctive features can be identified. There is no single diagnostic test or pathognomonic finding of CIPO, thus a stepwise approach including radiology, endoscopy, laboratory, manometry, and histopathology should be considered in the diagnostic work-up. Treatment of patients with CIPO is challenging and requires a multidisciplinary effort with participation of appropriately experienced gastroenterologists, pathologists, dieticians, surgeons, psychologists, and other subspecialists based on the presence of comorbidities. Current treatment options invariably involve surgery and specialized nutritional support, especially in children. Medical therapies are mainly aimed to avoid complications such as sepsis or intestinal bacterial overgrowth and, where possible, restore intestinal propulsion. More efficacious therapeutic options are eagerly awaited for such difficult patients.
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Affiliation(s)
- G Di Nardo
- Pediatric Unit, Orvieto Hospital, Orvieto, Italy
- Pediatric Gastroenterology Unit, International Hospital Salvator Mundi, Rome, Italy
| | - C Di Lorenzo
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Nationwide Children's Hospital, Columbus, OH, USA
| | - A Lauro
- Liver and Multiorgan Transplant Unit, St. Orsola-Malpighi University Hospital, Bologna, Italy
| | - V Stanghellini
- Department of Medical and Surgical Sciences, Centro di Ricerca BioMedica Applicata (C.R.B.A.), University of Bologna, Bologna, Italy
| | - N Thapar
- Department of Gastroenterology, Great Ormond Street Hospital, Institute of Child Health, London, UK
| | - T B Karunaratne
- Department of Medical and Surgical Sciences, Centro di Ricerca BioMedica Applicata (C.R.B.A.), University of Bologna, Bologna, Italy
| | - U Volta
- Department of Medical and Surgical Sciences, Centro di Ricerca BioMedica Applicata (C.R.B.A.), University of Bologna, Bologna, Italy
| | - R De Giorgio
- Department of Medical and Surgical Sciences, Centro di Ricerca BioMedica Applicata (C.R.B.A.), University of Bologna, Bologna, Italy
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Treyaud MO, Duran R, Zins M, Knebel JF, Meuli RA, Schmidt S. Clinical significance of pneumatosis intestinalis - correlation of MDCT-findings with treatment and outcome. Eur Radiol 2016; 27:70-79. [PMID: 27106233 PMCID: PMC5127863 DOI: 10.1007/s00330-016-4348-9] [Citation(s) in RCA: 42] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/20/2015] [Revised: 03/14/2016] [Accepted: 03/29/2016] [Indexed: 12/18/2022]
Abstract
Objectives To evaluate the clinical significance of pneumatosis intestinalis (PI) including the influence on treatment and outcome. Method and Materials Two radiologists jointly reviewed MDCT-examinations of 149 consecutive emergency patients (53 women, mean age 64, range 21-95) with PI of the stomach (n = 4), small (n = 68) and/or large bowel (n = 96). PI extension, distribution and possibly associated porto-mesenteric venous gas (PMVG) were correlated with other MDCT-findings, risk factors, clinical management, laboratory, histopathology, final diagnosis and outcome. Results The most frequent cause of PI was intestinal ischemia (n = 80,53.7 %), followed by infection (n = 18,12.1 %), obstructive (n = 12,8.1 %) and non-obstructive (n = 10,6.7 %) bowel dilatation, unknown aetiologies (n = 8,5.4 %), drugs (n = 8,5.4 %), inflammation (n = 7,4.7 %), and others (n = 6,4 %). Neither PI distribution nor extension significantly correlated with underlying ischemia. Overall mortality was 41.6 % (n = 62), mostly related to intestinal ischemia (p = 0.003). Associated PMVG significantly correlated with underlying ischemia (p = 0.009), as did the anatomical distribution of PMVG (p = 0.015). Decreased mural contrast-enhancement was the only other MDCT-feature significantly associated with ischemia (p p < 0.001). Elevated white blood count significantly correlated with ischemia (p = 0.03). Conclusion In emergency patients, ischemia remains the most common aetiology of PI, showing the highest mortality. PI with associated PMVG is an alerting sign. PI together with decreased mural contrast-enhancement indicates underlying ischemia. Key Points • In emergency patients, PI may be caused by various disorders. • Intestinal ischemia remains the most common cause of PI in acute situations. • PI associated with decreased mural contrast-enhancement indicates acute intestinal ischemia. • PI associated with PMVG should alert the radiologist to possible underlying ischemia.
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Affiliation(s)
- Marc-Olivier Treyaud
- Department of Diagnostic and Interventional Radiology, Lausanne University Hospital, Rue du Bugnon 46, 1011, Lausanne, Switzerland
| | - Rafael Duran
- Department of Diagnostic and Interventional Radiology, Lausanne University Hospital, Rue du Bugnon 46, 1011, Lausanne, Switzerland
| | - Marc Zins
- Department of Radiology, Fondation Hôpital St Joseph, 185 Rue Raymond Losserand, 75014, Paris, France
| | - Jean-Francois Knebel
- Department of Diagnostic and Interventional Radiology, Lausanne University Hospital, Rue du Bugnon 46, 1011, Lausanne, Switzerland
| | - Reto A Meuli
- Department of Diagnostic and Interventional Radiology, Lausanne University Hospital, Rue du Bugnon 46, 1011, Lausanne, Switzerland
| | - Sabine Schmidt
- Department of Diagnostic and Interventional Radiology, Lausanne University Hospital, Rue du Bugnon 46, 1011, Lausanne, Switzerland.
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Paine P, McLaughlin J, Lal S. Review article: the assessment and management of chronic severe gastrointestinal dysmotility in adults. Aliment Pharmacol Ther 2013; 38:1209-29. [PMID: 24102305 DOI: 10.1111/apt.12496] [Citation(s) in RCA: 27] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/05/2013] [Revised: 02/27/2013] [Accepted: 08/30/2013] [Indexed: 12/12/2022]
Abstract
BACKGROUND The characterisation and management of chronic severe gastrointestinal (GI) dysmotility are challenging. It may cause intestinal failure requiring home parenteral nutrition (HPN). AIMS To review the presentation, aetiology, characterisation, management and outcome of chronic severe GI dysmotility, and to suggest a pragmatic management algorithm. METHODS PubMed search was performed up to December 2012 using appropriate search terms, restricted to human articles and reviewed for relevance. Segmental dysmotility, acute ileus, functional syndromes and non-English articles were excluded. Evidence and recommendations were evaluated using the GRADE system. RESULTS In total, 721 relevant articles were reviewed. A coherent and definitive picture is hampered by overlapping classification systems using multi-modal characterisation methods, subject to pitfalls and some requiring further validation. The literature is confined to case series with no randomised trials. Fewer than 20% undergo full thickness jejunal biopsy, which are otherwise labelled idiopathic. However, in studies with up to 80% biopsy rates, neuromuscular abnormalities may be found in 90%. Between 14% and 50% will require HPN, comprising 8-14% of all HPN patients, of which 2/3 are primary/idiopathic and 1/3 secondary, with scleroderma being the leading secondary cause. Ten-year mortality ranges from 13% to 35% and is worst in elderly scleroderma patients. Management includes limited treatments for secondary causes, prokinetics, symptom palliation, psychological support, nutrition, hydration and judicious surgery. CONCLUSIONS Severe dysmotility often remains idiopathic. It is rarely possible to alter disease trajectory; consequently, prognosis may be poor. Multi-disciplinary teams in a specialist setting can improve outcomes. Graded recommendations are enumerated and a pragmatic algorithm is suggested.
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Affiliation(s)
- P Paine
- Department of Gastroenterology, Salford Royal NHS Foundation Trust, Salford, UK; Manchester Academic Health Sciences Centre, University of Manchester, Manchester, UK
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De Giorgio R, Cogliandro RF, Barbara G, Corinaldesi R, Stanghellini V. Chronic intestinal pseudo-obstruction: clinical features, diagnosis, and therapy. Gastroenterol Clin North Am 2011; 40:787-807. [PMID: 22100118 DOI: 10.1016/j.gtc.2011.09.005] [Citation(s) in RCA: 93] [Impact Index Per Article: 6.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 05/31/2023]
Abstract
CIPO is the very “tip of the iceberg” of functional gastrointestinal disorders, being a rare and frequently misdiagnosed condition characterized by an overall poor outcome. Diagnosis should be based on clinical features, natural history and radiologic findings. There is no cure for CIPO and management strategies include a wide array of nutritional, pharmacologic, and surgical options which are directed to minimize malnutrition, promote gut motility and reduce complications of stasis (ie, bacterial overgrowth). Pain may become so severe to necessitate major analgesic drugs. Underlying causes of secondary CIPO should be thoroughly investigated and, if detected, treated accordingly. Surgery should be indicated only in a highly selected, well characterized subset of patients, while isolated intestinal or multivisceral transplantation is a rescue therapy only in those patients with intestinal failure unsuitable for or unable to continue with TPN/HPN. Future perspectives in CIPO will be directed toward an accurate genomic/proteomic phenotying of these rare, challenging patients. Unveiling causative mechanisms of neuro-ICC-muscular abnormalities will pave the way for targeted therapeutic options for patients with CIPO.
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Camera L, Calabrese M, Sarnelli G, Longobardi M, Rocco A, Cuomo R, Salvatore M. Pseudopneumoperitoneum in chronic intestinal pseudo-obstruction: A case report. World J Gastroenterol 2011; 17:2972-5. [PMID: 21734810 PMCID: PMC3129513 DOI: 10.3748/wjg.v17.i24.2972] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/04/2010] [Revised: 02/09/2011] [Accepted: 02/16/2011] [Indexed: 02/06/2023] Open
Abstract
Chronic intestinal pseudo-obstruction (CIPO) is a rare disease due to a severe gastrointestinal motility disorder which may mimic, on both clinical and radiological grounds, mechanical obstruction. We report a case of a 26-year-old woman who presented to our institution for plain abdominal radiography for referred long-lasting constipation with recurrent episodes of abdominal pain and distension. At X-ray, performed both in the upright and supine position, an isolated air-fluid level was depicted in the left flank, together with a number of radiological signs suggestive of pneumoperitoneum. First, subphrenic radiolucency could be observed in the upright film. Second, the intestinal wall of some jejunal loops appeared to be outlined in the right flank. Third, the inferior cardiac border was clearly depicted in the upright film. The patient however had no evidence of peritoneal signs but only hypoactive bowel movements. Unenhanced multi-detector computed tomography (MDCT) of the abdomen and pelvis was therefore performed. MDCT revealed abnormal air-driven distension of the small and large bowel, without evidence of extra-luminal air. All radiological signs of pneumoperitoneum turned out to be false-positive results. The patient was submitted to pan-colonoscopy and to anorectal manometry to rule out Hirshprung’s disease, and was finally discharged with a diagnosis of CIPO.
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Mompoint D, Carlier RY, Orlikowski D. [Neuromuscular diseases: imaging of thoracic, digestive and urinary complications]. JOURNAL DE RADIOLOGIE 2010; 91:1419-1436. [PMID: 21242939 DOI: 10.1016/s0221-0363(10)70221-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/30/2023]
Abstract
Neuromuscular diseases have different characteristics. Respiratory and cardiovascular manifestations are frequent and severe. Respiratory difficulties secondary to muscle involvement and bronchial secretions worsen complications related to a frequent scoliosis. CT is a useful complement to standard radiographs in the setting of acute symptomatic deterioration and for presurgical evaluation of a scoliosis. Echocardiography is performed to determine the presence of cardiomyopathy. Gastrointestinal motility disorders are assessed with standard barium studies or CT in the acute setting. Urinary tract involvement also is possible in these patients given the increased survival and follow-up imaging may be helpful for palliative management. The imaging features of frequent manifestations will be discussed along with the imaging appearance of a few specific entities.
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Affiliation(s)
- D Mompoint
- Service d'Imagerie Médicale, Hôpital Raymond Poincaré, 104 boulevard Raymond Poincaré, 92380 Garches, France.
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