Non‐transplantation surgical approach for chronic intestinal pseudo‐obstruction: analysis of 63 adult consecutive cases

Sabbagh, Charles; Amiot, Aurélien; Maggiori, Léon; Corcos, Olivier; Joly, Francisca; Panís, Yves

doi:10.1111/nmo.12191

Cited by 43 publications

(28 citation statements)

References 21 publications

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“…Whereas 1 patient died in the perioperative period, the remaining patients continued to manifest with symptoms of severe gastrointestinal dysmotility involving other parts of the gastrointestinal tract, including stoma and end‐stage intestinal failure. These findings concur with reported high morbidity and mortality identified in patients with all‐cause IPO that have undergone intestinal resection 31. Moreover, of the 8 patients who died during the time course of the study, aspiration pneumonia as a complication of severe gastrointestinal dysmotility may be attributed to cause of death in up to half of these patients.…”

Section: Discussionsupporting

confidence: 88%

Pseudo‐obstruction, stroke, and mitochondrial dysfunction: A lethal combination

Feeney

Schaefer

et al. 2016

Annals of Neurology

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ObjectivesThe m.3243A>G MTTL1 mutation is the most common cause of mitochondrial disease; yet there is limited awareness of intestinal pseudo‐obstruction (IPO) in this disorder. We aimed to determine the prevalence, severity, and clinical outcome of patients with m.3243A>G‐related mitochondrial disease manifesting with IPO.MethodsIn this large, observational cohort study, we assessed the clinical, molecular, and radiological characteristics of patients with genetically determined m.3243A>G‐related mitochondrial disease, who presented with severe symptoms suggestive of bowel obstruction in the absence of an occluding lesion.ResultsBetween January 2009 and June 2015, 226 patients harbouring the m.3243A>G mutation were recruited to the Medical Research Council Centre Mitochondrial Disease Patient Cohort, Newcastle. Thirty patients (13%) presented acutely with IPO. Thirteen of these patients had a preceding history of stroke‐like episodes, whereas 1 presented 27 years previously with their first stroke‐like episode. Eight patients developed IPO concomitantly during an acute stroke‐like episode. Regression analysis suggested stroke was the strongest predictor for development of IPO, in addition to cardiomyopathy, low body mass index and high urinary mutation load. Poor clinical outcome was observed in 6 patients who underwent surgical procedures.InterpretationOur findings suggest, in this common mitochondrial disease, that IPO is an under‐recognized, often misdiagnosed clinical entity. Poor clinical outcome associated with stroke and acute surgical intervention highlights the importance of the neurologist having a high index of suspicion, particularly in the acute setting, to instigate timely coordination of appropriate care and management with other specialists. Ann Neurol 2016;80:686–692

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Section: Discussionsupporting

confidence: 88%

Pseudo‐obstruction, stroke, and mitochondrial dysfunction: A lethal combination

Feeney

Schaefer

et al. 2016

Annals of Neurology

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“…Unfortunately, despite continuous medical advances, the prognosis for people and dogs with CIPO secondary to intestinal leiomyositis is poor, with mortality rates up to 35% documented in people . All dogs were euthanized as a consequence of their intestinal disease in our series, with a maximum survival time of 9 months in dog 3.…”

Section: Discussionmentioning

confidence: 99%

Intestinal Leiomyositis: A Cause of Chronic Intestinal Pseudo‐Obstruction in 6 Dogs

Zacuto

Hill

McAlister³

et al. 2015

Veterinary Internal Medicne

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BackgroundIntestinal leiomyositis is a suspected autoimmune disorder affecting the muscularis propria layer of the gastrointestinal tract and is a cause of chronic intestinal pseudo‐obstruction in humans and animals.ObjectiveTo characterize the clinical presentation, histopathologic features, and outcome of dogs with intestinal leiomyositis in an effort to optimize treatment and prognosis.AnimalsSix client‐owned dogs.MethodsRetrospective case series. Medical records were reviewed to describe signalment, clinicopathologic and imaging findings, histopathologic diagnoses, treatment, and outcome. All biopsy specimens were reviewed by a board‐certified pathologist.ResultsMedian age of dogs was 5.4 years (range, 15 months–9 years). Consistent clinical signs included vomiting (6/6), regurgitation (2/6), and small bowel diarrhea (3/6). Median duration of clinical signs before presentation was 13 days (range, 5–150 days). Diagnostic imaging showed marked gastric distension with dilated small intestines in 4/6 dogs. Full‐thickness intestinal biopsies were obtained in all dogs by laparotomy. Histopathology of the stomach and intestines disclosed mononuclear inflammation, myofiber degeneration and necrosis, and fibrosis centered within the region of myofiber loss in the intestinal muscularis propria. All dogs received various combinations of immunomodulatory and prokinetic treatment, antimicrobial agents, antiemetics, and IV fluids, but none of the dogs showed a clinically relevant improvement with treatment. Median survival was 19 days after diagnosis (range, 3–270 days).Conclusions and Clinical ImportanceIntestinal leiomyositis is a cause of intestinal pseudo‐obstruction and must be diagnosed by full‐thickness intestinal biopsy. This disease should be considered in dogs with acute and chronic vomiting, regurgitation, and small bowel diarrhea.

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“…Although its use may be indicated as a tool to collect gut biopsy specimens for histopathology, the need for surgical therapy is sometimes required in emergency situations (massive bowel distension and perforation/ischemia). In a retrospective study on 63 adult patients there was an overall postoperative mortality rate of 7.9%, while CIPO‐related re‐operation rate was 66% at 5 years . In children palliative procedures such as feeding/venting gastrostomies and jejunostomies are mainly used to relieve symptoms in half of the patients .…”

Section: Therapymentioning

confidence: 99%

Chronic intestinal pseudo‐obstruction in children and adults: diagnosis and therapeutic options

Nardo¹,

Lorenzo

Lauro

et al. 2016

Neurogastroenterology Motil

108

139

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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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Non‐transplantation surgical approach for chronic intestinal pseudo‐obstruction: analysis of 63 adult consecutive cases

Abstract: The surgical management of CIPO patients was associated with high postoperative morbidity and mortality rates and frequent re-operation. Attempts should be made to avoid surgery when possible and optimize nutritional status.

Cited by 43 publications

References 21 publications

Pseudo‐obstruction, stroke, and mitochondrial dysfunction: A lethal combination

Pseudo‐obstruction, stroke, and mitochondrial dysfunction: A lethal combination

Intestinal Leiomyositis: A Cause of Chronic Intestinal Pseudo‐Obstruction in 6 Dogs

Chronic intestinal pseudo‐obstruction in children and adults: diagnosis and therapeutic options

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