Anti-Hu-mediated paraneoplastic chronic intestinal pseudo-obstruction associated with extraskeletal myxoid chondrosarcoma.

Chronic intestinal pseudo-obstruction (CIPO) is characterized by symptoms and signs of bowel obstruction in the absence of an anatomical cause. Almost 50 % of cases are secondary to systemic diseases of neurological, paraneoplastic, autoimmune, metabolic, or infectious origin.

Paralytic ileus precipitating platypnoea-orthodeoxia after right pneumonectomy.

A 78-year-old female underwent a right pneumonectomy for a neuroendocrine tumour. In the early postoperative phase, she developed a paralytic ileus and went on to develop breathlessness and orthostatic desaturation, which characterizes platypnoea-orthodeoxia. She was found to have an intra-atrial shunt on bubble echo, with equal atrial pressures. This was managed by device closure of the atrial connection, following which her condition improved.

Chronic intestinal pseudo-obstruction in a patient with metastatic gastro-oesophageal junction cancer receiving treatment with pembrolizumab.

Acute gastrointestinal (GI) immune-related adverse events (irAE) are commonly reported by patients with cancer undergoing treatment with immune checkpoint inhibitors (CPI); however chronic irAEs are rare. We present a case of a 71-year-old woman with metastatic gastro-oesophageal junction (GOJ) adenocarcinoma who developed delayed-onset chronic intestinal pseudo-obstruction (CIPO) while receiving second-line pembrolizumab. Repeated CT scans of the abdomen/pelvis found no small bowel obstruction, and evaluations for bowel inflammation, infection and paraneoplastic syndrome were negative. Bowel rest and glucocorticoids were associated with transient symptom resolution; however, symptoms recurred within 1 month. The patient was ultimately supported with total parenteral nutrition and intestinal motility agents. After 4 months, the GOJ cancer remained stable with no signs of progression. As CPI use expands, the incidence of rare irAEs, such as CIPO, may increase.

Lupus intestinal pseudo-obstruction and hydronephrosis: Case report.

INTRODUCTION: Intestinal pseudo-obstruction (IPO) is a rare and life-threatening complication of lupus. PATIENT CONCERNS: A patient with long-standing lupus developed recurrent abdominal pain and distension as well as nausea and emesis. DIAGNOSIS: Imaging showed dilated small bowel loops with air-fluid levels and bowel wall thickening. She also had bilateral hydronephrosis. INTERVENTIONS: She was given high-doses of intravenous steroids and cyclophosphamide. OUTCOMES: Her symptoms resolved within a week of starting immunosuppression. She was eventually transitioned to mycophenolate mofetil. She remained in remission and immunosuppression was successfully stopped after 1 year. CONCLUSIONS: Intestinal pseudo-obstruction is a rare complication of lupus that is often seen in association with ureterohydronephrosis and interstitial cystitis. This clinical syndrome is thought to be because of smooth muscle dysmotility of the gastrointestinal and genitourinary tracts, although the exact mechanism of dysmotility remains unknown. This condition is often responsive to immunosuppression if recognized and treated promptly.

A mitochondrial neurogastrointestinal encephalomyopathy with intestinal pseudo-obstruction resulted from a novel splice site mutation.

Mitochondrial neurogastrointestinal encephalopathy (MNGIE) is an autosomal recessive disorder characterized by gastrointestinal dysmotility, cachexia, ptosis, peripheral neuropathy and leukoencephalopathy. The diagnosis is often not made until 5-10 years after the onset of symptoms. MNGIE is caused by mutations in thymidine phosphorylase gene TYMP. Here, we present a 19-year-old boy with MNGIE who had a chronic intestinal pseudo-obstruction, and we describe his family history. Genetic analysis revealed a novel homozygous c.765+1G>C intronic mutation which is expected to disrupt splicing of TYMP in the patient. Family screening revealed that the brother was also affected and the mother was a carrier. MNGIE should be considered and genetic testing instigated if individuals with cachexia have neuromuscular complaints or symptoms of chronic intestinal pseudo-obstruction.

Intestinal pseudo-obstruction as the initial manifestation of systemic lupus erythematosus.

Small bowel obstruction is common in emergency departments. However, the exact cause of intestinal pseudo-obstruction (IPO) is often misdiagnosed. IPO is considered a severe manifestation of systemic lupus erythematosus (SLE). However, IPO is rare as the initial manifestation of SLE. This paper reports a female patient who presented with IPO as the initial manifestation and was ultimately diagnosed with SLE. The 31-year-old female was definitively diagnosed with SLE after IPO symptoms for 1 month. She then presented multiple organ dysfunction syndrome (MODS) leading to a poor prognosis. Patients with unexplained SBO symptoms should be aware of systemic diseases. Early diagnosis and prompt medical treatment are crucial to avoid unnecessary surgery and obtain satisfactory outcomes.

Evaluation of gastrointestinal motility with MRI: Advances, challenges and opportunities.

Dynamic magnetic resonance imaging (MRI) of gastrointestinal motility has developed rapidly over the past few years. The non-invasive and non-ionizing character of MRI is an important advantage together with the fact that it is fast and can visualize the entire gastrointestinal tract. Advances in imaging and quantification techniques have facilitated assessment of gastric, small intestinal, and colonic motility in a clinical setting. Automated quantitative motility assessment using dynamic MRI meets the need for non-invasive techniques. Recently, studies have begun to examine this technique in patients, including those with IBD, pseudo-obstruction and functional bowel disorders. Remaining challenges for clinical implementation are processing the large amount of data, standardization and validation of the numerous MRI metrics and subsequently assessment of the potential role of dynamic MRI. This review examines the methods, advances, and remaining challenges of evaluation of gastrointestinal motility with MRI. It accompanies an article by Khalaf et al. in this journal that describes a new protocol for assessment of pan-intestinal motility in fasted and fed state in a single MRI session.

Neostigmine and glycopyrronium: a potential safe alternative for patients with pseudo-obstruction without access to conventional methods of decompression.

Intestinal pseudo-obstruction mimics bowel obstruction. However, on examination, no mechanical cause is identified. This condition will often resolve when managed conservatively, yet in some cases decompression is required to avoid the serious complications of bowel ischaemia and perforation. This is performed endoscopically, and due to the invasive nature and limited access to this service, an alternative treatment option is deemed appealing. Neostigmine has good efficacy in the decompression of pseudo-obstruction but is hindered by its wide side effect profile. In this context, neostigmine requires careful monitoring, which limits its appeal. This side effect profile is minimised when neostigmine is administered in conjunction with glycopyrronium.This case demonstrates the novel use of neostigmine and glycopyrronium in decompression of the bowel in a patient with pseudo-obstruction. Furthermore, it highlights its value, particularly when conventional techniques for decompression are not accessible.

Chronic Intestinal Pseudo-obstruction and Orthostatic Hypotension Associated with Small Cell Lung Cancer that Improved with Tumor Reduction after Chemoradiotherapy.

Chronic intestinal pseudo-obstruction (CIPO) is a rare disease with symptoms of ileus without obstruction. Most cases of CIPO are idiopathic, and CIPO as a paraneoplastic neurological syndrome (PNS) associated with small cell lung cancer (SCLC) is rare. A 63-year-old man was diagnosed with functional ileus and confined to bed due to orthostatic hypotension. Chest computed tomography revealed a right hilar mass suspected of being lung cancer. Based on detailed examinations, he was diagnosed with limited-stage SCLC. His symptoms were confirmed as PNS because his serum anti-Hu antibody was positive. His PNS was improved with complete tumor reduction by chemoradiotherapy.

A case of intussusceptions at two parts of the ileum caused by an ileus tube.

An 87-year-old woman was admitted to our hospital for paralytic ileus, and she was treated using an ileus tube. Although her symptoms improved, abdominal fullness developed again on day 3 after ileus tube insertion. Abdominal computed tomography indicated intussusceptions at the ileum and the terminal part of the ileum;therefore, an emergency surgery was performed. During the surgery, antegrade intussusceptions were found in the ileum 60cm from the ileocecal valve and the terminal part of the ileum into the ascending colon. The intussusception of the anal side was resolved by manual reduction, but the oral side needed a partial resection of small bowel because of the presence of necrosis. There were no lesions, such as tumors, at the intussusceptions sites. Therefore, the two intussusceptions were thought to be caused by the ileus tube. We diagnosed a rare case of intussusceptions in the two parts of the ileum as a complication of the placement of an ileus tube.

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

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.

The Potentially Fatal Ogilvie's Syndrome in Lateral Transpsoas Access Surgery: A Multi-Institutional Experience with 2930 Patients.

BACKGROUND: Ogilvie's syndrome (OS) is a relatively uncommon pathology characterized by significant colonic dilation in the absence of mechanical obstruction. If unrecognized and untreated, cecal perforation resulting in a mortality rate of 25%-71% may occur. It is a potentially underdiagnosed condition in the lateral transpsoas approach population because of its uncommon nature and imitation of other well-known pathologies. METHODS: Two thousand nine hundred and thirty patients from 6 separate institutions were retrospectively reviewed since 2007 and screened for OS. All patients underwent a minimum of single-level lateral transpsoas fusion. Diagnostic criteria included signs of a postoperative paralytic ileus combined with abdominal computed tomography showing a cecal diameter greater than 9 cm. Treatment modalities and outcomes were recorded. RESULTS: Eight cases (0.22%) of OS were diagnosed at 6 separate institutions. Most institutions recorded more than 350 lateral access procedures. Four cases were initially diagnosed as a routine postoperative ileus; however, they failed conservative therapy and underwent abdominal CT imaging. Neostigmine treatment was required for 1 patient in the intensive care unit setting, and 3 patients were managed conservatively without complications. Four other patients demonstrated bowel perforation at least 48 hours after surgery and required laparotomy with diversion ileostomy. CONCLUSION: Ogilvie's syndrome is a rare but potentially fatal complication that can mimic a postoperative ileus. It is likely underdiagnosed in the lateral transpsoas approach population because of its uncommon nature and a high index of suspicion should remain. Neostigmine can be administered safely under close observation with immediate and successful outcomes. Patients with perforation require urgent laparotomy and primary repair.