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Chronic intestinal pseudo-obstruction is a disorder of gut motility resulting in severe abdominal pain, bloating, nausea, and vomiting after eating. The avoidance of food in order to minimize symptoms causes malnutrition. To date, no medical or surgical treatment has been shown to be of lasting benefit. We treated 10 patients disabled by chronic intestinal pseudo-obstruction using home parenteral nutrition. All were rendered minimally symptomatic as long as they refrained from significant oral intake. Nine of the 10 patients were malnourished prior to the institution of treatment. Home parenteral nutrition increased mean total body weight from 74.7 +/- 2.9 to 93.5 +/- 3.7% (p less than 0.001), mean lean body mass from 78.4 +/- 6.5 to a mean of 92.7 +/- 2.6 (p less than 0.02), and mean body fat from 57.1 +/- 8.8 to 83.8 +/- 8.2% of expected values (p less than 0.05). Mean total body potassium increased from 68.8 +/- 13.1 to 80.5 +/- 10.7 g (p less than 0.05). We conclude that in chronic intestinal pseudo-obstruction, home parenteral nutrition coupled with minimal oral intake effectively relieves symptoms and significantly improves the nutritional depletion.
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PMID:Successful management of chronic intestinal pseudo-obstruction with home parenteral nutrition. 392 33

Chronic intestinal pseudo-obstruction denotes the clinical picture that results due to the failure of intestinal peristalsis to overcome the normal resistance to flow and is characterized by recurrent episodes of signs and symptoms of intestinal obstruction in the absence of any mechanical compromise of the intestinal lumen. The region(s) of the gut affected may be isolated or diffuse. It is not uncommon to find evidence of autonomic neuropathy and smooth muscle dysfunction with extraintestinal manifestations such as urinary symptoms from abnormal ureter or bladder function. Intestinal pseudo-obstruction can be caused by a variety of diseases, and for simplicity, certain authors have divided it into myopathic and neuropathic categories. Intestinal pseudo-obstruction may present at any age with a variable amount of abdominal pain, distension, nausea, diarrhea, or constipation and with laboratory abnormalities usually reflecting the degree of malabsorption and malnutrition present. The radiologic findings are varied but commonly include paralytic ileus or signs of apparent clinical obstruction with dilated loops of bowel. The number of pseudo-obstruction cases is dependent on how one defines the condition. It appears prudent to require radiographic abnormalities consistent with obstruction on a plain film of the abdomen for the diagnosis. More recently, studies have focused on the gastrointestinal manometric abnormalities of the stomach and small intestine in chronic intestinal pseudo-obstruction during fasting and fed states; however, sensitivity and specificity of these abnormalities are not well defined. Treatment is aimed at limiting symptoms and maintaining adequate nutrition. Prokinetic agents should be tried in an attempt to restore normal intestinal propulsion. However, their overall efficacy appears to be variable. It is still too premature to consider intestinal pacing or small bowel transplantation in this condition. Surgical approaches to chronic intestinal pseudo-obstruction should be limited to patients refractory to medical therapy, and even then, an approach focused on the patient's primary presenting symptoms should be considered.
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PMID:Chronic intestinal pseudo-obstruction. 854 80

We report a 56-year old female with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE), presenting with protein-losing gastroenteropathy and serum copper deficiency. There was no neuromuscular disease in her family members. Three years prior to admission, she developed severe gastrointestinal symptoms including diarrhea, nausea, vomiting and ascites, and was diagnosed as having protein-losing gastroenteropathy based on alpha(1)-antitrypsin clearance and other tests. She was referred to our department when neurological symptoms were apparent. Neurological examinations revealed bilateral ptosis, ophthalmoplegia, hearing loss, facial and limb muscle weakness, mild sensory deficit of vibration on her feet and hypoactive deep tendon reflexes. Pigmentary retinopathy, cerebellar ataxia and heart block were not seen. Serum copper level was decreased to 45 micrograms/dl (normal: 83-155). Chronic intestinal pseudo-obstruction was proven by X-ray studies, and diffuse leukoencephalopathy demonstrated on brain MRI. On EMG, motor nerve conduction velocities were prolonged with temporal dispersion. Her muscle biopsy from biceps brachii muscle showed both neuropathic and myopathic changes, scattered ragged-red fibers and focal cytochrome c oxidase deficiency. Southern blot and polymerase chain reaction analysis on mitochondrial DNA showed no deletions nor point mutations. The clinical and pathologic findings of the present patient fulfilled the diagnostic criteria of mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) proposed by Hirano et al. There are few reported patients with MNGIE in Japan, but none presented with protein-losing gastroenteropathy and serum copper deficiency. Since the copper is a cofactor of cytochrome c oxidase, decreased serum copper level may aggravate the respiratory chain enzyme metabolism in mitochondria. Therefore, treatment for gastrointestinal tract disturbance and copper administration may be necessary to prevent disease progression.
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PMID:[Mitochondrial neurogastrointestinal encephalomyopathy presenting with protein-losing gastroenteropathy and serum copper deficiency: a case report]. 949 Sep 4

Chronic intestinal pseudo-obstruction (CIP) is a gastrointestinal motility disturbance characterized by recurrent episodes of postprandial nausea and bloating in the absence of mechanical obstruction of the small bowel or colon. Weight loss and severe malnutrition are often seen in advanced stages of the disorder. This article discusses the nutritional management of patients with CIP, focusing on general dietary as well as enternal and parenternal nutritional support. Enteral access methods and various enteral formulas used in CIP are also discussed.
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PMID:Nutritional management of chronic intestinal pseudo-obstruction. 1037 26

Chronic intestinal pseudo-obstruction (CIPO) is a disease characterized by episodes resembling mechanical obstruction in the absence of organic, systemic, or metabolic disorders. Pseudo-obstruction is an uncommon condition and can result from primary (40%) or secondary (60%) causes. The most common symptoms are nausea, vomiting, abdominal distension, abdominal pain and constipation or diarrhea. These symptoms are usually present many years before CIPO diagnosis. They can lead to severe electrolyte disorders and malnutrition. Principles for management of patients with CIPO are: to establish a correct clinical diagnosis in excluding mechanical obstruction; to perform a symptomatic and physiologic assessment of the gastrointestinal tract involved; to look for extra-intestinal manifestations, especially for myopathy and neuropathy; to discuss in some cases a surgery for full-thickness intestinal biopsies, and/or a neuromuscular biopsy in case of mitochondrial cytopathy suspicion. The management is primarily focused on symptom control and nutritional support to prevent weight loss and malnutrition. Treatment of CIPO includes prokinetic agents which may help to reduce gastrointestinal symptoms Courses of antibiotics may be needed in patients with symptoms suggestive of bacterial overgrowth. When necessary, enteral nutrition is preferred. In carefully selected patients, feeding jejunostomy with or without decompression gastrostomy may be tried. Long term parenteral nutrition should be reserved for patients who can not tolerate enteral nutrition. Intestinal transplantation can be discussed in selected patients.
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PMID:[Chronic intestinal pseudo-obstruction]. 1707 44

Chronic intestinal pseudo-obstruction (CIP) is a rare and serious disorder of the gastrointestinal (GI) tract characterized as a motility disorder with the primary defect of impaired peristalsis; symptoms are consistent with a bowel obstruction, although mechanical obstruction cannot be identified. CIP is classified as a neuropathy, myopathy, or mesenchymopathy; it is a neuropathic process in the majority of patients. The natural history of CIP is generally that of a progressive disorder, although occasional patients with secondary CIP note significant symptomatic improvement when the underlying disorder is identified and treated. Symptoms vary from patient to patient depending on the location of the luminal GI tract involved and the degree of involvement; however, the small intestine is nearly always involved. Common symptoms include dysphagia, gastroesophageal reflux, abdominal pain, nausea, vomiting, bloating, abdominal distension, constipation or diarrhea, and involuntary weight loss. Unfortunately, these symptoms are nonspecific, which can contribute to misdiagnosis or a delay in diagnosis and treatment. Since many of the symptoms and signs suggest a mechanical bowel obstruction, diagnostic tests typically focus on uncovering a mechanical obstruction, although routine tests do not identify an obstructive process. Nutrition supplementation is required for many patients with CIP due to symptoms of dysphagia, nausea, vomiting, and weight loss. This review discusses the epidemiology, etiology, pathogenesis, diagnosis, and treatment of patients with CIP, with an emphasis on nutrition assessment and treatment options for patients with nutrition compromise.
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PMID:Chronic intestinal pseudo-obstruction. 2361 3

Chronic intestinal pseudo-obstruction is a known complication of patients with systemic sclerosis, manifested as nausea, vomiting, constipation, abdominal distension and pain. We report a series of cases with systemic sclerosis that presented with signs of intestinal obstruction. In all cases, the differentiation between a pseudo-obstruction and true mechanical obstruction remained a formidable challenge. Our goal was to present different scenarios of patients with systemic sclerosis and features of intestinal obstruction, with a review on its clinical approach.
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PMID:Intestinal obstruction and pseudo-obstruction in patients with systemic sclerosis. 2430 89

Chronic intestinal pseudo-obstruction (CIPO) is an uncommon disorder characterized by the presence of bowel dilatation and abnormal motility. It is an important cause of chronic intestinal failure in patients with systemic sclerosis (SSc). Although intestinal pseudo-obstruction is an infrequent reason for hospitalization in these patients, it has been correlated with high in-hospital mortality compared to SSc patients hospitalized for other reasons as well as patients with intestinal pseudo-obstruction arising from other causes. Patients present with signs and symptoms of mechanical bowel obstruction, such as nausea, vomiting, constipation, abdominal pain, and abdominal distension, in the absence of an anatomic lesion blocking the movement of intestinal contents. Despite breakthroughs in our understanding of this disorder, these patients continue to be treated largely with organ-based symptomatic therapy. Unfortunately, despite treatment, they often experience decreased quality of life and impairment in their everyday lives. Here, we present an interesting case of an individual with SSc for many years who presented with signs and symptoms of CIPO.
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PMID:Chronic Intestinal Pseudo-Obstruction in Systemic Sclerosis: An Uncommon Presentation. 3005 21