Chronic intestinal pseudo-obstruction

Opinion statement For many patients, nutritional support and relief of symptoms remain the primary management goal of pseudo-obstruction. Specific pharmacological agents for this disorder are, in general, lacking. Given that the efficacy of many of the individual available agents is far from excellent, several centers have turned to combination therapy. Though there is at present no evidence from controlled studies to support this strategy, it is, at the very least, theoretically attractive as these agents act through a number of separate mechanisms. The combination of a prokinetic and an emetic may prove especially useful. As the pseudo-obstruction syndromes are, individually, rare, and experience with any given prokinetic agent in these disorders limited, it is difficult to develop strict guidelines for their use in this context. It stands to reason that a response to a prokinetic agent would seem unlikely in a patient with an advanced myopathic process; anecdotal evidence suggests, however, that some patients with severe scleroderma may derive some symptomatic improvement. Where oral therapy is tolerated, cisapride would appear the best choice among available agents. When this fails, subcutaneous octreotide may be added or substituted. In the acute situation, intravenous erythromycin may alleviate gastroparesis, but probably exerts little beneficial effect beyond the pylorus; parenteral metoclopramide may be tried, but, here again, convincing evidence of efficacy is lacking. The roles of endoscopy and surgery are largely confined to facilitating nutrition and providing decompression.     

Chronic intestinal pseudo-obstruction

Opinion statement Chronic intestinal pseudo-obstruction (CIP) is a gastrointestinal motility disorder characterized by chronic symptoms and signs of bowel obstruction in the absence of a fixed, lumen-occluding lesion. Radiographic findings consist of dilated bowel with airfluid levels. Pseudo-obstruction is an uncommon condition and can result from primary or secondary causes. The management is primarily focused on symptom control and nutritional support to prevent weight loss and malnutrition. The principles of management of patients with CIP involve 1) establishing a correct clinical diagnosis and excluding mechanical obstruction; 2) differentiating between idiopathic and secondary forms; 3) performing a symptomatic and physiologic assessment of the parts of the gastrointestinal (GI) tract involved by manometric and whole gut transit scintigraphic studies; 4) careful assessment of nutritional status of the patient; and 5) developing a therapeutic plan addressing the patient’s symptoms and nutritional status. Treatment of CIP includes frequent small meals with a low-fat, low-fiber diet, liquid nutritional supplements may be needed; prokinetic agents such as metoclopramide may help to reduce upper GI symptoms. Trials of drugs such as erythromycin, domperidone, cisapride, and tegaserod may be considered if there is no response. Subcutaneous octreotide may be helpful to improve small bowel dysmotility especially in patients with scleroderma. In patients with symptoms suggestive of bacterial overgrowth, courses of antibiotics such as metronidazole, ciprofloxacin, and doxycycline may be needed. Nutritional assessment and support is an important aspect of management. Enteral nutrition is usually 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. Complications associated with total parenteral nutrition include infections, sepsis, and cholestatic hepatic dysfunction.     

Chronic intestinal pseudo-obstruction

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.     

Chronic intestinal pseudo-obstruction

Chronic intestinal pseudo-obstruction （CIPO） is a severe digestive syndrome characterized by derangement of gut propulsive motility which resembles mechanical obstruction, in the absence of any obstructive process. Although uncommon in clinical practice, this syndrome represents one of the main causes of intestinal failure and is characterized by high morbidity and mortality. It may be idiopathic or secondary to a variety of diseases. Most cases are sporadic, even though familial forms with either dominant or recessive autosomal inheritance have been described. Based on histological features in- testinal pseudo-obstruction can be classified into three main categories：neuropathies, mesenchymopathies, and myopathies, according on the predominant involvement of enteric neurones, interstitial cells of Cajal or smooth muscle cells, respectively. Treatment of intestinal pseu- do-obstruction involves nutritional, pharmacological and surgical therapies, but it is often unsatisfactory and the long-term outcome is generally poor in the majority of cases.     

Chronic intestinal pseudo-obstruction

Chronic intestinal pseudo-obstruction (CIPO) is a severe functional digestive syndrome characterised by a derangement of gut propulsive motility which resembles mechanical obstruction. It may be associated with disabling and potentially life-threatening complications. CIPO can be secondary to a variety of diseases, but it is more frequently idiopathic. Most cases are sporadic, but familial forms have also been described. Based on histological features CIPO can be classified into three major entities: neuropathies, mesenchymopathies, and myopathies depending on the predominant involvement of enteric neurones, interstitial cells of Cajal (ICC) or smooth muscle cells, respectively. Mitochondriopathies may be responsible for a syndromic form of CIPO, i.e. mitochondrial neurogastrointestinal encephalomyopathy. Management of CIPO involves nutritional, pharmacological and surgical therapies, but the long-term outcome turns out to be poor in the vast majority of cases. The main pathogenetic and clinical features of the syndrome, together with current management recommendations are reviewed in this chapter.     

Chronic intestinal pseudo-obstruction]

Chronic intestinal pseudo-obstruction (CIPO) is a syndrome characterized by the presence of recurrent episodes of clinical intestinal obstruction in the absence of obstructive lesions. Although this syndrome is rare, it causes a high morbidity. It is caused by a disturbance of the intestinal motility, that results in a failure of the progression of the intestinal content. Basically, the failure of the intestinal motility is a consequence of muscular disorder, neurological disorder or both. Usually, CIPO is secondary to other systemic disease; however, in the last years, many cases of primary CIPO have been described. The use of new manometric tecniques and specific histological procedures have allowed to clarify the pathogenesis of some of these entities including mitochondrial diseases and paraneoplasic syndromes. Clinical manifestations of CIPO are diverse, depending on the location and extension of the motility disorder. As the diagnosis of this disease is usually not an easy task, patients frecuently undergo unnecesary surgical interventions, are diagnosed of psyquiatric disorders, or the correct diagnosis is delayed several years after the first symptoms arise. The aims of the treatment are to maintain the nutritional condition and to improve symptoms using nutritional measures, drugs or, eventually, endoscopical or surgical procedures.     

Chronic idiopathic intestinal pseudo-obstruction

Opinion statement The definition of chronic idiopathic intestinal pseudo-obstruction (CIIP) is somewhat vague because it was based on clinical observations that preceded modern advances in the measurement of gut motility and neuromuscular integrity. Appropriate management of patients with CIIP requires an initial consideration, supported by pertinent diagnostic tests, of the tissue affected (muscle, nerves, both), extent and magnitude of gut propulsive failure, and extraintestinal disease.     

Chronic intestinal pseudo-obstruction in systemic lupus erythematosus

Intestinal pseudo-obstruction (IPO) is an uncommon and severe complication of systemic lupus erythematosus (SLE). We report a 24-year-old female with a 2 year SLE duration who presented with abdominal pain, vomiting, constipation and abdominal distention. Plain abdominal radiograph showed multiple air-fluid levels of the small bowel. Computed tomographic scan of the abdomen revealed dilated small bowel loops without mechanical obstruction. Urinary tract involvement was also demonstrated. IPO was diagnosed and the patient responded well to immunosuppressive treatment. IPO is a recently recognized manifestation of SLE that may be the presenting manifestation of the systemic disease or occur more commonly during disease course. Early recognition of IPO is necessary to institute appropriate medical treatment and to avoid inappropriate surgical intervention.     

Chronic intestinal pseudo-obstruction in systemic lupus erythematosus

Background/Aims—Chronicintestinal pseudo-obstruction (CIPO) reflects a dysfunction of thevisceral smooth muscle or the enteric nervous system. Gastrointestinalmanifestations are common in systemic lupus erythematosus (SLE)but CIPO has not been reported. Features of CIPO are reported in fivepatients with SLE.
Methods—From 1988 to1993, five patients with SLE or SLE-like syndrome were hospitalised forgastrointestinal manometric studies. CIPO was the onset feature in twocases. Antroduodenal manometry (three hours fasting, two hours fed) wasperformed in all patients, and oesophageal manometry in four.
Results—Intestinalhypomotility associated with reduced bladder capacity and bilateralureteral distension was found in four patients and aperistalsis of theoesophagus in three. Treatment, which consisted of high dosecorticosteroids, parenteral nutrition, promotility agents, andantibiotics, led to remission of both CIPO and urinary abnormalities inall cases. Antroduodenal manometry performed in two patients afterremission showed increased intestinal motility. One patient died, andpostmortem examination showed intestinal vasculitis.
Conclusions—CIPO inSLE is a life threatening situation that can be reversed by treatment.It may be: (a) a complication or onsetfeature of the disease; (b) secondary tosmooth muscle involvement; (c) associatedwith ureteral and vesical involvement; (d)the result of intestinal vasculitis.

Keywords:chronic intestinal pseudo-obstruction; systemiclupus erythematosus

     

Chronic primary intestinal pseudo-obstruction from visceral myopathy.

Chronic intestinal pseudo-obstruction is an uncommon syndrome characterized by relapsing episodes suggesting intestinal obstruction during which no mechanical causes are identified to account for symptoms. Etiologic factors may be manifold. Among them a number of neurologic conditions, gastrointestinal smooth muscle myopathies, endocrino-metabolic and autoimmune diseases, and the use of selected drugs stand out. We report a case of chronic intestinal pseudo-obstruction originating in a sporadic, primary intestinal myopathy that corresponds to no type thus far described. A histological study of the intestinal wall showed disrupted muscle bundles and the presence of interstitial edema. Myocytes had severe degenerative changes, and no alterations were seen in submucosal and myenteric plexus neurons. The activity of enzyme complexes in the mitochondrial respiratory chain, and of thymidine phosphorylase was normal. No mitochondrial DNA changes were seen.     

Chronic idiopathic intestinal pseudo-obstruction: clinical and intestinal manometric findings.

We report the clinical and intestinal manometric findings in a group of 42 patients with chronic idiopathic intestinal pseudo-obstruction evaluated at the Mayo Clinic. The main clinical manifestations in these patients were nausea and vomiting (83%), abdominal pain (74%), distension (57%), constipation (36%), diarrhoea (29%), and urinary symptoms (17%). These symptoms preceded surgery in all patients. Air fluid levels or distended bowel loops occurred in 57% and a dilated bladder or urinary excretory pathway in 17%. All patients showed intestinal manometric abnormalities none of which are seen in healthy individuals: aberrant configuration or propagation of interdigestive motor complexes in 25 patients; bursts (greater than 2 min duration) of non-propagated phasic pressure activity in fasting and/or fed state in 30 patients; sustained incoordinated fasting pressure activity in 15 patients; and inability of an ingested meal to convert fasting into fed pattern in 28 patients. We conclude that qualitative analysis of intestinal manometry provides evidence of gut dysmotility in patients with the clinical syndrome of chronic intestinal pseudo-obstruction. These abnormalities of motility can help to establish the correct diagnosis.     

Chronic intestinal pseudo-obstruction: pathogenesis, diagnosis and therapy

Chronic intestinal pseudo-obstruction (CIPO) is a rare disease in which a severe intestinal motility disorder impairs transit of chyme so that patients suffer from symptoms of a mechanical ileus without mechanical obstruction. CIPO may be a primary or secondary disorder due to muscular, neurologic, metabolic or endocrine disorders, but may also occur postinfectiously, postoperatively, following abdominal radiation or be caused by drugs or noxae. In severe cases, the typical history of (repeated) symptoms of mechanical obstruction leading to unsuccessful laparotomies will give key clues for diagnosis. If CIPO is suspected, mechanical obstruction must be searched for carefully by radiologic and endoscopic examinations. Histologic diagnosis usually demands full thickness biopsies of the intestinal wall. Small intestinal manometry allows diagnosis of CIPO even during oligosymptomatic intervals as well as differentiation between neuropathic and myopathic forms of the disease. The main therapeutic goals consist in: 1. Maintenance of an adequate nutritional state by oral and/or enteral nutrition; in severe cases home-parenteral nutrition may be required and particularly in children intestinal transplantation may be the ultima ratio. 2. Reconstitution of intestinal propulsion by prokinetic drugs. 3. Therapy of complications such as bacterial overgrowth and severe pain by antibiotics and specific surgical procedures. Unnecessary laparotomies should be strictly avoided because they may lead to adhesions and markedly complicate the clinical course.     

Chronic intestinal pseudo-obstruction due to buserelin-induced formation of anti-GnRH antibodies

BACKGROUND & AIMS: A 30-year-old woman, treated with buserelin, an analogue of gonadotropin-releasing hormone (GnRH) (also called luteinizing hormone-releasing hormone, LH-RH), developed chronic intestinal pseudo-obstruction (CIPO). The sudden onset of this disease in a previously healthy woman perplexed us. CIPO refers to a gastrointestinal disorder that can have a variety of causes, such as drugs, among others. Thus, we wanted to examine whether in this patient the development of CIPO is related to the treatment with buserelin. METHODS: The patient was examined using esophagogastroduodenoscopy, esophageal, and antroduodenojejunal manometry, gastric emptying tests, and histologic analyses and immunohistochemistry on full-thickness biopsies including staining with anti-GnRH antibody. Plasma samples were examined by the standard serologic analyses and specifically for the occurrence of anti-GnRH antibodies by enzyme-linked immunosorbent assay methods. RESULTS: CIPO was diagnosed based on symptoms (abdominal pain, vomiting, and constipation), and the results of the clinical examinations, such as signs of esophageal aperistalsis, delayed gastric emptying, and small intestinal bursts. Histologic examination revealed a decreased number of myenteric neurons as well as increased neuronal degeneration and an abnormal immune profile. There was a loss of GnRH-containing neurons. The patient had high plasma titers of anti-GnRH antibodies, which occurred on the occasions of the treatment with buserelin. CONCLUSIONS: Our findings suggest that the patient has developed CIPO due to buserelin-induced formation of anti-GnRH antibodies destroying GnRH-producing neurons of the myenteric plexus.     

Chronic intestinal pseudo-obstruction in systemic lupus erythematosus due to intestinal smooth muscle myopathy

We report the case of a woman with systemic lupus erythematosus initially manifesting with fever, rash and arthritis, and two years later with Class IV lupus nephritis. Following treatment with cyclophosphamide she developed symptoms and signs of chronic intestinal pseudo-obstruction (CIPO) that was initially thought to be due to a neutropenic enterocolitis. However, persistence of symptoms resulted in segmental resection of the ileum which showed widespread myocyte necrosis and active inflammation within the muscularis propria. A subsequent, more extensive ileocolic resection showed severe diffuse atrophy and fibrosis of the muscularis propria throughout the resected bowel. The absence of mesenteric vasculitis and the clinical response of the CIPO to the immunosupressive regimen of prednisolone and cyclosporin A suggest that the bowel muscle coat changes reflect an intestinal myopathy secondary to systemic lupus erythematosus, and may have an auto-immune etiology.