Idiopathic Intestinal Pseudo-obstruction Research Articles

Idiopathic constipation: any movement?

Severe constipation now attracts considerable research interest. As a consequence, there have been recent advances in most aspects of this disorder. The epidemiology of this condition is now better appreciated, and subgroups of patients with different epidemiologies, symptom complexes, aetiologies, and treatments can now be distinguished. Radioisotopes enable detailed transit studies to be obtained of the entire gastrointestinal tract. Careful psychologic evaluation is an essential part of the evaluation. In young women with severe idiopathic constipation there is a decrease in propulsive mass movements, and specific colonic neurotransmitter abnormalities have been identified. Biofeedback therapy is an effective treatment for many of these patients. The variable results of surgery are now also clearly defined. Patients with chronic idiopathic intestinal pseudoobstruction can be categorized on the basis of their pathologic findings; detailed tissue studies may be required. The recognition that severe constipation encompasses various different conditions is leading to a more precise understanding of pathogenesis and treatment.

Degenerative leiomyopathy in children. A clinico-pathological study

The syndrome of degenerative leiomyopathy (DL) causing intestinal obstruction clinically manifests in young indigenous African children as massive megacolon without aganglionosis. We report on a clinico-pathologic study of 18 children seen over a 15-year period. All patients came from the same geographic area. Mean age at presentation was 9.5 years with mean duration of symptoms 4.3 years. The youngest infant was 6 months old. Eighteen children presented with progressive abdominal distention and infrequent stooling; 11 had intermittent diarrhoea and 9 colicky abdominal pain. Gross colonic gaseous distention was noted with proximal extension into the small bowel in 9 and into the stomach and oesophagus in 4. The rectosphincteric reflex was present in all 7 patients studied with anorectal manometry. The diagnosis, confirmed on full-thickness rectal biopsy, showed characteristic features distinguishing it from chronic idiopathic intestinal pseudo-obstruction, progressive systemic sclerosis, and mixed connective-tissue disease of childhood. The bowel was dilated, thin-walled, and on microscopy showed smooth-muscle degeneration and necrosis with replacement fibrosis of muscular layers, displacement of ganglion cells, mild increase in numbers and thickness of nerve fibres, mild inflammatory change, and medial fibrosis of small muscular arteries. Immunohistochemical studies were within normal limits. One child died; 14 have been maintained with a combination of prokinetic agents, low-residue diet, laxatives, and enemas; and 3 patients did not respond to this medical therapy on short-term follow-up. Nine children required surgical intervention — 2 diagnostic laparotomies, 4 volvulus, and 3 adhesive bowel obstructions. Four of these required subsequent surgical procedures. DL is a distinctive entity with a classical clinical and histologic picture whose exact etiology is still obscure, Exploratory laparotomy should be avoided.

Pneumatosis and pneumoperitoneum in chronic idiopathic intestinal pseudoobstruction

Chronic idiopathic intestinal pseudoobstruction is a diagnosis of exclusion for the rare patient with severe small bowel atony without any demonstrable organic cause. The very poor prognosis associated with this disease has been somewhat improved with the advent of parenteral nutrition; nevertheless, these patients follow a stormy course characterized by malnutrition, recurrent obstruction, infection, hemorrhage, and perforation. We describe a 16-year-old boy with this disease who presented to us with vague abdominal pain and pneumoperitoneum. Laparotomy showed pneumatosis cystoides intestinalis without intestinal perforation. The presence of free air in the abdominal cavity is an almost pathognomonic sign of intestinal perforation. Pneumatosis cystoides intestinalis is one of the very few nonsurgical causes of pneumoperitoneum. Beyond the neonatal period, in which it is the hallmark of necrotizing enterocolitis, it has been described in patients with severe obstructive lung disease, in collagenous disorders, and in the short-bowel syndrome. In the present case, it may have resulted from chronic intestinal distension, allowing air under pressure to dissect through the bowel wall. The challenge of such a unique association of conditions lies in the avoidance of unnecessary surgery in a chronic, essentially nonsurgical disease while avoiding unnecessary delay in a possibly acute surgical event.

Surgery for idiopathic megarectum and megacolon

Many patients with IMM can be managed successfully with laxatives. In a few surgery is required. A subtotal colectomy with ileorectal anastomosis is the operation of choice if the bowel is not too dilated, with a good outcome in approximately 80% of patients. In those with a grossly dilated rectum, a Duhamel procedure is a reasonable alternative, but the results are not as satisfactory. A left iliac colostomy formed above the dilated bowel is an acceptable option for those patients happy to live with a stoma. Distal resection with coloanal anastomosis, or restorative proctocolectomy, are experimental procedures whose roles are yet to be defined. The selection of the right operation for the right patient depends on a very careful evaluation. This should include a psychological assessment, as many patients have intellectual, social or psychological problems. The upper gastrointestinal tract should be evaluated by at least a barium meal and follow through, to try and exclude patients who have idiopathic intestinal pseudo-obstruction. The anal sphincter should be assessed physiologically to detect those with a weak sphincter, in case diarrhoea or worsened incontinence supervenes postoperatively. Because these patients are uncommon and difficult to assess, they should be man-aged by practitioners with a special interest in the condition.

Recurrent Pancreatitis in Three Patients with Chronic Idiopathic Intestinal Pseudo-obstruction

Chronic idiopathic intestinal pseudo-obstruction is a syndrome with substantial morbidity and mortality associated both with the syndrome and with its therapy. Standard therapy has included prokinetic agents and intravenous nutritional support when oral feedings are inadequate to maintain nutriture. We report three children with chronic intestinal pseudo-obstruction who experienced one or more attacks of pancreatitis. Two patients developed pseudocysts. One patient died. All three patients underwent cholecystectomy; one had stones, one had acalculous cholecystitis, and one had a normal gallbladder. All patients received prokinetic agents and total parenteral nutrition as therapy for their pseudo-obstruction. Candidate mechanisms to explain the etiology for pancreatitis in chronic intestinal pseudo-obstruction include biliary dysmotility associated with pseudo-obstruction and excessive cholinergic stimulation due to therapy with prokinetic agents.

A case of chronic idiopathic intestinal pseudoobstruction and review of literatures in Japan

A case of chronic idiopathic intestinal pseudoobstruction and review of literatures in Japan

Total Parenteral Nutrition‐Induced Steatosis: Reversal by Parenteral Lipid Infusion

Prolonged use of total parenteral nutrition (TPN) may be associated with hepatic complications, primarily steatosis and cholestasis. A case is reported of an 18-year-old woman with chronic idiopathic intestinal pseudo-obstruction syndrome who was on prolonged home parenteral nutrition without lipid supplementation and developed steatosis. This finding was reversed by addition of lipid emulsion, at a dose of 0.5 g/kg/day, to the parenteral nutrition solution. The lack of lipid supplementation as a possible cause of steatosis, as well as other mechanisms of liver steatosis associated with TPN, are discussed.

Enteric neuronal autoantibodies in pseudoobstruction with small-cell lung carcinoma

Severe gastrointestinal dysmotility is a newly recognized paraneoplastic syndrome that occurs with small-cell lung carcinoma. Thirty-four patients with small-cell carcinoma, of whom 5 had chronic intestinal pseudoobstruction and 29 had no digestive symptoms, were studied serologically. Four of the 5 patients with gut dysmotility had immunoglobulin G antibodies reactive with neurons of the myenteric and submucosal plexuses of jejunum and stomach in an indirect immunofluorescence assay. Antibodies of this type were not found in any of the 29 patients who had no gut dysmotility, nor were they found in patients with chronic idiopathic intestinal pseudoobstruction (n = 8), ovarian cancer (n = 20), or epilepsy (n = 4) or in normal subjects (n = 9). In 4 of the patients with paraneoplastic pseudoobstruction, antibodies in highly diluted serum (1:4000–1:8000) bound selectively to nuclei and cytoplasm of neuronal elements in the gut. This novel autoantibody activity suggests that intestinal pseudoobstruction occurring in patients with small-cell carcinoma may have an autoimmune basis. From a clinical standpoint, serological testing offers a simple means for determining which patients with gut dysmotility syndromes may have associated small-cell carcinoma, thereby enabling earlier diagnosis and treatment of the tumor.

Cardiomyopathy associated with nonendemic selenium deficiency in a Caucasian adolescent

We describe a girl aged 17 y who died after a cardiac arrest secondary to septic shock. At autopsy, the enlarged, soft, and flabby heart showed microscopic evidence of acute myocardial infarction, myocardial edema, myocardiocyte loss, replacement fibrosis in the interventricular septum, and right and left ventricular hypertrophic nucleomegaly. The pathological diagnosis was that of cardiomyopathy due to prolonged selenium deficiency. The patient had been on total parenteral nutrition for 17 mo, following extensive bowel resection for intractable pain, nausea, and vomiting caused by chronic idiopathic intestinal pseudoobstruction. Seven months before death, when severe biochemical selenium deficiency was diagnosed, supplemental selenium was added to the infusion, and plasma selenium concentrations increased. In long-standing selenium deficiency, sepsis may contribute the final insult to a damaged myocardium, triggering symptomatic cardiac failure and sudden death.

Jejunal Water and Sodium Secretion Occurs in Chronic Idiopathic Intestinal Pseudo-obstruction

We observed jejunal water and sodium secretion in two patients with chronic idiopathic intestinal pseudo-obstruction by standard perfusion techniques. The ability of glucose to reverse this secretory state was impaired. These observations suggest that a jejunal secretory state may contribute to the diarrhea in chronic idiopathic intestinal pseudo-obstruction.

Chronic idiopathic intestinal pseudoobstruction: A commonly misdiagnosed disease in infants and children

Chronic idiopathic intestinal pseudoobstruction: A commonly misdiagnosed disease in infants and children

A case of chronic idiopathic intestinal pseudo-obstruction with Sjögren's syndrome

A case of chronic idiopathic intestinal pseudo-obstruction with Sjögren's syndrome

Chronic Idiopathic Intestinal Pseudoobstruction: A Commonly Misdiagnosed Disease in Infants and Children

Intestinal pseudoobstruction is characterized by signs and symptoms of recurrent intestinal obstruction in the absence of an identifiable mechanical lesion. Underlying illnesses that have been associated with intestinal pseudoobstruction include scleroderma, amyloidosis, various endocrinopathies (eg, hypothyroidism), narcotic or laxative abuse, and electrolyte disturbances (eg, hypokalemia). Although the underlying cause of idiopathic intestinal pseudoobstruction is unknown, the primary pathophysiologic dysfunction in patients with idiopathic pseudoobstruction is a disorganization or lack of smooth muscle contractility that results in disordered peristalsis or aperistalsis involving bowel and/or urinary bladder. The clinical manifestations of the motility disorder associated with chronic idiopathic intestinal pseudoobstruction include periodic vomiting, abdominal distension, constipation, urinary retention, and weight loss.

Effect of six weeks of treatment with cisapride in gastroparesis and intestinal pseudoobstruction

We have investigated the effect of oral cisapride (10 mg t.i.d.) in a double-blind, placebo-controlled trial in 26 patients with upper gut dysmotility: 11 with gastroparesis (8 diabetic, 3 idiopathic) and 15 with chronic idiopathic intestinal pseudoobstruction. Patients were evaluated at entry and at the end of the 6-wk study by upper gastrointestinal manometry, scintigraphic evaluation of gastric emptying of solids and liquids, measurement of body weight, and scoring of the following symptoms: abdominal pain, nausea, vomiting, early satiety, bloating, and distention. Cisapride and placebo groups were strictly comparable for all parameters assessed. Cisapride resulted in a significant increase in the gastric emptying of solids (p < 0.05) compared with placebo; cisapride also tended to increase the postcibal antral motility and normalize the abnormal manometric features in the patients with intestinal dysmotility, particularly the characteristics of fasting interdigestive motor complexes and the fed motor pattern. Both cisapride and placebo groups showed an improvement in total symptom scores and there was no significant difference in overall symptom response between the two groups. However, the change in abdominal pain was greater with cisapride (p = 0.07). Cisapride facilitates gastric emptying in patients with upper gut dysmotility. The overall symptomatic benefit during a 6-wk trial of cisapride, 10 mg t.i.d., was not greater than that of placebo, and dose-response as well as longer term trials are necessary to determine the clinical efficacy of this medication.

Effect of Six Weeks of Treatment With Cisapride in Gastroparesis and Intestinal Pseudo obstruction

We have investigated the effect of oral cisapride (10 mg t.i.d.) in a double-blind, placebo-controlled trial in 26 patients with upper gut dysmotility: 11 with gastroparesis (8 diabetic, 3 idiopathic) and 15 with chronic idiopathic intestinal pseudoobstruction. Patients were evaluated at entry and at the end of the 6-wk study by upper gastrointestinal manometry, scintigraphic evaluation of gastric emptying of solids and liquids, measurement of body weight, and scoring of the following symptoms: abdominal pain, nausea, vomiting, early satiety, bloating, and distention. Cisapride and placebo groups were strictly comparable for all parameters assessed. Cisapride resulted in a significant increase in the gastric emptying of solids (p less than 0.05) compared with placebo; cisapride also tended to increase the postcibal antral motility and normalize the abnormal manometric features in the patients with intestinal dysmotility, particularly the characteristics of fasting interdigestive motor complexes and the fed motor pattern. Both cisapride and placebo groups showed an improvement in total symptom scores and there was no significant difference in overall symptom response between the two groups. However, the change in abdominal pain was greater with cisapride (p = 0.07). Cisapride facilitates gastric emptying in patients with upper gut dysmotility. The overall symptomatic benefit during a 6-wk trial of cisapride, 10 mg t.i.d., was not greater than that of placebo, and dose-response as well as longer term trials are necessary to determine the clinical efficacy of this medication.

Primary intestinal myopathy, a cause of chronic idiopathic intestinal pseudoobstruction syndrome (CIPS): clinicopathological studies of seven cases in children.

Clinicopathological data on seven instances of primary intestinal myopathy in children are reported. The ages of the patients ranged from eleven months to thirteen years. A persistent intestinal obstruction was the main and constant clinical feature. An ineffective intestinal propulsion was documented on manometric studies. Various urological abnormalities were present in three cases. One patient died and six survive but are dependent on enteral and parenteral nutrition. The morphological findings consisted of degenerative changes involving the muscular layers of the intestinal wall. These changes varied from cytoplasmic vacuolation to definite atrophy and disappearance of the muscular fibers. An extensive interstitial fibrosis underlined these atrophic changes in the late stages of the disease. A familial history was identified in three cases, one consistent with an autosomal dominant transmission.

Alterations in Cholinergic Muscarinic and Somatostatin Binding Sites in a Patient with Idiopathic Intestinal Pseudo-obstruction

We present a comparative study of cholinergic muscarinic and somatostatin binding sites on isolated membranes from mucosa and tunica muscularis of normal and dilated parts of the proximal jejunum obtained at surgery from a patient with idiopathic intestinal pseudo-obstruction (IIP) syndrome. We found a statistically significant diminution of cholinergic muscarinic and somatostatin binding sites in mucosa taken from the dilated part of the jejunum, compared with those taken from the normal part. Tunica muscularis of the dilated part of the jejunum contained a significantly higher concentration of peripheral cholinergic muscarinic binding sites (M2) than the normal part did, whereas concentration of M1 cholinergic muscarinic and somatostatin binding sites was similar in both examined parts. These results indicate that IIP-syndrome may be related to alterations in cholinergic muscarinic binding sites in the tunica muscularis of the intestine.

Esophageal motility disorders.

Esophageal motility disorders consist of a complex array of disturbances in normal esophageal function associated with dysphagia, gastroesophageal reflux, and noncardiac chest pain. A thorough knowledge of normal esophageal anatomy and physiology is important to a full understanding of these motility derangements. Through a complicated interaction of neuromuscular and hormonal influences, the voluntary act of swallowing transforms into an automated sequence of peristaltic waves propelling food and liquids into the stomach in concert with coordinated relaxation of the sphincters. Anatomic and physiologic barriers exist within the esophagus protecting against gastroesophageal reflux and aspiration. With improvements in diagnostic tools such as barium contrast radiography, scintigraphy, pH measurements, and esophageal manometrics with provocative testing, motility disorders have become better defined and understood. Primary motility disorders consist of achalasia, diffuse esophageal spasm (DES), "nutcracker esophagus," hypertensive lower esophageal sphincter, and nonspecific esophageal motility dysfunction (NEMD). A host of secondary and miscellaneous motility disorders also affect the esophagus, including scleroderma and other connective tissue diseases, diabetes mellitus, Chagas' disease, chronic idiopathic intestinal pseudo-obstruction, and neuromuscular disorders of striated muscle. Gastroesophageal reflux disease (GERD) may also be promoted by associated motility disturbances. Treatment modalities include surgical myotomy; dilatation; and pharmacologic manipulations, including use of nitrates, calcium-channel blockers, H2-blockers, and psychotropic drugs where appropriate.

Successful management of idiopathic intestinal pseudo-obstruction with cisapride

This is a case report of a previously asymptomatic 11-year-old boy who developed chronic intestinal pseudo-obstruction. Barium studies revealed grossly disordered motility of the proximal small bowel, and ganglion cells in a gastric biopsy were mildly abnormal. Treatment with conventional prokinetic agents and gastrojejunostomy were ineffective. Intravenous cisapride induced an immediate remission, which has been maintained subsequently by rectal administration of the drug.

僧帽弁逸脱症候群を伴った慢性特発性偽性腸閉塞症の１例

僧帽弁逸脱症候群を伴った慢性特発性偽性腸閉塞症の１例