Chronic intestinal pseudo-obstruction. A report of 27 cases and review of the literature.

Twenty-seven cases of chronic intestinal pseudo-obstruction are reported. The causes of pseudo-obstruction were progressive systemic sclerosis in 14, hollow visceral myopathy in 4, visceral neuropathy in 2, sclerosing mesenteritis in 1, and jejunal diverticulosis in 1. No identifiable cause was found in five. Chronic pseudo-obstruction is a long-term illness characterized by vomiting, abdominal distention, abdominal pain and weight loss. Involvement is often present throughout the intestine so that patients may present with a variety of symptoms deriving from the esophagus, stomach, small intestine, and colon. Hollow visceral myopathy and visceral neuropathy are usually familial and urologic involvement is sometimes present in the former. Abnormalities of smooth muscle function can be discerned by radiography and esophageal manometry. The pattern and distribution of the abnormalities are helpful in differentiating pseudo-obstruction from true mechanical obstruction. They may also be helpful in differentiating one form of pseudo-obstruction from another. The majority of cases have identifiable pathology within either the smooth muscle or myenteric plexus of the bowel wall. The natural history of pseudo-obstruction is variable. Remissions and exacerbations occur and may be unrelated to anything that is done therapeutically. The illness is unresponsive to any drug known to have an effect on intestinal motility. Antibiotic treatment of small intestinal bacterial overgrowth and selected surgical procedures may occasionally be palliative. Many patients develop malnutrition and require home parenteral nutrition in order to survive.

The etiology of anorectal infections in homosexual men.

The infectious etiology of symptomatic anorectal disease was studied in 52 homosexual men who did not have gonococci on initial Gram stain of anorectal exudate. Herpes simplex virus (HSV) was isolated from the anal canal or rectum in 15 of the 52 (29 percent) men and characteristically caused severe anorectal pain and focal ulcerations visible on sigmoidoscopy. Despite negative initial Gram stains, seven men (14 percent) had anorectal gonococcal infection. Six (12 percent) had syphilis, including two with dark-field positive anal lesions. Four were infected with enteric pathogens, including Giardia lamblia, Entamoeba histolytica or Campylobacter fetus ssp. jejuni. Chlamydia trachomatis (LGV 2 strain) was isolated from one patient with severe granulomatous proctitis. One or more etiologic pathogens were identified in 28 (67 percent) of 42 men who had anorectal leukocytic exudate and in two of 10 who did not (p = 0.01). A review of the prominent features of different etiologic forms of anorectal infection in homosexuals is presented.

Gallium-67 scanning at 6 hr in active inflammatory bowel disease: case report.

Gallium citrate scanning at 6 hr was used to evaluate a patient with active ulcerative colitis. The localization of 67Ga in the colon correlated with the extent of the inflammatory process. When either colonoscopy or radiographic contrast studies are contraindicated, 67Ga scanning at 6 hr may prove useful in the evaluation of active inflammatory bowel disease.

Pathologic features of familial visceral myopathy.

Familial visceral myopathy is the most common cause of chronic primary (idiopathic) intestinal pseudo-obstruction. We studied four family groups with this disease and found that it has a characteristic morphologic appearance. Grossly, there is segmental dilatation of the alimentary tract, often involving multiple sites and most commonly producing a megaduodenum. Microscopically, the involved areas show a characteristic change consisting of degenerating muscle cells and fibrosis, which may involve the full thickness of the muscularis propria but is often more prominent in or limited to the external layer. Degenerating muscle cells appear pale, poorly defined, and fragmented. As residual thread-like remnants become surrounded by collagen or as muscle cells are destroyed, leaving apparent spaces surrounded by collagen, the longitudinal and circular muscles take on a vacuolated appearance easily recognized at low magnifications. Recognition of this change is greatly facilitated by use of a trichrome stain, and mild lesions may be recognized only with such stains. The nondilated segments of intestine show similar changes but of a less severe degree. Neural and vascular structures are apparently normal. Although the lesion most closely resembles progressive systemic sclerosis, the degenerating muscle cells and vacuolated appearance of the muscle serve to distinguish familial visceral myopathy from the latter entity.

Long-term response to subtotal colectomy in colonic inertia.

The purpose of this study was to determine the long-term outcome of patients who had previously undergone subtotal colectomy for severe idiopathic constipation at the University of Florida between 1983 and 1987. In addition, we aimed to determine whether preoperative motility abnormalities of the upper gastrointestinal tract are more common among those patients who have significant postoperative complications after subtotal colectomy. We evaluated 13 patients who underwent subtotal colectomy for refractory constipation between 1983 and 1987 at the University of Florida. Preoperatively, all patients exhibited a pattern consistent with colonic inertia as demonstrated by means of radiopaque markers. Each patient was asked to quantitate the pain intensity and frequency of their bowel movements before and after surgery. In seven patients an ileosigmoid anastomosis was performed, whereas in six patients an ileorectal anastomosis was used. Abdominal pain decreased after subtotal colectomy. Patients with abnormal upper gastrointestinal motility preoperatively experienced greater postoperative pain than those with normal motility regardless of the type of anastomosis. In addition, the number of postoperative surgeries was similar in those patients with abnormal upper motility compared to those with normal motility. Overall, the total number of bowel movements per week increased from 0.5 +/- 0.03 preoperatively to 15 +/- 4.5 (P < 0.007) postoperatively. The results of our study suggest that patients with isolated colonic inertia have a better long-term outcome from subtotal colectomy than patients with additional upper gastrointestinal motility abnormalities associated with their colonic inertia.

Progressive systemic sclerosis of the gastrointestinal tract and hereditary hollow visceral myopathy: two distinguishable disorders of intestinal smooth muscle.

The purpose of this study was to determine whether progressive systemic sclerosis (PSS) of the gastrointestinal tract and hereditary hollow visceral myopathy are two distinguishable disorders of intestinal smooth muscle. We coded and blindly reviewed 50 specimens of tissue from the small intestine of 15 normal controls, 4 patients with visceral myopathy, 5 patients with PSS and intestinal pseudoobstruction, and 5 patients with PSS but no pseudoobstruction. We determined that there is a fundamental difference between the pathology of these two disorders. In visceral myopathy, the smooth muscle is characterized by vacuolar degeneration and fibrosis. In PSS, smooth muscle fibrosis is present but vacuolar degeneration is absent. Although smooth muscle cells are decreased in number in both disorders, those that are present in PSS are morphologically normal by light microscopy, whereas most of those present in visceral myopathy are degenerated. Visceral myopathy and gastrointestinal PSS are two distinct disorders of smooth muscle which are easily distinguished by conventional light microscopy. Their dissimilar appearances and the familial nature of visceral myopathy suggest that they result from quite different causes.

Paraneoplastic visceral neuropathy as a cause of severe gastrointestinal motor dysfunction.

The purpose of this study was to define the cause of severe gastrointestinal motor dysfunction in 7 patients with lung cancer. Six patients had small cell carcinoma and 1 patient had pulmonary carcinoid. Their ages ranged from 58 to 74 yr. All had intestinal pseudoobstruction and obstipation/constipation; 6 of 7 patients had gastroparesis; 4 of 4 patients had esophageal peristaltic abnormalities; and 2 patients had neurogenic bladders, autonomic insufficiency, and peripheral neuropathy. Five of 7 patients had dilated small bowel with 4 of them showing slow transit of barium; 2 of 7 patients had dilated colons; and 3 of 7 patients had slow colonic transit. Five patients died 4-9 mo after onset of gastrointestinal symptoms, and 2 survived. Post-mortem or surgical samples of the esophagus, stomach, small bowel, and colon showed neuron and axon degeneration and dropout, lymphoplasmacytic infiltration, and glial cell proliferation within the myenteric plexus of 6 patients. The antrum from the seventh patient had inflammatory cells within the myenteric plexus but without neuron dropout. Neuron numbers were significantly less than normal in each area of the gastrointestinal tract. Thus, we conclude that lung cancer may be complicated by severe gastrointestinal motor dysfunction resulting from visceral neuropathy of the myenteric plexus, a paraneoplastic effect of the cancer.

Visceral myopathy of the colon mimicking Hirschsprung's disease. Diagnosis by deep rectal biopsy.

A 51-year-old man presented with a history and physical findings consistent with adult Hirschsprung's disease. An inadvertent transmural rectal biopsy led to the unexpected diagnosis of a visceral myopathy, a diagnosis which was confirmed by subsequent colectomy. The pathological findings are reviewed, and the potential use of transmural rectal biopsy in the diagnosis of smooth muscle disorders of the colon is discussed.

Pathology of neuromuscular disorders of the small intestine and colon.

A variety of pathological abnormalities of the smooth muscle and myenteric plexus result in clinical syndromes of disordered small intestinal and colonic motility. These pathological abnormalities have been noted by conventional light microscopy and by utilization of Smith's technique for visualizing the myenteric plexus with silver. We have classified the neuromuscular disorders into two major categories, i.e., those affecting the myenteric plexus and those affecting the smooth muscle. The classification is further developed based on the variety of clinicopathological features of the various disorders. Although we can now identify the underlying pathology of these motor disorders and thus understand these illnesses better than we did a decade ago, we have much more to learn. With the great strides being made to understand the normal structure, function, and development of the myenteric plexus and smooth muscle, there is hope that we will be able to learn much more about the etiology and pathogenesis of these neuromuscular disorders in the decade to come.

Esophageal motor dysfunction in idiopathic intestinal pseudoobstruction.

Five patients with idiopathic intestinal pseudoobstruction were studied with esophageal manometry and all had abnormalities of esophageal motility. In 2, primary peristalsis was replaced by simultaneous contractions and repetitive spontaneous activity was marked. The lower esophageal sphincter failed to relax and the Mecholyl test was positive in both. In the other 3, primary peristalsis was absent, low amplitude simultaneous waves followed swallows, no spontaneous activity was present, and defects in lower esophageal sphincter relaxation were found in two. The Mecholyl test was negative in the one patient tested. The esophagus appears to be abnormal in idiopathic intestinal pseudoobstruction and could prove useful as an organ for diagnostic study in patients who may have this syndrome.

Studies of idiopathic intestinal pseudoobstruction. II. Hereditary hollow visceral myopathy: family studies.

Twelve relatives of a 15-year-old girl with idiopathic intestinal pseudoobstruction were studied with esophageal manometry or cine-esophagography to determine whether the disease was genetically transmitted. Four maternal relatives, including the patient's mother, 13-year-old brother, one aunt, and one of that aunt's children had mild dysphagia and esophageal motor dysfunction. In addition, the patient's mother and 13-year-old brother had a flaccid bladder and bilateral ureteral reflux, respectively. The brother had abnormal bladder smooth muscle by light microscopy. We conclude that idiopathic intestinal pseudoobstruction in this family is secondary to a generalized disease of smooth muscle which is transmitted as a dominant trait of variable expressivity, manifested in some family members as a mild disorder of esophageal smooth muscle dysfunction, at times accompanied by bladder dysfunction. We suggest that this form of idiopathic intestinal pseudoobstruction be called "hereditary hollow visceral myopathy." Esophageal manometry may prove useful as a tool for studying the inheritance of this disorder.

Chronic idiopathic intestinal pseudo-obstruction caused by a degenerative disorder of the myenteric plexus: the use of Smith's method to define the neuropathology.

In this paper we report the pathologic basis of chronic idiopathic intestinal pseudo-obstruction in a patient who had a subtotal colectomy and ileorectal anastomosis for severe obstipation. Conventional light microscopy of the resected intestine showed an increased thickness of the longitudinal muscle, minimal amounts of smooth muscle fibrosis, and normal smooth muscle cells. The morphology of the myenteric plexus was difficult to interpret with this technique, but quantification of colonic neurons revealed a significantly decreased number compared with controls. Silver stains of the myenteric plexus by Smith's method showed: (a) patchy loss of nerve tracts with replacement by Schwann cells, (b) degeneration and decreased numbers of both argryophilic and argyrophobic neurons, (c) fragmentation and dropout of many axons, and (d) increased thickness and disorganized spatial arrangement of other axons. The pathology of this intestinal neuropathy could be missed by conventional light microscopy and may be apparent only when a silver technique is used to visualize the myenteric plexus.

Chronic idiopathic intestinal pseudo-obstruction. A surgical approach.

Chronic idiopathic intestinal pseudo-obstruction is an increasingly recognized syndrome in which patients usually present with an acute or chronic history suggestive of intestinal obstruction, although no obstructing lesion is found at surgery. The diagnosis can be suspected in most cases from the clinical presentation. A diagnostic evaluation should be undertaken and exploratory laparotomy avoided if the diagnosis is confirmed on the basis of the radiographic and manometric data. If, in the acute presentation, exploratory laparotomy proves unavoidable, and dilated, nonmechanically obstructed bowel is found, a full-thickness biopsy specimen should usually be taken and the abdomen closed. A carefully chosen, palliative procedure should be reserved for patients who have well defined clinicoanatomic patterns of involvement, and who are incapacitated by their symptoms despite medical management.

Small intestinal biopsy in a patient with Crohn's disease of the duodenum. The spectrum of abnormal findings in the absence of granulomas.

The case is reported of a patient who presented with an occult anemia that was due to Crohn's disease of the duodenum. The initial evaluation revealed low serum levels of iron, folate, and carotene, and a small bowel series was abnormal but not diagnostic of Crohn's disease. Numerous small intestinal biopsy specimens were obtained from the duodenum and proximal jejunum in an unsuccessful attempt to make a diagnosis. It was shown by radiography and laparotomy 2 yr later that the patient had Crohn's disease of the proximal small intestine. This report provides a detailed analysis of the spectrum of abnormalities found by peroral mucosal biopsy in this patient. These abnormalities were patchy and included flattened mucosa, an abnormal surface epithelium which was infiltrated by large numbers of polymorphonuclear leukoyctes, increased plasma cells and polymorphonuclear leuckocytes within the lamina propria, crypt abscesses, erosions, granulation tissue, and pyloric gland metaplasia, all in the absence of granulomas. Crohn's disease should always be considered in the differential diagnosis of a proximal small bowel mucosal disease, especially when a constellation of acute inflammatory changes is present.

There are no morphologic abnormalities of the gastric wall or abdominal vagus in patients with diabetic gastroparesis.

Because there is evidence for vagal autonomic neuropathy as the cause of diabetic gastroparesis, we hypothesized that this disorder should be associated with morphologic abnormalities of the abdominal vagus nerve or gastric myenteric plexus, or both. We studied the smooth muscle and myenteric plexus of the stomach in 18 nondiabetic controls and 16 patients with long-standing diabetes. Five of the diabetics had gastroparesis and 11 did not. We utilized conventional histology and Smith's silver technique for visualizing the myenteric plexus. Neurons within the myenteric plexus were quantified in sections stained with each technique. The abdominal vagus nerves from 5 diabetics (2 with gastroparesis) and 12 nondiabetic controls were stained with hematoxylin and eosin, Gomori trichrome, luxol-fast blue, and Holmes' silver stains. There were no abnormalities in the numbers or appearance of neurons or axons in the myenteric plexus of the stomach of diabetics, with or without gastroparesis. Also absent were abnormalities of the smooth muscle or vagus nerve. Thus, no morphologic abnormalities of the gastric wall or abdominal vagus were identified in diabetic gastroparesis.

Chronic intestinal pseudo-obstruction in infants and children caused by diverse abnormalities of the myenteric plexus.

BACKGROUND: Chronic intestinal pseudo-obstruction is a motility disorder that leads to severe disability in infants and children. Our purpose was to study the myenteric plexus in infants and children with pseudo-obstruction METHODS: Using cross sections stained with H&E and frozen tangential sections of the myenteric plexus processed using Smith's silver technique, the myenteric plexus of the stomach, small intestine, and colon from 26 affected infants and children was analyzed quantitatively. RESULTS: Under H&E staining, there were decreased neuron scores of 34.1 +/- 7.4 and 25.3 +/- 7.1 in the jejunum and ileum (normal, 57.5 +/- 6.2 and 55.0 +/- 4; P = 0.005 for both). Using Smith's technique, the specimens had decreased argyrophilic neuron counts of 3.1 +/- 1.4, 3.6 +/- 1.1, 3.4 +/- 1.0, and 2.8 +/- 0.6 in the duodenum, jejunum, ileum, and colon, respectively (normal, 14.0 +/- 1, 14.8 +/- 1, 14.7 +/- 0.5, and 13.6 +/- 1.1; P < 0.0003). The abnormalities included (1) absence of myenteric plexus in 3 patients; (2) small numbers of neuronal structures present on H&E stains but the absence of the plexus on silver stains in 2; and (3) myenteric plexus present but decreased argyrophilic neurons and axons on silver stains in 18. The abnormalities would have been missed in 10 patients if H&E had been used alone, whereas Smith's technique identified the deficiency of argyrophilic neurons in all patients tested. CONCLUSIONS: Pseudo-obstruction in infants and children may be caused by diverse abnormalities of the myenteric plexus.

Subtotal colectomy for severe idiopathic constipation. A follow-up study of 13 patients.

We obtained follow-up information on 13 patients who underwent subtotal colectomy for severe idiopathic constipation 19-45 months previously. Stool frequency increased from one bowel movement per 11.5 days before colectomy to 5.3 bowel movements per day after colectomy. Nine patients have required readmissions for abdominal pain and four have required further surgery for symptoms of small bowel obstruction. Ten patients consider that their quality of life is improved, although five have variable amounts of fecal incontinence. Preoperative studies did not predict the three patients who failed to improve. Subtotal colectomy palliates constipation in most patients with severe idiopathic constipation but patients should be cautioned that not all improve and some are left with significant abdominal pain, obstructive symptoms, diarrhea, and fecal incontinence.

Familial enteric neuropathy with pseudoobstruction.

We report a case of autosomal dominant chronic intestinal pseudoobstruction secondary to a familial enteric neuropathy. Esophagogastrointestinal manometry studies in the index case showed decreased postprandial contractile frequency with normal amplitude of pressure activity in the stomach and small bowel. Pupillary function and autonomic reflexes were all normal, excluding an extrinsic autonomic neuropathy of the viscera. Histologic examination of the small intestine by hematoxylin and eosin stains revealed normal smooth muscles but a reduced number of neurons in the myenteric plexus without inflammatory cells or neuroNal intranuclear inclusions. Histologic examination of the myenteric plexus using the sections taken along the longitudinal axis of the intestine, stained with silver by the Smith technique, disclosed decreased numbers of argyrophilic neurons and degeneration of neurons and axons; however, there was no reactive increase in the number of glial cell nuclei. The patient's mother had suffered from chronic intestinal pseudoobstruction, which did not abate following extensive small bowel resection. This is the third family reported with an autosomal dominant enteric neuropathy unassociated with evidence of extrinsic autonomic or peripheral neuropathy. Subtotal resection of the small bowel was followed by recurrence of the pseudoobstruction syndrome in both affected members of the family.

Small bowel resection for relief of chronic intestinal pseudo-obstruction.

Chronic intestinal pseudo-obstruction refractory to medical therapy is a debilitating problem for patients and a challenge for clinicians. We report a case of chronic idiopathic intestinal pseudo-obstruction with giant upper intestinal diverticula, complicated by hypersecretion refractory to medical therapy and requiring 10 yr of home parenteral nutrition. Resection of the chronically dilated small bowel with giant diverticula and construction of improved gastric and duodenal drainage was performed. This not only relieved the hypersecretory state and improved gastric drainage, but allowed the patient to stop parenteral nutrition and eat regular food for the first time in 10 yr. Select patients with chronic intestinal pseudo-obstruction will respond favorably to palliative surgical intervention.