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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., (Copyright 2002 The Society for Surgery of the Alimentary Tract, Inc.) more...

Pneumatosis intestinalis (PI) is an uncommon but important condition in which gas is found in a linear or cystic form in the submucosa or subserosa of the bowel wall. PI is a sign, not a disease; therefore, its relevance should be interpreted within the whole clinical context. PI has been found in several distinctive clinical settings: 1) in premature infants with necrotizing enterocolitis; 2) in adults with obstructive pulmonary disease; 3) in adults and children with a wide variety of associated conditions, including pyloric stenosis, jejunoileal bypass, progressive systemic sclerosis, transplantation, ischemic bowel, and drug therapy, particularly steroids, chemotherapy, and immunosuppression; 4) in adults as a primary benign problem; and 5) as an incidental finding in endoscopic mucosal biopsies. The two most important tasks of the physician include: 1) recognition of the entity of PI so that patients are not misdiagnosed and mismanaged as having malignancy or polyposis; and 2) differentiation of the benign variety, in which no intervention is indicated, from the life-threatening form, in which immediate surgery is necessary. Once life-threatening illnesses such as bowel necrosis, perforation, and infections are excluded, patients symptomatic from the cysts per se may be treated with oxygen and/or antibiotics. Because the reports of treatment of PI are at best anecdotal, the decision to treat and the treatment chosen should be carefully balanced with the risks. more...

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. more...

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. more...

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. more...

Acute herpes simplex virus (HSV) infection was detected in 23 of 102 consecutively examined, sexually active male homosexuals who presented with anorectal pain, discharge, tenesmus, or hematochezia, as compared with 3 of 75 homosexual men without gastrointestinal symptoms (P less than 0.01). Findings that were significantly more frequent in men with HSV proctitis than in men with proctitis due to other infectious causes included fever (48 per cent), difficulty in urinating (48 per cent), sacral paresthesias (26 per cent), inguinal lymphadenopathy (57 per cent), severe anorectal pain (100 per cent), tenesmus (100 per cent), constipation (78 per cent), perianal ulcerations (70 per cent), and the presence of diffuse ulcerative or discrete vesicular or pustular lesions in the distal 5 cm of the rectum (50 per cent). Serologic evidence indicated that 85 per cent of the men with symptomatic HSV proctitis were having their first episode of HSV-2 infection. The diagnosis of HSV proctitis is suggested by the presence of severe anorectal pain, difficulty in urinating, sacral paresthesias or pain, and diffuse ulceration of the distal rectal mucosa. more...

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. more...

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. more...

A previously well 39-yr-old man presented with a 4-wk history of abdominal pain, nausea, vomiting, and weight loss. An upper gastrointestinal examination showed retained food in the stomach and duodenal dilatation. A radioisotope meal showed little gastric emptying; esophageal manometry was normal. Because of persistent symptoms, a duodenojejunostomy was done. However, the patient remained symptomatic and after an episode of profuse vomiting, aspirated and died 10 wk after initial presentation. At autopsy, no tumor was found. Hematoxylin and eosin stains throughout the gastrointestinal tract showed many lymphocytes and plasma cells within the myenteric plexus. Silver stains showed the argyrophilic and argyrophobic neurons to be normal, but axons showed beading, fragmentation, and dropout in all areas. We therefore concluded the following: intestinal pseudoobstruction can be caused by an inflammatory neuropathy of the myenteric plexus, not associated with a distant carcinoma, and this process produced an axonopathy while sparing neuron bodies. more...

Disabling esophageal symptoms ((dysphagia, painful swallowing, and severe restrosternal pain) developed in 8 of 63 patients with chronic graft-versus-host disease after allogeneic bone marrow transplantation. At endoscopy 7 patients had characteristic desquamation of the upper esophagus; 2 of these also had distal esophagitis; and 3 had distinctive upper esophageal webs. No infectious pathogens were detected in esophageal biopsies or brushings. Abnormalities of esophageal motility were seen in 5 of 7 patients studied including 3 with aperistalsis. Retrosternal pain in 3 patients resulted from acid reflux. Esophageal histology from 5 autopsied patients showed no muscle or neuronal abnormalities by silver stain or conventional light microscopy. There was increased submucosal fibrosis associated with mucosal esophagitis and ulceration. Blind microscopic review of histology clearly distinguished the esophagus of chronic graft-versus-host disease from that of progressive systemic sclerosis. We conclude that esophageal epithelium, like skin and mucous membranes, is a target organ in chronic graft-versus-host disease. This immunologic disease results in desquamative esophagitis with web formation. Peptic esophagitis, a cause of severe pain and perhaps distal esophageal strictures in these patients, may be related to poor acid clearing from the esophagus. Diagnostic endoscopy and disruption of webs should be performed carefully to avoid perforation. Treatment should be directed toward suppressing the underlying immunologic disorder and at preventing acid-peptic reflux. more...

A 37-yr-old homosexual man presented with mild anorectal symptoms, a diffuse maculopapular rash, constitutional symptoms, and a reactive serologic test for syphilis. Sigmoidoscopy revealed a 2 x 2-cm indurated rectal mass 5 cm above the anal verge. Darkfield examination of rectal exudate revealed motile treponemes and rectal biopsy of the mass showed diffuse infiltration of plasma cells and lymphocytes. Large numbers of spirochetes were present on silver stain of the rectal biopsy specimen and these were shown to be Treponema pallidum by indirect immunofluorescence. The rectal mass and symptoms resolved following penicillin therapy. This case and a review of previously reported cases of anorectal syphilis emphasize the significance of these often neglected and misdiagnosed lesions of syphilis. more...

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. more...

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. more...

A 58-year-old woman who had presented with intestinal pseudo-obstruction died 9 months later from rapidly progressive neurologic symptoms and autonomic insufficiency. Her gastric emptying had been markedly delayed and transit of markers had been slowed throughout the small bowel. A 5-hour manometric recording of the antrum and duodenum had shown absence of the normal interdigestive motor complex, which was replaced by irregular contractile activity of reduced amplitude. A small-cell carcinoma of the lung was found at autopsy. Pathologic study of the gut showed widespread degeneration of the myenteric plexus, which was infiltrated by plasma cells and lymphocytes and contained significantly reduced numbers of neurons. The extra-intestinal nervous system had neuronal loss and lymphocytic infiltrates in dorsal root ganglia. Thus, a gastrointestinal neuropathy causing intestinal pseudo-obstruction may be the presenting manifestation of a paraneoplastic syndrome associated with small-cell carcinoma. more...

We report a patient with an unusual cause of chronic intestinal pseudoobstruction, i.e., neuronal intestinal dysplasia. This disorder is characterized by hyperplasia of the nerve plexuses of the intestine or colon, or both. Detailed morphologic and manometric studies are provided. The discussion emphasizes the various motor abnormalities that may be found in chronic intestinal pseudoobstruction. We propose that rectal biopsy may be of value in the diagnosis of this unusual form of pseudoobstruction. more...

We report a case of Duchenne's muscular dystrophy complicated by intestinal pseudoobstruction. The patient had recurrent attacks of nausea, vomiting, and abdominal distention for many years, and abdominal films repeatedly showed a dilated and fluid-filled small intestine and colon. Barium studies showed an esophageal diverticulum, reduced esophageal and gastric motility, and a dilated small bowel and colon. Pathologically, the entire gastrointestinal tract had smooth muscle fibrosis, but this was most marked in the esophagus and stomach. We conclude that Duchenne's muscular dystrophy may involve intestinal smooth muscle and produce pseudoobstruction. more...

We describe 4 infants who had chronic intestinal pseudoobstruction caused by visceral myopathy. Three of the 4 were girls. Two were symptomatic at birth and 2 were symptomatic by 3 wk of age. All had abdominal distention and emaciation, 3 of the 4 had severe obstipation and fecal impactions, and 3 had signs of urologic obstruction. All had gaseous distention of the small bowel and colon, and barium studies showed dilated small bowel and colon, with slow transport through the small intestine. Two of 3 had enlarged stomachs and slowed gastric emptying, and 3 had dilated bladders and ureters. The 1 infant studied by esophageal manometry had absence of esophageal contractions. Despite total parenteral nutrition in 3, all died within 10-18 mo. The pathologic features of visceral myopathy were identified in variable sample sites from the esophagus, stomach, small intestine, colon, bladder, and ureter of the 4 infants. Of 170 family members related to 3 of the infants, there was no consanguinity and no one appeared to be clinically affected. Thus, an infantile form of visceral myopathy exists which, pathologically, is identical to the familial and sporadic forms of visceral myopathy previously identified in adolescents and adults. more...

A 32-yr-old man with myotonic dystrophy had a left hemicolectomy performed because of a megacolon. The colonic mucosa, smooth muscle, and connective tissue appeared normal by hematoxylin and eosin and trichrome stains and transmission electron microscopy. In contrast, the myenteric plexus had markedly fewer neurons than normal on the hematoxylin and eosin stains. Silver staining of the plexus revealed degeneration and decreased numbers of argyrophilic neurons, which were smaller and had fewer processes and a more uneven staining quality than controls. Many axons were fragmented, and increased numbers of glial cell nuclei were present in the plexus. Degenerative changes in the neurons were present in a patchy distribution on transmission electron microscopy. Immunohistochemistry revealed a decrease of the substance P- and enkephalin-immunoreactive fibers in the muscularis externa. This suggests that colonic motor dysfunction associated with myotonic dystrophy may be caused by a visceral neuropathy that involves the substance P- and enkephalin-immunoreactive fibers of the smooth muscle. more...

This study presents the case of a patient who had jejunal diverticulosis with perforation and abscess formation as a complication of Fabry's disease. Light microscopy disclosed glycolipid deposition in the neurons and nerve fibers of the intestinal nerve plexuses and smooth muscle. Silver stains of the myenteric plexus in the involved segment of the bowel showed enlarged, granular argyrophobic neurons and a marked decrease in the number of argyrophilic neurons, with those remaining being enlarged and distorted by the cytoplasmic glycolipid accumulation. These abnormalities of the myenteric plexus suggest that jejunal diverticulosis may be the result of a variety of disorders of the smooth muscle or myenteric plexus, or both. We propose that jejunal diverticulosis in our patient was a consequence of uncoordinated smooth muscle activity resulting from Fabry's involvement of myenteric plexus neurons, with mucosal protrusion through the smooth muscle. more...

A 15-year-old girl with idiopathic intestinal pseudoobstruction is reported. She presented with a long term history of low grade obstructive symptoms, diarrhea, and poor nutrition culminating in an acute obstructive attack leading to exploratory laparotomy. At surgery, the small bowel and colon were dilated, with no mechanical obstruction found. Further evaluation revealed her to have a diffuse disorder of gastrointestinal smooth muscle function involving esophagus, small bowel, and colon. Because medical management failed to control symptom, a gastrojejunostomy was done to bypass a megaduodenum. A third laparotomy was necessary 2 months later because of intractable obstructive symptoms. At this last laparotomy, the afferent loop was taken down and a duodenoplasty and duodenojejunostomy were performed, resulting in clinical improvement. Light and electron microscopic study of the excised small intestine showed marked thinning and degeneraton of the smooth muscle, with replacement by fibrous tissue. The myenteric plexus was normal. This case demonstrates that a degenerative disease of smooth muscle may be one cause of idiopathic intestinal pseudoobstruction. more...

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. more...

In studies of the cause of gastrointestinal symptoms in homosexual men, Campylobacter jejuni was recovered from 10 of 158 men with, and 2 of 75 men without, intestinal symptoms. In addition, a heterogeneous group of Campylobacter-like organisms was identified in 26 symptomatic and 6 asymptomatic homosexual men, but in none of 150 heterosexual men and women. Infections with Campylobacter-like organisms, like infections with C. jejuni, were significantly associated with the sexual practice of anilinctus and were usually associated with diarrhea, abdominal cramps, and hematochezia; proctocolitis seen at sigmoidoscopy; increased numbers of leukocytes seen on rectal smears; acute inflammatory changes seen on rectal biopsy specimens; and a serum antibody response to the infecting organism. more...

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. more...

Homosexually active men have frequent intestinal and rectal symptoms resulting from sexually acquired gastrointestinal infections. We evaluated the histologic findings in rectal biopsy specimens obtained from 89 homosexual men with intestinal symptoms and 11 homosexual men without intestinal symptoms. All had undergone comprehensive microbiologic evaluation for rectal and enteric pathogens. Rectal biopsy specimens were evaluated without knowledge of clinical or microbiologic data by a standardized method for the presence or absence of abnormal histologic features. Forty-six percent of specimens from symptomatic men and 27% of those from asymptomatic men were abnormal. Acute inflammation was the most frequent histologic abnormality and was more frequent in men who had pathogens (51%) than men without pathogens (24%, p less than 0.02). Acute but not chronic inflammation was seen also in specimens from homosexual men without intestinal symptoms. Intestinal spirochetosis was present in specimens from 23 (26%) of the symptomatic and 5 (45%) of the asymptomatic men. In 5 of the 89 symptomatic men, biopsy features of idiopathic inflammatory bowel disease (IIBD) were present; all 5 of these men were infected with either Treponema pallidum or Chlamydia trachomatis. Features of IIBD were present in 25% of those infected with C. trachomatis or T. pallidum. Chronic inflammation was more frequent in men infected with C. trachomatis, syphilis, or herpes simplex virus type II: 31% vs. 3%, p = 0.0002. Acute inflammation was present in specimens from men with proctitis or proctocolitis and enteritis as well as in those from asymptomatic men, whereas chronic inflammation was present only in specimens from men with proctitis or proctocolitis. Both acute and chronic inflammation were more frequent when biopsy specimens of the abnormal mucosa were examined. When specimens from men with single infections were analyzed, histology was rarely diagnostic. We conclude that acute inflammation is frequent in rectal biopsy specimens from symptomatic and asymptomatic homosexual men; chronic inflammation is infrequent, but when present is significantly associated with syphilis, herpes simplex virus type II, and C. trachomatis infection. more...

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. more...

Forty patients with neuromuscular disorders of the gastrointestinal tract were evaluated histologically and radiologically. Eighteen patients with progressive systemic sclerosis had predominant circular muscle thinning and fibrosis. Visceral myopathy (11 patients) was characterized by vacuolar degeneration of the smooth muscle cells with thinning and fibrosis typically affecting the longitudinal layers. Visceral neuropathy (five patients) had degeneration of myenteric plexus neurons with various patients having intranuclear inclusions, Schwann cell proliferation, or inflammatory cell infiltration. Radiologically, these syndromes had diffuse abnormalities of gastrointestinal motor function manifested by small and large intestinal dilatation, esophageal hypomotility (progressive systemic sclerosis and visceral myopathy), or disordered hypercontractility (visceral neuropathy). Marked duodenal enlargement typified visceral myopathy, and although all types may have dilated small intestine, only progressive systemic sclerosis has packing of valvulae. Colonic sacculations were found in progressive systemic sclerosis, lack of haustrations and increased colonic caliber in visceral myopathy, and hypercontractility in visceral neuropathy. Complete barium contrast examination will assist in differentiation of true obstruction from pseudoobstruction, will define the diffuse nature of the syndrome, and will help establish an accurate diagnosis by identifying features specific for these entities. more...

Six patients with idopathic intestinal pseudoobstruction underwent extensive radiographic evaluation of the gastrointestinal tract. Propulsive motor activity was consistently absent. All had smooth muscle dysfunction of the esophagus, small bowel, and colon, and two had abnormal gastric emptying. Two forms of the syndrome were observed, characterized by either hyper- or hypoactive smooth muscle. In the hyperactive form chaotic, spontaneous contractions of the esophagus and small intestine occurred and extensive diverticular disease of the colon was present. In the hypoactive form the esophagus was atonic and there was marked widening and hypomotility of the small intestine and colon. The presence of two forms of smooth muscle dysfunction suggests that the syndrome has a heterogeneous pathology and pathophysiology. more...

Esophageal involvement in systemic amyloidosis is common. Manometric studies have been few in number and have revealed a variety of findings compatible with deposition of amyloid in myopathic as well as neuropathic patterns. This report describes a patient with primary amyloid whose esophageal dysfunction was limited to the lower esophageal sphincter, with both a hypertensive sphincter and impaired relaxation after swallows. Pharmacologic testing confirmed a mild abnormality of the inhibitory pathway to the LES with an intact excitatory pathway and sphincter muscle. This case suggests that amyloidosis, like idiopathic achalasia and carcinoma of the lower esophagus, can produce dysphagia by selective impairment of the inhibitory neural pathway to the lower esophageal sphincter. more...

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. more...

The purpose of this investigation was to determine the frequency and severity of small intestinal mucosal damage in pseudoobstruction syndromes. One hundred eighty-nine interpretable biopsies from 12 patients were blindly reviewed by two investigators. The underlying disorders were scleroderma in 7 and idiopathic intestinal pseudoobstruction in 5. All 12 had small-intestinal dilatation on small-bowel series. Eight of the 12 patients had biopsies characterized by moderate to severe mucosal damage; 3 of these had some biopsies which were flat. The damage did not correlate with: (1) types and numbers of organisms recovered from small intestinal aspirates; (2) duration of illness; (3) degree of dilatation of the proximal small bowel; (4) concentrations of deconjugated bile salts in small intestinal fluid; or (5) amount of fat absorbed in fat-balance studies. We conclude that mucosal damage is common in pseudoobstruction syndromes. The pathogenesis of the damage and its relationship to intraluminal bacteria remain undefined. more...

The purpose of this paper is to describe 2 siblings who had a generalized neurological disease which presented as intestinal pseudoobstruction. The siblings had 40-year histories of abdominal pain, distention, and vomiting as well as gait ataxia, small, irregular, poorly reactive pupils, dysarthria, absent deep tendon reflexes, and impaired vibratory and position senses. Compared with age-matched controls, they had inappropriate blood pressure responses to phenylephrine, the Valsalva maneuver, and upright posture, lack of sweating on warming, and pupillary denervation hypersensitivity. Radiographs revealed hyperactive, nonpropulsive contractions of a dilated esophagus and small intestine and extensive colonic diverticulosis. Esophageal manometry recorded repetitive, spontaneous, nonperistaltic waves and positive Mechyolyl tests. Postmortem examinations showed degeneration of the myenteric plexuses of the esophagus, small intestine, and colon of both patients. Myenteric plexus neurons were significantly reduced in number compared with 7 controls. About one-third of the siblings' neurons contained round, eosinophilic intranuclear inclusions, which, by histochemistry, were composed of protein by lacked RNA, DNA, carbohydrate, and fat. By electron microscopy the inclusions consisted of an irregular array of nonviral, nonmembrane-bounded filaments. Neurons and glial cells of the brain, spinal cord, dorsal root, and celiac plexus ganglia contained identical intranuclear inclusions. Intestinal smooth muscle was normal. These 2 siblings represent a unique disease in which degeneration of the myenteric plexus resulted in hyperactive but uncoordinated smooth muscle activity and the clinical syndrome of intestinal pseudoobstruction, the presenting manifestation of their neurological disease. more...

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. more...

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. more...

We analyzed the clinical, radiographic, esophageal manometric, and pathologic features of 26 women with severe, idiopathic constipation. Twenty-four patients were between 19 and 39 yr of age. Stool frequency was once every 5-28 days. On barium enema examination, 9 of 24 patients had colons of increased length and 4 of these 9 patients had colons of increased width (greater than 10 cm). Radionuclide solid-meal gastric-emptying studies were normal in 23 patients tested. Esophageal manometry demonstrated high-amplitude waves in 10 of 22 patients and long-duration waves in 3 of these 10 patients. Rectal biopsy specimens showed normal submucosal neurons in all patients and melanosis coli in 6. Twelve patients underwent subtotal colectomies for constipation. Conventional light microscopy using hematoxylin and eosin serial sections showed (a) melanosis coli in 4 patients; (b) normal smooth muscle in 11; (c) thinning of the circular muscle in 1; and (d) no apparent abnormalities of the myenteric plexus in any. In contrast, silver stains of the myenteric plexus showed (a) quantitatively reduced numbers of argyrophilic neurons in 10 patients; (b) morphologically abnormal argyrophilic neurons in 11; (c) decreased numbers of axons in 11; and (d) increased numbers of variably sized nuclei within ganglia in all 12. A coded analysis of the silver stains of colons from 8 patients with constipation and 19 control cases demonstrated that the pathologic abnormalities of severe idiopathic constipation could be differentiated from controls. Thus, severe idiopathic constipation is associated with a pathologically identifiable abnormality of the myenteric plexus. This abnormality appears different from anything previously described in intestinal pseudoobstruction. more...

A case of intestinal pseudoobstruction occurring 30 yr after radiation therapy is described. Mechanical causes of obstruction were excluded by laparotomy. Histology of full-thickness sections of the small bowel revealed vascular ectasia and sclerosis, serosal fibrosis, neuronal proliferation within the submucosa, and degeneration of the muscle fibers of the circular layer of the muscularis propria. On the basis of the clinical and histologic findings we conclude that, in this patient, intestinal pseudoobstruction was due to muscular and neuronal injury from abdominal irradiation. more...

One hundreds seventy-one homosexual men, 96 of whom had symptoms suggestive of proctitis and 75 of whom had no such symptoms, were consecutively enrolled in a study of the prevalence, clinical spectrum, and histopathology of Chlamydia trachomatis rectal infections. C. trachomatis was isolated from the rectums of 14 men. Three of the isolates, which had lymphogranuloma venereum (LGV) immunotypes, were recovered from three men with symptoms and signs of severe proctitis, two of whom had granulomatous inflammation that was observed at rectal biopsy and was initially suggestive of Crohn's disease. Eleven isolates, which had non-LGV immunotypes, were obtained from eight symptomatic and three asymptomatic men, all of whom had fecal leukocytes and mild abnormalities of the mucosa present at sigmoidoscopy, usually with mild nongranulomatous inflammatory changes that were seen on rectal biopsy. These observations suggest that the presence of LGV immunotypes of C. trachomatis in the rectum is associated with severe acute proctitis that mimics Crohn's disease of the rectum, whereas the non-LGV immunotypes are associated with a mild proctitis with or without symptoms. more...

We report a case of chronic intestinal pseudoobstruction caused by a newly recognized type of degenerative neuropathy of the myenteric plexus. Failure to improve despite aggressive medical management led to radical resection of the clinically involved small intestine. At follow-up 10 mo later, the patient is doing well without the need for parenteral nutrition. Radical resection of the small intestine may be necessary for palliation in rare patients with intractable pseudoobstruction. more...

We analyzed the clinical, radiographic, esophageal manometric, and pathological features of 10 patients referred with jejunal diverticulosis. Nine patients were over age 59 yr and had symptoms of intestinal pseudoobstruction of 5-43 yr duration. Seven had surgery for mechanical obstruction, although none was found. Eight had diarrhea, steatorrhea, and weight loss. Five had Raynaud's phenomenon and heartburn, and 2 had dysphagia. At radiography, 9 had jejunal diverticula with or without duodenal or ileal diverticula, or both. Two each had abnormal structure or motility of the esophagus or stomach. At manometry, 3 of 7 had a nonspecific motor abnormality, and 1 other had low amplitude peristaltic waves. Light microscopy of small intestinal tissue in 7 patients showed that 4 had fibrosis and decreased numbers of normal-appearing muscle cells, findings consistent with progressive systemic sclerosis. Two others had fibrosis associated with degenerated smooth muscle cells, findings consistent with a visceral myopathy. The seventh patient had neuronal and axonal degeneration and neuronal intranuclear inclusions, findings consistent with a visceral neuropathy. We conclude that (a) intestinal pseudoobstruction is a major clinical manifestation of jejunal diverticulosis, (b) jejunal diverticulosis is a heterogenous disorder associated with at least three abnormalities of the smooth muscle or myenteric plexus, (c) in contrast to intestinal pseudoobstruction without diverticulosis, the esophagus, stomach, and colon are less frequently involved in jejunal diverticulosis, and (d) some patients with jejunal diverticulosis probably have clinically inapparent progressive systemic sclerosis. more...

Four young women presented with diarrhea, malabsorption, and intestinal pseudoobstruction. Intestinal biopsy specimens (both peroral and full-thickness) showed flat small intestinal mucosa, sparsity of crypts, and a widespread lymphoid infiltrate in the lamina propria, muscularis propria, and myenteric plexus. There was no neuron or nerve fiber loss or damage in the plexus; muscle cell absence in the vicinity of lymphoid cell infiltration in the muscularis propria probably accounted for the pathogenesis of pseudoobstruction. Immunochemical stains showed that the infiltrate was polyclonal, and none of the patients has developed lymphoma on clinical follow-up of 4-16 yr. Transient improvement in symptoms occurred after antibiotic therapy in 3 patients, and 1 patient had improvement after treatment with cyclophosphamide and prednisone; however, symptoms of pseudoobstruction persist in all. These cases illustrate yet another cause of intestinal pseudoobstruction which is histologically distinct from visceral myopathies and neuropathies. The pathogenesis of this illness may be related to that of diffuse immunoproliferative diseases seen in Third World countries. more...

To determine the microbial cause and the clinical and pathologic correlates of anorectal and intestinal symptoms in homosexually active men, we performed comprehensive microbiologic studies, anoscopy, sigmoid-oscopy, and rectal biopsy in men examined in a clinic for sexually transmitted diseases. Enteric pathogens were found in 95 of 119 consecutive homosexual men with anorectal or intestinal symptoms and in 29 of 75 randomly selected homosexual men without such symptoms (P less than 0.001). The syndromes of proctitis, proctocolitis, and enteritis were differentiated on the basis of predominant symptoms and findings on anoscopy and sigmoidoscopy. Neisseria gonorrhoeae, herpes simplex virus, Chlamydia trachomatis (non-lymphogranuloma venereum serotypes), and Treponema pallidum were associated with 80 per cent of cases with symptomatic proctitis. Known causes of colitis, including Campylobacter jejuni, Campylobacter fetus fetus, Shigella flexneri, Chl. trachomatis (lymphogranuloma venereum serotypes), Entamoeba histolytica, and Clostridium difficile, were identified in 60 per cent of the cases of proctocolitis. Giardia lamblia was the only agent significantly correlated with enteritis. These data demonstrate that intestinal symptoms in homosexual men are attributable to a complex spectrum of microorganisms, but that careful clinical classification can serve as a guide to the selection of microbiologic studies and to a rational initial choice of therapy. more...