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.

Pneumatosis intestinalis: a review.

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.

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.

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.

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.

Amyloidosis mimics achalasia's effect on lower esophageal sphincter.

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.

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.

Megacolon in myotonic dystrophy caused by a degenerative neuropathy of the myenteric plexus.

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.

Visceral myopathy of the gastrointestinal and genitourinary tracts in infants.

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.

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.

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.

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.

Neuronal dysplasia and chronic intestinal pseudoobstruction: rectal biopsy as a possible aid to diagnosis.

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.

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.

Familial visceral neuropathy with autosomal dominant transmission.

This report describes a family with a visceral neuropathy without extraintestinal manifestations transmitted over at least four generations in an autosomal dominant manner. Four of 7 living patients underwent extensive evaluation including histology, radiography, gastric emptying and secretory studies, esophageal and jejunal manometry, and measurements of plasma levels of gastrointestinal hormones. The only characteristic radiologic abnormality in 7 patients was dilatation of jejunum and ileum. Gastric emptying studies were normal in 2 patients, whereas 2 others showed accelerated emptying of liquids either alone or in combination with grossly delayed solid emptying. Manometry of the esophagus and proximal small intestine and gastric secretory studies were normal. Histologic studies showed hypertrophy of the smooth muscle, a markedly reduced number of argyrophilic neurons, and degenerative changes of argyrophilic neurons and nerve fibers but without Schwann cell proliferation, intranuclear inclusions, or inflammatory cells. This appears to be a familial visceral neuropathy characterized by distinct involvement of the jejunum and ileum as defined radiographically, histology different from that of two previously described forms of familial visceral neuropathy, autosomal dominant transmission, and no evidence for extraintestinal neurologic manifestations.

Radiation-induced intestinal pseudoobstruction.

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.

Spectrum of rectal biopsy abnormalities in homosexual men with intestinal symptoms.

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.

An inflammatory axonopathy of the myenteric plexus producing a rapidly progressive intestinal pseudoobstruction.

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.