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Biomedical subjects

M D Schuffler

Publications and source records attributed to M D Schuffler.

At least 37 records · Page 2Linked to original sources
PubMed1986

Chronic intestinal pseudoobstruction as a complication of Duchenne's muscular dystrophy.

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.

Adolescent↗
PubMed1986

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.

Adult↗
PubMed1985

Intestinal pseudoobstruction caused by a new form of visceral neuropathy: palliation by radical small bowel resection.

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.

Adult↗
PubMed1985

Intestinal pseudoobstruction caused by diffuse lymphoid infiltration of the small intestine.

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.

Adolescent↗
PubMed1985

Severe idiopathic constipation is associated with a distinctive abnormality of the colonic myenteric plexus.

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.

Adult↗
PubMed1984

Radiologic and histologic differentiation of neuromuscular disorders of the gastrointestinal tract: visceral myopathies, visceral neuropathies, and progressive systemic sclerosis.

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.

Esophagus↗
PubMed1984

Infections with Campylobacter jejuni and Campylobacter-like organisms in homosexual men.

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.

Adult↗
PubMed1984

Jejunal diverticulosis with perforation as a complication of Fabry's disease.

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.

Abscess↗
PubMed1984

Megacolon and neurofibromatosis: a neuronal intestinal dysplasia. Case report and review of the literature.

This study presents the case of a patient with neurofibromatosis and megacolon. A diffuse, but patchy abnormality of neural tissue was present throughout the colon, especially in the myenteric plexus, but also in the submucosal plexus, smooth muscles, and lamina propria. This consisted of (a) a marked decrease in the number of argyrophilic neurons within the myenteric plexus, enlargement and deformity of those neurons present, and a marked increase of nerve fibers and nerve tract size in the myenteric plexus; (b) a proliferation of neurons and nerve fibers within the smooth muscle and submucosa; and (c) a proliferation of nerve fibers within focal areas of the lamina propria. The relationship of this case to previously reported cases of neuronal intestinal dysplasia and plexiform neurofibromatosis is discussed. Surgical treatment may be necessary and the surgical options are reviewed.

Colonic Neoplasms↗
PubMed1983

The polymicrobial origin of intestinal infections in homosexual men.

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.

Adult↗
PubMed1983

Herpes simplex virus proctitis in homosexual men. Clinical, sigmoidoscopic, and histopathological features.

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.

Acute Disease↗
PubMed1983

Intestinal pseudo-obstruction as the presenting manifestation of small-cell carcinoma of the lung. A paraneoplastic neuropathy of the gastrointestinal tract.

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.

Autonomic Nervous System Diseases↗
PubMed1983

Jejunal diverticulosis. A heterogenous disorder caused by a variety of abnormalities of smooth muscle or myenteric plexus.

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.

Adult↗
PubMed1982

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.

Adolescent↗
PubMed1982

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.

Colon↗
PubMed1982

Rectal mass caused by Treponema pallidum: confirmation by immunofluorescent staining.

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.

Adult↗
PubMed1981

Chlamydia trachomatis proctitis.

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.

Adult↗