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Biomedical subjects

C A Rohrmann

Publications and source records attributed to C A Rohrmann.

At least 37 records · Page 2Linked to original sources

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↗

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↗

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↗

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↗

Diagnosis and treatment of retroperitoneal perforation complicating the double-contrast barium-enema examination.

A case of retroperitoneal air caused by rectal perforation during a double-contrast barium-enema examination is reported. In 9 similar reported cases, radiographic signs included perirectal, mediastinal, and cervical emphysema. Because of the frequent absence of clinical signs, radiographic recognition may be crucial for prompt management. Reported experience suggests that asymptomatic patients with these radiographic findings may be managed with hospitalization and close observation rather than immediate laparotomy.

Aged↗

Double duct sign: reassessed significance in ERCP.

The double duct sign, defined as an abnormality of both the pancreatic duct and the contiguous part of intrapancreatic common bile duct, was found in 52 of 1180 patients studied by endoscopic retrograde cholangiopancreatography. Thirty patients were proved to have pancreatic malignancy and 22, benign pancreatic disease. Specific ductal characteristics found to indicate a malignant process were ductal obstruction, especially of the common bile duct, close proximity of the biductal lesions, a short stenotic segment of the common bile duct removed from the papilla, an abrupt, irregular transition from normal to stenotic or obstructed duct. Ductal characteristics suggesting a benign process were long length of common bile duct stenosis, calcium deposits, pseudocyst formation, and ectasia of pancreatic ductal branches central to the main duct lesion. This analysis indicates that the double duct sign per se is not disease specific, but when other ductal characteristics are assessed as a component of this finding, the ability to differentiate benign from malignant pancreatic disease is enhanced.

Adenocarcinoma↗

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.

Adolescent↗

Significance of the nonopacified gallbladder in endoscopic retrograde cholangiography.

Failure to visualize the cystic duct and gallbladder during endoscopic retrograde cholangiography, while obtaining satisfactory opacification of the rest of the biliary system, is a radiographic sign of undetermined meaning. The diagnostic implication of this finding was analyzed in 63 patients with pathologically proven diagnoses. One patient was normal at surgery. Three groups of abnormal patients had: (1) obstructing lesions of the distal common bile duct (35 patients); (2) primary lesions of the cystic duct or gallbladder (19 patients); or (3) obstructing lesions about the common hepatic/cystic duct junction (8 patients). The results indicate that obstructing lesions of the distal common bile duct may cause stasis of bile within the biliary system, increased biliary pressure, and sludge formation that prevent the flow of contrast material through the cystic duct and into the gallbladder, which can cause nonopacification. If the extrahepatic biliary system is of normal caliber without evidence of an obstructing process, nonfilling of the cystic duct and gallbladder is highly predictive of pathology.

Biliary Tract Diseases↗

Cholangiographic abnormalities in patients with inflammatory bowel disease.

Twenty patients with inflammatory bowel disease, abnormal liver function tests and abnormal endoscopic retrograde cholangiograms were found to have a spectrum of abnormalities affecting the intra- and extrahepatic biliary trees. The intrahepatic systems were abnormal in all patients and demonstrated ductal stenosis, ectasia, decreased arborization and major duct obstruction. The extrahepatic systems were abnormal in 75 percent of cases with stenosis, diverticula formation and mural irregularity being the most frequent abnormalities. The value of endoscopic retrograde cholangiography in this patient group is to exclude extrahepatic obstruction, establish a nonoperative diagnosis, and assist in determining the method of treatment.

Adenoma, Bile Duct↗

Chronic mucocutaneous candidiasis: radiologic abnormalities in the esophagus.

Chronic mucocutaneous candidiasis is a disorder characterized by chronic recurrent infection with Candida. The esophagus may be involved, resulting in chronic candidal esophagitis, which is clinically and radiographically distinct from acute candidal esophagitis. The clinical and radiographic features of four new and four previously reported cases are presented. Esopahgeal narrowing was found in all patients; it was focal in six and diffuse in two. Motor abnormalities were common, but mucosal changes seen characteristically in acute candidal esophagitis were unusual. Esophageal intramural pseudodiverticulosis was documented in one patient, substantiating the belief that Candida may be the etiologic agent in some patients with this condition.

Adolescent↗

Endoscopic retrograde intrahepatic cholangiogram: radiographic findings in intrahepatic disease.

Endoscopic retrograde intrahepatic cholangiograms were evaluated in 107 patients and correlated with intrahepatic diagnoses determined by liver biopsy. Included were normal livers (six), cirrhosis (38) portal fibrosis (14), cholangitis (22), metastases (11), and miscellaneous diagnoses (16). Results suggest that differentiation of the normal from the abnormal intrahepatic biliary system using the endoscopic retrograde intrahepatic cholangiogram is possible, and that certain patterns of abnormality prevail within given disease categories. The cholangiogram in cirrhosis is marked by ductular stenosis, diminished arborization, tortuosity, and approximation of the intrahepatic ducts. Sclerosing cholangitis demonstrates focal stenoses with concomitant ectasias and frequent similar involvement of the extrahepatic system. Chronic cholangitis and portal fibrosis are frequently associated with extrahepatic obstructing lesions and increased intrahepatic ductal caliber, but demonstrate no distinguishing intrahepatic characteristics. Intrahepatic metastases, polycystic liver disease, and primary hepatic neoplasm produce mass effects consisting of ductal displacement, narrowing, and obstruction. The potential of endoscopic retrograde intrahepatic cholangiography in evaluating the intraheptic biliary tree is significant; specifically in separating normal from abnormal, in distinguishing between intrahepatic processes, and as an adjunct to liver biopsy in determining the extent and location of intrahepatic abnormalities.

Bile Ducts, Intrahepatic↗