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1/65. Four cases with chronic intestinal pseudo-obstruction due to hollow visceral myopathy.

    BACKGROUND/AIMS: Chronic intestinal pseudo-obstruction is a rare clinical syndrome characterized by symptoms and signs of intestinal obstruction without any organic lesion obstructing the intestine. Visceral myopathy is one of the etiological causes and full thickness intestinal biopsy is essential for reaching a diagnosis. In this article we describe 4 cases of hollow visceral myopathy; our aim is to stress the importance of full thickness biopsy. METHODOLOGY: Four cases of hollow visceral myopathy are studied herein. All the patients had recurrent abdominal pain and constipation. The onset of symptoms was early in life or in the second to third decade. A diagnosis was established in all cases by full thickness intestinal biopsy obtained during laparotomy. Associated disorders were noted in 2 cases. One patient had Axenfelt syndrome, non-descended testicles and primary hypogonadism, and another had a diagnosis of Kleinfelter syndrome. RESULTS: All of the 4 cases were diagnosed to be suffering from hollow visceral myopathy by full thickness intestinal biopsy and 2 had additional disorders as well. CONCLUSIONS: patients with chronic intestinal pseudo-obstruction should be carefully evaluated as to whether there is an associated disorder and the diagnosis may be delayed unless full thickness intestinal biopsy is obtained.
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keywords = pain
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2/65. intestinal pseudo-obstruction and acute pandysautonomia associated with Epstein-Barr virus infection.

    We report the association of neurological and intestinal disorders with the reactivation of Epstein-Barr virus (EBV) in a child. This previously healthy 13-yr-old boy presented with pharyngitis and acute abdominal ileus. laparotomy excluded a mechanical obstruction. Postoperatively, he suffered from prolonged intestinal obstruction, pandysautonomia, and encephalomyelitis. Histological examination of the appendix and a rectal biopsy taken 3 months after the onset showed an absence of ganglion cells (appendix) and hypoganglionosis (rectum), with a mononucleate inflammatory infiltrate in close contact with the myenteric neural plexuses. EBV-PCR was positive in the blood and cerebrospinal fluid, and in situ hybridization with the Epstein-Barr virus encoded rna probe showed positive cells throughout the appendix wall including the myenteric area, in a mesenteric lymph node, and in the gastric biopsies. EBV spontaneous lymphocytic proliferation was noted in the blood. The serology for EBV showed previous infection but anti-early antigen antibodies were present. No immunodeficiency was found. Neurological and GI recovery occurred after 6 months of parenteral nutrition and bethanechol. The omnipresence of EBV associated with the neurointestinal symptoms suggest that the virus was the causal agent. This is the first documented case of acquired hypoganglionnosis due to EBV reactivation.
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ranking = 327.84967421125
keywords = plexus, area
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3/65. Ogilvie's syndrome after cesarean delivery.

    Ogilvie's syndrome is a rare postsurgical complication that can be associated with cesarean delivery. It is characterized by massive dilation of the colon, much like that which occurs with an obstruction but in the absence of a mechanical obstruction. Early detection and intervention are necessary to avoid serious morbidity and/or mortality. Conservative treatment is effective in many cases, but surgical intervention may be required. nursing assessment of the gastrointestinal system in the postsurgical patient is reviewed using a case report of a patient who developed Ogilvie's syndrome after a cesarean delivery.
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ranking = 0.23119341563786
keywords = area
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4/65. Primary hypertrophic colonopathy.

    We report a 45-year-old man and a 60-year-old woman who presented with features of intermittent intestinal obstruction. barium enema revealed narrowing at the pelvic-rectal junction in the man, and from the pelvic colon to the anal verge in the woman. histology of the resected sections showed marked hypertrophy of the muscularis propria in both cases, with normal mucosa, submucosa and myenteric plexus. Both patients are asymptomatic at 4 years' and 2 years' follow up. This entity of primary hypertrophic colonopathy may be a variant of primary visceral myopathy.
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ranking = 327.81114197531
keywords = plexus
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5/65. Gastrointestinal pseudo-obstruction: report of a patient with postoperative pseudo-obstruction.

    Postoperative pseudo-obstruction is a rare state of protracted gastrointestinal paresis that may progress to paralysis without the presence of obstructive lesions. Pseudo-obstruction is usually, but not exclusively, associated with an abdominal operative procedure (laparotomy), however, it may occasionally occur following extra-abdominal operations. As differentiated from the usual, 'physiologic'postoperative paresis, pseudo-obstruction persists for more than 7 days. The pathogenesis of postoperative pseudo-obstruction is complex and as yet partially unknown. Whereas the 'physiologic' postoperative gastrointestinal paresis includes short-term functional cholinergic depression of the visceral organs, in pseudo-obstruction focal lesions in the region of Auerbach's plexus, manifesting as visceral neuromyopathy, are involved. That is why the 'physiologic' postoperative paresis never transforms into paralytic ileus, while in pseudo-obstruction such a risk is potentially involved. The treatment for pseudo-obstruction is as a rule conservative. Surgical treatment (cecostomy) is rarely required. Colonoscopic decompresive suction is usually enough to eliminate the risk of colon rupture due to extensive distention by fast growing meteorism. A patient with postoperative pseudo-obstruction is presented.
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ranking = 327.81114197531
keywords = plexus
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6/65. Submucosal hypoganglionosis causing chronic idiopathic intestinal pseudo-obstruction.

    A 39-year-old woman presented with recurrent symptoms suggestive of intestinal obstruction. She was put on total parenteral nutrition (TPN) and consequently developed sepsis and endocarditis. TPN was stopped and a venting enterostomy was performed. Biopsies of mucosa and submucosa were taken at surgery; immunohistochemistry for neuronal proteins, protein gene product 9.5 (PGP 9.5) and the glial S-100-protein was done. Many enlarged nerve fiber strands were found in the submucosa. Few small ganglia containing a small number of nerve cells could be observed, suggesting hypoganglionosis. This patient with chronic idiopathic intestinal pseudoobstruction of neurogenic type had a defect in the submucous plexus, whereas visceral neuropathies are usually characterized by defects of the myenteric plexus with normal submucous plexus.
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ranking = 983.43342592593
keywords = plexus
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7/65. Abnormal distribution of the interstitial cells of cajal in an adult patient with pseudo-obstruction and megaduodenum.

    interstitial cells of cajal (ICC) are fundamental regulators of GI motility. Here, we report the manometrical abnormalities and abnormalities of ICC distribution and ultrastructure encountered in a 30-yr-old patient with megaduodenum and pseudo-obstruction. Full thickness biopsies taken during laparoscopic placement of a jejunostomy showed vacuolated myocytes and fibrosis predominantly in the outer third of the circular muscle layer of the duodenum, suggestive for visceral myopathy. The distribution of ICC was also strikingly abnormal: by light microscopy, ICC surrounding the myenteric plexus were lacking in the megaduodenum, whereas ICC were normally present in the duodenal circular muscle and in the jejunum. By electron microscopy, very few ICC were identified around the duodenal myenteric plexus. These findings suggest that abnormalities in ICC may contribute to the disturbed motility in some myopathic forms of intestinal pseudo-obstruction.
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ranking = 655.62228395062
keywords = plexus
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8/65. Chronic intestinal pseudoobstruction ileus in a patient with rheumatoid arthritis: outcome of a stoma construction and its histopathological features.

    We report the first case of a chronic intestinal pseudoobstruction (CIPO) in a patient with rheumatoid arthritis (RA) in whom a stoma was constructed at the sigmoid colon to decompress intracolonic pressure. The treatment improved quality of life in this patient, suggesting that surgical resection of the colon to decompress intracolonic pressure would be one of the options for the treatment of intractable CIPO. The resected specimen obtained from the sigmoid colon was found to be palisaded, with nuclei crowded in some areas of the inner circular muscle layer and thickened, with zonal or focal myxoid regions with interstitial fibrosis, composed of collagenous and elastic fibers, in the outer longitudinal muscle layer. Some of the smooth muscle cells in the outer longitudinal muscle layer showed mild degeneration with cytoplasmic vacuolar changes, but no inflammatory changes. However, further studies are required to determine whether these histopathological changes are unique to RA.
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ranking = 0.038532235939643
keywords = area
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9/65. Intestinal pseudoobstruction as a paraneoplastic syndrome in ganglioneuroblastoma.

    Intestinal pseudoobstruction may be part of a paraneoplastic syndrome. We report a teenage girl with ganglioneuroblastoma who presented with severe constipation. The intestinal pseudoobstruction was presumed to be due to inflammation of the myenteric plexus with destruction of the ganglion cells caused by antineuronal nuclear antibodies (ANNA or Anti-Hu).
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ranking = 327.81114197531
keywords = plexus
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10/65. Treatment of intestinal pseudo obstruction by segmental resection.

    intestinal pseudo-obstruction refractory to medical therapy is a debilitating problem for specialists dealing with gastrointestinal disorders. We report the case of a newborn who developed severe, recurrent symptoms of intestinal obstruction, due to visceral myopathy. The case was persistently intractable to medical management, leading to repeated laparotomies. Gastrointestinal lesions showed marked dilatation of the entire digestive tract, with enlarging to enormously distended segments at two areas. Resection of these segments improved bowel function, facilitating enteral nutrition. Long-term hyperalimentation and repetitive hospitalizations were also avoided with this procedure. These results suggest that segmental resections can save unnecessary intestinal resections in cases with extensive gastrointestinal involvement.
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ranking = 0.038532235939643
keywords = area
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