1/53. 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.- - - - - - - - - - ranking = 1keywords = myopathy (Clic here for more details about this article) |
2/53. The striking effect of hyperbaric oxygenation therapy in the management of chronic idiopathic intestinal pseudo-obstruction.Chronic idiopathic intestinal pseudo-obstruction is one of the disorders that is most refractory to medical and surgical treatment. Even when patients are given nutritional support, including total parenteral nutrition, obstructive symptoms seldom disappear. We report a case of chronic idiopathic intestinal pseudo-obstruction, due to myopathy, in which hyperbaric oxygenation therapy was strikingly effective. The presence of myopathy was histologically confirmed on the surgically resected jejunal specimen. hyperbaric oxygenation resulted not only in relief of the patient's obstructive symptoms but also in a rapid decrease of abnormally accumulated intestinal gas. At last, he could resume oral intake without any critical adverse effects. These observations strongly suggest that hyperbaric oxygenation can be an effective therapy in the management of chronic idiopathic intestinal pseudo-obstruction.- - - - - - - - - - ranking = 0.25keywords = myopathy (Clic here for more details about this article) |
3/53. 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.- - - - - - - - - - ranking = 0.125keywords = myopathy (Clic here for more details about this article) |
4/53. Chronic intestinal pseudo-obstruction in systemic lupus erythematosus due to intestinal smooth muscle myopathy.We report the case of a woman with systemic lupus erythematosus initially manifesting with fever, rash and arthritis, and two years later with Class IV lupus nephritis. Following treatment with cyclophosphamide she developed symptoms and signs of chronic intestinal pseudo-obstruction (CIPO) that was initially thought to be due to a neutropenic enterocolitis. However, persistence of symptoms resulted in segmental resection of the ileum which showed widespread myocyte necrosis and active inflammation within the muscularis propria. A subsequent, more extensive ileocolic resection showed severe diffuse atrophy and fibrosis of the muscularis propria throughout the resected bowel. The absence of mesenteric vasculitis and the clinical response of the CIPO to the immunosupressive regimen of prednisolone and cyclosporin A suggest that the bowel muscle coat changes reflect an intestinal myopathy secondary to systemic lupus erythematosus, and may have an auto-immune etiology.- - - - - - - - - - ranking = 0.625keywords = myopathy (Clic here for more details about this article) |
5/53. Application of the Malone antegrade continence enema principle in degenerative leiomyopathy.An 1-year-old boy with degenerative leiomyopathy (DL) presented with a volvulus of the transverse colon. After derotating the volvulus, we constructed a tube colostomy (TC) from the transverse colon. This TC has been used for the past 2 years by the patient for regular deflation of the colon and antegrade colonic enemas (ACE). We present this as a preliminary report of the use of the Malone ACE procedure in a patient with DL and review the relevant literature.- - - - - - - - - - ranking = 0.625keywords = myopathy (Clic here for more details about this article) |
6/53. 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.- - - - - - - - - - ranking = 0.125keywords = myopathy (Clic here for more details about this article) |
7/53. Familial visceral myopathy with carcinoma of unknown primary.We report an autopsy case of a 35-yr-old man with familial visceral myopathy, a cause of primary intestinal pseudo-obstruction. The patient died from complications of familial visceral myopathy, sepsis, and generalized signet-ring cell carcinomatosis. The patient had massive distension of the large and small intestines, a dilated thickened esophagus, and fibroneoplastic adhesions between intra-abdominal and thoracic structures. This case provides an observation, not previously described in cases of familial visceral myopathy, which is fibrosis and atrophy of the outer longitudinal smooth muscle of the small bowel, alternating to involve only the inner smooth muscle layer of the large bowel. This case shows how a malignant neoplasm can compound the pathology of familial visceral myopathy.- - - - - - - - - - ranking = 1keywords = myopathy (Clic here for more details about this article) |
8/53. 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.- - - - - - - - - - ranking = 0.125keywords = myopathy (Clic here for more details about this article) |
9/53. intestinal pseudo-obstruction and urinary retention: cardinal features of a mitochondrial dna-related disease.The syndrome of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) is a multisystemic disorder associated in most of the patients with an A to G transition at nucleotide position 3243 in the transfer rna (tRNA)Leu(UUR) (A3243G) of the mitochondrial dna. This syndrome is characterized by the preponderant involvement of skeletal muscle and central nervous system, but urinary or gastrointestinal symptoms are seldom documented. Here we report an unusual case of a 52-year-old woman with a clinical phenotype characterized by encephalopathy, left hemiparesis, urinary retention and gastrointestinal pseudo-obstruction. She had the classical A3243G mitochondrial dna point mutation of melas syndrome. We also present a clinically heterogeneous multigenerational pedigree with several affected members in the maternal lineage.- - - - - - - - - - ranking = 0.125keywords = myopathy (Clic here for more details about this article) |
10/53. 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.- - - - - - - - - - ranking = 0.125keywords = myopathy (Clic here for more details about this article) |
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