1/33. 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.- - - - - - - - - - ranking = 1keywords = plexus (Clic here for more details about this article) |
2/33. 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 = 1keywords = plexus (Clic here for more details about this article) |
3/33. 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 = 1keywords = plexus (Clic here for more details about this article) |
4/33. 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.- - - - - - - - - - ranking = 3keywords = plexus (Clic here for more details about this article) |
5/33. 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 = 2keywords = plexus (Clic here for more details about this article) |
6/33. 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).- - - - - - - - - - ranking = 1keywords = plexus (Clic here for more details about this article) |
7/33. Mitochondrial myopathy (complex I deficiency) associated with chronic intestinal pseudo-obstruction.We report a patient presenting with severe muscular impairment and chronic intestinal pseudo-obstruction (CIP) at the age of eight months. Due to the aggravated symptoms, assisted ventilation, an ileostomy and total parenteral nutrition were required. Later on, the patient developed a locked-in syndrome (Leigh's subacute necrotising encephalomyelopathy) and finally died due to recurrent pneumonia and chronic renal failure. The assessment of muscle biopsies revealed a moderate single-fibre type II atrophy, a variation of muscle fibre calibre with focal fatty degeneration and a decreased reactivity of cytochrome-c oxidase. Although ragged red fibres had not been found, mitochondrial enzyme activities were markedly decreased with the lowest residual activity detected for NADH:Q1 oxidoreductase and NADH:O2 oxidoreductase (complex I deficiency), thereby confirming the diagnosis of mitochondrial myopathy. A molecular genetic analysis could not identify known mutations of mitochondrial dna. Gastrointestinal full-thickness biopsies revealed myenteric hypoganglionosis of the colon and stomach and hyperplasia of the submucosal plexus of the ileum. Some of the intestinal smooth muscle cells displayed bulbous protrusions filled with lateralised mitochondria. mitochondrial myopathies are known to be associated with a variety of clinical syndromes including CIP. However, in contrast to previous reports in which CIP has been attributed to visceral intestinal myopathies, the present case is characterised by neuronal intestinal malformations. Therefore, a mitochondrial myopathy associated with CIP requires a subtle assessment of both the intestinal smooth muscle and the enteric nervous system to identify the underlying pathology.- - - - - - - - - - ranking = 1keywords = plexus (Clic here for more details about this article) |
8/33. Autoimmune enteric leiomyositis: a rare cause of chronic intestinal pseudo-obstruction with specific morphological features.Autoimmune enteric leiomyositis is an extraordinary rare cause of acquired chronic intestinal pseudo-obstruction in children. We report a 5-year-old girl who developed chronic intestinal pseudo-obstruction 3 years after an autoimmune hepatitis. Mucosal biopsies of the upper gastrointestinal tract and colon showed minimal inflammatory changes. On full-thickness biopsies of the small intestine, a dense lymphocytic infiltrate of the muscularis propria was seen, mainly consisting of cytotoxic T lymphocytes. Smooth muscle fibers were degenerated and diminished, but the myenteric plexus was intact. The coexistence of an autoimmune hepatitis in our case indicates an expansion of autoreactive T cells to homologous self-antigens. It is of practical importance for histopathological diagnosis that inflammation in autoimmune enteric leiomyositis affects the muscularis propria of the small intestine, whereas mucosa and submucosa do not show severe inflammatory changes. Therefore, correct diagnosis may be missed in peroral and peranal mucosal biopsies, but full-thickness biopsies are required.- - - - - - - - - - ranking = 1keywords = plexus (Clic here for more details about this article) |
9/33. Familial visceral neuropathy: a defined entity?Familial visceral neuropathy (FVN) is a heterogeneous group of disorders due to abnormalities of the myenteric plexus. FVN with neuronal intranuclear inclusions is one particular form of FVN with a variable phenotype that includes achalasia, gastro-esophageal reflux, intestinal dysmotility and pseudo-obstruction, dysarthria, peripheral neuropathy and pupillary defects, and the presence of intranuclear inclusions within the neurons of the enteric nervous system. We present a four-generation family in which 10 individuals (7 of whom have been examined) are affected with FVN. The family was previously reported as familial esophageal achalasia, an autosomal recessive condition (MIM200400). At that time, several individuals in a single sibship were affected and there were no manifestations in either parent. Since that report, two individuals have had affected children and the mother has developed symptoms and has abnormalities on electromyography, thus enabling us to reclassify the family. This family provides further evidence of autosomal dominant inheritance, with marked variation in expression.- - - - - - - - - - ranking = 1keywords = plexus (Clic here for more details about this article) |
10/33. Paraneoplastic chronic intestinal pseudoobstruction as a rare complication of bronchial carcinoid.This report describes paraneoplastic visceral neuropathy including achalasia, gastroparesis, subileus and constipation in a 59 year old patient with metastasising atypical bronchial carcinoid. Achalasia was successfully treated by cardiomyotomy and fundoplication; additionally, extramucosal pylorectomy was undertaken to improve gastric emptying. Endoscopic papillotomy was necessary because of a functional stenosis of the sphincter of oddi with development of obstructive jaundice. Symptoms of intestinal pseudoobstruction did not improve with cisapride or corticosteroid treatment. Histological examination of gastrointestinal specimens revealed a lymphocytic infiltration of the myenteric plexus associated with loss of neurones. The rheumatoid factor was positive, there was evidence of circulating immune complexes and antibodies to Sm-antigen were present, suggesting a possible autoimmune pathogenesis.- - - - - - - - - - ranking = 1keywords = plexus (Clic here for more details about this article) |
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