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Toxic megacolon caused by cytomegalovirus colitis in a multiply injured patient.

Cytomegalovirus (CMV) colitis is a rare event that has been described mainly in immunocompromised patients with immunosuppressive medication or HIV infection. An association with severe trauma has not been described previously. We report a formerly healthy, multiply injured 75-year-old male who subsequently developed what appeared to be pseudomembranous colitis. By the time the diagnosis of toxic megacolon on the basis of CMV colitis was established, he had succumbed to multiple organ failure. Whenever pseudomembranous colitis is clinically suspected but not confirmed in a critically ill formerly healthy patient, CMV colitis should be excluded. Once the diagnosis is confirmed, generous resection of all affected colon is mandatory in view of the limited benefit of antiviral therapy in CMV-induced toxic megacolon.

Aged↗

Idiopathic megacolon associated with oesophageal achalasia.

We report a rare case of achalasia coexistent with megacolon. The patient, a 25-year-old woman, presented at our hospital with a history of abdominal pain with distension, and was finally operated on for a megacolon. Five months later she presented symptoms of progressive dysphagia and heartburn. Oesophageal manometry of the upper and lower oesophageal sphincter and X-ray studies showed images compatible with achalasia. Oesophagomyotomy of the oesophagogastric junction (Heller procedure with Dor haemifundoplication technique) was performed. In the specimens taken for biopsy, neither pathology of the myenteric plexuses, nor atrophy of the muscle fibres was evident. Chagas' disease serological diagnosis for Trypanosoma cruzii, neurological disease, diabetes and all the pathological events related with neuromuscular disorders of the gastrointestinal tract proved negative. We believe that the pathological findings are related to a dysfunction of the physiological mediators of the upper and lower digestive tract motility. The present case is extraordinary and, to our knowledge, extremely rare. The association of the two pathological diseases is questionable, and the literature is reviewed.

Adult↗

Megacystis and megacolon in an infant with Ehlers-Danlos syndrome.

We report a 14 month old male infant with Ehlers-Danlos syndrome who became 'anuric' due to an acutely dilated urinary bladder. Although the patient was also found to have megacolon, no diverticulum was seen in his gastrointestinal tract or urinary bladder. In order to decompress the urinary bladder and colonic wall, we put an indwelling urinary catheter in place for 2 months, and carried out daily glycerin enema for 3 months. All urological and gastrointestinal symptoms subsided with this intensive medical treatment. The diagnosis of megacystis and megacolon was made very early in life for this patient. This may indicate that the striking extension of gastrointestinal and bladder wall may lead to the development of diverticula of gastrointestinal and urinary tracts in later life.

Barium Sulfate↗

Oesophageal manometric studies in patients with chronic Chagas disease and megacolon.

Intraluminal manometric studies performed on 12 patients with chronic Chagas disease and megacolon without clinical or radiological abnormalities of the oesophagus showed no alteration in oesophageal physiology under basal conditions when compared with a control group of 30 healthy subjects. Oesophageal peristalsis was unimpaired. Patients with chronic Chagas disease and megacolon differed from normals in their reaction to a small dose of a cholinergic drug. This agent induced an isolated but significant increase in resting pressure at the level of the oesophagogastric sphincter, accompanied in most cases by an increase in spontaneous activity but not by a change in resting pressure in the body of the oesophagus. This is interpreted as a subclinical expression of oesophageal pathology related to Chagas disease.

Adult↗

Lower oesophageal sphincter response to pentagastrin in chagasic patients with megaoesophagus and megacolon.

Intraluminal manometric studies were performed in 14 chagasic patients with megaoesophagus, 10 chagasic patients with megacolon, and 15 control subjects. Basal lower oesophageal sphincter pressure was 20.27+/-1.16 mmHg (mean+/-SEM) in controls as compared wtih 15.16+/-1.53 mmHg in chagasics with megaoesophagus and 14.38+/-1.50 mmHg in chagasics with megacolon. Dose-response studies to intravenous pentagastrin showed that the chagasic patients exhibited a lower sensitivity to the stimulant than did the controls, as demonstrated by shifting of the dose-response curve to the right and higher individual values of the dose for half maximal contraction (D50). No difference was noted between the calculated maximal contraction (Vmax) of oesophageal sphincter of controls and chagasics. These data are compatible with the hypothesis of an interaction between pentagastrin and cholinergic nervous excitation on oesophageal sphincteric smooth muscle.

Adolescent↗

Colectomy for idiopathic megarectum and megacolon.

The outcome in 40 patients who underwent colectomy for idiopathic megacolon and megarectum over an 18 year period was evaluated. All patients had a radiologically dilated bowel and a bowel frequency of less than two per week. Twenty two patients had a caecorectal anastomosis, 11 had an ileorectal anastomosis (including one with a previous caecorectal anastomosis and four with a previous sigmoid resection), and seven had a sigmoid resection. The mean (range) age at operation was 35 (17-69) years. All three operations resulted in a normal bowel frequency in more than 80% of patients but no patient with an ileorectal anastomosis experienced recurrent constipation. Thirty four patients experienced pain preoperatively and this was still present in 14 patients postoperatively. One patient died and four required subsequent laparotomy for bowel obstruction. The functional outcome in patients with dilatation of the whole colon and in those with dilatation of the left colon did not differ. Subsequent surgery for constipation was performed in three patients. Colectomy offers good results with few complications in the treatment of idiopathic megacolon, and an ileorectal anastomosis is the preferred operation.

Adolescent↗

Loss of interstitial cells and a fibromuscular layer on the luminal side of the colonic circular muscle presenting as megacolon in an adult patient.

BACKGROUND: Animal studies have shown that the neuromuscular structures on the luminal side of the colonic circular muscle coordinate circular muscle activity. These structures have been identified by electron microscopy in the normal human colon, but have never been thoroughly studied in patients with acquired intestinal hypoganglionosis. AIMS: To perform histological, immunocytochemical, and electron microscopic examinations of the colon of a patient with acquired intestinal hypoganglionosis presenting as megacolon. PATIENT: A 32 year old man with a one year history of constipation and abdominal distention, a massively dilated ascending and transverse colon, and a normal calibre rectum and descending and sigmoid colon. He had a high titre of circulating serum anti-neuronal nuclear antibodies. METHODS: Histology, immunocytochemistry (for neurofilaments, neurone specific enolase, synaptophysin, glial fibrillar acidic protein, S100 protein, and smooth muscle alpha-actin), and electron microscopic examinations on the resected colon. RESULTS: The number of ganglion cells and nerve trunks was decreased throughout the colon. Disruption of the neural network and a loss of interstitial cells of Cajal were observed on the luminal side of the circular muscle; in their place, the non-dilated colon contained a hypertrophic fibromuscular layer. CONCLUSIONS: Striking architectural alterations occurred at the site regarded as the source of the coordination of colonic circular muscle activity in an adult patient with acquired intestinal hypoganglionosis presenting as megacolon.

Adult↗

A newborn with ring chromosome 10, aganglionic megacolon, and renal hypoplasia.

A newborn infant is reported who had aganglionic megacolon, renal hypoplasia, severe growth retardation, generalised hypotonia, and various dysmorphic features. Chromosome analysis of lymphocytes and fibroblasts showed a ring chromosome 10 with breakpoints at p13-15 and q26. AluI digestion showed that the ring chromosome was monocentric. FISH with an alpha satellite probe specific for chromosome 10 showed one signal only in about 20% of interphase nuclei. It is suggested that aganglionic megacolon could result from dynamic somatic mosaicism owing to loss of the ring chromosome.

Chromosome Mapping↗

Toxic megacolon complicating chemotherapy for acute myeloid leukaemia.

A 43 year old woman in remission from acute myeloid leukaemia developed abdominal pain, severe melaena, diarrhoea and gram-negative septicaemia whilst severely pancytopenic following consolidation chemotherapy. Subsequently, serial abdominal X-rays showed a progressive toxic megacolon. Conservative management was attempted but, because of radiological evidence of increasing colonic dilatation and incipient perforation, an emergency defunctioning colostomy was performed. The patient recovered and 2 months later the caecostomy was reversed and a right hemicolectomy performed. This first described case of toxic megacolon following leukaemia treatment is compared with three previously described cases following cytotoxic chemotherapy for other conditions.

Adult↗

Ano-rectal profile in adult megacolon.

A number of parameters of ano-rectal function were measured in 10 patients with adult megacolon and the values compared to those in an age- and sex-matched group of normal volunteers. The absence of inhibition of force in the anal canal on rectal distention in patients with adult Hirschsprung's disease was the most constant finding although a group of patients were shown to have high resting and canal pressures resulting in megacolon. No constant sensory or myoelectrical abnormality was apparent within the anal canal. However, an unusual electrical rhythm was noted from the rectum in 1 patient with adult Hirschsprung's disease.

Adolescent↗

Megacolon in rats.

Four months of sigmoid colon intrinsic denervation of young male Wistar rats resulted in typical megacolon. Average 46.0% denervation of the Auerbach plexus was obtained by serosal application of benzalkonium chloride (0.2% v/v). Denervation was assessed by ganglion cell counts in a 480-nm extension of the treated segment. Increased thickness of the megacolon wall was due to muscle hypertrophy (+117.4%) and mucosal hyperplasia (+53.9%) as shown by morphometry. The potential usefulness of this experimental model is emphasized.

Animals↗

A novel pathogenesis of megacolon in Ncx/Hox11L.1 deficient mice.

The Ncx/Hox11L.1 gene, a member of the Hox11 homeobox gene family, is mainly expressed in neural crest-derived tissues. To elucidate the role of Ncx/Hox11L.1, the gene has been inactivated in embryonic stem cells by homologous recombination. The homozygous mutant mice were viable. These mice developed megacolon with enteric ganglia by age 3-5 wk. Histochemical analysis of the ganglia revealed that the enteric neurons hyperinnervated in the narrow segment of megacolon. Some of these neuronal cells degenerated and neuronal cell death occurred in later stages. We propose that Ncx/Hox11L.1 is required for maintenance of proper functions of the enteric nervous system. These mutant mice can be used to elucidate a novel pathogenesis for human neuronal intestinal dysplasia.

Animals↗

Recurrent severe gastrointestinal bleeding and malabsorption due to extensive habitual megacolon.

Dilatation of the colon and the rectum, which is not attributable to aganglionosis, is a rare finding and can be the result of intractable chronic constipation. We report a rare case of a 29-year-old male patient with impressive megacolon, in whom Hirschsprung's or Chagas disease was ruled out. In the present case, dilatation of the colon was most likely due to a behavioral disorder with habitual failure of defecation. Chronic stool retention led to a bizarre bulging of the large bowel with displacement of the other abdominal organs and severe occult blood loss. Because of two episodes of life-threatening gastrointestinal bleeding despite conventional treatment of constipation, a surgical approach for bowel restoration was necessary. Temporary loop ileostomy had to be performed for de-pressurization of the large bowel and the subsequent possibility for effective antegrade colonic lavage to remove impacted stools. Shortly after the operation, the patient was healthy and could easily manage the handling of the ileostomy. However, the course of the megacolon in this young adult cannot be predicted and the follow-up will have to reveal if regression of this extreme colonic distension with reestablishment of regular rectal perception will occur.

Adult↗

Chagasic megacolon associated with colon cancer.

Two cases of chagasic megacolon associated with colon cancer are reported. This is the first communication with complete clinical details of this association. Our two cases presented tumors (adenocarcinomas) in a nondilated segment of the transverse colon. The associated tumor lesions were diagnosed by a barium enema. Both patients had a typical clinical epidemiology and history of chagasic megacolon.

Adenocarcinoma↗

[Clinical and roentgenological aspects of toxic megacolon (author's transl)].

Ulcerative colitis may occur on several forms. Toxic megacolon is roentgenologically characterized by dilatation usually in the area of transverse colon and toxic symptoms. At the first appearance of these symptoms the patient should be treated or operated upon. Under normal circumstances the roentgenological diagnosis may be obtained only by a full erect or a left lateral decubitus film of the abdomen. A barium enema could lead to performation of the colon, peritonitis or provocation of toxic megacolon. In doubtful cases the examination should be performed with water-solubel contrast media (i. e. Gastrografin, Propyliodon etc.) The therapeutical internal and surgical possibilities are discussed.

Colitis, Ulcerative↗

[Toxic megacolon presenting as Crohn's disease].

We introduce a young patient, without history of inflammatory bowel disease (I.B.D.) who started with an acute gastroenteritis, which in the following days progressed to a toxic megacolon. The patient had come to hospital with nausea, vomiting, fever and liquid, explosive diarrhoea without pathologic products. There was no clinical remission with astringent diet, hydroelectrolitic reposition and antidiarrheic opiates. The patient was admitted in hospital when he had blood in the diarrhoea. This progressed to a toxic megacolon in three days and the patient had to be operated on urgently. The surgeons found perforations in the colon and the pathologists diagnosed Crohn disease. Even without previous E.B.D. history we reached the diagnosis from the clinical and analytical data and the plain abdominal radiology. It was impossible to confirm the diagnosis with a colonoscopy because of the high risk of perforation. In cases like this, early surgery may save the life of the patient.

Adult↗

Acquired segmental megacolon in an adult patient with cystic fibrosis.

Cystic fibrosis (CF) is characterized by symptoms related to pulmonary dysfunction and pancreatic insufficiency. Constipation, though a frequent complaint of patients with CF, receives less attention. We report a case of acquired segmental megacolon and constipation necessitating surgical colonic resection in an adult patient with CF. The differential diagnosis and possible causes of megacolon in this setting are discussed.

Adult↗

Toxic megacolon: a rare presentation of primary lymphoma of the colon.

Primary colonic lymphoma is rare and accounts for less than 1% of colon malignancies. Moreover, diffuse neoplastic invasion of the colon is exceptional. This case describes a patient with primary non-Hodgkin's lymphoma of the colon presenting as toxic megacolon. This unique presentation is the first case reported in the literature of a diffuse colonic lymphoma diagnosed in the setting of a toxic megacolon. Histological confirmation was performed on the piece of colectomy.

Colonic Neoplasms↗