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[The megacolon in myotonic dystrophy: case report and review of the literature].

Myotonic dystrophy (MD) is an autosomal dominant inherit disease, slowly progressive, involving multiple organ systems. Disorders at any level of the gastrointestinal tract are relatively common and manifest as disturbances in motility, such as impaired esophageal transport, delayed gastric emptying, and megacolon. A 51 years-old man was admitted to our surgical department with obstructive symptoms. Diagnostic evaluation showed megacolon and the typical clinical features of the MD, such as weakness, myotonia, frontal baldness and testicular atrophy. Risk of perforation and dehydration led to emergency total colectomy with ileorectal stapled anastomosis. The patient didn't suffer for compliance related to surgical treatment but, after 1 month in intensive care, died of pneumonia and myocardial infarct. The overall frequency of perioperative complications in patients with MD ranges from 8.2 to 42.9%. The risk of perioperative pulmonary complications is particularly high. Thus, we believe that the conservative treatment of motility disorders of the bowel in patients with MD is to be justified and that surgical treatment should be reserved, as last resort, performing a early diagnosis and careful monitoring during perioperative period.

Fatal Outcome↗

[Toxic megacolon: report of 2 clinical cases and review of the literature].

The authors present two cases of toxic megacolon. A proctocolectomy with ileostomy was performed in both cases. One of the two cases was detected late; the patient underwent surgery in desperate conditions and died on postoperative day one. The authors regard a prompt diagnosis as fundamental for the correct timing of the surgical approach in patients with toxic megacolon and severe acute colitis. Clinical and laboratory findings and, above all, plain films of the abdomen should be evaluated very carefully. The authors suggest total proctocolectomy with section-suture of the rectal stump at levator level and ileostomy, which makes it possible to avoid postoperative rectal haemorrhages and recurrences.

Female↗

[Toxic megacolon].

Reference to the literature dealing with idiopathic rectocolitis and its complications, with particular attention to megacolon, is followed by the presentation of two cases of ulcerous colitis complicated by toxic megacolon and a picture of acute abdomen. Emphasis is placed on the clinical features of such complications and the surgical treatment required in accordance with the degree of seriousness of the case.

Colitis, Ulcerative↗

[Incidence of symptomatic megacolon in patients with chagasic megaesophagus].

The authors analyze the incidence of megacolon associated to megaesophagus during a 7 year period. Of the 192 patients with megaesophagus only 15 who presented associated intestinal symptoms were further investigated through barium enema. No statistically significant differences were found between the group with megaesophagus and the group presenting association of megaesophagus and megacolon as to sex and age of the individuals and severity of megaesophagus. The esophageal symptoms preceded the intestinal symptoms in 93.3% of the cases. Many treatments were used, but recently good results were obtained by the association of Heller's cardiomyotomy with modified Lortat-Jacob's technique and left hemicolectomy.

Adult↗

[Surgical treatment of toxic megacolon complicating pseudomembranous colitis. Apropos of a case, review of the literature].

Toxic megacolon complicating pseudomembranous colitis has been rarely observed. Only 36 cases have been previously reported. We present herein a new case report in which pseudomembranous colitis was secondary to prophylactic antibiotherapy with pefloxacin for hip prosthesis. Despite specific oral treatment (against Clostridium difficile) by vancomycin, toxic megacolon required urgent subtotal colectomy with ileostomy and sigmoidostomy. Postoperative course was uneventful. Analysis of the reported cases demonstrates the high overall mortality of the series (32%); the procedure of choice seems to be subtotal colectomy, which removes the septic focus, with a 12% operative mortality rate.

Aged↗

Hirschsprung's disease and congenital megacolon.

Hirschsprung's disease and congenital megacolon are recognized as separate entities. They differ one from another in clinical, roentgenographic and histologic features. Conservative treatment of patients with Hirschsprung's disease is ineffective; surgical therapy with a new technique is beneficial. In congenital megacolon, operation is futile and may be harmful, whereas patients may be benefited by conservative measures, and improvement may occur spontaneously after adolescence.

Adolescent↗

Infliximab in the treatment of ulcerative colitis with toxic megacolon.

Toxic megacolon is a gastrointestinal emergency requiring prompt management to avoid fatal outcome. Although a majority of patients respond to conservative treatment, those not responding have been treated with intravenous cyclosporine or emergency surgery. Infliximab has been tried in patients with severe steroid-refractory ulcerative colitis. We report the successful use of this drug in the management of toxic megacolon in a 48-year-old woman not responding to the routine measures and who refused surgery.

Antibodies, Monoclonal↗

[Surgical treatment of idiopathic megacolon].

The authors discuss own experience in diagnosis and surgical treatment of 32 patients with idiopathic megacolon. Three surgical techniques were compared: 1) partial excision of the colon, 2) nearly complete excision of the colon with cecum-rectal anastomosis, and 3) total colectomy with ileorectal anastomosis. Basing on the authors' own experience and available literature the third approach is recommended as the most appropriate in the treatment of the persistent, chronic constipation accompanying idiopathic megacolon.

Adult↗

[Surgical treatment of toxic megacolon].

From 1978 to 1989, 11 patients suffering from toxic megacolon were treated. In eight patients it was based on ulcerative colitis, while Crohns' disease, infectious colitis and pseudomembranous colitis were seen once each. Patients were initially treated with intravenous fluids and corticosteroids. They were closely followed both clinically and radiologically. The plain abdominal X-ray was of great help. Conservative regimen was successful in 3 patients, the remaining had to be operated on in due course. Three patients proved to have a perforation of the bowel at the time of operation. The initial operations performed were one proctocolectomy, four times a subtotal colectomy, twice a partial colectomy and in one instance a diverting colostomy. Two patients had to undergo an additional colon resection. Postoperative complications consisted mainly of intra-abdominal abscesses. Two of the operated patients died. Follow-up is between 2 and 9 years. None of the patients had a recurrence of the toxic megacolon. Four patients had a recurrence of their underlying disease, which however could be resolved in most cases by medical means. In one patient total colectomy is being considered.

Adolescent↗

Fulminant colitis and toxic megacolon.

Twenty-seven cases of fulminant colitis are reported. All had at least five of the seven criteria of severity. Four developed toxic megacolon. Sixty-three per cent failed to respond to parenteral steroids, parenteral nutrition, and vigorous resuscitation and required surgery. Two of the patients with megacolon perforated and died. Prompt attention to these patients and a willingness to rely on surgery are important approaches. The experience reported here reiterates that previously published.

Adult↗

[Surgical treatment of toxic megacolon in ulcerative rectocolitis. Analysis of 16 patients].

The clinical aspects, surgical treatment and outcome of 16 consecutive patients treated for toxic megacolon secondary to ulcerative colitis at the 1st department of surgery at the university of Rome between 1976 and 1987 were reviewed. The surgical management consisted in total colectomy and immediate ileo-rectal anastomosis, without protective ileostomy in 14 patients and total colectomy with terminal ileostomy and ileorectal anastomosis in 2 patients, 5 months later. No postoperative mortality was observe the immediate complications were: anastomotic leakage, (one case) and rectal bleeding (three cases). The late complications were: ileo-anastomotic stump fistula and perforation (two cases). Protectomy, due to colitis recrudescence, in the rectal remnant 10 months after surgery. These results encourage the total colectomy with immediate ileorectal anastomosis for the treatment of toxic megacolon.

Adolescent↗

Toxic megacolon in cytomegalovirus colitis.

We report a case of toxic megacolon manifesting in cytomegalovirus (CMV) colitis in a 55-yr-old man with steroid-dependent chronic obstructive pulmonary disease. He presented to the hospital with increasing dyspnea and low-grade fever. His hospital course was characterized by the poor response of his symptoms to treatment, and by the subsequent development of intermittent hematochezia and, eventually, acute abdomen. The surgical specimen showed dilatation of the cecum and ascending colon with a solitary mucosal ulcer in the latter. The major histologic changes were limited to the area of ulceration. In addition to classical CMV inclusions. vasculitis manifested in two forms, namely, leukocytoclastic type and fibrinoid necrosis. The patient died shortly thereafter, due to multi-organ system failure. To our knowledge, this represents the first reported case of toxic megacolon due to CMV infection without underlying inflammatory bowel disease. The pathogenesis of toxic colonic dilatation remains unknown.

Colitis↗

Idiopathic megacolon presenting with fatal inferior vena caval obstruction and colonic perforation. Case report.

A 73-year-old woman with chronic constipation and associated megarectum and megacolon presented as an emergency with colonic perforation, inferior vena caval obstruction and urinary retention. Soon after admission the patient sustained a massive feculent vomit and died. Intractable constipation demands referral for further investigation, if similarly neglected cases are to be avoided. Idiopathic megacolon presenting in this way has not been previously reported.

Aged↗

[Unusual dilatation of the colon in idiopathic megacolon in adulthood].

This is a report on a 20-year old patient who had been suffering from chronic constipation from her childhood. After radiography of the colon in double contrast in December 1986 the patient was admitted to hospital under suspicion of a perforation of the colon. The radiographic demonstration of the abdomen showed an exceptional dilatation of some segments of the colon but without any signs of perforation by free intraperitoneal gas. Subsequent examination led to the diagnosis of an idiopathic megacolon whereas a congenital as well as symptomatic and toxic megacolon could be excluded.

Adult↗

Megacolon at high altitude.

Megacolon occurs frequently in high altitude areas. This report describes observations made in 60 cases seen in La Paz, Bolivia (3,600 meters above sea level). Motility of both the large and small bowel was found to be increased and the feces had a low pH. No histologic abnormalities were noted in the nervous plexus or smooth muscle. It is assumed that megacolon in these circumstances is acquired and not congenital.

Altitude↗

Primary myenteric plexus alterations as a cause of megacolon in Von Recklinghausen's disease.

This is a case report of a megacolon in a 26 years old male patient having typical von Recklinghausen's disease. Exploratory laparotomy shows no neurofibroma on digestive tract. Myenteric plexus study of colic resection specimen following B. Smith technique reveals marked alterations. These alterations are both quantitative (hyperhypoganglionosis) and qualitative (ganglion cells are irregular in shape and size, dendritic processes are hypertrophic, irregular; schwannosis; no inflammatory infiltration). The patient suffers no more transit disorders after adequate resection of this megacolon.

Adult↗

[Histochemical demonstration of acetylcholinesterase activity applied to the study of human biopsies. I. Rectal suction biopsy in the diagnosis of congenital megacolon].

The application of the histochemical demonstration of AChE activity to the diagnosis of congenital megacolon (Hirschsprung's disease) is discussed, both from the point of view of the technical aspects as well as the interpretation of the results obtained, using the method of Karnovsky and Roots, as modified by El Badawi-Schenk, applied to rectal suction biopsy. The authors adhere to the original ideas of Meier-Ruge et al., that the diagnosis of Hirschsprung's disease can be done solely with the evaluation of the AChE positive parasympathetic network of the lamina propria, with the additional consideration of the submucosal plexus to include cases of hypoganglionar megacolon, where that plexus is clearly diminished. The use of phase contrast is also emphasized.

Acetylcholinesterase↗