Hirschsprung's disease, aganglionic or hypoganglionic megacolon. Animal model: aganglionic megacolon in piebald and spotted mutant mouse strains.
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Treatment of toxic megacolon. A comparative review of 29 patients.
A review of 29 patients with toxic megacolon complicating ulcerative colitis was undertaken to (1) compare the results of medical and surgical treatment; (2) determine the optimal timing for surgical intervention, and (3) identify possible precipitating factors. Twenty-one patients were treated medically with nasogastric suction, steroids, parental fluids, blood transfusions, and antimicrobial agents. Of the 21 patients, 11 (53%) showed improvement by subjective and objective criteria and 10 (47%) failed to respond. Sixteen patients were treated surgically. This group was subdivided into 8 patients who failed to respond to medical treatment and 8 treated surgically. Total proctocolectomy with ileostomy was performed in 8 and subtotal colectomy and ileoproctostomy in 8, with subsequent proctectomy and ileostomy in 6 patient. Six of 8 patients (75%) treated primarily surgically improved, and 2 (25%) died. Seven of 8 patients (87.5%) treated surgically after failure of medical trial showed definite postoperative improvement, and 1 (12.5%) failed. Those who were operated on within the first 48-72 hr after the diagnosis of toxic megacolon was made responded uniformly well. Anticholinergics, opiates,, barium enema, and colonoscopy were identified as possible precipitating factors in 70% of cases. The results of this tudy in this patient population indicate that early surgical therapy in toxic megacolon is associated with better results than medical therapy (P less than 0.025). Although intensive, optimal medical therapy plays a significant role in the management of toxic megacolon, failure to induce rapid improvement within 48-72 hr constitutes an indication for definitive surgical treatment.
Adolescent↗
A manometric assessment of anorectal pressures and its significance in the diagnosis of Hirschsprung's disease and idiopathic megacolon.
Anorectal manometric studies were performed on nine patients with Hirschsprung's disease and ten patients with idiopathic megacolon for the purpose of differentiation, comparing with those of the normal subjects. The anorectal reflex was absent in all nine of the patients with Hirschsprung's disease while in all of ten patients with idiopathic megacolon rectal distension produced a relaxation in the anal canal like that of the normal subjects. Manometric studies proved to be a reliable and non-invasive technique for the diagnosis of Hirschsprung's disease and were of particular value as simple screening tests in patients with a clinical suggestion of this disease. The manometric studies of the patients with Hirschsprung's disease or idiopathic megacolon in the present series also demonstrated that the pronounced internal sphincter contraction combined with a total absence of reflex relaxation or an inadequate response of the sphincter to rectal distension might be responsible for the obstructive symptoms in these diseases.
Adolescent↗
Toxic idiopathic megacolon: fatal outcome in a mentally retarded adolescent.
The case of an 18-year-old mentally retarded boy who had long standing idiopathic megacolon and pica is presented. Acute toxic dilatation of the colon complicating pseudomembranous colitis developed, with a rapidly fatal outcome. No similar case of relatively benign idiopathic megacolon complicated by pseudomembranous colitis and toxic megacolon has been reported. The pathophysiology of this rare combination is discussed.
Adolescent↗
The treatment and postoperative complications of congenital megacolon: A 25 year followup.
In 1948 one of us (O.S.) proposed a new method of treatment, abdominoperineal resection, for patients with congential megacolon. Since then, 483 patients have been treated by 13 pediatric surgeons in Chicago and Boston using this technique. Two hundred and eight-two of the patients were last interviewed and examined more than 5 years after the resection. There were 16 postoperative deaths (3.3%) and 6 late deaths (1.2%) from enterocolitis. Both early and late complications were infrequent and are discussed in detail. Almost 90% of the patients reported that they now have normal bowel habits. None of the patients developed urinary incontinence or impotence, although ten patients (2.1%) reported permanent fecal soiling. This is the first large group of patients treated for congenital megacolon who have been followed to adulthood. The low incidence of postoperative complications and minimal frequency of long-term complications indicate that the abdominoperineal resection is a safe, effective method of treatment for congenital megacolon.
Adolescent↗
[Behcet's disease and toxic megacolon].
The gastrointestinal symptoms of Behçet's disease are ancillary manifestations of this disorder reflected principally in the form of diarrhea, abdominal pain, meteorism, nausea, and loss of appetite. If radiological changes can be detected they generally appear as dilatation of the small intestine or ulceration at different levels of the digestive tract. In our patient the intestinal symptoms started with dilation of the ileum and then toxic megacolon developed. At later follow-up examinations the radiological picture resembled Crohn's disease and ischemic colitis of the entire organ. It has been repeatedly, and wrongly, stated that there is an association between Behçet's disease and Crohn's disease or ulcerative colitis. Rather, it should be assumed that the intestinal manifestations of Behçet's disease correspond to those of Crohn's disease and ulcerative colitis without these diseases being actually present. The evolution towards toxic megacolon may be the consequence of a transmural infection across the colonic wall deriving from the mucosal ulcerations of colon and sigmoid, and proves that toxic megacolon is not a pecific complication of ulcerative colitis but may appear in the course of any acute inflammatory ulcerative lesion of the colonic wall.
Adrenal Cortex Hormones↗
[Adrenergic receptors in the pathogenesis of congenital megacolon (author's transl)].
Contractions of fresh specimens of taenial musculature from human colon were investigated under the influence of depolarization (bathing in solutions high in potassium), of acetylcholine, and of adrenaline; definite changes of contractile tension could be induced by these agents. The investigations were carried out using normal musculature as well as specimens from patients with congenital megacolon taken from the narrow segment. Contractions after depolarization and after cholinergic stimulation were equal in all specimens investigated, pre-extension of these specimens not differing significantly. Relaxation of colon musculature mediated by adrenergic receptors was very pronounced in normal musculature, but was lacking in specimens taken from the narrow segments of congenital megacolon. Sympathetic stimulation of normal taenial musculature does abolish completely cholinergically induced contractions. On the other hand, a preparation relaxed by adrenaline contracts very little after additional application of acetylcholine. These findings do suggest a severe disturbance of adrenergic receptors in the colon, or of adrenergic neuromuscular transmission in congenital megacolon.
Humans↗
Surgical management of toxic megacolon.
Toxic megacolon developed in ten of 220 patients (4.5%) admitted for chronic ulcerative colitis over the past 11 years. Nine of these patients came under the care of the Surgical Department. Only three of these 10 patients had previously been treated with steroids. Steroid therapy reversed the acute process in three patients (33%). All three patients later came to surgery. Toxic megacolon developed during the first episode of ulcerative colitis in seven of ten patients (70%). Three of the seven (43%) had perforated their colons prior to operation. Two patients died after a subtotal colectomy and one without operation. A delayed diagnosis was associated with sepsis in five patients (50%) and with all three deaths. Seven patients survived proctocolectomy. Prolonged medical management without dramatic response appeared to correlate with a high postoperative morbidity. This study supports the concept of aggressive diagnosis and early surgical intervention for toxic megacolon.
Acute Disease↗
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↗
[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].
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↗
[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↗
Toxic megacolon: results of emergency colectomy.
In order to assess the results of emergency colectomy for toxic megacolon in ulcerative colitis, the records of 40 patients treated over a 16-year period were reviewed. The diagnosis of toxic;colon was confirmed by a combination of clinical, radiologic, operative and pathologic examinations. Steroids were part of the treatment before operation in 36 patients. The surgical procedures included subtotal celectomy in 37 patients and total proctocolectomy in 3. Fecal spillage was recognized in 10 procedures. Two patients died postoperatively, but in neither had fecal contamination been observed. The findings of this review support the use of colectomy as the surgical treatment for toxic megacolon.
Adolescent↗
Toxic megacolon of ulcerative colitis in infancy.
A 12-week-old white male infant with ulcerative colitis and toxic megacolon is described. His diarrhea and rectal bleeding responded to prednisone. He subsequently developed toxic dilatation of the transverse colon while on salicylazosulfapyridine and one week after discontinuation of prednisone. His toxic megacolon disappeared during close medical observation, readministration of prednisone, and avoidance of repeated abdominal examinations. One year later sigmoidoscopy showed only friable mucosa and the barium enema showed the presence of ulcerative colitis but with improvement from the initial study. Currently he is taking 375 mg of salicylazosulfapyridine daily. Growth and development are normal and he is asymptomatic.
Colitis, Ulcerative↗
Current surgical approach to toxic megacolon.
Presented evidence illustrates that proctocolectomy performed as an emergency procedure is frequently less than adequate in salvaging patients with toxic megacolon, and a current mortality of 27 per cent is recorded. The mortality and morbidity of this disease are directly related to the delay in recognition and treatment of this complication and to iatrogenic perforation of the colon during colectomy, which results in fecal peritonitis. This is particularly evident when definitive operation is postponed for more than five days after the initial diagnosis of toxic megacolon. The rationale of current adherence to proctocolectomy for this complication is questioned, and a plea is made for the recognition of the life-saving potential for ileostomy-colostomy for selected patients and also for closer and earlier collaboration between surgeon and internist during the management of these patients.
Adult↗
Total proctocolectomy and ileostomy: procedure of choice for acute toxic megacolon.
Of 42 critically ill patients with toxic megacolon, 31 required emergency surgery, and 11 were treated without operation. Of the 11 patients treated by medical measures alone, there were two deaths (18%): one early, secondary to undiagnosed colonic perforation, and one late death from recurrent ulcerative colitis. Nine of ten survivors (90%) experienced continued morbidity, and five (50%) required subsequent elective surgery. Thirty-one patients required emergency operation for failure of medical treatment (19), colonic perforation (ten), and uncontrolled hemorrhage (two). Early and late morbidity was 74% (two-thirds occurring after subtotal colectomy and ileostomy). Surgical mortality was 19% (8% over the last ten years).
Acute Disease↗
The association of Waardenburg syndrome and Hirschsprung megacolon.
We describe four patients with Waardenburg syndrome and Hirschsprung aganglionic megacolon. In view of pathophysiologic relationships and animal studies, we conclude that the association of these two uncommon disorders is pathophysiologically significant.
Abnormalities, Multiple↗