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Idiopathic constipation: To cut is to cure?
SELECTED SUMMARIES
March 1989
and 8 mo after discharge. Of the 26 patients without cirrhosis, there was one death (hospital mortality, 3.9%). The mean serum bilirubin level in this latter group of patients was 9.9 mgldl preoperatively and decreased to 4.3 mg/dl 5 yr postoperatively. Two patients required liver transplantation 48 and 80 mo after biliary reconstruction, respectively, because of progressive liver disease. There have been five additional deaths from chronic liver failure. Silastic biliary stents are changed over guidewires as an outpatient procedure every 3 mo. An occasional patient has required changes every 2 mo. Episodes of fever have been unusual. Among the 26 patients without cirrhosis, mean survival was 46 mo. Actuarial l-, 3-, and 5-yr survival rates were 92%, 87%, and 71%, respectively. Comment.
The indications for surgery in patients with PSC are unclear. There are several possible reasons to avoid operation in patients with PSC. First, the natural history is unpredictable, and patients may remain asymptomatic for years. Second, prospective randomized trials are underway that may lead to beneficial medical treatments. Third, the operation as described by Cameron et al. introduces bacteria into the biliary tract, which may result in cholangitis and actually hasten the progression to secondary biliary sclerosis in these patients. Fourth, liver transplantation is now widely available and may significantly palliate patients with end-stage PSC. In my opinion, each of these four reasons not to operate on patients with PSC should be critically evaluated. Without question, asymptomatic patients with PSC should not be operated on. However, in most patients the disease progresses relentlessly with a dismal outcome. In a recently published prospective randomized trial by LaRusso et al. (Gastroenterology patients with PSC 1988;95:1036-42), >80% of placebo-treated experienced progressive symptoms, deterioration of hepatic laboratory values, and histologic progression on sequential liver biopsies. What about medical treatment of PSC? There are no published controlled trials that demonstrate any effect of medical treatment of PSC on survival. Penicillamine and steroids have no role in PSC. Similarly, anecdotal reports of good responses to methotrexate or azathioprine have not been confirmed in controlled trials. Until the results of ongoing prospective randomized trials are available, no medical treatment can be recommended. Operative intervention, including percutaneous transhepatic or endoscopic dilatation or stenting introduces bacteria into the biliary tree, potentially producing a syndrome of chronic cholangitis. Whether cholangitis hastens the progression to secondary biliary cirrhosis has never, to my knowledge, been proven. Nevertheless, after operation, most patients require frequent stent changes as indicated by fever or jaundice. What about hepatic transplantation for PSC? The University of Pittsburgh recently reported their results with 55 consecutive liver transplants for patients with PCS (Ann Surg 1988;207:21-5). Actuarial survival rates for 1 and 2 yr were 71% and 57%, respectively. The authors concluded that, “orthotopic liver transplantation has emerged as the sole form of therapy that offers patients who had failed treatment a chance of returning to normalcy at the cost of the least number of lives.” In my opinion, a treatment that results in death within 2 yr in 43% of patients should not be considered the “sole form of therapy.” Furthermore, several transplantation centers have shown that the operative mortality in patients with previous hepatobiliary surgery is not significantly increased. Therefore, a surgical treatment that might alter the natural history of PSC should not be withheld because of the availability of liver transplantation. The key question is whether the surgical procedure advocated by Cameron
et al. alters
the
natural
history
of PSC.
If it does
not
949
delay
progression to secondary biliary cirrhosis, surgery should be avoided in anticipation of liver transplantation. In the present series, the estimated 3-yr actuarial survival rate in patients without cirrhosis was 87%, not dissimilar from the control population recently reported by LaRusso et al. Although the mean serum bilirubin level fell from 9.9 to 4.0 mg/dl at 3 yr, 2 patients required and there have been five deaths from chronic liver failure. Thus, it is unclear whether survival or quality of life was improved by operation. When is this operation indicated? Patients are not candidates for operation if (a] they are asymptomatic; (b) they have significant intrahepatic stenoses; or (c) they have secondary biliary cirrhosis. At the Johns Hopkins Hospital, 31 of 100 patients seen during a 7-yr period were operated on. All were deeply jaundiced or had recurrent episodes of cholangitis. More importantly, all had a critical stenosis of hepatic duct bifurcation as originally described by Cameron and coworkers (Ann Surg 1984;200:54-60). In these select patients, I believe operation may delay the progression to secondary biliary cirrhosis. In my opinion, the procedure should be modified slightly so that the Roux-en-Y limb is attached to the anterior abdominal wall, making percutaneous radiologic access easier (Surgery 1983;94:324-30). The availability of repetitive percutaneous cholangiograms through the accessible (Hutson) loop allows frequent percutaneous balloon dilatation of recurrent strictures, thus avoiding external stents. Regardless, this paper from the Johns Hopkins Hospital is a landmark surgical series and is the gold standard to which all other surgical approaches to PSC must be compared. liver transplantation
K. C.
IDIOPATHIC TO CURE?
CONSTIPATION:
THIRLBY, M.D.
TO CUT IS
Kamm MA, Hawley PR, Lennard-Jones JE. [St. Marks Hospital, London, U.K. Outcome of colectomy for severe idiopathic constipation. Gut 1988:29:969-73 (July). This paper is a retrospective review of outcome in 44 women aged 18-74 yr treated with colectomy for severe at St. Mark’s Hospital from 1972 to 1987. constipation Patients with idiopathic megarectum or megacolon or Hirschspring’s disease were excluded. The median interval between spontaneous bowel movements was 4 wk, and most of these women also had abdominal pain (43 of 44), bloating (42 of 44), and straining to defecate (42 of 44) preoperatively. Therapy with laxatives and fiber was of no benefit. Many patients had had previous colon or rectal surgery (11 of 44) and 21 of 44 patients had a total of 41 noncolorectal abdominal surgeries including laparotomy for pain, hysterectomy, appendectomy, and ovarian cystectomy. Serious psychiatric disturbances were found in 6 of 44 patients preoperatively. Most of these patients had whole gut transit studied with radiopaque markers (abnormally long in 35 of 36) and the dynamics of defecation assessed with a balloon expulsion test [abnormal in 21 of 29) and puborectalis electromyography [abnormal in 13 of 20). Surgery consisted of colectomy with cecorectal or ileorectal anastomosis. Mean follow-up postoperatively was 38 mo. Bowel frequency returned to normal in 22 patients (50%). Diarrhea (>3 stools daily) occurred in 17 of 44 (39%) and was
950 SELECTED SUMMARIES
complicated by fecal incontinence in 6 (14%). Constipation (Cl stool every 3 days) persisted in 5 patients (11%). Abdominal pain, bloating, and straining continued in 71%, 45%, and 32%, respectively, of patients with these symptoms before surgery. Laxative use persisted in 3 of 20 patients (15%) followed up to 2 yr and in 9 of 20 patients (45%) followed for >2 yr. Further surgery was done in 17 of 44 patients including eight exploratory laparotomies to exclude bowel obstruction, six ileostomies for persistent constipation or diarrhea, and three puborectalis divisions [all unsuccessful) for continued difficulty with evacuation. Balloon expulsion tests or puborectalis electromyography were not predictive of success or failure. Psychiatric disturbances [depression, suicide, surreptitious laxative abuse) developed in 4 additional patients postoperatively. Comment. Refractory chronic constipation is often as frustrating for the treating physician as for the affected patient. In spite of the profusion of physiologic tests of colon motility and defecation mechanics, we still do not know the mechanism of constipation in most patients with this complaint. Attempts to classify patients with constipation mechanistically work well on paper, but flounder in clinical practice when multiple pathophysiologic problems are discovered. Therapies conceived to remedy specific suspected mechanisms of constipation remain poorly developed at present and are untested scientifically. Desperate people do desperate things and surely colectomy for constipation falls into this category. Although recent reports of small series of patients from the United States (Dig Dis Sci 1987; 32:1104-12 and 1249-54) have emphasized the potential benefit
GASTROENTEROLOGYVol. 96, No. 3
of such an approach in somewhat different patient groups, the paper by Kamm et al. clearly shows that the scalpel is a two-edged sword. The overall effect of colectomy was to increase stool frequency, but many of these patients remained symptomatic. No patient should have a colectomy for stubborn idiopathic constipation without knowing that they have only a 50% chance of having normal stool frequency and only a 30% chance of having their pain relieved. The authors rightly emphasize the presence of psychologic disturbances in these patients, but it is not clear what the physician can do about these problems. My personal opinion is that any patient being considered for colectomy for idiopathic constipation should be evaluated by a psychiatrist and that surgery should be delayed or cancelled in patients with clear-cut psychiatric disturbances. The role of physiologic testing in patients with constipation is unsettled by this paper. The three tests used in this report were of little help in these patients, but this may be an artifact of retrospective analysis. Whether these or other tests of colonic function and the mechanisms of continence are worthwhile in the evaluation of constipated patients remains moot, at least until better therapies are devised. For now I think that clinicians should be cautious in advising patients with idiopathic constipation to have their colons removed. It may be that we should consider ileostomy (without colectomy) more often in these patients when medical therapy has failed; at least then no bridges would be burned and new developments might allow restoration of more normal bowel function in the future. L. R. SCHILLER,M.D.
March 1989
and 8 mo after discharge. Of the 26 patients without cirrhosis, there was one death (hospital mortality, 3.9%). The mean serum bilirubin level in this latter group of patients was 9.9 mgldl preoperatively and decreased to 4.3 mg/dl 5 yr postoperatively. Two patients required liver transplantation 48 and 80 mo after biliary reconstruction, respectively, because of progressive liver disease. There have been five additional deaths from chronic liver failure. Silastic biliary stents are changed over guidewires as an outpatient procedure every 3 mo. An occasional patient has required changes every 2 mo. Episodes of fever have been unusual. Among the 26 patients without cirrhosis, mean survival was 46 mo. Actuarial l-, 3-, and 5-yr survival rates were 92%, 87%, and 71%, respectively. Comment.
The indications for surgery in patients with PSC are unclear. There are several possible reasons to avoid operation in patients with PSC. First, the natural history is unpredictable, and patients may remain asymptomatic for years. Second, prospective randomized trials are underway that may lead to beneficial medical treatments. Third, the operation as described by Cameron et al. introduces bacteria into the biliary tract, which may result in cholangitis and actually hasten the progression to secondary biliary sclerosis in these patients. Fourth, liver transplantation is now widely available and may significantly palliate patients with end-stage PSC. In my opinion, each of these four reasons not to operate on patients with PSC should be critically evaluated. Without question, asymptomatic patients with PSC should not be operated on. However, in most patients the disease progresses relentlessly with a dismal outcome. In a recently published prospective randomized trial by LaRusso et al. (Gastroenterology patients with PSC 1988;95:1036-42), >80% of placebo-treated experienced progressive symptoms, deterioration of hepatic laboratory values, and histologic progression on sequential liver biopsies. What about medical treatment of PSC? There are no published controlled trials that demonstrate any effect of medical treatment of PSC on survival. Penicillamine and steroids have no role in PSC. Similarly, anecdotal reports of good responses to methotrexate or azathioprine have not been confirmed in controlled trials. Until the results of ongoing prospective randomized trials are available, no medical treatment can be recommended. Operative intervention, including percutaneous transhepatic or endoscopic dilatation or stenting introduces bacteria into the biliary tree, potentially producing a syndrome of chronic cholangitis. Whether cholangitis hastens the progression to secondary biliary cirrhosis has never, to my knowledge, been proven. Nevertheless, after operation, most patients require frequent stent changes as indicated by fever or jaundice. What about hepatic transplantation for PSC? The University of Pittsburgh recently reported their results with 55 consecutive liver transplants for patients with PCS (Ann Surg 1988;207:21-5). Actuarial survival rates for 1 and 2 yr were 71% and 57%, respectively. The authors concluded that, “orthotopic liver transplantation has emerged as the sole form of therapy that offers patients who had failed treatment a chance of returning to normalcy at the cost of the least number of lives.” In my opinion, a treatment that results in death within 2 yr in 43% of patients should not be considered the “sole form of therapy.” Furthermore, several transplantation centers have shown that the operative mortality in patients with previous hepatobiliary surgery is not significantly increased. Therefore, a surgical treatment that might alter the natural history of PSC should not be withheld because of the availability of liver transplantation. The key question is whether the surgical procedure advocated by Cameron
et al. alters
the
natural
history
of PSC.
If it does
not
949
delay
progression to secondary biliary cirrhosis, surgery should be avoided in anticipation of liver transplantation. In the present series, the estimated 3-yr actuarial survival rate in patients without cirrhosis was 87%, not dissimilar from the control population recently reported by LaRusso et al. Although the mean serum bilirubin level fell from 9.9 to 4.0 mg/dl at 3 yr, 2 patients required and there have been five deaths from chronic liver failure. Thus, it is unclear whether survival or quality of life was improved by operation. When is this operation indicated? Patients are not candidates for operation if (a] they are asymptomatic; (b) they have significant intrahepatic stenoses; or (c) they have secondary biliary cirrhosis. At the Johns Hopkins Hospital, 31 of 100 patients seen during a 7-yr period were operated on. All were deeply jaundiced or had recurrent episodes of cholangitis. More importantly, all had a critical stenosis of hepatic duct bifurcation as originally described by Cameron and coworkers (Ann Surg 1984;200:54-60). In these select patients, I believe operation may delay the progression to secondary biliary cirrhosis. In my opinion, the procedure should be modified slightly so that the Roux-en-Y limb is attached to the anterior abdominal wall, making percutaneous radiologic access easier (Surgery 1983;94:324-30). The availability of repetitive percutaneous cholangiograms through the accessible (Hutson) loop allows frequent percutaneous balloon dilatation of recurrent strictures, thus avoiding external stents. Regardless, this paper from the Johns Hopkins Hospital is a landmark surgical series and is the gold standard to which all other surgical approaches to PSC must be compared. liver transplantation
K. C.
IDIOPATHIC TO CURE?
CONSTIPATION:
THIRLBY, M.D.
TO CUT IS
Kamm MA, Hawley PR, Lennard-Jones JE. [St. Marks Hospital, London, U.K. Outcome of colectomy for severe idiopathic constipation. Gut 1988:29:969-73 (July). This paper is a retrospective review of outcome in 44 women aged 18-74 yr treated with colectomy for severe at St. Mark’s Hospital from 1972 to 1987. constipation Patients with idiopathic megarectum or megacolon or Hirschspring’s disease were excluded. The median interval between spontaneous bowel movements was 4 wk, and most of these women also had abdominal pain (43 of 44), bloating (42 of 44), and straining to defecate (42 of 44) preoperatively. Therapy with laxatives and fiber was of no benefit. Many patients had had previous colon or rectal surgery (11 of 44) and 21 of 44 patients had a total of 41 noncolorectal abdominal surgeries including laparotomy for pain, hysterectomy, appendectomy, and ovarian cystectomy. Serious psychiatric disturbances were found in 6 of 44 patients preoperatively. Most of these patients had whole gut transit studied with radiopaque markers (abnormally long in 35 of 36) and the dynamics of defecation assessed with a balloon expulsion test [abnormal in 21 of 29) and puborectalis electromyography [abnormal in 13 of 20). Surgery consisted of colectomy with cecorectal or ileorectal anastomosis. Mean follow-up postoperatively was 38 mo. Bowel frequency returned to normal in 22 patients (50%). Diarrhea (>3 stools daily) occurred in 17 of 44 (39%) and was
950 SELECTED SUMMARIES
complicated by fecal incontinence in 6 (14%). Constipation (Cl stool every 3 days) persisted in 5 patients (11%). Abdominal pain, bloating, and straining continued in 71%, 45%, and 32%, respectively, of patients with these symptoms before surgery. Laxative use persisted in 3 of 20 patients (15%) followed up to 2 yr and in 9 of 20 patients (45%) followed for >2 yr. Further surgery was done in 17 of 44 patients including eight exploratory laparotomies to exclude bowel obstruction, six ileostomies for persistent constipation or diarrhea, and three puborectalis divisions [all unsuccessful) for continued difficulty with evacuation. Balloon expulsion tests or puborectalis electromyography were not predictive of success or failure. Psychiatric disturbances [depression, suicide, surreptitious laxative abuse) developed in 4 additional patients postoperatively. Comment. Refractory chronic constipation is often as frustrating for the treating physician as for the affected patient. In spite of the profusion of physiologic tests of colon motility and defecation mechanics, we still do not know the mechanism of constipation in most patients with this complaint. Attempts to classify patients with constipation mechanistically work well on paper, but flounder in clinical practice when multiple pathophysiologic problems are discovered. Therapies conceived to remedy specific suspected mechanisms of constipation remain poorly developed at present and are untested scientifically. Desperate people do desperate things and surely colectomy for constipation falls into this category. Although recent reports of small series of patients from the United States (Dig Dis Sci 1987; 32:1104-12 and 1249-54) have emphasized the potential benefit
GASTROENTEROLOGYVol. 96, No. 3
of such an approach in somewhat different patient groups, the paper by Kamm et al. clearly shows that the scalpel is a two-edged sword. The overall effect of colectomy was to increase stool frequency, but many of these patients remained symptomatic. No patient should have a colectomy for stubborn idiopathic constipation without knowing that they have only a 50% chance of having normal stool frequency and only a 30% chance of having their pain relieved. The authors rightly emphasize the presence of psychologic disturbances in these patients, but it is not clear what the physician can do about these problems. My personal opinion is that any patient being considered for colectomy for idiopathic constipation should be evaluated by a psychiatrist and that surgery should be delayed or cancelled in patients with clear-cut psychiatric disturbances. The role of physiologic testing in patients with constipation is unsettled by this paper. The three tests used in this report were of little help in these patients, but this may be an artifact of retrospective analysis. Whether these or other tests of colonic function and the mechanisms of continence are worthwhile in the evaluation of constipated patients remains moot, at least until better therapies are devised. For now I think that clinicians should be cautious in advising patients with idiopathic constipation to have their colons removed. It may be that we should consider ileostomy (without colectomy) more often in these patients when medical therapy has failed; at least then no bridges would be burned and new developments might allow restoration of more normal bowel function in the future. L. R. SCHILLER,M.D.