Complications and results

The best results are obtained when a bile duct injury is discovered immediately and when a suitable tension-free repair is performed, which heals with minimal scarring. The worst results arise in patients who have undergone multiple previous repairs and who have evidence of liver failure and portal hypertension. Injuries in the porta hepatis have a worse prognosis than more proximal damage probably because they are technically more difficult to repair.

 

The operative mortality is at least 5 per cent and uncontrollable haemorrhage and renal failure are common causes of death, often associated with infection and an external biliary fistula. Many patients experience one or more major complications. Bile duct repairs are notorious for the formation of a recurrent stricture. In the past about one in three patients could expect further trouble; recently this has fallen to one in 10 patients. Despite the difficulties they should be offered a further attempt at operation: previous failure does not preclude a successful outcome.

 

Postinflammatory stricture

Narrowing of the bile duct is often seen at ERCP in association with chronic inflammation in or around the duct usually from bile duct stones and sometimes from chronic pancreatitis. In patients with stones the stricture tends to be in the retroduodenal portion of the duct, and the important point from the endoscopist's point of view is to be sure that the stone will come through the narrow area if it is engaged in a basket. Failure to realize this problem is the most common cause of a trapped basket during attempted endoscopic removal of a bile duct stone (Fig. 48) 1271. Significant and short inflammatory strictures of the duct appear to respond well to balloon dilatation, although if dilatation fails surgery is needed. Rarely, chronic pancreatitis may cause narrowing of the proximal bile duct. Jaundice developing during an acute exacerbation usually fades spontaneously. When there is evidence of long-term obstruction from severe fibrosis, an end-to-side choledochoduodenostomy is necessary.

 

Ampullary stenosis

The incidence of this condition is controversial. The main symptom that leads to investigation is episodic pain with features which strongly suggest a biliary origin. Most of these patients will have undergone previous cholecystectomy. There are no absolute diagnostic criteria, but the most useful are the combination of abnormal liver function tests, dilatation of the bile duct, delayed emptying of contrast, and difficulty in cannulating the ampulla at an ERCP performed by an experienced endoscopist. In specialist units manometric studies of ampullary function and special provocation tests may help to identify these patients and indicate those who will benefit from a sphincterotomy. The precise histological changes are uncertain, but in most cases there is excessive fibrosis and inflammation of both the mucosa and the muscle of the ampulla. If the diagnosis is well established before operation the results are good.

 

Sclerosing cholangitis

Sclerosing cholangitis is characterized by an obliterative inflammatory fibrosis of the biliary tract that leads to chronic liver disease. Sometimes the fibrosis is clearly secondary to stones in the bile duct or previous biliary surgery, but primary sclerosing cholangitis, in which these predisposing causes are absent, is a disease entity on its own. The appearance on cholangiography is diagnostic, although occasionally only time will exclude cholangiocarcinoma (Fig. 49) 1272. Primary sclerosing cholangitis was regarded as a rare disease but the advent of ERCP has resulted in greater recognition of the condition.

 

Aetiology

The cause of primary sclerosing cholangitis is unknown. The association with ulcerative colitis in two-thirds of patients suggests that chronic low-grade portal bacteraemia or the absorption of toxic bile acids from the diseased colon might be significant aetiological factors, but neither hypothesis has much experimental support. Recently, phenotyping studies have shown a much higher frequency of HLA-B8, DR3, DQ2, and DRw52A in patients with primary sclerosing cholangitis than in controls. These findings not only confirm the role of genetic factors but also suggest that the disease is immunologically mediated, as this phenotype is closely associated with a number of autoimmune diseases. Overall, current evidence suggests that primary sclerosing cholangitis is an immunologically mediated disease, perhaps triggered in genetically susceptible subjects by acquired toxic or infectious agents.

 

Diagnosis

Men are twice as commonly affected as are women and most patients present between the ages of 25 and 40. The usual symptoms are fatigue, intermittent jaundice, weight loss, upper abdominal pain, and pruritus. Attacks of acute cholangitis are surprisingly rare, unless there has been instrumental biliary intervention. Approximately half of all symptomatic patients have jaundice or hepatosplenomegaly. Many patients are discovered because of an abnormally high alkaline phosphatase on routine testing, usually during investigation of ulcerative colitis. Serum levels of bilirubin and alkaline phosphatase are usually elevated, the latter more than the former. These levels also fluctuate during the course of the disease. The cholangiogram is diagnostic, with typical beading from irregular stricturing and dilatation of both the intra- and extrahepatic ducts (Fig. 49) 1272. Occasionally only the intrahepatic ducts are involved; very rarely the disease affects only the extrahepatic system. Liver histology is not often diagnostic. The early features are periductal fibrosis, portal oedema, and bile ductular proliferation. Later fibrosis spreads into the liver parenchyma leading ultimately to biliary cirrhosis. Although primary sclerosing cholangitis and ulcerative colitis are closely linked the course of each disease is apparently independent. The colitis usually extends throughout the colon but causes few symptoms. Colectomy makes no difference to the course of the cholangitis.

 

Treatment

There is no curative treatment. Trials of corticosteroids, immunosuppressants, cholecystogogues, and antibiotics, either alone or in combination, have been universally disappointing. Management is directed towards minimizing symptoms and treating complications. Pruritus responds to cholestyramine; antibiotics are needed during episodes of cholangitis. Mechanical relief of a well-defined stricture is well worthwhile. In many patients the best approach is to place a stent across the obstruction either percutaneously or endoscopically. Balloon dilatation of the strictures may also be very effective.

 

Surgical treatment is controversial. Resection of extrahepatic strictures and reconstruction over Silastic stents produces good results in some series, but orthotopic liver transplantation is the only option available to young patients with primary sclerosing cholangitis and advanced liver disease. Recently, a 4-year survival rate of 70 per cent in 75 transplanted patients has been reported.

 

The average time between the onset of symptoms and death is about 7 years, and most patients die from hepatic failure. About one-quarter of patients with primary sclerosing cholangitis eventually develop a bile duct carcinoma, which frequently follows a very aggressive course.

 

Biliary fistula

Leakage of bile from the biliary tract can occur from the liver, the gallbladder, or the bile duct itself, and it may leak to the skin via the peritoneum or to the bowel. Some fistulae are created deliberately, such as a choledochoduodenostomy. Others develop from a pathological process, either from surgical complications, from ulceration of a stone, or from drainage of pus into an adjacent structure.

 

External biliary fistula

The most common external fistula develops following surgery. Even after a straightforward cholecystectomy there may be a little bile in the drain the following day. Larger volumes of bile occasionally drain, presumably because the tie on the cystic duct stump has slipped. Providing a stone has not been left in the bile duct and that there is no other cause of biliary obstruction the volume will decrease and the fistula will close spontaneously.

 

A t-tube in the common bile duct is technically a fistula. Normally a cholangiogram will be performed before the t-tube is removed to confirm that there is free flow into the duodenum; the fistula closes rapidly once the t-tube is removed. Any delay in closure implies some degree of obstruction, such as a residual stone, and an ERCP is necessary.

 

The late development of a fistula after an open cholecystectomy almost always signifies unrecognized damage to the bile duct and comes to light after the drainage of an abscess (Fig. 50) 1273. These patients are usually ill and septic. They need careful evaluation and investigation before any further surgical intervention. Biliary leaks from the cystic duct stump are a complication of laparascopic cholecystectomy. Placing a stent in the bile duct at ERCP normally stops these leaks at once.

 

Severe cholangitis occasionally leads to an intrahepatic abscess, which ruptures first into the perihepatic peritoneum. Biliary peritonitis rarely ensues because of surrounding adhesions, but when the abscess is drained externally a fistula results. Such a fistula will only close when the proximal obstruction that caused the cholangitis is removed or relieved. This may not be possible with a malignant obstruction.

 

Any significant bile loss externally is accompanied by rapid fluid and electrolyte depletion which must be vigorously replaced. If the patient will tolerate it bile can be returned to the bowel via a nasogastric tube.

 

Internal biliary fistula

Spontaneous internal fistulae are uncommon and are usually discovered at cholecystectomy when a communication between the gallbladder and the duodenum becomes apparent as Hartmann's pouch is dissected away from the bowel. This usually results when a stone has ulcerated into the duodenum and disappeared in the faeces. There are no specific symptoms to suggest that this has happened, except when a large stone escapes and impacts in the terminal ileum, giving rise to gallstone ileus. Rarely, the stone ulcerates into the stomach or the colon. In the latter instance patients have profuse diarrhoea as the bile is irritant to the colon.

 

The treatment is to remove the gallbladder and to close the hole in the bowel. It is very rarely necessary to resect the bowel, but it is wise to leave a drain in the wound.

 

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