Symposium on Surgery at the Lahey Clinic
Changing Patterns of Surgery of the Gallbladder, Bile Ducts, and Liver
Dudley T. Moorhead, M.D.,* and Kenneth W. Warren, M.D.t
More than 15 million persons are estimated to have cholelithiasis and cholecystitis at the present time, and each year more than 500,000 cholecystectomies are performed. Gallstones form from the less soluble components of bile , namely, cholesterol, bile pigments, and calcium; 80 to 90 per cent are mixed and multiple (Fig. 1). Pigment calculi predominate in patients with hemolytic conditions because of increased levels of free bilirubin in the bile. Organisms, such as Ascaris and E. coli that possess beta-glucuronidase activity, also predispose to pigment calculi. Cholesterol calculi form when an imbalance occurs in the concentrations of cholesterol, lecithin, and bile salts, leading to supersaturation. A decrease in the size of the bile salt pool, caused by decreased resorption in the distal small bowel or by congenital factors, usually creates the imbalance. However, a supersaturated solution of cholesterol will not precipitate unless nucleation on bacteria, suture material, precipitated bile salts or pigments, or other foreign bodies has occurred or unless there has been stasis. Cholesterol calculi can begin to grow decades before they produce symptoms. Wenckert and Robinson 19 studied records of 781 patients in Malmo, Sweden, who had demonstrated either cholelithiasis or nonvisualization of the gallbladder on oral cholecyst~graphy but in whom elective cholecystectomy was not considered because recurrent or persistent jaundice or severe symptoms were not present. In the ll-year follow-up period, one half had mild or no symptoms, one third had severe symptoms, and 18 per cent had serious complications. The incidence of severe complications was twice as high for patients with nonvisualized gallbladders as it was for those with normal function despite cholelithiasis. The number of complications also increased with age.
*Resident, New England Deaconess Hospital-Harvard Surgical Service, Boston, Massachusetts tDepartment of Surgery, Lahey Clinic Foundation, Boston, Massachusetts
Surgical Clinics of North America-Vol. 56, No.3, June 1976
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Plrcent Bile Salt
,
Figure 1. Method for presenting three major components of bile (bile salts, lecithin, and cholesterol) on triangular coordinates. Each component expressed as percentage mole of total bile salt, lecithin, and cholesterol. Line ABC represents maximum solubility of cholesterol in varying mixtures of bile salt and lecithin. Point P represents bile composition containing 5% cholesterol, 15% lecithin, and 80% bile salt and falls within the zone of a single phase of micellar liquid. Bile having a composition falling above line would contain excess cholesterol in either supersaturated or precipitated form (crystals of liquid crystals). (Reproduced from Redinger, R. N., and Small, D. M.: Bile composition, bile salt metabolism and gallstones. Arch. Intern. Med., 130:620,1972. Used with permission.)
MEDICAL VERSUS SURGICAL THERAPY Dissolution of asymptomatic gallstones with chenodeoxycholic acid is efficacious in approximately 60 per cent of patients. However, the safety of this agent has not been thoroughly established, and recent reports indicate that treatment must be continued indefinitely or calculi will reform. Moreover, most studies have shown that medical therapy is not beneficial6 when the calculi are radiopaque, when the gallbladder does not function, when the bile ducts contain calculi, or when complications ensue. Because the mortality rate for complications of gallstones in patients more than 60 years of age is approximately 15 per cent and the mortality rate for routine cholecystectomy is less than 1 per cent,2 arguments against cholecystectomy for asymptomatic calculi are weak. We have long believed that cholecystectomy is the procedure of choice for asymptomatic cholelithiasis as well as for acute and chronic cholecystitis. Some surgeons still advocate cholecystostomy for acute cholecystitis in elderly patients. Malstrom and Olsson 1o recently reviewed results of 63 cholecystostomies performed at the University of Lund on patients with a mean age of 71 years. The mortality rate at initial operation was 20 per cent. Sixteen patients later required cholecystectomy, nine had one attack each of cholecystitis, four had continuous symptoms, and five died of gallbladder disease. The authors concluded that "cholecystostomy may be as dangerous and unsuitable for the patient as it is comfortable and easy for the surgeon."
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Some surgeons advocate prolonged medical treatment for acute cholecystitis and then cholecystectomy. Although approximately 80 per cent of these patients have remission after nasogastric aspiration, intravenous fluid replacement, and adequate .analgesia and antibiotics, those who do not respond are likely to fare poorly; the ultimate mortality rate is between 5 and 20 per cent when emergency cholecystectomy is performed. Braasch et aP reported 324 cases of acute cholecystitis treated by early operation at the Lahey Clinic with an operative mortality rate ofless than 1 per cent. The complication rate in this series was 13.4 per cent; three fourths of complications were postoperative atelectasis, pneumonitis, phlebitis, and minor wound infections. These results are certainly acceptable, because 8 per cent of patients had perforated gallbladders and 15 per cent had choledocholithiasis. These data support our preference for early cholecystectomy for acute cholecystitis; it shortens the period of disability and prevents further deterioration. However, cholecystostomy under local anesthesia may be life-saving in patients with severe pulmonary, cardiac, and renal disease.
SAFETY FACTORS IN CHOLECYSTECTOMY The abdomen is entered through a right paramedian rectusretracting incision extending from the xiphoid process to a point approximately 2 inches below the umbilicus. The greatest hazard in biliary tract surgery is associated with the anatomic relationships of the cystic duct and the cystic artery to the right lateral wall of the hepatic duct. The abdominal contents are explored carefully in the same order at each operation; normal and abnormal findings are recorded in the operative notes in detail. Proper exposure is the hallmark of a safe cholecystectomy (Figs. 2 and 3). The fundus of the gallbladder is clamped with a Pennington clamp,
Figure 2. The most frequent causes of bile duct injury. They can be prevented by taking the steps mentioned in the text.
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B
Figure 3. The safe way to control cystic artery bleeding.
and gentle traction is applied in an inferior direction. A Deaver retractor is placed so that its point impinges at the angle made by the junction of the distal common bile duct with the duodenum, and traction is applied downward and to the left. This places the common bile duct under enough tension to facilitate delineation of its course. A large right-angled retractor is placed along the right costal margin, and a moist abdominal pad is placed over the hepatic flexure. A Deaver retractor is placed at the top of the incision immediately to the left of the gallbladder. Next a right-angled clamp is applied to the ampulla in a manner that allows the operator to control direction and magnitude of tension placed on the cystic duct and artery. When exposure of the right upper quadrant has been obtained, the peritoneum covering the free edge of the gastrohepatic omentum is incised and the common bile duct is exposed. If the safety of the procedure is to be assured, no structure in this region should be ligated or divided until the cystic duct and its junction with the common bile duct, the cystic artery, and the right hepatic artery are identified with certainty. As the cystic duct is invariably longer than the cystic artery, division of the cystic artery between paired clamps is the logical next step. If the common bile duct cannot be identified at its junction with the cystic duct because of edema, induration, or scarring, it is usually safely and easily identifiable immediately superior to the duodenum. In this event, we prefer a choledochostomy in the supraduodenal area and insertion of a Bakes dilator of appropriate size to the presumed junction of the cystic and common hepatic ducts. If bleeding ensues, the bleeding point is never clamped under direct vision or blindly. The first maneuver is application of gentle pressure to the area if bleeding is venous or compression of the common hepatic artery ifbleeding is arterial. Accumulated blood is then aspirated, and the
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bleeding point is identified by gradually releasing pressure. Suture ligation is employed under direct vision with a fine atraumatic needle. The cystic duct is divided near its junction with the common bile duct and is ligated with silk sutures. The gallbladder is stripped from its bed, leaving margins of serosa of 1 cm. The gallbladder bed is sutured for hemostasis and is drained, usually through the superior pole of the incision.
CHOLEDOCHOLITHIASIS The basic indications for common bile duct exploration are palpable calculi in the duct, recent jaundice, recent pancreatitis, a dilated common bile duct, calculi in the gallbladder smaller than the caliber of the cystic duct, thickened bile sediment in the gallbladder or duct, thickened common bile duct, obvious bile duct lesion or fistula, and acalculous cholecystitis. However, criteria for exploration of the bile ducts need not be so rigid with presently available techniques of operative cholangiography. Kakos et aU recently reported results of 3,012 operative cholangiograms obtained between 1951 and 1970. Although the incidence of routine cholangiography increased from 2.9 per cent in the first five years to 93 per cent in the last five years of the study, the yield of common bile duct calculi recovered remained the same (13 per cent of cholecystectomies). The number of negative explorations, however, decreased from 72 to 38 per cent. The main contribution of operative cholangiography is not to prevent the surgeon from "missing" calculi but to allow more security in electing not to explore the duct when the diagnosis is in doubt. However, negative common bile duct exploration causes minimal increased risk and is preferred to cholangiography by many surgeons. Most retained calculi are overlooked at the time of choledocholithotomy. One series l2 reported 28 per cent retained calculi after positive bile duct exploration. Completion cholangiography detects retained calculi in 14 per cent of patients and fails to detect calculi in another 7 per cent. IS Proper technique of common bile duct exploration is essential if retained calculi are to be kept to a minimum.
TECHNIQUE OF CHOLEDOCHOSTOMY The common bile duct has already been identified during cholecystectomy. Exposure is obtained, and the loose areolar tissue and peritoneum are cleared away from the site of the proposed choledochostomy. The common bile duct is aspirated with a fine needle to-confirm its identity and for culture. The anterior wall is incised parallel to its longitudinal axis, and the assistant continually aspirates the bile as it flows from the opening. Traction sutures of fine silk are inserted carefully in the edges ofthe opening, which is then enlarged to permit adequate ductal exploration. The intrahepatic and common hepatic ducts are explored first. A silver scoop is introduced, and the proximal common and common hepa-
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tic ducts are cleared of calculi and debris to avoid pushing them into the intrahepatic ducts. The right and left hepatic ducts are identified early in the exploration. When the intrahepatic ductal system is greatly dilated, the duct to the quadrate lobe is also identified and explored. If calculi are found, gentle scooping is continued until no further calculi are recovered. The proximal ducts are irrigated with a bulb syringe forced tightly into the choledochostomy and injected into the liver under pressure. Frequently, more calculi are forced out of the intrahepatic ducts using this maneuver. Small Bakes dilators are used to probe the smaller branches of the main hepatic ducts. With experience, the operator can feel the small dimples and tell if they are plugged with calculi. Steady pressure will frequently break through these plugs, allowing more calculi and debris to be recovered. These three maneuvers with dilators, scoops, and irrigation are repeated until all calculi are removed. Only then is attention turned to the distal ductal system. After all calculi have been removed or it is demonstrated that no calculi are present, attention is directed toward the ampulla of Vater and the sphincter ofOddi. If the 3 mm Bakes dilator does not pass readily into the duodenum, the distal duct should be explored with a small scoop and with calculus forceps. If no calculus is obtained, the duodenum and head of the pancreas should be mobilized by the, Kocher maneuver. This straightens out the intrapancreatic portion of the distal common bile duct and facilitates identification of the papilla, which can be palpated more easily with the index finger posterior to the head of the pancreas. If the 3 mm probe still cannot be passed readily into the duodenum without undue pressure, a duodenotomy should be performed. Usually, however, the dilator will pass. Dilation should be incremental up to a 7 mm diameter or, for a small common bile duct, 1 to 2 mm smaller than the diameter of the common bile duct. When the surgeon is satisfied that the duct has been completely cleared of calculi and debris and that the sphincter of Oddi has been dilated to a normal caliber, a short-limb T tube is inserted through the choledochotomy, which is closed with interrupted sutures of fine catgut.
RETAINED CALCULI Even after the most careful bile duct exploration, calculi occasionally remain. We usually favor surgical removal soon after their discovery. However, nonsurgical extraction and chemical dissolution are promising alternatives. Cholic acid has been shown to render the bile more capable of dissolving cholesterol. Clinical disappearance of calculi occurs in two thirds of patients when cholic acid, 100 mM, is infused through the T tube for two to three weeks. Mazzariello l l has reported 220 patients with retained calculi treated by extraction under fluoroscopic control through the T-tube tract with successful results in 204 patients. The T-tube tract is dilated first; finetipped calculus forceps or Dormia baskets are then used to crush and to extract the calculi. Calculi up to 2 cm in diameter and calculi from
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virtually every location within the intrahepatic or extrahepatic ducts have been extracted in this manner.
BENIGN BILIARY TRACT STRICTURES Although the incidence of benign biliary tract strictures has decreased in recent years presumably because of a more widespread use of acceptable techniques during cholecystectomy, the tragic dimensions of the problem are unchanged. The morbidity and ultimate mortality rates are high, and the disease can devastate the patient's finances. Moreover, 97 per cent of the 958 biliary tract strictures repaired in a 25-year period at the Lahey Clinic were essentially iatrogenic in origin.l7 The strictures were usually located high in the ductal system; 10 per cent were in the region of the bifurcation or in the intrahepatic ducts, 40 per cent were in the common hepatic duct, 28 per cent were in the common hepatic and common bile ducts, and 23 per cent were in the common bile duct. Ironically, in 85 per cent of the patients referred here, the original surgeon had no knowledge of the biliary duct injury at the time it occurred. In 38 per cent of these patients, cause of injury was deduced retrospectively at the time of repair. Of these strictures, 34 per cent were believed to have arisen during blind clamping for brisk bleeding in the porta hepatis, 22 per cent were associated with a technically difficult cholecystectomy, 21 per cent were caused by a ligature around the bile duct at the time of repair, and 5 per cent were associated with a difficult gastrectomy. The consequences of biliary strictures included choledochitis in nearly 100 per cent of patients, internal and external biliary fistulas in 35 per cent of patients, and biliary cirrhosis in 35 per cent of patients. Anemia and hypoprothrombinemia were also seen frequently. In our experience, 64 per cent of patients had chills and fever, 48 per cent had abdominal pain, 43 per cent had pruritus, 38 per cent had hepatomegaly, 24 per cent had external biliary fistulas, and 19 per cent had portal hypertension. Less than 1 per cent of patients were asymptomatic. Long-standing strictures, especially those with external biliary fistulas, can cause extreme debility with septicemia, weight loss, anemia, coagulopathies, base deficit-producing acidosis, and electrolyte abnormalities. Treatment is exclusively surgical. The physician may be tempted to correct all these medical problems before operation. However, after correction of bleeding tendencies or electrolyte abnormalities, which would make operation hazardous, expeditious decompression of the dilated bile ducts to prevent progressive biliary cirrhosis and liver abscess is the wisest course of action. If an external biliary fistula exists, a fistulogram through a No.8 Foley catheter with a balloon inflated subcutaneously frequently produces excellent visualization ofthe upper ductal system. Frequently, the lower ducts can be studied through endoscopic retrograde catheterization or with a barium cholangiogram, if a duct to small bowel anastomosis has previously been constructed. More often than not, however, anatomic
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data, such as size of ducts, amount of scarring, and location of stricture, are not known until the tedious dissection has been completed at the time of repair. Since each unsuccessful repair poses a certain risk of morbidity and mortality and reduces the chance of success of subsequent repairs, the surgeon who undertakes this procedure must have a thorough mastery of the principles of stricture repair, be familiar with the variety of available anastomoses and intubation techniques, and be able to make· sound intraoperative judgments. Those lacking such qualifications are wise to refer patients to an institution specializing in this type of surgery.
Operative Procedure The outcome of stricture repair depends primarily on the anatomic location of the stricture; the caliber of the proximal duct; the degree of cholangitis, pericholangitis, and local abscess formation; the time interval between injury and repair; the degree of biliary cirrhosis present; and the experience of the surgeon involved in assessing, selecting, and executing the procedure of choice (Figs. 4-8). Before operation, fluid, electrolyte, and acid-base abnormalities are corrected; chloramphenicol is given to neutralize the prevailing bile or-
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COMMON BILE DUCT
Figure 4. End-to-end repair of recent common bile duct transection.
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A
B
Figure 5.
Heineke-Mikulicz repair of duct narrowing.
Figure 6. For most strictures, end-to-side hepaticojejunostomy is used. A, The posterior row is placed first. B, The modified T tube is placed first in the intrahepatic ducts and the solid limb is brought out through a jejunostomy. C, The distal arm is next placed into the jejunal lumen.
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For lesions at .the bifurcation, the septum may be incised to produce a single
?pening.
L.HEPDUCT R.HEP.
A
Figure 8.
For lesions of intrahepatic ducts, each duct must be treated separately.
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ganisms; and vitamin K is administered. The abdomen is best entered through a long, vertical right paramedian muscle-retracting incision extending from the xiphoid process to several inches below the umbilicus. The peritoneum is entered through the lower pole as this is the least likely place to encounter intra-abdominal adhesions. Adhesions to the parietal peritoneum are disconnected as the operator 'proceeds toward the free edge of the liver in the extreme right of the abdomen. Dissection is continued to expose the base and dome of the right lobe. The portal triad is approached from its right lateral and anterior surfaces. If a fistulous tract exists, a probe inserted in it at this time can greatly facilitate finding the site of stricture. The position of the hepatic artery can usually be determined by palpation. The structure believed to be the common bile duct should always be aspirated with a small needle before it is incised. If the stricture is high, the dissection on the anterior surface of the duct must be continued past the bifurcation. The position of the distal bile duct can be determined between the common duct node and the gastroduodenal artery after an extensive Kocher maneuver has been performed on the duodenum. After the site of stricture has been found, operative cholangiography is usually essential to determine the exact location and condition of the duct. The bile duct is opened, the intrahepatic ducts are explored, and the calculi and sludge that are usually present are removed. At this point, the surgeon must decide what type of repair to perform. End-to-end repair is rarely possible except when transection of the duct is recent. In longstanding strictures, the proximal duct is dilated and the distal duct is thin and atretic, producing a discrepancy in size and tension on the suture line that makes end-to-end anastomosis hazardous. A Heineke-Mikulicz type of revision is occasionally possible on a recently partially transected duct or for a stricture at an intact biliary intestinal anastomosis. The majority of patients with common bile duct or common hepatic duct strictures seen at the Lahey Clinic are treated with an antecolic, end-to-side hepaticojejunostomy over an internal splint. A loop of jejunum, approximately 12 inches from the ligament of Treitz, is brought to the severed end of the bile duct. A small enterotomy is created on the antimesenteric border. A posterior row of interrupted, simple chromic sutures is placed first and then tied, approximating the two structures. An internal splint is selected and inserted. For high strictures, a modified Y tube is usually used, whereas for strictures of the common bile duct a T tube is frequently employed. The size of the tube is chosen so that it lies loosely in the duct. The external limb never exits through the anastomosis; instead, it exits through the jejunum directly opposite the anastomosis for a high stricture or, occasionally, through the distal common bile duct just above the anastomosis for alow stricture. Next, the anterior row of sutures is placed and, if possible, reinforced with interrupted silk sutures between Glisson's capsule and the jejunal serosa. The afferent limb is defunctionalized above an enteroenterostomy with four interrupted silk sutures. The enteroenterostomy is placed 16 inches distal to the hepaticojejunostomy. The abdomen is copiously irrigated with saline, and Penrose and sump drains are placed near the anastomosis in case a bile fistula develops.
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Strictures at the bifurcation ofthe right and left hepatic ducts can be treated more easily if the septum is incised to produce a single opening. Strictures slightly above the bifurcation, where the left and right hepatic ducts are still extrahepatic, can be treated by anastomosing each duct separately to the jejunal loop. For still higher strictures, where the ducts are intrahepatic, drainage is frequently accomplished transhepatically by guiding a curved calculus forceps through the stricture into a small biliary radicle and by pushing the tip of the forceps through the dome of the liver. A drainage tube is then brought back through the stricture and into the distal duct. Between 1940 and 1967, 1,553 operations on biliary strictures were performed at the Lahey Clinic, 78 per cent of patients had an ultimately successful result, and 17 per cent had an unsuccessful result, frequently because of operative mortality. Many patients required more than one procedure to restore normal function; those patients in whom function had not returned after the third operation were practically never improved. Our more recent series! of 119 patients operated on between 1967 and 1970 reported 88 successful results in 148 operations. Only 2 per cent operative mortality was recorded, and 65 per cent of patients eventually had a successful result. Wexler and Smith20 recently reported 61 repairs using a sutureless technique combining the principle of the transhepatic tube with a mucosal graft. The procedure is performed by removing a seromuscular patch near the end of a Roux-en-Y loop of jejunum, thereby creating a mucosal outpouching. The transhepatic tube is drawn through the liver and anchored to the Roux-en-Y loop through the previously created mucosal diverticulum. The tube is pulled back into the liver, carrying with it the sleeve of jejunal mucosa into the ductal system so that it comes in contact with the epithelium of the intrahepatic ducts. This technique eliminates some of the tedious dissection and provides mucosa-to-mucosa approximation, avoiding sutures that might compromise the blood supply. Wexler and Smith reported an 85 per cent success rate with this technique.
CARCINOMA OF THE GALLBLADDER Carcinoma of the gallbladder is a highly lethal disease with a predilection for elderly women. A recent study of 1,629 cases from the California Tumor Registry8 showed the women to men ratio to be 1.9 to 1.0 and the median age at diagnosis to be 70 years. Workers in the automotive, rubber, and textile industries have a statistically greater than expected chance of contracting this disease. The overall five-year survival rate is 7 per cent. Results of treatment are poor, because tumors are frequently asymptomatic until late in their course. The tumor spreads into contiguous parts of the liver, common bile duct, and duodenum. Lymphatic spread carries tumor cells to the nodes of the cystic and common bile ducts, to periaortic nodes, mediastinal nodes, and, eventually, to the bloodstream. Hematogenous metastases are
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mostly to the lungs. Death is usually caused by fatigue, anorexia, dehydration, malnutrition, and pneumonia. In a recent series of 100 cases, Pemberton et al. 13 reported that no patient died from replacement or failure of a major organ resulting from the neoplasm itself. The only clue to early diagnosis obtainable without operation is a calcified gallbladder, which harbors the disease in 25 per cent of instances. Although some workers have been enthusiastic about wedge resection of the liver and skeletonization of the portal triad, simple cholecystectomy has provided the highest survival rate.
CARCINOMA OF THE BILE DUCTS Although cancer of the bile duct is relatively uncommon, we have had considerable experience with it at the Lahey Clinic. Of 103 patients with supraduodenal carcinoma of the bile ducts treated in a recent 15-year period, 93 presented with jaundice, 40 with pain, and 29 with pruritus. It is most often seen in patients in their fifties and is associated with ulcerative colitis and the presence or history of gallstones. At operation, 70 per cent of cancers were found to be at the bifurcation or above, 22 per cent were in the central bile ducts, and 8 per cent were in the distal common bile duct. Eight per cent of patients had disease throughout the extrahepatic ductal system. 14 The true nature of this condition is frequently missed at the first operation, especially if biopsy is not performed. Because of the dense fibroblastic reaction around the tumor, the pathologist often confuses it with fibrosis or sclerosing cholangitis. Results of treatment are disappointing. Of 101 patients in our follow-up study, 21 died in the immediate postoperative period (the majority from hepatocellular failure), and the median length of survival was only 13 months. Nine patients lived four or more years. All of the 12 patients who remained asymptomatic for five years had had resection. Resection offers the only chance of cure. Occasionally, hepatic lobectomy is possible for carcinoma of one duct or for carcinoma at the bifurcation. However, most resectable tumors are located ih the periampullary area where they are amenable to pancreatoduodenectomy. In these instances, tissue can frequently be obtained for a positive disgnosis by vigorous scraping of the inside ofthe distal duct with a bone curet inserted through the choledochotomy. Reanastomosis after the Whipple procedure is made difficult by the normally thin-walled pancreatic duct. Because of the proximity of the portal vein and hepatic artery and because of the predilection of the tumor to infiltrate widely submucosally, curative resection is not possible for cancers in most other locations. In 19 of 103 patients, bypass was possible and provided the best palliation. The procedure of choice is hepaticojejunostomy performed just below the bifurcation. In 52 of our patients, the tumor extended so high that neither resection nor bypass was possible. Instead, the neoplastic stricture was dilated and patency was maintained with a buried Y tube, modified Y tube,
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transhepatic tube, or T tube (Fig. 9). Whatever procedure is performed, stricture often recurs as the disease progresses. Reoperation is frequently necessary to provide further palliation. Some of our patients underwent as many as six palliative procedures in a nine-year period before they eventually died of disease. Eleven patients had such extensive disease that neither resection, bypass, nor dilatation was possible. The best procedure for these patients is biopsy only. The prognosis is poor.
SCLEROSING CHOLANGITIS Sclerosing cholangitis is an uncommon reaction of the biliary duct precipitated by unknown stimuli and characterized by an intense submucosal fibrosis causing progressive encroachment on the lumen. This may be seen alone or in association with previous biliary tract surgery, gallstones, retroperitoneal fibrosis, Riedel's struma, reticulum cell sarcoma, familial immunologic deficiency, Crohn's disease, or ulcerative colitis. A recent review15 of 84 patients treated at the Lahey Clinic for this condition revealed that the process involved the entire biliary tract in 45, the bifurcation in 22, the extrahepatic ducts in 5, the distal common bile
THROUGH ABDOMINAL· WALL
Figure 9. Transhepatic tubes are placed in the bile duct to stent the anastomosis.
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duct in 5, and the intrahepatic ducts in 3. Of the patients presenting with sclerosing cholangitis, 93 per cent had intermittent obstructive jaundice, . 66 per cent had pain, 50 per cent had chills and fever, 63 per cent had weight loss, 59 per cent had anorexia and malaise, and 45 per cent had nausea and vomiting. Preoperative endoscopic retrograde choledochography showed a narrowed biliary tract with attenuation of the peripheral branches in the liver and a beaded appearance. At operation, the duct may be reduced to a fibrous cord. Despite previous enthusiasm for treating this condition with steroids, we now explore the biliary tract in almost every instance. The common bile duct is identified, and operative choledochography is performed. Choledochostomy and choledocholithotomy are performed next if calculi are present (which they were in 33 of our 84 patients). Mucosal scrapings of the duct wall are taken and submitted for frozen section examination to exclude carcinoma. The ducts are dilated with care, irrigated, and intubated with a loosely fitting T tube or with a modified Y tube. A bypass procedure is performed primarily for distal ductal strictures. Only 13 per cent of our patients have had continued improvement after operation. An additional 25 per cent of patients were stabilized but with sporadic mild choledochitis. However, more than 50 per cent of patients progress to biliary cirrhosis, portal hypertension, and liver failure. The mean length of survival has been six years after the onset of symptoms despite surgical treatment. The operative mortality rate was nearly 8 per cent.
HEPATOMA Although primary carcinoma of the liver is still comparatively rare in the United States, deaths from this disease have increased from 2,500 in 1964 to 7,500 in 1973. Its incidence is up to 500 times greater than this in Mozambique where it is believed to be largely environmentally induced. 5 The tumor may be solitary, nodular, or diffuse. Usually, the first symptom is vague right hypochondrial or epigastric pain. One third of patients present with spontaneous rupture of the liver and intraperitoneal hemorrhage. Surgical resection of hepatoma has produced few cures. In a large series of 271 patients, Lin 9 found that 82 hepatomas were resectable, but only 8 patients survived five years. The mortality rate within 30 days of operation was 12 per cent. 9 These poor results are consistent with the advanced state at which hepatoma is first clinically apparent. Systemic chemotherapy of hepatomas with nitrogen mustard and prednisolone has increased the mean length of survival from 4.6 to 7.4 months from the onset of symptoms. Regional infusion chemotherapy with intra-arterial 5-fluorodeoxyuridine in 12 patients seen at the Lahey Clinic in a recent five-year period produced a 14.5 month median length of survival from the onset of treatment. All infusion catheters were placed intraoperatively after exploration revealed metastatic or bilobar involvement precluding curative hepatic lobectomy. The patients were then given prolonged ambulatory infusion using a portable chronometric pump.4
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BENIGN CYSTIC DISEASES OF THE LIVER AND BILE DUCTS Choledochal cysts arise from defective embryologic development and are frequently accompanied by other abnormalities (Fig. 10), Cysts contain from 100 ml to several liters of fluid and are usually localized to the common bile duct. They present in children or in young adults as a right upper quadrant mass frequently accompanied by jaundice. Surgical treatment can be difficult because the cyst may actually be a tremendously dilated common bile duct; communications with accessory bile ducts are a frequent occurrence; and the connection between the distal bile duct and the duodenum can rarely be found. We have found side-to-side choledochojejunostomy to be effective treatment. Solitary nonparasitic cysts of the liver are believed to arise from congenitally faulty fusion of the ductal elements or of the accessory bile ducts. However, cystadenomas and cystic carcinomas of the liver have also been reported. Benign cysts usually arise from the inferior surface of the liver and contain up to four gallons offluid. If they communicate with functioning liver cells the fluid will be thick and bile stained; ifnot, it may be colorless. Although we formerly recommended complete extirpation by
Figure 10. Various types of choledochal cysts.
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enudeation or partial hepatectomy,1S we recently have begun to use unroofing and marsupialization extensively, unless the cysts contain bile.
HEPATIC METASTASES Approximately 20, 000 persons in the United States die each year from colonic carcinoma with hepatic metastases. Although patients are occasionally cured by hepatic resection, the majority have bilobar involvement. Systemic chemotherapy has produced unimpressive results with response rates of only 20 per cent, and the duration of remission, when achieved, is brief. Hepatic artery ligation has been tried with equivocal results. Fifty-five patients at the Lahey Clinic underwent regional arterial infusion chemotherapy of 5-fluorodeoxyuridine. They represented 80 per cent of patients referred to us for evaluation of hepatic metastases. 'Those not treated had either distant metastases, massive ascites, portal hypertension, or lack of symptoms. Of the 51 patients surviving the immediate postoperative period, clinical response was noted in 71 per cent. Nonresponders had a median length of survival of 5 months, and responders survived for a median of 16 months. These rates are approximately double those reported for systemic chemotherapy in patients with similarly staged disease. Factors that predicted response to infusion were young age, absence of ascites, presence of jaundice, and minimal replacement of the liver.3 These results are encouraging and we believe that such chemotherapy has a place in the therapeutic armamentarium.
REFERENCES 1. Braasch, J. W., Warren, K. W., Blevins, P. K.: Progress in biliary stricture repair. Am. J. Surg., 129:34-37 (Jan.) 1975. 2. Braasch, J. W., Wheeler, W. M., Colcock, B. P.: Acute cholecystitis. Surg. Clin. NorthAm., 44:707-716 (June) 1964. 3. Cady, B., Oberfield, R. A.: Regional infusion chemotherapy of hepatic metastases from carcinoma of the colon. Am. J. Surg., 127:220-227 (Feb.) 1974. 4. Cady, B., Oberfield, R A.: Arterial infusion chemotherapy of hepatoma. Surg. Gynecol. Obstet., 138:381-384 (March) 1974. 5. Higginson, J., Svoboda, D. J.: Primary carcinoma of the liver as a pathologist's problem. Pathol. Annu., 5:61-89, 1970. 6. Iser, J. H., Dowling, R. N., Mok, H. Y. 1., et al.: Chenodeoxycholic acid treatment of gallstones. A follow-up report and analysis offactors influencing response to therapy. N. Engl. J. Med., 293:378-383 (Aug. 21) 1975. 7. Kakos, G. S., Tompkins, R K., Turnipseed, W., et al.: Operative cholangiography during routine cholecystectomy: a review of3,012 cases. Arch. Surg., 104:484-488(April) 1972. 8. Krain, L. S.: Gallbladder and extrahepatic bile duct carcinoma. Analysis of 1,808 cases. Geriatrics, 27:111-117 (Nov.) 1972. 9. Lin, T.-Y.: Primary cancer of the liver. Scan. J. Gastroenterol., 5 (Suppl. 6):223-241, 1970. 10. Malstrom, P., Olsson, A. M.: Cholecystostomy for acute cholecystitis. Am. J. Surg., 126:397-402 (Sept.) 1973. 11. Mazzariello, R: Review of 220 cases of residual biliary tact calculi treated without reoperation: an eight-year study. Surgery, 73 :299-306 (Feb.) 1973. 12. Paine, J. R, Firrne, C. N.: The problem of the overlooked common duct stone. Am. Surgeon, 20:1171-1179 (Nov.) 1954. 13. Pemberton, L. B., Diffenbaugh, W. F., Strohl, E. L.: The surgical significance of carcinoma of the gallbladder. Am. J. SUrg., 122:381-383 (Sept.) 1971.
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14. Ross, A. P., Braasch, J. W., Warren, K. W.: Carcinoma of the proximal bile ducts. Surg. Gynecol. Obstet., 136:923-928 (June) 1973. 15. Warren, K. W., Tan, E. G. C.: Diseases of the gallbladder and bile ducts. In Schiff, L. (ed.): Diseases of the Liver, 4th ed. Philadelphia, J. B. Lippincott Co., 1976, pp. 1278-1335. 16. Warren, K. W., Kune, G. A., Hardy, K. J.: Biliary duct cysts. Surg. Clin. North Am., 48:567-577 (June) 1968. 17 . Warren, K. W., Mountain, J. C., Midell, A.!,: Management of strictures of the biliary tract. Surg. Clin. North Am., 51 :711-731 (June) 1971. 18. Way, L. W., Admirand, W. H., Dunphy, J. E.: Management of choledocholithiasis. Ann. Surg., 176:347-359 (Sept.) 1972. 19. Wenckert, A., Robertson, B.: The natural course of gallstone disease: eleven-year review of 781 nonoperated cases. Gastroenterology, 50:376-381 (March) 1966. 20. Wexler, M., Smith, R: Jejunal mucosal graft: a sutureless technic for repair of high bile duct strictures. Am. J. Surg., 129 (2):204-211 (Feb.) 1975. Lahey Clinic Foundation 605 Commonwealth Avenue Boston, Massachusetts 02215
Changing Patterns of Surgery of the Gallbladder, Bile Ducts, and Liver
Dudley T. Moorhead, M.D.,* and Kenneth W. Warren, M.D.t
More than 15 million persons are estimated to have cholelithiasis and cholecystitis at the present time, and each year more than 500,000 cholecystectomies are performed. Gallstones form from the less soluble components of bile , namely, cholesterol, bile pigments, and calcium; 80 to 90 per cent are mixed and multiple (Fig. 1). Pigment calculi predominate in patients with hemolytic conditions because of increased levels of free bilirubin in the bile. Organisms, such as Ascaris and E. coli that possess beta-glucuronidase activity, also predispose to pigment calculi. Cholesterol calculi form when an imbalance occurs in the concentrations of cholesterol, lecithin, and bile salts, leading to supersaturation. A decrease in the size of the bile salt pool, caused by decreased resorption in the distal small bowel or by congenital factors, usually creates the imbalance. However, a supersaturated solution of cholesterol will not precipitate unless nucleation on bacteria, suture material, precipitated bile salts or pigments, or other foreign bodies has occurred or unless there has been stasis. Cholesterol calculi can begin to grow decades before they produce symptoms. Wenckert and Robinson 19 studied records of 781 patients in Malmo, Sweden, who had demonstrated either cholelithiasis or nonvisualization of the gallbladder on oral cholecyst~graphy but in whom elective cholecystectomy was not considered because recurrent or persistent jaundice or severe symptoms were not present. In the ll-year follow-up period, one half had mild or no symptoms, one third had severe symptoms, and 18 per cent had serious complications. The incidence of severe complications was twice as high for patients with nonvisualized gallbladders as it was for those with normal function despite cholelithiasis. The number of complications also increased with age.
*Resident, New England Deaconess Hospital-Harvard Surgical Service, Boston, Massachusetts tDepartment of Surgery, Lahey Clinic Foundation, Boston, Massachusetts
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Plrcent Bile Salt
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Figure 1. Method for presenting three major components of bile (bile salts, lecithin, and cholesterol) on triangular coordinates. Each component expressed as percentage mole of total bile salt, lecithin, and cholesterol. Line ABC represents maximum solubility of cholesterol in varying mixtures of bile salt and lecithin. Point P represents bile composition containing 5% cholesterol, 15% lecithin, and 80% bile salt and falls within the zone of a single phase of micellar liquid. Bile having a composition falling above line would contain excess cholesterol in either supersaturated or precipitated form (crystals of liquid crystals). (Reproduced from Redinger, R. N., and Small, D. M.: Bile composition, bile salt metabolism and gallstones. Arch. Intern. Med., 130:620,1972. Used with permission.)
MEDICAL VERSUS SURGICAL THERAPY Dissolution of asymptomatic gallstones with chenodeoxycholic acid is efficacious in approximately 60 per cent of patients. However, the safety of this agent has not been thoroughly established, and recent reports indicate that treatment must be continued indefinitely or calculi will reform. Moreover, most studies have shown that medical therapy is not beneficial6 when the calculi are radiopaque, when the gallbladder does not function, when the bile ducts contain calculi, or when complications ensue. Because the mortality rate for complications of gallstones in patients more than 60 years of age is approximately 15 per cent and the mortality rate for routine cholecystectomy is less than 1 per cent,2 arguments against cholecystectomy for asymptomatic calculi are weak. We have long believed that cholecystectomy is the procedure of choice for asymptomatic cholelithiasis as well as for acute and chronic cholecystitis. Some surgeons still advocate cholecystostomy for acute cholecystitis in elderly patients. Malstrom and Olsson 1o recently reviewed results of 63 cholecystostomies performed at the University of Lund on patients with a mean age of 71 years. The mortality rate at initial operation was 20 per cent. Sixteen patients later required cholecystectomy, nine had one attack each of cholecystitis, four had continuous symptoms, and five died of gallbladder disease. The authors concluded that "cholecystostomy may be as dangerous and unsuitable for the patient as it is comfortable and easy for the surgeon."
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Some surgeons advocate prolonged medical treatment for acute cholecystitis and then cholecystectomy. Although approximately 80 per cent of these patients have remission after nasogastric aspiration, intravenous fluid replacement, and adequate .analgesia and antibiotics, those who do not respond are likely to fare poorly; the ultimate mortality rate is between 5 and 20 per cent when emergency cholecystectomy is performed. Braasch et aP reported 324 cases of acute cholecystitis treated by early operation at the Lahey Clinic with an operative mortality rate ofless than 1 per cent. The complication rate in this series was 13.4 per cent; three fourths of complications were postoperative atelectasis, pneumonitis, phlebitis, and minor wound infections. These results are certainly acceptable, because 8 per cent of patients had perforated gallbladders and 15 per cent had choledocholithiasis. These data support our preference for early cholecystectomy for acute cholecystitis; it shortens the period of disability and prevents further deterioration. However, cholecystostomy under local anesthesia may be life-saving in patients with severe pulmonary, cardiac, and renal disease.
SAFETY FACTORS IN CHOLECYSTECTOMY The abdomen is entered through a right paramedian rectusretracting incision extending from the xiphoid process to a point approximately 2 inches below the umbilicus. The greatest hazard in biliary tract surgery is associated with the anatomic relationships of the cystic duct and the cystic artery to the right lateral wall of the hepatic duct. The abdominal contents are explored carefully in the same order at each operation; normal and abnormal findings are recorded in the operative notes in detail. Proper exposure is the hallmark of a safe cholecystectomy (Figs. 2 and 3). The fundus of the gallbladder is clamped with a Pennington clamp,
Figure 2. The most frequent causes of bile duct injury. They can be prevented by taking the steps mentioned in the text.
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B
Figure 3. The safe way to control cystic artery bleeding.
and gentle traction is applied in an inferior direction. A Deaver retractor is placed so that its point impinges at the angle made by the junction of the distal common bile duct with the duodenum, and traction is applied downward and to the left. This places the common bile duct under enough tension to facilitate delineation of its course. A large right-angled retractor is placed along the right costal margin, and a moist abdominal pad is placed over the hepatic flexure. A Deaver retractor is placed at the top of the incision immediately to the left of the gallbladder. Next a right-angled clamp is applied to the ampulla in a manner that allows the operator to control direction and magnitude of tension placed on the cystic duct and artery. When exposure of the right upper quadrant has been obtained, the peritoneum covering the free edge of the gastrohepatic omentum is incised and the common bile duct is exposed. If the safety of the procedure is to be assured, no structure in this region should be ligated or divided until the cystic duct and its junction with the common bile duct, the cystic artery, and the right hepatic artery are identified with certainty. As the cystic duct is invariably longer than the cystic artery, division of the cystic artery between paired clamps is the logical next step. If the common bile duct cannot be identified at its junction with the cystic duct because of edema, induration, or scarring, it is usually safely and easily identifiable immediately superior to the duodenum. In this event, we prefer a choledochostomy in the supraduodenal area and insertion of a Bakes dilator of appropriate size to the presumed junction of the cystic and common hepatic ducts. If bleeding ensues, the bleeding point is never clamped under direct vision or blindly. The first maneuver is application of gentle pressure to the area if bleeding is venous or compression of the common hepatic artery ifbleeding is arterial. Accumulated blood is then aspirated, and the
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bleeding point is identified by gradually releasing pressure. Suture ligation is employed under direct vision with a fine atraumatic needle. The cystic duct is divided near its junction with the common bile duct and is ligated with silk sutures. The gallbladder is stripped from its bed, leaving margins of serosa of 1 cm. The gallbladder bed is sutured for hemostasis and is drained, usually through the superior pole of the incision.
CHOLEDOCHOLITHIASIS The basic indications for common bile duct exploration are palpable calculi in the duct, recent jaundice, recent pancreatitis, a dilated common bile duct, calculi in the gallbladder smaller than the caliber of the cystic duct, thickened bile sediment in the gallbladder or duct, thickened common bile duct, obvious bile duct lesion or fistula, and acalculous cholecystitis. However, criteria for exploration of the bile ducts need not be so rigid with presently available techniques of operative cholangiography. Kakos et aU recently reported results of 3,012 operative cholangiograms obtained between 1951 and 1970. Although the incidence of routine cholangiography increased from 2.9 per cent in the first five years to 93 per cent in the last five years of the study, the yield of common bile duct calculi recovered remained the same (13 per cent of cholecystectomies). The number of negative explorations, however, decreased from 72 to 38 per cent. The main contribution of operative cholangiography is not to prevent the surgeon from "missing" calculi but to allow more security in electing not to explore the duct when the diagnosis is in doubt. However, negative common bile duct exploration causes minimal increased risk and is preferred to cholangiography by many surgeons. Most retained calculi are overlooked at the time of choledocholithotomy. One series l2 reported 28 per cent retained calculi after positive bile duct exploration. Completion cholangiography detects retained calculi in 14 per cent of patients and fails to detect calculi in another 7 per cent. IS Proper technique of common bile duct exploration is essential if retained calculi are to be kept to a minimum.
TECHNIQUE OF CHOLEDOCHOSTOMY The common bile duct has already been identified during cholecystectomy. Exposure is obtained, and the loose areolar tissue and peritoneum are cleared away from the site of the proposed choledochostomy. The common bile duct is aspirated with a fine needle to-confirm its identity and for culture. The anterior wall is incised parallel to its longitudinal axis, and the assistant continually aspirates the bile as it flows from the opening. Traction sutures of fine silk are inserted carefully in the edges ofthe opening, which is then enlarged to permit adequate ductal exploration. The intrahepatic and common hepatic ducts are explored first. A silver scoop is introduced, and the proximal common and common hepa-
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tic ducts are cleared of calculi and debris to avoid pushing them into the intrahepatic ducts. The right and left hepatic ducts are identified early in the exploration. When the intrahepatic ductal system is greatly dilated, the duct to the quadrate lobe is also identified and explored. If calculi are found, gentle scooping is continued until no further calculi are recovered. The proximal ducts are irrigated with a bulb syringe forced tightly into the choledochostomy and injected into the liver under pressure. Frequently, more calculi are forced out of the intrahepatic ducts using this maneuver. Small Bakes dilators are used to probe the smaller branches of the main hepatic ducts. With experience, the operator can feel the small dimples and tell if they are plugged with calculi. Steady pressure will frequently break through these plugs, allowing more calculi and debris to be recovered. These three maneuvers with dilators, scoops, and irrigation are repeated until all calculi are removed. Only then is attention turned to the distal ductal system. After all calculi have been removed or it is demonstrated that no calculi are present, attention is directed toward the ampulla of Vater and the sphincter ofOddi. If the 3 mm Bakes dilator does not pass readily into the duodenum, the distal duct should be explored with a small scoop and with calculus forceps. If no calculus is obtained, the duodenum and head of the pancreas should be mobilized by the, Kocher maneuver. This straightens out the intrapancreatic portion of the distal common bile duct and facilitates identification of the papilla, which can be palpated more easily with the index finger posterior to the head of the pancreas. If the 3 mm probe still cannot be passed readily into the duodenum without undue pressure, a duodenotomy should be performed. Usually, however, the dilator will pass. Dilation should be incremental up to a 7 mm diameter or, for a small common bile duct, 1 to 2 mm smaller than the diameter of the common bile duct. When the surgeon is satisfied that the duct has been completely cleared of calculi and debris and that the sphincter of Oddi has been dilated to a normal caliber, a short-limb T tube is inserted through the choledochotomy, which is closed with interrupted sutures of fine catgut.
RETAINED CALCULI Even after the most careful bile duct exploration, calculi occasionally remain. We usually favor surgical removal soon after their discovery. However, nonsurgical extraction and chemical dissolution are promising alternatives. Cholic acid has been shown to render the bile more capable of dissolving cholesterol. Clinical disappearance of calculi occurs in two thirds of patients when cholic acid, 100 mM, is infused through the T tube for two to three weeks. Mazzariello l l has reported 220 patients with retained calculi treated by extraction under fluoroscopic control through the T-tube tract with successful results in 204 patients. The T-tube tract is dilated first; finetipped calculus forceps or Dormia baskets are then used to crush and to extract the calculi. Calculi up to 2 cm in diameter and calculi from
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virtually every location within the intrahepatic or extrahepatic ducts have been extracted in this manner.
BENIGN BILIARY TRACT STRICTURES Although the incidence of benign biliary tract strictures has decreased in recent years presumably because of a more widespread use of acceptable techniques during cholecystectomy, the tragic dimensions of the problem are unchanged. The morbidity and ultimate mortality rates are high, and the disease can devastate the patient's finances. Moreover, 97 per cent of the 958 biliary tract strictures repaired in a 25-year period at the Lahey Clinic were essentially iatrogenic in origin.l7 The strictures were usually located high in the ductal system; 10 per cent were in the region of the bifurcation or in the intrahepatic ducts, 40 per cent were in the common hepatic duct, 28 per cent were in the common hepatic and common bile ducts, and 23 per cent were in the common bile duct. Ironically, in 85 per cent of the patients referred here, the original surgeon had no knowledge of the biliary duct injury at the time it occurred. In 38 per cent of these patients, cause of injury was deduced retrospectively at the time of repair. Of these strictures, 34 per cent were believed to have arisen during blind clamping for brisk bleeding in the porta hepatis, 22 per cent were associated with a technically difficult cholecystectomy, 21 per cent were caused by a ligature around the bile duct at the time of repair, and 5 per cent were associated with a difficult gastrectomy. The consequences of biliary strictures included choledochitis in nearly 100 per cent of patients, internal and external biliary fistulas in 35 per cent of patients, and biliary cirrhosis in 35 per cent of patients. Anemia and hypoprothrombinemia were also seen frequently. In our experience, 64 per cent of patients had chills and fever, 48 per cent had abdominal pain, 43 per cent had pruritus, 38 per cent had hepatomegaly, 24 per cent had external biliary fistulas, and 19 per cent had portal hypertension. Less than 1 per cent of patients were asymptomatic. Long-standing strictures, especially those with external biliary fistulas, can cause extreme debility with septicemia, weight loss, anemia, coagulopathies, base deficit-producing acidosis, and electrolyte abnormalities. Treatment is exclusively surgical. The physician may be tempted to correct all these medical problems before operation. However, after correction of bleeding tendencies or electrolyte abnormalities, which would make operation hazardous, expeditious decompression of the dilated bile ducts to prevent progressive biliary cirrhosis and liver abscess is the wisest course of action. If an external biliary fistula exists, a fistulogram through a No.8 Foley catheter with a balloon inflated subcutaneously frequently produces excellent visualization ofthe upper ductal system. Frequently, the lower ducts can be studied through endoscopic retrograde catheterization or with a barium cholangiogram, if a duct to small bowel anastomosis has previously been constructed. More often than not, however, anatomic
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data, such as size of ducts, amount of scarring, and location of stricture, are not known until the tedious dissection has been completed at the time of repair. Since each unsuccessful repair poses a certain risk of morbidity and mortality and reduces the chance of success of subsequent repairs, the surgeon who undertakes this procedure must have a thorough mastery of the principles of stricture repair, be familiar with the variety of available anastomoses and intubation techniques, and be able to make· sound intraoperative judgments. Those lacking such qualifications are wise to refer patients to an institution specializing in this type of surgery.
Operative Procedure The outcome of stricture repair depends primarily on the anatomic location of the stricture; the caliber of the proximal duct; the degree of cholangitis, pericholangitis, and local abscess formation; the time interval between injury and repair; the degree of biliary cirrhosis present; and the experience of the surgeon involved in assessing, selecting, and executing the procedure of choice (Figs. 4-8). Before operation, fluid, electrolyte, and acid-base abnormalities are corrected; chloramphenicol is given to neutralize the prevailing bile or-
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COMMON BILE DUCT
Figure 4. End-to-end repair of recent common bile duct transection.
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A
B
Figure 5.
Heineke-Mikulicz repair of duct narrowing.
Figure 6. For most strictures, end-to-side hepaticojejunostomy is used. A, The posterior row is placed first. B, The modified T tube is placed first in the intrahepatic ducts and the solid limb is brought out through a jejunostomy. C, The distal arm is next placed into the jejunal lumen.
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For lesions at .the bifurcation, the septum may be incised to produce a single
?pening.
L.HEPDUCT R.HEP.
A
Figure 8.
For lesions of intrahepatic ducts, each duct must be treated separately.
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ganisms; and vitamin K is administered. The abdomen is best entered through a long, vertical right paramedian muscle-retracting incision extending from the xiphoid process to several inches below the umbilicus. The peritoneum is entered through the lower pole as this is the least likely place to encounter intra-abdominal adhesions. Adhesions to the parietal peritoneum are disconnected as the operator 'proceeds toward the free edge of the liver in the extreme right of the abdomen. Dissection is continued to expose the base and dome of the right lobe. The portal triad is approached from its right lateral and anterior surfaces. If a fistulous tract exists, a probe inserted in it at this time can greatly facilitate finding the site of stricture. The position of the hepatic artery can usually be determined by palpation. The structure believed to be the common bile duct should always be aspirated with a small needle before it is incised. If the stricture is high, the dissection on the anterior surface of the duct must be continued past the bifurcation. The position of the distal bile duct can be determined between the common duct node and the gastroduodenal artery after an extensive Kocher maneuver has been performed on the duodenum. After the site of stricture has been found, operative cholangiography is usually essential to determine the exact location and condition of the duct. The bile duct is opened, the intrahepatic ducts are explored, and the calculi and sludge that are usually present are removed. At this point, the surgeon must decide what type of repair to perform. End-to-end repair is rarely possible except when transection of the duct is recent. In longstanding strictures, the proximal duct is dilated and the distal duct is thin and atretic, producing a discrepancy in size and tension on the suture line that makes end-to-end anastomosis hazardous. A Heineke-Mikulicz type of revision is occasionally possible on a recently partially transected duct or for a stricture at an intact biliary intestinal anastomosis. The majority of patients with common bile duct or common hepatic duct strictures seen at the Lahey Clinic are treated with an antecolic, end-to-side hepaticojejunostomy over an internal splint. A loop of jejunum, approximately 12 inches from the ligament of Treitz, is brought to the severed end of the bile duct. A small enterotomy is created on the antimesenteric border. A posterior row of interrupted, simple chromic sutures is placed first and then tied, approximating the two structures. An internal splint is selected and inserted. For high strictures, a modified Y tube is usually used, whereas for strictures of the common bile duct a T tube is frequently employed. The size of the tube is chosen so that it lies loosely in the duct. The external limb never exits through the anastomosis; instead, it exits through the jejunum directly opposite the anastomosis for a high stricture or, occasionally, through the distal common bile duct just above the anastomosis for alow stricture. Next, the anterior row of sutures is placed and, if possible, reinforced with interrupted silk sutures between Glisson's capsule and the jejunal serosa. The afferent limb is defunctionalized above an enteroenterostomy with four interrupted silk sutures. The enteroenterostomy is placed 16 inches distal to the hepaticojejunostomy. The abdomen is copiously irrigated with saline, and Penrose and sump drains are placed near the anastomosis in case a bile fistula develops.
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Strictures at the bifurcation ofthe right and left hepatic ducts can be treated more easily if the septum is incised to produce a single opening. Strictures slightly above the bifurcation, where the left and right hepatic ducts are still extrahepatic, can be treated by anastomosing each duct separately to the jejunal loop. For still higher strictures, where the ducts are intrahepatic, drainage is frequently accomplished transhepatically by guiding a curved calculus forceps through the stricture into a small biliary radicle and by pushing the tip of the forceps through the dome of the liver. A drainage tube is then brought back through the stricture and into the distal duct. Between 1940 and 1967, 1,553 operations on biliary strictures were performed at the Lahey Clinic, 78 per cent of patients had an ultimately successful result, and 17 per cent had an unsuccessful result, frequently because of operative mortality. Many patients required more than one procedure to restore normal function; those patients in whom function had not returned after the third operation were practically never improved. Our more recent series! of 119 patients operated on between 1967 and 1970 reported 88 successful results in 148 operations. Only 2 per cent operative mortality was recorded, and 65 per cent of patients eventually had a successful result. Wexler and Smith20 recently reported 61 repairs using a sutureless technique combining the principle of the transhepatic tube with a mucosal graft. The procedure is performed by removing a seromuscular patch near the end of a Roux-en-Y loop of jejunum, thereby creating a mucosal outpouching. The transhepatic tube is drawn through the liver and anchored to the Roux-en-Y loop through the previously created mucosal diverticulum. The tube is pulled back into the liver, carrying with it the sleeve of jejunal mucosa into the ductal system so that it comes in contact with the epithelium of the intrahepatic ducts. This technique eliminates some of the tedious dissection and provides mucosa-to-mucosa approximation, avoiding sutures that might compromise the blood supply. Wexler and Smith reported an 85 per cent success rate with this technique.
CARCINOMA OF THE GALLBLADDER Carcinoma of the gallbladder is a highly lethal disease with a predilection for elderly women. A recent study of 1,629 cases from the California Tumor Registry8 showed the women to men ratio to be 1.9 to 1.0 and the median age at diagnosis to be 70 years. Workers in the automotive, rubber, and textile industries have a statistically greater than expected chance of contracting this disease. The overall five-year survival rate is 7 per cent. Results of treatment are poor, because tumors are frequently asymptomatic until late in their course. The tumor spreads into contiguous parts of the liver, common bile duct, and duodenum. Lymphatic spread carries tumor cells to the nodes of the cystic and common bile ducts, to periaortic nodes, mediastinal nodes, and, eventually, to the bloodstream. Hematogenous metastases are
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mostly to the lungs. Death is usually caused by fatigue, anorexia, dehydration, malnutrition, and pneumonia. In a recent series of 100 cases, Pemberton et al. 13 reported that no patient died from replacement or failure of a major organ resulting from the neoplasm itself. The only clue to early diagnosis obtainable without operation is a calcified gallbladder, which harbors the disease in 25 per cent of instances. Although some workers have been enthusiastic about wedge resection of the liver and skeletonization of the portal triad, simple cholecystectomy has provided the highest survival rate.
CARCINOMA OF THE BILE DUCTS Although cancer of the bile duct is relatively uncommon, we have had considerable experience with it at the Lahey Clinic. Of 103 patients with supraduodenal carcinoma of the bile ducts treated in a recent 15-year period, 93 presented with jaundice, 40 with pain, and 29 with pruritus. It is most often seen in patients in their fifties and is associated with ulcerative colitis and the presence or history of gallstones. At operation, 70 per cent of cancers were found to be at the bifurcation or above, 22 per cent were in the central bile ducts, and 8 per cent were in the distal common bile duct. Eight per cent of patients had disease throughout the extrahepatic ductal system. 14 The true nature of this condition is frequently missed at the first operation, especially if biopsy is not performed. Because of the dense fibroblastic reaction around the tumor, the pathologist often confuses it with fibrosis or sclerosing cholangitis. Results of treatment are disappointing. Of 101 patients in our follow-up study, 21 died in the immediate postoperative period (the majority from hepatocellular failure), and the median length of survival was only 13 months. Nine patients lived four or more years. All of the 12 patients who remained asymptomatic for five years had had resection. Resection offers the only chance of cure. Occasionally, hepatic lobectomy is possible for carcinoma of one duct or for carcinoma at the bifurcation. However, most resectable tumors are located ih the periampullary area where they are amenable to pancreatoduodenectomy. In these instances, tissue can frequently be obtained for a positive disgnosis by vigorous scraping of the inside ofthe distal duct with a bone curet inserted through the choledochotomy. Reanastomosis after the Whipple procedure is made difficult by the normally thin-walled pancreatic duct. Because of the proximity of the portal vein and hepatic artery and because of the predilection of the tumor to infiltrate widely submucosally, curative resection is not possible for cancers in most other locations. In 19 of 103 patients, bypass was possible and provided the best palliation. The procedure of choice is hepaticojejunostomy performed just below the bifurcation. In 52 of our patients, the tumor extended so high that neither resection nor bypass was possible. Instead, the neoplastic stricture was dilated and patency was maintained with a buried Y tube, modified Y tube,
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transhepatic tube, or T tube (Fig. 9). Whatever procedure is performed, stricture often recurs as the disease progresses. Reoperation is frequently necessary to provide further palliation. Some of our patients underwent as many as six palliative procedures in a nine-year period before they eventually died of disease. Eleven patients had such extensive disease that neither resection, bypass, nor dilatation was possible. The best procedure for these patients is biopsy only. The prognosis is poor.
SCLEROSING CHOLANGITIS Sclerosing cholangitis is an uncommon reaction of the biliary duct precipitated by unknown stimuli and characterized by an intense submucosal fibrosis causing progressive encroachment on the lumen. This may be seen alone or in association with previous biliary tract surgery, gallstones, retroperitoneal fibrosis, Riedel's struma, reticulum cell sarcoma, familial immunologic deficiency, Crohn's disease, or ulcerative colitis. A recent review15 of 84 patients treated at the Lahey Clinic for this condition revealed that the process involved the entire biliary tract in 45, the bifurcation in 22, the extrahepatic ducts in 5, the distal common bile
THROUGH ABDOMINAL· WALL
Figure 9. Transhepatic tubes are placed in the bile duct to stent the anastomosis.
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duct in 5, and the intrahepatic ducts in 3. Of the patients presenting with sclerosing cholangitis, 93 per cent had intermittent obstructive jaundice, . 66 per cent had pain, 50 per cent had chills and fever, 63 per cent had weight loss, 59 per cent had anorexia and malaise, and 45 per cent had nausea and vomiting. Preoperative endoscopic retrograde choledochography showed a narrowed biliary tract with attenuation of the peripheral branches in the liver and a beaded appearance. At operation, the duct may be reduced to a fibrous cord. Despite previous enthusiasm for treating this condition with steroids, we now explore the biliary tract in almost every instance. The common bile duct is identified, and operative choledochography is performed. Choledochostomy and choledocholithotomy are performed next if calculi are present (which they were in 33 of our 84 patients). Mucosal scrapings of the duct wall are taken and submitted for frozen section examination to exclude carcinoma. The ducts are dilated with care, irrigated, and intubated with a loosely fitting T tube or with a modified Y tube. A bypass procedure is performed primarily for distal ductal strictures. Only 13 per cent of our patients have had continued improvement after operation. An additional 25 per cent of patients were stabilized but with sporadic mild choledochitis. However, more than 50 per cent of patients progress to biliary cirrhosis, portal hypertension, and liver failure. The mean length of survival has been six years after the onset of symptoms despite surgical treatment. The operative mortality rate was nearly 8 per cent.
HEPATOMA Although primary carcinoma of the liver is still comparatively rare in the United States, deaths from this disease have increased from 2,500 in 1964 to 7,500 in 1973. Its incidence is up to 500 times greater than this in Mozambique where it is believed to be largely environmentally induced. 5 The tumor may be solitary, nodular, or diffuse. Usually, the first symptom is vague right hypochondrial or epigastric pain. One third of patients present with spontaneous rupture of the liver and intraperitoneal hemorrhage. Surgical resection of hepatoma has produced few cures. In a large series of 271 patients, Lin 9 found that 82 hepatomas were resectable, but only 8 patients survived five years. The mortality rate within 30 days of operation was 12 per cent. 9 These poor results are consistent with the advanced state at which hepatoma is first clinically apparent. Systemic chemotherapy of hepatomas with nitrogen mustard and prednisolone has increased the mean length of survival from 4.6 to 7.4 months from the onset of symptoms. Regional infusion chemotherapy with intra-arterial 5-fluorodeoxyuridine in 12 patients seen at the Lahey Clinic in a recent five-year period produced a 14.5 month median length of survival from the onset of treatment. All infusion catheters were placed intraoperatively after exploration revealed metastatic or bilobar involvement precluding curative hepatic lobectomy. The patients were then given prolonged ambulatory infusion using a portable chronometric pump.4
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BENIGN CYSTIC DISEASES OF THE LIVER AND BILE DUCTS Choledochal cysts arise from defective embryologic development and are frequently accompanied by other abnormalities (Fig. 10), Cysts contain from 100 ml to several liters of fluid and are usually localized to the common bile duct. They present in children or in young adults as a right upper quadrant mass frequently accompanied by jaundice. Surgical treatment can be difficult because the cyst may actually be a tremendously dilated common bile duct; communications with accessory bile ducts are a frequent occurrence; and the connection between the distal bile duct and the duodenum can rarely be found. We have found side-to-side choledochojejunostomy to be effective treatment. Solitary nonparasitic cysts of the liver are believed to arise from congenitally faulty fusion of the ductal elements or of the accessory bile ducts. However, cystadenomas and cystic carcinomas of the liver have also been reported. Benign cysts usually arise from the inferior surface of the liver and contain up to four gallons offluid. If they communicate with functioning liver cells the fluid will be thick and bile stained; ifnot, it may be colorless. Although we formerly recommended complete extirpation by
Figure 10. Various types of choledochal cysts.
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enudeation or partial hepatectomy,1S we recently have begun to use unroofing and marsupialization extensively, unless the cysts contain bile.
HEPATIC METASTASES Approximately 20, 000 persons in the United States die each year from colonic carcinoma with hepatic metastases. Although patients are occasionally cured by hepatic resection, the majority have bilobar involvement. Systemic chemotherapy has produced unimpressive results with response rates of only 20 per cent, and the duration of remission, when achieved, is brief. Hepatic artery ligation has been tried with equivocal results. Fifty-five patients at the Lahey Clinic underwent regional arterial infusion chemotherapy of 5-fluorodeoxyuridine. They represented 80 per cent of patients referred to us for evaluation of hepatic metastases. 'Those not treated had either distant metastases, massive ascites, portal hypertension, or lack of symptoms. Of the 51 patients surviving the immediate postoperative period, clinical response was noted in 71 per cent. Nonresponders had a median length of survival of 5 months, and responders survived for a median of 16 months. These rates are approximately double those reported for systemic chemotherapy in patients with similarly staged disease. Factors that predicted response to infusion were young age, absence of ascites, presence of jaundice, and minimal replacement of the liver.3 These results are encouraging and we believe that such chemotherapy has a place in the therapeutic armamentarium.
REFERENCES 1. Braasch, J. W., Warren, K. W., Blevins, P. K.: Progress in biliary stricture repair. Am. J. Surg., 129:34-37 (Jan.) 1975. 2. Braasch, J. W., Wheeler, W. M., Colcock, B. P.: Acute cholecystitis. Surg. Clin. NorthAm., 44:707-716 (June) 1964. 3. Cady, B., Oberfield, R. A.: Regional infusion chemotherapy of hepatic metastases from carcinoma of the colon. Am. J. Surg., 127:220-227 (Feb.) 1974. 4. Cady, B., Oberfield, R A.: Arterial infusion chemotherapy of hepatoma. Surg. Gynecol. Obstet., 138:381-384 (March) 1974. 5. Higginson, J., Svoboda, D. J.: Primary carcinoma of the liver as a pathologist's problem. Pathol. Annu., 5:61-89, 1970. 6. Iser, J. H., Dowling, R. N., Mok, H. Y. 1., et al.: Chenodeoxycholic acid treatment of gallstones. A follow-up report and analysis offactors influencing response to therapy. N. Engl. J. Med., 293:378-383 (Aug. 21) 1975. 7. Kakos, G. S., Tompkins, R K., Turnipseed, W., et al.: Operative cholangiography during routine cholecystectomy: a review of3,012 cases. Arch. Surg., 104:484-488(April) 1972. 8. Krain, L. S.: Gallbladder and extrahepatic bile duct carcinoma. Analysis of 1,808 cases. Geriatrics, 27:111-117 (Nov.) 1972. 9. Lin, T.-Y.: Primary cancer of the liver. Scan. J. Gastroenterol., 5 (Suppl. 6):223-241, 1970. 10. Malstrom, P., Olsson, A. M.: Cholecystostomy for acute cholecystitis. Am. J. Surg., 126:397-402 (Sept.) 1973. 11. Mazzariello, R: Review of 220 cases of residual biliary tact calculi treated without reoperation: an eight-year study. Surgery, 73 :299-306 (Feb.) 1973. 12. Paine, J. R, Firrne, C. N.: The problem of the overlooked common duct stone. Am. Surgeon, 20:1171-1179 (Nov.) 1954. 13. Pemberton, L. B., Diffenbaugh, W. F., Strohl, E. L.: The surgical significance of carcinoma of the gallbladder. Am. J. SUrg., 122:381-383 (Sept.) 1971.
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14. Ross, A. P., Braasch, J. W., Warren, K. W.: Carcinoma of the proximal bile ducts. Surg. Gynecol. Obstet., 136:923-928 (June) 1973. 15. Warren, K. W., Tan, E. G. C.: Diseases of the gallbladder and bile ducts. In Schiff, L. (ed.): Diseases of the Liver, 4th ed. Philadelphia, J. B. Lippincott Co., 1976, pp. 1278-1335. 16. Warren, K. W., Kune, G. A., Hardy, K. J.: Biliary duct cysts. Surg. Clin. North Am., 48:567-577 (June) 1968. 17 . Warren, K. W., Mountain, J. C., Midell, A.!,: Management of strictures of the biliary tract. Surg. Clin. North Am., 51 :711-731 (June) 1971. 18. Way, L. W., Admirand, W. H., Dunphy, J. E.: Management of choledocholithiasis. Ann. Surg., 176:347-359 (Sept.) 1972. 19. Wenckert, A., Robertson, B.: The natural course of gallstone disease: eleven-year review of 781 nonoperated cases. Gastroenterology, 50:376-381 (March) 1966. 20. Wexler, M., Smith, R: Jejunal mucosal graft: a sutureless technic for repair of high bile duct strictures. Am. J. Surg., 129 (2):204-211 (Feb.) 1975. Lahey Clinic Foundation 605 Commonwealth Avenue Boston, Massachusetts 02215
