The diverse causes of biliary obstruction and their remedies

THE DIVERSE CAUSES OF BILIARY OBSTRUCTION AND THEIR REMEDIES WILLIAM P. LONGMIRE, JR.

TABLE INTRODUCTION

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SPECIFIC DIAGNOSTIC TESTS .

CONTENTS .

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RETAINED COMMON DUCT STONES

Diagnosis

Discussion .

Operative

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Management

Summary

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Special Diagnostic Tests .

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Pathology

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Clinical Course Diagnosis

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SCLEROSlNG CHOLANGITIS

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IATROGENIC TRAUMA

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12 20

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36 39

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39

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40

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52

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53

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54

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56

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59

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43

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Summary

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Results

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Long-Term

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Treatment

SUMMARY

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CONGENITAL BILIARY ATRESIA .

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and Treatment

Prognosis

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CANCER OF THE BILE DUCT . Treatment

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LESIONS OF THE SPHINCTER OF ODDI Background

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QUESTIONS OF CLINICAL USEFULNESS ANSWERED IN THIS ISSUE

1. List 3 special diagnostic procedures that may be utilized in the preoperative evaluation of patients with obstructive jaundice and the advantages and disadvantages of each. p. 7. 2. Outline a plan of investigation of the patient with obstructive jaundice, p. 12. 3. List 3 methods that may be utilized to eliminate a retained common duct stone and the advantages and disadvantages of each. pp. 17 and 18. 4. In what type of patient are common duct stones most frequently overlooked? p. 13. 5, WhatLfactors are responsible for not visualizing retained stones in postexplm'atory operative cholangiograms? p. 17. 6. Sphincteroplasty may be of value in the treatment of chronic pancreatitis under what specific conditions? p. 21. 7. List the advantages and disadvantages of sphincteroplasty and choledochoduodenostomy,pp. 23 and 24. 8. List 5 diagnostic findings that may support the diagnosis of malfunction of the sphincter of Oddi. pp. 24 and 25. 9. Name 2 important technical considerations in performing a sphincteroplasty that will aid in avoiding serious postoperative complications, pp. 27 and 28. 10. What is the most common site in the biliary tree for the development of cancer? p. 29. 11. What is the importance of endoscopy in the treatment of cancer of the bile duct? p. 30. 12. Are there characteristic features in either the history or the physical examiriation of the patient with obstructive jaundice to allow the physician to m~ke a diagnosis of carcinoma of the bile ducts? p. 30. 3

13. What are the characteristic findings ofa transhepatic cholangiogram to suggest the diagnosis of bile duct carcinoma in its most frequent location? p. 31. 14. For purposes of prognosis and treatment, how may bile duct cancer be classified? pp. 31-33. 15. How does a U tube differ from a T tube? pp. 33 and 34. 16. Bile duct cancer is frequently confused at operation with what benign condition of the bile ducts? p. 29. 17. Of what value is ERCP in sclerosing cholangitis? p. 29. 18. What is the value of prolonged T-tube drainage for sclerosing cholangitis, and what is the chief hazard of this therapy? p. 41.

19. What is the prognosis ofsclerosing cholangitis?pp. 41 and 42. 20. Name 3 common anomalies of the extrahepatic bile ducts that may confuse .the surgeon during ch.olecystectomy. pp. 43 and 44.

21. If recognized at the time, how should a n injury of the bile duct be managed? p. 45. 22. Under what conditions is long-term intubation of a duct enteric anastomosis indicated? pp. 47 and 48. 23, Certain cases of biliary atresia are thought to be due to something other than congenital anomalous development. What? p. 53. 24. What is the principle of the Kasai operation? pp. 53 and 54. 25. Depending upon the condition of the bile ducts as found a t operation, what operative procedures are indicated in the patient with neonatal jaundice? p. 57.

is Professor of Surgery, UCLA School of Medicine, where he previously served as Chairman of the Department of Surgery. He has served as President of the American College of Surgeons, the American Surgical Association, and the Society for Surgery of the Alimentary Tract. Doctor Longmire received his medical degree and training at The Johns Hopkins School of Medicine. His interest in the biliary tract dates back to the introduction in 1945 of the operation of intrahepatic cholangiojejunostomy, and his published interests include gastric and pancreatic cancer and tissue transplantation as well as early contributions to the field of cardiac surgery.

INTRODUCTION THE BILIARY DUCTS are essentially a simple system of inert tubes, with the the sole function of transporting bile passively fr0m the liver into the duodenum. Symptoms related to obstruction of the system are few and straightforward: jaundice and pain and, when the system becomes infected, chills and fever. The possible causes of obstruction are numerous, however, and diagnostic methods are complex. Management varies widely based on the underlying cause. Although Bobbs, 2 an American '~Hoosier," is credited with performing the'first operation on the biliary tract in 1868 (an operation using chloroform anesthesia that took place on the 3d floor of a wholesale drug company building in downtown Indianapolis), biliary operations'were rare in this country for a number of years thereafter. Fourteen years after Bobbs's successful procedure, Langenbuch 6 performed the first planned cholecystectomy. However, it was not until 1886 that biliary tract operations were first mentioned in the yearly Transactions of the American Surgical Association. At that time Parkes, H professor of anatomy at Rush Medical College, r e p o r t e d on 2 operations for gallstones, each with a fatal ending, and emphasized the difficulties with which the surgeon must contend when the gallbladder is atrophied or undistended with fluid. Seven years passed before the next 2 papers on biliary tract surgery appeared (in the 1893 Transactions). Richardson, 12 of the 5

Massachusetts General Hospital, discussed at length cholecystostomy and cholecystectomy and described for the first time before the American Surgical Association operations on the cystic, hepatic and common bile ducts. In addition to the 14 cases presented by Richardson, the personal operative experience of the 5 surgeons who discussed his paper indicated that biliary surgery was on the increase in America. Two years later, in 1895, Elliot 3 reported on 2 patients in whom stones had been extracted from the hepatic or common duct with immediate suture of the duct. Thereby began an argument not completely resolved in the minds of all surgeons even today: Is drainage of the common duct with a T or other tube mandatory after common duct exploration? Elliot lists Kummell's unsuccessful effort in 1890 as the first choledocholithotomy and Thornton's report of 3 cases in 1891 as the first successful removal of stones from the common duct. Lacking a definitive answer as to the genesis of gallstones, some surgeons, led by Lawson Tait of London, contended that all gallstones were formed in the liver and passed down into the gallbladder and more distal ducts. Others, such as Langenbuch, believed the gallbladder to be the primary site of stone formation. This difference of opinion gave rise to the controversy ofcholecystostomy versus cholecystectomy and led to strong expressions of opinion on both sides for a number of years. For example, in his 1902 paper, "Why should w e not treat the gallbladder as we do the appendix?" Roswell Park 1~vigorously urged the use of routine cholecystectomy. A reliable method of visualizing the gallbladder on radiographic examination developed by Graham and co-workers in 19235 aided immeasurably in the preoperative evaluation and selection of patients for operations upon the biliary tract. In addition, the introduction of operative cholangiography by Mirizzi ~ of Argentina in 1931 was important in directing the surgeon's efforts during operations on the bile ducts for stones and other sources of obstruction. Fenger,4 in 1896, proposed the following modification of Schiippel's 13classification of bile duct obstruction: 1. Obstruction from within: (a) bodies that fill the lumen, gallstones, parasites; (b) cicatricial strictures following inflammation; (c) tumors within the ducts, benign or malignant. 2. Obstruction from without: compression by masses, tumors, adhesions, scars. 3. Deviations of the bile ducts in the hilus: bending, displacement, oblique insertion. The third category, no longer accepted today, obviously was an attempt to explain symptoms of bile duct obstruction in which the usually readily observable stones, tumors or strictures were not found. 6

TABLE 1 . - T H E DIVERSE CAUSES OF BILIARY OBSTRUCTION 1. Lesions within the lumen a) Stones b) Parasites c) Hematobilia 2. Lesions without the duct a) Pancreatic lesions, tumors, inflammation b) Lesions of sphincter of Oddi c) Peptic ulcer 3. Lesions of the duct wall a) Tumors: malignant, benign b) Sclerosing cholangitis c) Iatrogenic trauma d) Abdominal trauma e) Congenital lesions: atresia, hypoplasia, cysts

Today we may use Schiippel's '3 original classification as the basis for an almost overwhelming list of possible causes ofbiliary obstruction. From the array of causes of biliary obstruction listed in Table 1, certain topics have been selected arbitrarily for further discussion, primarily on the basis of current interest or controversial features in etiology, diagnosis and management. These are: retained common duct stones, lesions of the sphincter of Oddi, cancer of the bile duct, sclerosing cholangitis, iatrogenic trauma, and congenital biliary atresia.

SPECIFIC DIAGNOSTIC TESTS The follOwing specific diagnostic techniques frequently utilized in the preoperative evaluation of the patient with bile duct obstruction require comment: (1) "Thin needle" percutaneous cholangiography, (2) endoscopic retrograde choledochopancreatography (ERCP) and (3) ultrasonography. "THIN NEEDLE" PERCUTANEOUS CHOLANGIOGRAPHY.--The "thin TABLE 2.-SUCCESS RATE OF PERCUTANEOUS TRANSHEPATIC CHOLANGIOGRAPHY IN DIFFERENT DISEASE GROUPS* GROUP

Malignancies of liver, bile duct, pancreas Cholelithiasis Other surgical diseases Nonsurgical diseases

Total

NO. O F PATIENTS

i%[O. O F SUCCESSES

SUCCESS R A T E (%)

95

95

100.0

121 18 80

104 15 54

85.9 83.3 67.5

314

268

85.3

*From Okuda and associates)

TABLE 3 . - C O M P L I C A T I O N S OF PERCUTANEOUS TRANSHEPATIC CHOLANGIOGRAPHY* CO.MPLICATION

NO. OF PATIENTS

% OF TOTAL~

Fevers Blood pressure, 25 cm Hg Bile leakage Bile peritonitis Bleeding Total

11 6 3 2 2 24

3.5 1.9 0.95 0.64 0.64 7.63

*Modified from Okuda and associates2 T314 cases. SAbove 38C.

needle" (Chiba needle) technique, introduced by Ohto and Tsuchiya in 1969, s has greatly improved the accuracy and safety of the procedure of percutaneous transhepatic cholangiography. Utilizing this technique, we have had 100% success in demonstrating the intrahepatic biliary system in patients with ductal obstruction and 60% success in patients with nonobstructed systems. We have encountered 1 case of transient bacteremia and 1 of questionable intraperitoneal bleeding, neither of which required early operation. Okuda et al., 9 in their report of 314 such examinations, documented an excellent Success rate in the diagno~ of various diseases (Table 2). The complications they encountered in this large series are listed in Table 3. Operation for bile leakage was required in 1 patient. Intraperitoneal bleeding that did not require operation was identified in 2 patients. The so-called Chiba needle is 15 cm in length with an outside diameter of 0.7 mm, an inner diameter of 0.5 mm and a bevel angle of 30 degrees. With the patient in the supine position and with use of local infiltration anesthesia, the needle is introduced in the right flank at the level of the 7th or 8th interspace and directed parallel to the plane of the table 11/2 to 2 vertebral body thicknesses below the vertebrodiaphragmatic junction to a point just short of the vertebral column. The styler is removed, a syringe containing opaque medium is attached to the needle, and slight injection pressure is maintained as the needle is withdrawn. When. the needle enters a blood vessel, the injected dye is quickly diluted and carried away; dye injected into the parenchyma remains. When a bile duct is entered, the dye moves slowly toward the hilar region of the liver. Should the first attempt be unsuccessful, the needle may be moved 3 - 5 mm above or below the first site and additional injections attempted. When a bile duct is entered (Fig. 1), several x-ray films are made as the medium is being injected. The needle is then re8

Fig 1.-Percutaneous transhepatic cholangiogram provides one of the most precise methods of diagnosing the cause of obstructive jaundice. Complications of gram-negative sepsis and intraperitoneal collections of infected bile and blood have been greatly reduced with the "thin needle" technique. Normal or constricted intrahepatic ducts may be missed by the needle and the ductal system may not be visualized.

moved and additional exposures made with patient supine, prone, erect and at angles, if indicated. Antibiotics are administered for 2 days before and 3 days after the examination. Visualization of the ductal system by percutaneous cholangiography provides the most accurate method available for the preoperative determination ofthe site and cause ofbiliary obstruction. ENDOSCOPIC

RETROGRADE

CHOLEDOCHOPANCREATOGRAPHY. - -

The technique of ERCP is another useful diagnostic procedure (Fig. 2). BlumgarL1 stated that ERCP has the advantages of (1) being useful early in a patient's illness, (2) posing few hazards, (3) providing an unequivocal preoperative diagnosis in experienced hands in up to 80% of jaundiced patients and (4) being particularly valuable in demonstrating a patient's normal biliary ductal system. His group reported the investigation of 146 jaundiced patients and the successful performance of duodenoscopy in 144. Intravenous cholangiography gave a positive diagnosis in 1 patient; in another, pyloric stenosis due to carcinoma of the common bile duct prevented entrance into the duodenum. The ampulla of Vater was located in 140 of the patients and its papilla successfully cannulated in 114 (80%). In 3 patients the cannulation was not attempted, since carcinoma of the papilla of Vater was diagnosed at endoscopy. 9

B

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Fig 2.-Endoscopic retrograde choledochopancreatography (ERCP) permits visual examination of the papilla of Vater; if the orifice is successfully cannulated, x-ray visualization of the common bile and pancreatic ducts is possible. In "experienced hands the ampulla can be cannulated in approximately 80% of the ~ases. Significant complications are infrequent. This endoscopic retrograde cholangiogram shows catheter passing well up into common bile duct; ductal system is slightly dilated with delayed emptying into duodenum.

Duodenography, retrograde choledochopancreatography, or both, gave a definitive diagnosis in 109 patients and useful information in another 11. Misleading information was obtained in 1 patient, and endoscopy did not contribute to diagnostic information in 24. The technique is well described by Blumgart; however, the procedure requires not only proper equipment and facilities but also considerable skill and practice. A physician who wishes to make the substantial investment in time and effort required to learn and to maintain his proficiency in ERCP is well advised to start his training in an apprentice relationship to an established, skilled endoscopist or to enroll in a formal endoscopic training program. ULTRASONOGRAPHY.--The technique of ultrasonography has been greatly improved in recent years and is still undergoing refinement. As a relatively simple noninvasive technique, it is of value in demonstrating dilatations of the extrahepatic biliary system, stones in the gallbladder or in dilated extrahepatic bile 10

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Fig 3 . - a , Longitudinal gray scale sonogram to the right of the midline showing a dilated common bile duct (CBD) anterior to the portal vein (PV) containing noncalcified common duct stones (CDS)o A mass present in the head of the pancreas (PM) contains calcifications (Cal). Other features visible: acoustical shadows (AS), head (H), feet (F), centimeter marker (cm). b, transverse computer tomogram 2 cm showing a dilated common bile duct (CBD). Other features visible: duodenal bulb {DB), pancreas (Pa), inferior vena cava (IVC), aorta (Ao), superior mesenteric artery (SMA), stomach (St), right side (R), left side (L). (Both photographs courtesy of William F. Sample, Department of Radiology, University of California'at Los Angeles.)

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ducts and masses in the region of the ductal system (Fig. 3, A). Thus far, it lacks the specificity of a good cholangiogram. Computerized tomography may also become a useful diagnostic procedure, but it has not been as valuable as sonography in our hands to date (Fig. 3, B). Radioactive scans and arteriograms have been of little value in the establishment of preoperative diagnoses. Our plan of investigation for the patient with obstructive jaundice includes a full range of liver function tests, upper gastrointestinal X-ray studies and, in patients with a serum bilirubin value below 2.5 mg/100 ml, intravenous cholangiography with tomography. If the cause of the jaundice remains uncertain, ERCP and ultrasonography are performed. If cannulation of the papilla is unsuccessful or if the ductal system cannot be adequately visualized, a percutaneous transhepatic cholangiogram is considered. REFERENCES 1. Blumgart, L. H.: Endoscopy of the Upper Gastrointestinal Tract, in Longmire, W. P., Jr. (ed.): Advances in Surgery ~Chicago: Year Book Medical Publishers, Inc., 1975), Vol. 9, p. 97. 2. Bobbs, J. S.: A case of lithotomy of the gallbladder, Trans. Indiana State Med. Soc. 18:68, 1868. 3. Elliot, J. W.: Immediate suture of the gall ducts and the gallbladder after the extraction of stones, with cases, Trans. Am. Surg. Assoc. 13:361, 1895. 4. Fenger, C.: Retention from displacement, bending and valve-formation (oblique insertion) in the biliary tract, Trans. Am. Surg. Assoc. 14:683, 1896. 5. Graham, E. A., Cole, W. H., Colpher, G. H., and Moore, S.: Diseases of the Gallbladder a~d Bile Ducts (Philadelphia: Lea & Febiger, 1928). 6. Langenbuch, C.: Ein Fall yon Exstirpation der Gallenblase wegen chronischer Cholelithiasis-Heilung,Berl. Klin. Wochenschr. 19:725, 1882. 7. Mirizzi, P. L.: La exploracion de las vias biliares principales en el eurso. Presented at Argentine Surgical Congress, October 1931. 8. Ohto, hi., and Tsuchiya, Y.: Medical cholangiography. Technique and cases, Medicina 6:735, 1969. 9. Okuda, K., Tanikawa, E., Emura, T., et al.: Non-surgical percutaneous transhepatic cholangiography-diagnostic significance in medical problems of the liver, Am. J. Dig. Dis. 19:21, 1974. 10. Park, R:: Why should we not treat the gallbladder as we do the appendix? Trans. Am. Surg. Assoc. 20:374, 1902. 11. Parkas, C. T.: Two cases of cholecystotomy, Trans. Am. Surg. Assoc. 4:297, 1886. 12. Richardson, hl. H.: Surgery of the gallbladder, Trans. Am. Surg. Assoc. 11:81, 1893. 13. Schiippel, O.: Die Krankheiten der Gallenwege und der Pfortader, in von Ziemssen, H. (ed.); Handbuch der speziellen Pathologie und Therapie, lief. xxii, 4 (Leipzig: F. C. W. Vogel, 1878).

RETAINED COMMON DUCT STONES One of life's greatest'embarrassments for the biliary surgeon is to discover on postoperative cholangiogram a retained common 12

duct stone in a patient who has had a recent meticulous and thorough common duct exploration. Halsted, 1~ who in 1880 or 1881 performed one of the earliest gallbladder operations in this country, stated a problem then that, unfortunately, still exists: "Some sure and simpler method must be devised for determining positively the presence of stones in the ductus choledochus after excision of the gallbladder." How prophetic was this statement! A little less t h a n 20 years later, Halsted himself died after an operation for a retained common duct stone. Every surgeon who performs biliary tract operations will have, sooner or later, a patient who falls into the group of the 5 - 9% of Fig 4 . - A , 70-year-old female, known to have stones in the gallbladder for more than 20 years, had an attack of jaundice, after which multiple stones forming a cast of the dilated common duct were removed. It is in such cases of multiple stones that 1 or more of the stones may be missed at exploration, only to be visualized on the postoperative cholangiogram as a retained stone. B, sketch of multiple stones in common duct and retained stone after exploration.

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MULTIPLE STONES Pr eoperative

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patients who have retained common duct stones after duct exploration. There are an estimated 3,000-4,000 such patients each y e a r in America. More frequent use of operative cholangiography has lessened the incidence of retained stones, and it is hoped that more widespread use of the choledochoscope in patients with multiple common duct stones (the patients in whom stones are most apt to be missed) will further reduce the incidence of these incomplete operations (Fig. 4). After repeating the postoperative cholangiograms and allowing sufficient time to elapse to assure both surgeon and patient that t h e stone will not pass spontaneously (spontaneous disappearance of stones has been reported to occur in 10-20% of patients [Fig. 5]), what is the next step? Probably simplest have been the attempts of Way e t a l . 1~ and others to increase the solubility of cholesterol by irrigating the common bile duct and retained stones with a sodium cholate solution (200 mM, in 0.15N NaC1 buffered to pH 7.5, prefiltered by passage through a 0.45-t~ millipore filter and sterilized by passage through a 0.22-tt millipore filter) administered via continuous drip through a T tube at the rate of 30 ml/hour over a 3 - 1 4 day period. Biliary pressure should not be allowed to rise above 30 cm H20 (Fig. 6). Although the method is only recommended for the t r e a t m e n t of cholesterol stones, Way and his co-workers 15 have reported the disappearance or reduction in size of retained stones in 33 of 49 patients so treated. If the duct is not obstructed, Fig 5.-Enlarged photographs of T-tube cholangiograms following removal of common duct stones. A, retained stone visualized on T-tube cholangiogram 1 week after operation. (Presence of stone was confirmed on repeated x-rays.) B, subsequent cholangiogram demonstrates the spontaneous passage of the stone and clearing of the duct.

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Sodium cholote solution

Fi 9 6.-Continuous irrigation method recommended by Way. Sodium cholate solution is administered via continuous drip through the T tube at a rate of 30 ml/hour~over a 3-14-day period. Biliary pressure of the perfusate is monitored.

the infusing solution is permitted to run through the T tube and into the duodenum. If a stone obstructs and is located between the tube and the duodenum, or if the stone is proximal to the T tube, a fine catheter is passed through the T tube and positioned against the stone under fluoroscopic control. The cholate solution is infused via the small tube, washing the stone and escaping back through the T tube. The effect of the treatment is monitored with cholangiograms at 3-day intervals. The troublesome diarrhea that occurs from the infusion of this large amount of bile acid is reduced (but, unfortunately, not eliminated) by the simultaneous oral administration of cholestyramine. The reported complications of sodium cholate infusions include diarrhea, impaction of the stone at the ampulla of Vater, pancreatitis and cholangitis. It is unlikely that 14 days would be enough t i m e for sodium cholate infusion to dissolve completely a sizable" retained stone. More likely, in the successful cases, the stones are reduced in size and are passed into the duodenum as a result of the flushing action of the infusion solution. A variety of other solutions have been used in recent years. Gardner and associates9 have recently reviewed their results of 15

the treatment for retained stones by means of a continuous T-tube drip of 25,000 units of heparin in 250 cc of saline every 8 hours. In a series of 43 patients, stones were reported to have disappeared in 31. Of the 12 who failed to respond, 7 were treated for less than 1 week before undergoing reoporation. Although its action is not completely understood, heparin supposedly encourages the fragmentation of stones as a result of its action on the suspension stability of particles suspended in bile. The heparin solution has the advantage of being readily available, it should be equally effective in treating noncholesterol stones and it does not have the side effects of sodium cholate. Gardner et a l 2 report bleeding about the T tube in 1 patient. No changes were observed in the clotting times in any patient in whom they were measured. The purely mechanical effects of T-tube irrigation were demonstrated in the report of Catt et al. ~ from Brisbane. Stones disappeared in 6 of 10 patients given probanthine systemically and infused daily through the T tube with 1,000 cc of saline containing a local anesthetic. Stones in the ductal system on the hepatic side of the T tube are rarely affected by irrigation techniques with any of the solutions described here. The current interest in systemic dissolution of stones in the gallbladder raises the question of a similar systemic treatment for retained common duct stones. The 1 report available at this time indicates that orally administered cholic acid was associated with the disappearance of common duct stones i n 1 of 7 patients treated, s An additional nonoperative technique of current interest is the instrumental removal of stones via the tract l:emaining to the common duct after removal of the T tube (Fig. 7). This procedure, described by Mondet 14 in 1962 and Magarey u in 1969, has been successfully utilized in large numbers of patients by Mazzariello~2, ~3 and Burhenne. 3, 4 Utilizing a specially designed polyethylene steerable catheter and a variety of extraction instruments, Burhenne et a l 2 have successfully retrieved all retained stones from 115 of 120 patients. More than one session was required in one third of the cases. In a collected review of 612 cases from 38 hospitals (204 from his institution) Burhenne 5 reported a 5% morbidity with postextraction fever in 12 patients, sepsis in 2 patients, pancreatitis in 2, ~'vasovagal" reaction in 2, subhepatic bile collection in 2 and perforation of the sinus tract in 7. When extravasation from the sinus tract occurred (particularly after extraction of large stones through a small sinus tract), the contrast medium remained contained except in 2 patients, in whom there was free intra-abdominal extravasation. All patients were placed on antibiotics, and no clinical signs or symptoms developed after sinus tract extravasation. There were no bile duct perforations. Papillomas of the common duct were mistaken for stones in 3 patients. Three com16

Troct creoted lti"i"

Fig 7.--Extraction of a retained stone with a Dormia basket. If postoperative cholangiogram demonstrates a retained stone, the T tube is left in place for 4 - 6 weeks to permit the formation of a well-established fibrous tract. The T tube is withdrawn and a Dormia stone basket introduced into the duct with the Burhenne steerable catheter to engage and extract the stone. A T tube size 14 or 16 F should be used and the long arm brought out lateral to the right midclavicular line.

plications required reoperation, 2 of these for removal of an entrapped snare. There were no deaths. Reoperation for failure of stone extraction was required in 9% of patients. The calculated radiation dosage to both patient and roentgenologist was within generally accepted levels. Because dissolution of retained stones by T-tube irrigation was unsuccessfully attempted in 64 of the 120 p a t i e n t s - a n d because irrigation requires prolonged hospitalization, whereas instrumental extraction is performed as an outpatient or brief inpatient p r o c e d u r e - B u r h e n n e endorses nonoperative extraction under roentgenologic control as the method of choice for the removal of retained biliary tract stones. The operative cholangiograms of 83 of the patients referred to Burhenne with retained stones were reviewed in retrospect and showed the stone to be visible in only 20% of the patients. The main reason for overlooked biliary duct stones was not the lack of operative cholangiography but, rather, poor technique. Motion, poor positioning, over- or underexposure and insufficient contrast filling of major hepatic radicles were the chief factors responsible for the poor technical quality of operative cholangiograms. When skilled radiologic extraction techniques are available, Burhenne suggests that the surgeon should not persist in repeated fruitless attempts to extract intrahepatic stones at the operating table b u t rather that his technique should be utilized subsequently as an outpatient procedure. When multiple stones are present, and when a possibility of retained stones exists, a T tube, at least 14 and preferably 16 F should be used to create a fistu17

lous tract large enough to permit the passage of the steerable catheter. The long arm of the T tube should be brought out laterally in the right subcostal area in the midclavicular line or lateral to it. The tube is left in place a minimum of 4 weeks to ensure a well-established fistulous tract. Britton e t al. 2 reported the disappearance of all retained stones in 4 of 7 patients treated with sodium cholate solution within 8 - 1 2 days after initiation of irrigations. In the fifth patient a stone distal to the T tube disappeared, and a stone on the hepatic side of the T tube was removed with a Burhenne catheter. In the sixth patient the infusion method failed, and the stone was later removed by the extraction catheter method. In the final patient 2 attempts with the Burhenne catheter failed to remove a stone impacted at the ampulla. They found the infusion method to be unsuccessful when the stone was located proximal to the T tube. In all patients treated by infusion diarrhea developed, often severe. Cholestyramine lessened but did not eliminate this troublesome side effect. All patients became nauseated and anorexic and vomited intermittently despite medication. Four of 7 patients experienced some abdominal pain, although it was never severe, and the serum amylase was not elevated. In 1 patient signs of bile peritonitis were attributed to disruption of the T-tube tract 2 hours after a Burhenne catheter and Dormia basket had been utilized. No deaths or long-term complications occurred in their series. Britton e t al. 2 thus concluded that the use of sodium cholate infusion is~simple and requires little expertise apart from the ability to manage the not-insignificant side effects. On the other hand, successful use of the Burhenne catheter appears to require considerable practice and expertise and, therefore, should be reserved for centers with a particular interest in its use. Way and Motson 16 have recently commented, "About the same time that cholate was introduced to d i s s o l v e retained stones, techniques for their instrumental extraction by fluoroscopic manipulation through the T-tube tract were refined. The latter approach has now evolved to the point where it is successful in about two" thirds Of cases. The technique, is safe and if instrumental extraction is unsuccessful, the patient can still be treated by dissolution or reoperation. Therefore, we generally recommend that an attempt at mechanical extraction be the first step in management of the patient with a retained stone. If that fails, the irrigation technique should be used." It is obvious that the mechanical extraction technique has great merit but that it requires proper equipment and facilities and, most important, expertise on the part of the individual performing the procedure. Fortunately, the n u m b e r of these cases is insufficient to warrant' maintenance of a competent "extractionist" in every hospital; thus, this is a procedure that should be con18

c e n t r a t e d in t h e h a n d s of an interested, qualified t e a m in each p o p u l a t i o n center. A l t h o u g h r e o p e r a t i o n for r e t a i n e d stones carries a low morbidity, it would s e e m t h a t n o n o p e r a t i v e e x t r a c t i o n is indicated w h e n p e r s o n n e l experienced in the procedure are available. B a r t l e t t a n d Q u i n b y , ' who h a v e r e p o r t e d 96 s e c o n d a r y b i l i a r y o p e r a t i o n s w i t h o u t a fatality, n e v e r t h e l e s s c o m m e n t : "The absence of f a t a l cases in this series of 96 o p e r a t i o n s is not s t a t i s t i c a l l y significant a n d s h o u l d not be i n t e r p r e t e d as m e a n i n g t h a t no risk is associated w i t h r e p e a t e d o p e r a t i o n s in the b i l i a r y system. It does suggest, however, t h a t the risk of s e c o n d a r y o p e r a t i o n s is not excessive a n d gives some a s s u r a n c e t h a t a n overlooked c o m m o n duct stone is not n e c e s s a r i l y a t r a g i c o c c u r r e n c e - c e r t a i n l y it is less t r a g i c t h a n a n o p e r a t i v e i n j u r y to t h e c o m m o n duct." I f a qualified '~extractionist" is not a v a i l a b l e a n d a small-tom o d e r a t e - s i z e d stone does not s e e m to be i m p a c t e d at the a m p u l l a or on t h e h e p a t i c side of the T tube, the r e p o r t e d results of the infusion of h e p a r i n or s o d i u m cholate solutions, or even saline, h a v e been sufficiently e n c o u r a g i n g to m a k e a case for t r e a t i n g p a t i e n t s w i t h one or the o t h e r of the solutions for a period of up to 7 d a y s before r e s o r t i n g to reoperation. REFERENCES 1. Bartlett, M. K., and Quinby, W. C.: Secondary operations on tl~e common bile duct, N. Engl. J. Med. 256:11, 1957. 2. Britton, D. C., Gill, B. S., Taylor, R. M. R., and James, O.: The removal of retained gallstones from the common bile duct: Experience with sodium cholate infusion and the Burhenne catheter, Br. J. Surg. 62:520, 1975. 3. Burhenne, H. J.: Nonoperative retained biliary tract stone extraction: A new roentgenologic technique, Am. J. Roentgenol. Radium Ther. Nucl. Med. 117: 388, 1973. 4. Burhenne, H. J.: Nonoperative roentgenologic instrumentation technics of the postoperative biliary tract, Am. J. Surg. 128:111, 1974. 5. Burhenne, H. J.: Complications of nonoperative extraction of retained common duct stones, Am. J. Surg. 131:260, 1976. 6. Burhenne, H. J., Richards, V., Mathewson, C., Jr., and Westdahl, P. R.: Nonoperative extraction of retained biliary tract stones requiring multiple sessions, Am. J. Surg, 128:288, 1974. 7. Catt, P. B., Hogg, D. F., Clunie, G. J. A., and Hardie, I. R.: Retained biliary calculi: Removal by a simple nonoperative technique, Ann. Surg. 180:247, 1974. 8. Faloon, W. W.: Gallstone prophylaxis and therapy: Report of a conference, Am. J. Dig. Dis. 19:81, 1974. 9. Gardner, B., Dennis, C. R., and Patti, J.: Current status ofheparin dissolution ofgallstones, Am. J. Surg. 130:293, 1975. 10. ttalsted, W. S.: Discussion of paper by J. M. T. Finney, Excision of the Gallbladder, Johns Hopkins Hosp. Bull. 13:54, 1902. 11. Magarey, C. J.: Removal of retained bile duct calculus without operation, Br. J. Surg. 56:312, 1969. 12. Mazzariello, R.: Removal of residualbiliary tract calculi without reoperation, Surgery 69:566, 1970. 13. Mazzariello, R.: Residual Biliary Tract Stones: Nonoperative Treatment of 19

570 Patients, in Nyhus, L. M. (ed.), Surgery Annual (New York: AppletonCentury-Crofts, Inc., 1976), VoL 8, p. 113. 14. Mondet, A.: Tecnica de la extracci6n incruenta de los cSlculos en la litiasis residual del coledoco, Bol. Soc. Cir. Buenos Aires 46:278, 1962. 15. Way, L. W., Admirand, W. H., and Dunphy, J. E.: Management ofcholedocholithiasis, Ann. Surg. 176:347, 1972. 16. Way, L. W., and Motson, R. W.: Dissolution of Retained Common Duct Stones; Symposium on Gallstones, Part III, in Longmire, W. P., Jr. (ed.): Advances in Surgery (Chicago: Year Book Medical Publishers, Inc., 1976), Vol. 10, p. 99.

LESIONS OF THE SPHINCTER OF ODDI One of the most controversial problems of biliary tract surgery relates to the correlation of clinical symptoms with dysfunction of the sphincter of Oddi. The problem is made even more complex by the fact that symptoms of sphincter dysfunction may be related to the biliary tract, to the pancreas or to both systems. Nardi ~~stated, "Fibrosis, inflammation, or spasm of the sphincter of Oddi produces a discrete clinical syndrome which is not well recognized. Patients with this condition complain of recurrent and persistent bouts of epigastric pain frequently radiating to the back. They have often been subjected to cholecystectomy, hiatus hernia repair, and other operations without relief of their symptoms. Physical examination is not revealing, and routine radiologic and laboratory examinations are frequently normal." Nardi continued, "It is important to realize that partial or intermittent obstruction to the outflow ofbiliary and pancreatic secretions by the sphincter of Oddi at the ampulla of Vater may produce these bouts of recurrent abdominal pain and that this obstruction, and the symptoms as well, may more often than not be relieved by operation." Many may feel that such a straightforward analysis of the problem is a much too simplistic answer to one of the most difficult differential diagnostic problems with which the abdominal surgeon is faced. Certainly if one resorts to sphincteroplasty in treating the majority of the patients described a b o v e - t h a t is, with recurrent epigastric pain unrelieved by previous operation and with negative routine radiologic and laboratory examinat i o n s - the results will be extremely disappointing. Are there, then, any patients whose biliary tract symptoms are related to sphincter of Oddi obstruction? If so, how may they be detected? Most surgeons concerned with hepatopancreatic problems are convinced that dysfunction of the sphincter of Oddi can be responsible for the clinical symptoms outlined by Nardi. How frequently this condition occurs, what objective tests can be used to select those patients whose symptoms are specifically related to a malfunctioning sphincter and the reliability of currently available tests are unsolved problems that dictate an extremely cautious surgical approach to this problem. 20

BACKGROUND DISCUSSION Since McBurney's9initial report in i891, division of the sphincter of Oddi has been a commonlyutilized procedure for the removal of common duct stones impacted in or near the ampulla of Vater. Doubilet and Mulholland3 for a time popularized the use of sphincterotomy in the treatment of chronic pancreatitis, but the unreliable results of the procedure in the hands of most surgeons led to general dissatisfaction with and abandonment of the operation. Jones and Smith7 revived interest in the procedure for certain forms of recurrent pancreatitis and introduced the term sphincteroplasty rather than sphincterotomy. They emphasized the needto divide completely the entire sphincteric mechanism, which normally varies in size from 10 to 30 mm, depending upon the obliquity of the bile and pancreatic ducts as they pass through the duodenal wall. They recommended an incision in the lower end of the common bile duct from the orifice of the papilla through the entire thickness of the duodenal wail, with careful suture approximation of the duodenal and bile duct mucosa. Jones and Smith recognized that the procedure would not benefit patients with pancreatitis who had intraductal obstruction and that the choiceof operation for pancreatitis should be determined on the basis of a pancreatic ductogram and the condition of the pancreatic parenchyma. Only that small group of patients with evidence of obstruction due to the constricting action of the sphincteric mechanism and without gross evidence of advanced fibrosis and parenchyma] destruction were considered to be candidates for sphincteroplasty. Jones and Smith's subsequent experience indicated that the procedure was of greatest value for the prophylaxis and treatment of residual common duct stones. Jones and Smith8studied 268 sphincteroplasty procedures performed over a 19-year period. Ninety-eight were carried out for acute recurrent pancreatitis, with 87.7% of the patients reported as improved or asymptomatic, and 170 were performed for obstruction of the biliary duct, with relief obtained in all patients. Multiple commor/duct stones were present in 161 patients from the total group. (Twenty of these were seen originally with residual stones, and 5 had irremovable hepatic duct calculi.) Neither residual nor recurrent stones developedpostoperatively in any of these patients. Twenty-two patients with biliary obstruction attributable to ampu]lary stenosis alone, without common duct stones, were all apparently relieved of their symptomsby sphincterop]asty. Although the number of cases was small, results were least satisfactory in the nonealculous, recurrent pancreatitis group. Unimproved were 14% of alcoholic patients and 30% of nonalcoholic patients. 21

Twenty of the 60 patients on whom Rutledge '1 performed sphincteroplasty had stones in the lower end of the common bile duct; primary stones or sludge were present in 10 and multiple secondary stones "in 4. Failure of passage of no. 3 or 4 Bakes dilators was the indication for division of the sphincter in 18 patients. The patients usually also had large bile ducts, abnormal operative cholangiograms or elevated alkaline phosphatase levels to indicate partial biliary obstruction. Three also had positive morphine-prostigmine tests. Fifty-two of the 60 procedures were primary operations. There were no serious postoperative complications, and 49 of these 52 patients were classified as having good or excellent results. Three patients, all of whom had positive morphine-Prostigmin tests preoperatively as the main indication for operation, continued to complain of varying degrees of abdominal pain but did not have objective evidence of bile duct problems. Of particular interest in the report of Rutledge were 8 patients who had sphincteroplasty as a secondary procedure an average of 17 years after their original operations. All of these patients had pain similar to their original gallbladder pain. One patient was jaundiced. At operation the common bile ducts varied from 13 to 18 mm i n diameter. A no. 4 Bakes dilator failed to pass the sphincter in all 8 patients. Six of the 8 were symptom free following sphincteroplasty. Two still had abdominal pain but did not have objective evidence of duct obstruction. Rutledge prefers sphincteroplasty to choledochoduodenostomy because it provides dependent drainage of the bile duct, ready access to impacted ampullary stones, and good visualization of the papilla and distal ends of the bile and pancreatic ducts to ensure that no pathologic condition will be overlooked. The greatest value has been in preventing residual or recurrent bile duct stones and in relieving true ampullary stenosis. Choledochoduodenostomy also yielded good results in Rutledge's series. It is the simpler procedure, and there is less manipulation of the pancreas and less postoperative pancreatitis. It more adequately drains a huge bile duct and can be used for unusually long strictures of the distal duct as well as in cases where a duodenal diverticulum is immediately adjacent to the ampulla. The distal bypassed segment of the bile duct, however, can become filled with inspissated bile and debris and cause symptoms. Five of the 60 patients who had sphincteroplasty still had varying degrees of pain. In all 5 a positive morphine-Prostigmin test had been a prime indication for operation. Rutledge believes that these failures represent more a fault of patient selection than of the operation itself. Stuart and Hoerr, TM in comparing the results of 44 choledochoduodenostomies with the results of 46 sphincterotomies, found a higher incidence of satisfactory results with the lateral duct-en22

teric anastomosis. Seven of the patients who underwent sphincterotomy required additional operations, 4 for continuing or new pancreatitis and 3 for retained or recurrent calculi in the common duct. However, the illustrations accompanying Stuart and Hoerr's article suggest that their sphincterotomy procedure would not have divided the entire sphincteric mechanism. Fortyeight of their patients, of whom 40 had associated choledocholithiasis or pancreatitis, were treated for stenosis at the ampulla of Vater. Results for this specific group of cases are not identified. White '4 generally prefers sphincteroplasty to choledochoduodenostomy, although the latter procedure is used in older and more debilitated patients and when there is stenosis of the intrapancreatic portion of the bile duct. In his series of 129 patients undergoing sphinct.eroplasty procedures, only 7 of 36 patients who were acute or chronic alcoholics were completely relieved of pain. Some degree of success was achieved in treating all but 5 of 62 patients with complications of gallstone disease. The results in 22 patients operated upon for the postcholecystectomy syndrome are not specifically listed. In the discussion of White's paper, Boyden2 stressed that the indications for, and results from, sphincteroplasty must be identified with the primary pathologic problem for which the procedure is being considered. Division of the sphincter i s indicated for fibrosis of the sphincter or true ampullary stenosis. Boyden indicated that this condition is much rarer than some reports would suggest. ~ailure of a small dilator to pass through the sphincter does not alone confirm the diagnosis of ampullary stenosis. Fibrosis should be proved by microscopic evidence, and spasm should not be confused with stenosis. In the various reports reviewed above, the majority of patients were first seen with common duct stones, history of jaundice with elevations of serum bilirubin and alkaline phosphatase, and chills and fever, thus demonstrating a clear relation between the common bile duct disease and the patient's symptoms. Operative cleansing of the biliary system in such cases is imperative. The only question to be raised, when continued decompression of the biliary tract seems indicated, concerns the preferred procedure, sphincteroplasty or choledochoduodenostomy. It is clear from the foregoing reports that opinions vary as to the merits of the two procedures. Most authors agree that choledochoduodenostomyis a simpler procedure, with less chance of operative complications. Sphincteroplasty has the advantage of (1) providing for direct examination of the papilla and ducts for tumor or other concealed abnormality and (2) obviating the "sump syndrome" of biliary debris filling the lower bypassed segment of the common duct. With these.points in mind, the surgeon must select the procedure he believes to be best suited for his individu23

al patient. In general, for primary bile duct stones or unusually large numbers of secondary common duct stones we have utilized a choledochoduodenostomy. Jones and Smith 8 have described in detail the rare case of pancreatitis that will be benefited by sphincteroplasty. The role of sphincteroplasty in the treatment of sphincteric stenosis or spasm remains an unresolved enigma. In a study of the pathologic changes in the papilla of Vater and a correlation with the clinical diagnosis and operative results, Acosta e t al. 1 noted that sphincterotomy was a much more effective procedure when performed in patients with biliary tract disease with or without pancreatitis than in patients with pancreatitis alone. The association of papillary fibrosis with dilatation and thickening of the common bile duct and the good result of sphincterotomy suggested a cause-effect relationship between pathologic and operative findings. Difficulty in the passage of a small dilator through the ampulla was found to be a less reliable criterion than the presence of dilatation and thickening of the common bile duct in assessing pathologic changes in the papillary sphincter. Of the four reports herein reviewed, the results of the sphincteroplasty for ampullary stenosis'are recorded in only two. Jones and Smith 8 performed sphincteroplasty for treatment of ampullary stenosis in 22 patients, with apparently satisfactory results in all. In a somewhat more detailed report, Rutledge I1 recorded 6 of 8 patients free from symptoms followingsphincteroplasty; 2 patients continued to have pain without any demonstrable organlC abnormahty. DIAGNOSIS As already noted, symptoms referrable to ampullary stenosis are usually associated with mild transient rises in serum bilirubin or alkaline phosphatase levels. Chills and fever may or may not be present; usually they are absent. In part, the diagnosis is one of exclusion, and the more common causes of abdominal pain (such as peptic ulcer, gallstones, hiatus hernia and renal calculi) must be ruled out by meticulously performed upper gastrointestinal x-ray series, cholecystograms and intravenous pyelograms. Intravenous cholangiograms with tomographic films of the ductal system are helpful if they demonstrate dilatation of the duct with delay of the dye at the sphincter and poor emptying of the duct into the duodenum~ The evocative test of Nardi (the morphine-Prostigmin test), in which pancreatic secretion is stimulated by the injection of I mg of Prostigmin methylsulfate just as sphincter of Oddi spasm or obstruction "is enhance~ by the injection of 10 mg morphine, is widely used as a diagnostic test of sphincter obstruction. Serum levels of amylase and lipase are determined before the test and at 24

1-hour intervals for 4 hours after the injection. Interpretation of the results depends on the extent of the enzyme elevations and, of equal importance, on whether or not the patient's symptoms are reproduced. Nardi considers elevation of the amylase to a level twice normal or above or reproduction of the clinical symptoms to be a positive reaction. In our experience, marked elevations of serum enzymes have been correlated with satisfactory response to sphincteroplasty. Reproduction of the patient's clinical symptoms or slight-to-moderate elevations of enzyme levels have not been reliable guides. Rutledge found the test to be unreliable in all five of his unsuccessful cases. Endoscopic retrograde cholangiopancreatography has been an important diagnostic aid in evaluating the condition of the papilla of Vater and the sphincter of Oddi prior to operation. Direct vision of the papilla will detect any undue prominence due to hypertrophy, edema or excessive scar tissue. Cannulation of the orifice gives some indication of the diameter of its lumen, and retrograde cholangiograms will give information regarding the diameter of the duct, the appearance of its distal end and whether or not there is any obstructive delay in the emptying of the duct. Periampullary duodenal diverticula may also be associated with sphincter malfunction and their presence considered another bit of presumptive evidence suggesting that the patient's symptoms m a y be related to an abnormal function of the distal common bile duct and its related structures (Fig. 8). We a:oTee with Boyden ~ that passage of dilators at operation to Fig 8 . - I n man, 48, with persistent right upper quadrant pain following cholecystectomy, ERCP demonstrates mild dilatation of the common bile duct with dilatation of the distal end, delayed emptying into the duodenum and mild dilatation of the pancreatic duct. Note the adjacent duodenal diverticulum. Symptoms were relieved by sphincteroplasty.

Dilated common bile duct I I

ic \

i~/.~_~--Duodenal I diverticulum I

No drainage 25

determine the presence of sphincter stenosis is not only an unreliable indicator but also potentially hazardous. Perforation of the duct wall with passage of the dilator into the pancreas may cause a severe, even fatal, pancreatitis. Ultrasound scans to identify intra-abdominal structures, both normal and abnormal, are becoming increasingly precise. As yet, however, their chief value in the differential diagnosis of this disease is to rule out other abnormalities that might be responsible for upper abdominal symptoms. Changes in the diameter and configuration of the common duct and pancreas, as well as the presence of intraductal stones, m a y be reliably detected by this technique. OPERATIVE MANAGEMENT

The diagnosis of sphincteric obstruction should be confirmed intraoperatively prior to sphincteroplasty or other appropriate procedures. A thorough general examination of the intra-abdominal viscera to rule out any previously undiagnosed cause of the patient's symptoms should be followed by operative cholangiogram, unless the preoperative findings of sphincter obstruction are so convincing that the surgeon feels justified in proceeding without further confirmatory evaluation. The gallbladder is removed, if cholecystectomy has not been previously performed, and a catheter inserted through the stump of the cystic duct to obtain the cholangiogram. The roentgenogram is carefully inspected for-evidence of small calculi in the distal end of the duct, and any delay in passage of the dye into the duodenum is noted. In addition, rate of flow through the common bile duct and manometric studies have been utilized in an attempt to define more accurately sphincter of Oddi dysfunction. White e t al. ~3 defined normal resting pressure as 9 - 1 5 cm of water and normal flow rate as greater than 10 ml/minute at a pressure of 30 cm of water. Lower flow ratio and higher pressures found in 15 of the first 206 consecutive patients tested were the only indication of Sphincteric or common duct disease. These flow and pressure changes were associated with multiple small stones not visualized on the usual cholangiograms and with fibrosis and s p a s m of the sphincter treated by sphincteroplasty. Yvergneaux e t a l . ~5 in Belgium report utilization of a combination manometric and x-ray cholangiographic technique in which the dye is injected u n d e r specific pressures and x-ray studi.es are made both with conventional film techniques and by placing dental films directly beneath the bile duct. In a series of 1,150 such radiologic-manometric studies, '~benign Oddi stenosis" was diagnosed in 65 cases (5.7%). In these 65 cases.there was often a causative agent, such as lithiasis, debris or ministones (46 cases); ulcer in the second portion of the duodenum (2 cases); inflammation in periampul26

lary diverticulosis (2 cases). When the serum bilirubin was significantly elevated, the obstruction was never due to benign Oddi stenosis. The authors note that the incidence of benign Oddi stenosis has been reported to vary widely, between 2% and 33.5% of biliary tract operations. Finally, the technique reported by Hess s evaluated "opening pressure" and flow pressures by means of manometric dye cholangiograms performed under fluoroscopic control. After completion of the cholangiographic studies, the hepatic flexure of the colon is freed, the duodenum is mobilized by incising the lateral peritoneal reflection and the papilla is palpated through the intact lateral duodenal wall. Having located the papilla, an appropriate longitudinal incision is made through the lateral duodenum and a small plastic catheter introduced into the ampulla, whence it will usually pass directly into the duct of Wirsung. Abnormalities of the duct of Wirsung demonstrated by pancreatogram will, if present, support the need for sphincteroplasty. The technical principles of sphincteroplasty, as described by Jones et al., 6 are followed, with division of the sphincter and infundibular region of the common bile duct until the opening is equal to the maximum diameter of the duct (Fig,' 9). This usually entails extending the incision up the common duct for a distance of 2-2.5 cm, completely through the wall of the duodenum. The orifice of the duct of Wirsung must be identified; if the pancreatogram has demonstrated the duct to be abnormal, its orifice should also be incised. The duodenal and common duct mucosa are careFig 9.--Technique of sphincteroplasty after Jones and Smith. Incision of the sphincter of Oddi mechanism through the duodenal wall with suture of duct and duodenal mucosa. The orifice of the pancreatic duct is visualized and protected. The important apex suture carefully approximates the duct and duodenal walls at the upper end of the incision. I

Pencreotic duct 'd

'/

Apex _ sufure~

"~

i~ll~

,.,,

27

fully sutured along both sides of the incision, with particular attention to the angle at the apex where the incision m a y extend beyond the wall of the duodenum into the pancreas. Care must be taken in the placement of clamps and sutures not to occlude or compromise the pancreatic duct orifice. If the common duct has been opened, a short-limb T tube is placed in the duct, and the duodenotomy is closed longitudinally in 2 layers. Postoperative edema at the site of the sphincteroplasty and duodenotomy is sufficient to cause (1) a temporary elevation of the serum amylase, alkaline phosphatase and serum bilirubin and (2) a variable degree and persistence of gastric retention. For this reason, gastrostomy has been advocated, but has been used by us only in selected cases. Duodenotomy and sphincteroplasty are operations t h a t carry morbidity and mortality risks. They should not be undertaken without careful evaluation of the risks in relation to the potential benefits to the patient. In the experienced hands of Jones and associates, 6 the mortality was 1.24% and morbidity 4.9% in 241 patients. H a f f a n d Torma 4 reported 9 complications and no deaths in 23 patients. A postoperative abscess or fistula developed in 5 patients, and there were 3 deaths in the 129 patients reported by White '3 in sphincteroplasties performed for a variety of conditions. SUMMARY Stenosis of the sphincter of Oddi without demonstrable common duct stones or debris is a rare cause of recurrent episodic upper abdominal pain with limited specific physical findings. Objective evidence (for example, cholangiographic demonstration of common duct dilatation with delayed emptying into the duoden u m and elevations of serum bilirubin or alkaline phosphatase levels) should be detectable. The morphine-Prostigmin test is unreliable and should not be depended upon alone. Markedly positive tests may be considered as additional supportive evidence of Stenosis. Of the special preoperative diagnostic tests available, ERCP seems to hold the most promise. Manometric and cholangiographic studies made at operation m a y also be helpful in properly identifying patients who m a y be helped by sphincteroplasty. Sphincteroplasty is a procedure with potentially serious risk t h a t should be used with utmost discrimination and care. REFERENCES 1. Acosta,J. M., Civantos, F., Nardi, G. L., and Castleman, B.: Fibrosis of the papilla of Vater, Surg. Gynecol.Obstet. 124:787, 1967. 2. Boyden,A. hi.: Discussion!p White,T. T.,'~ Am. J. Surg. 126:165, 1973. 3. Doubilet, H., and Mulholland, J. H.: Eight-year study of pancreatitis and sphincterotomy,J.A.M.A.160:521, 1956. 28

4. Haft, R. C., and Torma, M. J.: Oddi sphincteroplasty in the management of complicated biliary and pancreatic disease, Am. J. Surg. 129:509, 1975. 5. Hess, W.: Surgery of the Biliary Passages and the Pancreas (Princeton: D. Van Nostrand Company, 1965). 6. Jones, S. A., Steedman, R. A., Keller, T. B., and Smith, L. L.: Transduodenal sphincteroplasty (not sphincterotomy) for biliary and pancreatic disease, Am. J. Surg. 118:292, 1969. 7. Jones, S.. A., and Smith, L. L.: Transduodenal sphincteroplasty for recurrent pancreatitis: A preliminary report, Ann. Surg. 136:937, 1952. 8. Jones, S. A., and Smith, L. L.: A reappraisal ofsphincteroplasty (not sphincterotomy), Surgery 71:565, 1972. 9. McBurney, C. L.: Section of the intestine for the removal of a gallstone, N.Y. State J. Med. 53:520, 1891. 10. Nardi, G. L.: Papillitis and stenosis ofthe sphincter of Oddi, Surg. Clin. North Am. 53:1149, 1973. 11. Rutledge, R. H.: Sphincteroplasty and choledochoduodenostomy for benign biliary obstructions, Ann. Surg. 183:476, 1976. 12. Stuart, hi., and Hoerr, S. 0.: Late results of side to side choledochoduodenostomy and of transduodenal sphincterotomy for benign disorders, Am. J. Surg. 123:67, 1972. 13. White, T. T., Waisman, H., Hopton, D., and Kavlie, H.: Radiomanometry flow rates and cholangiogram in evaluating common bile duct pathology, 220 cases, Am. J. Surg. 123:73, 1972. 14. White, T. T.: Indications for sphincteroplasty as opposed to choledochoduodenostomy, Am. J. Surg. 126:165, 1973. 15. Yvergneaux, J. P., Bouwens, E., and Yvergneaux, E.: Diagnostic de la stdnose oddienne benigne dans une sdrie homog6ne de 1150 interventions biliaires sous radiomanometrie, Ann. Chir. 28:545, 1974.

CANCER OF THE BILE DUCT Another of the perplexing and frustrating causes of obstructive jaundice is carcinoma of the bile duct. About 50% of these lesions occur high in the hilus of the liver, involve the right or left hepatic duct or their confluence and are sufficiently well localized to make them difficult to visualize at operation. Once the site of obstruction has been identified, histologic confirmation of the malignant nature of the lesion is difficult to obtain, and the positive differentiation from sclerosing cholangitis may have to await the eventual outcome of the case. The tumors, although small and apparently well localized, usually involve the adjacent blood s u p ply of the liver and frequently extend into the parenchyma, making complete excision of the tumor impossible without either extensive resection of the liver and its component blood supply or hepatic transplantation. On the basis of their gross characteristics, bile duct carcinomas, more than 90% of which are adenocarcinomas, may be divided into 3 types: 1. Local or nodular. The tumor forms a small, well-localized, firm mass involving some localized portion of the duct system. 2. Diffuse. The wall of the duct is thickened over an extensive area, the lumen is narrowed, and the surrounding tissues of the 29

hepatoduodenal ligament are inflamed. It is this type of tumor that can be so difficult to differentiate from sclerosing cholangitis. 3. P a p i l l a r y . This tumor grows within the lumen of the duct. Endoscopic examination of the lumen of the duct at operation m a y reveal multiple sites of tumor growth. Tompkins et a l 2 recently reported a series of 5 cases of bile duct carcinoma in which the lumen of the ductal system was examined by means of the choledochoscope. Multiple tumors were found in the ducts of 4 patients who would have been judged by conventional criteria to be curable. The observation that these tumors may frequently b e multifocal m a y also explain in part the poor results attained with tumor excision in the past. Certainly, careful endoscopic examination is indicated before embarking on any radical surgical resection for bile duct carcinoma. Although the majority of these patients die within 2 years, regardless of the method of treatment, the symptoms until the terminal stage are related solely to biliary obstruction. Excellent palliation can be obtained by a variety of methods that restore bile flow. There m a y be as many as 4,500 new cases of primary bile duct carcinoma in this country this year. 2 The tumor is more frequent in men than in women, with a 3:2 ratio. The age range has been reported to be 2 3 - 76 years with an average in the early 60s. The principal signs and symptoms are those of obstructive jaundice, but there is nothing unique in the clinical picture to suggest that the obstructive process m a y be malignant. Sako et al. 7 described jaundice in 90% of their patients, usually occurring within 4 months before admission and becoming progressively more severe. Pain occurred in approximately one half of the cases. Often severe pruritus is the most distressing symptom. Anorexia and weight loss occur as the disease progresses. Cholang i t i s is a common complication after operation, b u t the signs thereof (chills and fever) are rare until the biliary tract has been opened surgically. Approximately one half of the 63 patients in our series ~ consulted a physician within the first 3 months after onset of symptoms, and about one half received some definitive treatment within 4 months. Abnormal physical signs were noted to be few in the series of 103 patients reported by Ross et al. 6 Enlargement of the liver,.a palpable gallbladder and, in the later stages, signs of portal hypertension were noted. Elevation of the serum bilirubin and alkaline phosphatase levels is routinely present, b u t there are no specific laboratory abnormalities. SPECIAL DIAGNOSTIC TESTS

The most helpful of the preoperative diagnostic studies has been the percutaneous transhepatic cholangiogram. With the 30

technique introduced by Ohto and Tsuchiya 4 and Okuda et al., ~ the intrahepatic biliary system has been demonstrated in 100% of our patients whose ducts were obstructed and in 60% of those with a nonobstructed intrahepatic biliary system. Demonstration of a dilated ductal system above a constricting obstructing lesion without filling defects in a patient who has not been operated upon is evidence of a neoplastic obstruction. In a patient on this continent, if the lesion is in the common hepatic duct or above, it will almost certainly be a bile duct carcinoma. Endoscopic retrograde choledochopancreatography is also a very useful diagnostic procedure. The ampulla of Vater can be cannulated and the bile or pancreatic ducts (or both) visualized by radiography in 7 5 - 96% ofcases. Our plan of investigation is to perform ultrasonography and ERCP in the patient with unspecified obstructive jaundice after the basic laboratory and radiologic procedures have been performed. If the papilla cannot be cannulated or if the ductal system is not adequately visualized, a percutaneous transhepatic cholangiogram is considered. Liver scans and arteriograms have been of little value. TREATMENT

The treatment and prognosis of bile duct tumors are related to the location of the tumor in the ductal system. Division of the extrahepatic biliary system into three areas has been suggested (Fig. 10).l TUMORS

OF THE DISTAL THIRD.--Tumors located in the intra-

pancreatic portion of the c o m m o n duct are generally grouped Clinically with periampullary tumors. Differentiation of this tumor from the far more c o m m o n carcinoma of the head of the pancreas m a y be difficult.However, because of their location, tumors of the intrapancreatic duct produce jaundice early. They a r e s l o w to metastasize and remain localized. Eighteen of the 77 malignant bile duct tumors reported by Warren et al. 1~ were located in the intrapancreatic common duct. Fifteen patients were treated by pancreaticoduodenectomy, 9 of whom had survived for more than 1 year and 2 for more than 3 years at the time of their report. Eleven of our own 13 patients with distal-third lesions were operated upon. 3 A duct-enteric bypass was utilized for 1 patient, a cholecystojejunostomy for another; pancreaticoduodenectomy was performed for the other 9 patients (Fig. 11). Jaundice was relieved, and palliation was considered good or excellent in all 11 patients. Of the 8 patients who had died at the time of our report, 5 had survived for more thah 1 year; 5 were alive from 1 to 15 years after operation. The en bloc type of resection that is possible 31

Lower Third

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Fig 10.-Localized bile duct carcinoma treatment is related to the position of the tumor in the ductal system. The extrahepatic biliary tree may be roughly divided into thirds. The upper third, where one half of the tumors arise, includes the hepatic ducts, their confluence and the common hepatic duct. The middle thi{d of the system includes the section between the common hepatic duct and intrapancreatic portions of the common duct. The lower third is composed of the intrapancreatic portion of the common duct. Fig 11.-Excision of bile duct cancer. En bloc resection of bile duct cancer in the distal third of the duct by pancreaticoduodenectomy has given the best survival rate for these tumors. A shows the usual method of reconstruction. Unfortunately, en bloc resection can rarely be utilized in the treatment of tumors in other parts of the biliary system due to early involvement of the immediately adjacent inflow blood supply to the liver, i.e., the hepatic artery and the portal vein. B, if local excision of a tumor in the middle or upper third of the duct system can be performed, th e hepatic duct is anastomosed to a Roux-en-Y jejunal limb. A

./

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32

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with tumors in the intrapancreatic bile duct gives the best results in the treatment of bile duct carcinoma. Endoscopic evaluation of the ductal system at the time of operation may reduce the number of tumors considered for resection b u t will undoubtedly increase the survival rate of those patients whose tumors are resected. TUMORS OF THE MIDDLE THIRD.--In 13 of our patients the bile duct tumor was classified as occupying the middle third of the duct system. Three of these patients were treated by dilatation of the tumor and T-tube intubation of the duct. Duct-enteric bypass was performed in 3 cases. Resection of the tumor and duct-enteric anastomosis was possible in 4 patients; all other patients were treated with palliative bypass procedures. Of our 13 patients, jaundice was relieved in 11 and partially relieved in 2. Palliation was considered excellent in 3 patients, good in 7 and fair in 1. Six of them survived for 3 years or more and 1 for more than 11 years. TUMORS OF THE HEPATIC DUCTS (UPPER-THIRD TUMORS). - - A s

in tumors of the middle third, the first problem to be faced at operation for tumors of the hepatic ducts is location of the site of obstruction and identification of the true nature of the lesion. In 9 of the 13 cases recorded by Klatskin I and 8 of our 25 cases 3 the cause of the biliary obstruction was not identified at the initial operative biliary exploration for obstructive jaundice. In 17 other patients in our series undergoing preliminary operations, a neoplasm waa suspected b u t not proved, either because a biopsy was not taken or the biopsy did not contain tumor. When the site of the obstruction has been localized by preoperative cholangiograms and when the intrahepatic ducts above this area are uniformly dilated, the obstructing lesion is most certainly malignant (Fig: 12). The majority of tumors that occur in this area will not be resectable because of (1) their extension up into the parenchyma of the liver, (2) their involvement of the hepatic blood supply (Fig. 13) or (3) their widely scattered multicentric location inside the duct. We were able'to resect the tumor in this region in only 6 of 33 patients, and in 2 of these, major hepatic resections were combined with the local tumor excision. Duct-enteric bypass could be performed in only 3 patients. Intrahepatic cholangiojejunostomy was performed 8 times. The results of this procedure were generally poor, as the tumor invariably obstructed the confluence of the left and right hepatic ducts. The most frequently performed procedure was dilatation of the site of obstruction and intubation of the ducts above and below the tumor, usually with a T tube. The U-tube technique described by Terblanche e t al. s has been utili'zed in recent cSses. A long tube is passed through the tumor into the intrahepatic duct and out the liver parenchyma and anterior abdominal wall to the outside (Fig. 14). Through-and33

Fig 12.-Cholangiogram of a patient showing a typical uniformly dilated intrahepatic duct with stenosis at the confluence of the left and right hepatic ducts. In a patient without a previous history of an operation in the right upper quadiant, such a cholangiogram is pathognomonic of bile duct cancer. (Figs. 12 and 13 from Longmire, W; P., Jr.: Tumors of the Extrahepatic Biliary Radicals, in Hickey, R. C. (ed.): Current Problems in Cancer (Chicago: Year Book Medicat Publishers, Inc., August, 1976). Fig 13.-Cross section of hepatoduodenal ligament indicates how a small, well-localized bile duct carcinoma in the middle and upper thirds of the ductal system quickly involves the walls of the adjacent hepatic artery and portal vein. Ao IVC

of

34

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Fig 14.-Transhepatic intubation of an obstructing unresectable carcinoma at the confluence of the hepatic duct via a Roux-en-Y anastomosis of a jejunal limb to the ductal system below the main obstruction (after Terblanche). Metal clips are placed at the site of the tumor to direct postoperative irradiation.

through irrigation permits cleansing of the lumen of the tube. When both ends are brought to the outside, the tube can be replaced by attaching another tube to one e n d and p u l l i n g it through the established sinus tract as the old tube is removed. This procedure has been combined with a hepatico-Roux-en-Y jejunostomy when the anastomosis could be made above the major site of obstruction; the lower end of the U tube is then brought down the jejunal limb through the anterior abdominal wall. We have also been encouraged with the results of irradiation concentrated to the site of these localized tumors after they have been intubated and the site of the tumor marked with metal clips at operation. The results of chemotherapy have thus far been disappointing. Jaundice was relieved in 15 of our patients with tumors of the upper third of the ductal system. Partial relief was obtained in 9 and no relief in 6. At the time of our report 5 patients were alive, 4 of them from 2 to 71/2 months and 1 for 41/2 years after operation. In summary, carcinoma of the bile ducts is an unusual b u t not rare cause of obstructive jaundice. Characteristic uniform dilatation of the intrahepatic ductal system above the site of obstruction as demonstrated by cholangiography is a most helpful diagnostic sign. Positive diagnosis by gross appearance and histologic confirmation are difficult to obtain. Treatment depends upon the location of the tumor. En bloc resection of the most distal tumors gives the best results. The more proximal tumors can rarely be excised, b u t acceptable palliation can be achieved by intubation 35

of the obstructed duct. Extension of the period of palliation by localized irradiation appears promising. REFERENCES 1. Klatskin, G.: Adenocarcinoma of the hepatic duct at its bifurcation within the porta hepatis, Am. J. Med. 38:241, 1965. 2. Longmire, W. P., Jr.: Tumors ofthe Extrahepatic Biliary Radicals, in Hickey, R. C. (ed.): Current Problems in Cancer (Chicago: Year Book Medical Publishers, Inc., August, 1976). 3. Longmire, W. P., Jr., McArthur, hi. S., Bastounis, E. A., and Hiatt, J.: Carcinoma of the extrahepatic biliary tract, Ann. Surg. 178:333, 1973. 4. Ohto, M., and Tsuchiya, Y.: Medical cholangiography. Technique and cases, Medicina 6:735, 1969. 5. Okuda, K., Tanikawa, R., Emura, T., et al.: Nonsurgical percutaneous transhepatic cholangiography-diagnostic significance in medical problems of the liver, Am. J. Dig. Dis. 19:21, 1974. 6. Ross, A. P., Braasch, J. W., and Warren, K. W.: Carcinoma of the proximal bile ducts, Surg. Gynecol. Obstet. 136:923, 1973. 7. Sako, S., Seitzinger, G. L., and Garside, E.: Carcinoma ofthe extrahepatic bile ducts. Review of the literature and report of six cases, Surgery 41:416, 1957. 8. Terblanche, J., Saunders, S. J., and Louw, J. H.: Prolonged palliation in carcinoma of the main hepatic duct junction, Surgery 71:720, 1972. 9. Tompkins, R. K., Johnson, J., Storm, F. K., and Longmire, W. P., Jr.: Operative endoscopy in the management of biliary tract neoplasms, Am. J. Surg. 132:174, 1976. 10. Warren, K. W., Mountain, J. C., and Lloyd-Jones, W.: Malignant tumors of the bile ducts, Br. J. Surg. 59:501, 1972.

SCLEROSING CHOLANGITIS One of the most confusing causes of extrahepatic biliary obstruction is sclerosing cholangitis (SC), a disease of the biliary system that may involve any part or all of the ducts from the intralobular ductules to the ampulla of Vater. The disease, when confined to the intrahepatic ductal system, is referred to by some authors as primary biliary cirrhosis (PBC) or cholangiolitic hepatitis: When the disease is discovered at operation to involve the extrahepaticbile ducts, it is called sclerosing cholangitis (Fig. 15). Most authors who have had an opportunity to analyze more than a few cases of this disease and to follow the patients over a period of years believe that although the disease may, when initially recognized, involve only a portion of the ductal system, it frequently progresses to involve all of the intra- and extrahepatic ducts. Caroli and Rosner 3 have classified chronic SC as (1) segmental sclerosing cholangitis with subcategories of (a) fibroinflammatory stenosis of the hepatic duct junction, (b) selective stenosis of the common hepatic duct and (c) sclerosing choledochitis; and (2) di~tse chronic sclerosing cholangitis. They indicate that, in early cases with little involvement of the intrahepatic ducts, surgical biliary drainage coupled with corticosteroid therapy may be the 36

--Sinusoid

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Fig 15.-Diagrammatic representation of sclerosing cholangitis and cholangiolitic-hepatitis as manifestations of a similar disease process affecting different po~rtions of the ductal system. W h e n the extrahepatic and major intrahepatic ducts are involved, the process is labeled sclerosing cholangifis. If the primary involvement is in the most distal intrahepatic portion of the ductal system, it is called cholangiolitic hepatitis. (The process may also be called cholestatic jaundice, or primary biliary cirrhosis.) (From Longmire, W. P., ,Jr.,and Rangel, D. M.: Difficult Problems Encountered in the Management of Biliary Obstruction Due to Stones and Other Benign Conditions, in Welch, C. E. (ed.): Advances in Surgery [Chicago: Year Book Medical Publishers,. Inc., 1970],

Vol. 4.)

best therapeutic approach. Grodsinsky e t a l 5 also suggested that several anatomical patterns of involvement of the extrahepatic biliary tract m a y occur. The walls of the ducts m a y be involved segmentally or diffusely, or the ducts may be involved in a fibrotic process that is primarily external to the ducts, for example, a pericholedochitis. The diffuse involvement of the extrahepatic biliary tract, as well as the intrahepatic ducts in certain of the cases, make the ultimate outlook poor. However, in many instances the disease is compatible with years of life. Thus, all possible avenues of mechanical relief of obstruction by operative procedures should be provided. Steroids are recommended when operative aid is not possible or only incompletely effective. Fee e t a l 4 noted that patients have been found at operation to have involvement of either the intra- or extrahepatic biliary sys37

tems. Later, at autopsy or at laparotomy, these same patients were found to have progressed to fibrosis of both the intra- and extrahepatic biliary tree. Fee and his associates compared the clinical pictures of 21 patients with SC with those of 14 patients with PBC. Females predominated in the PBC group, whereas the sex ratio was equal in the SC patients. In both groups, patients had chemical evidence of obstructive jaundice. The most common symptoms in both groups were pruritus, jaundice and right upper quadrant pain. Hepatic failure and hepatorenal syndrome were the most frequent causes of death in both series. At the time of their review, 43% of the PBC p a t i e n t s a n d 33% of the SC patients were dead. The mean time from onset of symptoms until death was 91/2 years for PBC patients and 21/2 years for patients in the SC group, indicating that, in general, patients with the diagnosis of PBC followed a more benign protracted course. Sherlock, 13in a discussion of chronic cholangitis, differentiated primary SC'from PBC by the presence of a positive mitochondrial immunofluorescent test and preponderance of female patients in the latter disease. Thorpe e t al. 14 in discussing primary SC associated with ulcerative colitis, stated that primary SC is a general disease of the biliary tree that can affect both the intra- and extrahepatic ducts as well as the gallbladder. The cause of the disease is unknown, although current opinion supports an autoimmune reaction in the wall of the duct rather than a bacterial or viral infectious process. Association of the disease with retroperitoneal fibrosis, Riedel's thyroiditis, ulcerative colitis and regional ileitis, as well as its occurrence in kidney transplant recipients, has been advanced as evidence in favor of the autoimmune mechanism in the etiology of the process. Unnecessary confusion has been added to this already puzzling disease by the inclusion of patients with thickened bile ducts due to a wide variety of other conditions. It is important to recognize that not every case of thickening of the wall of t h e common bile duct can properly be diagnosed as SC. In an attempt to define more clearly a specific pathologic and clinical entity, Holubitsky and McKenzie Glisted the following diagnostic criteria: (1) absence of previous operative t r a u m a to the biliary tract, (2) absence of calculi in the g a l l b l a d d e r and common duct, (3) sclerosis and stenosis involving all or almost all of the extrahepatic ducts and (4) exclusion of malignancy. Other authors 9 would exclude those cases associated with biliary cirrhosis, regional enteritis, ulcerative colitis, and retroperitoneal fibrosis. However, since the biliary tract disease in these cases follows the clinical and pathologic course of SC so closely, this exclusion does not seem justified. As indicated, the etiologic mechanism in these diseases may be related. Although reports have varied, the disease in collected series is most common in middle-aged male patients. In our previous re38

port of 21 patients, 8 the intrahepatic aspects of the disease were emphasized. There were 8 males and 13 females, and one half of the patients were 4 0 - 60 years of age at the time of their admission to the hospital. PATHOLOGY

The disease is characterized by a dense, diffuse thickening of the wall of all or a part of the common bile duct with a marked reduction in the diameter of its lumen. The contents of the duct are related to the degree of stenosis of the duct and to the extent of intrahepatic ductal involvement. In the severe case, a scant amount of dark, thick, sludgelike bile is present in the markedly stenotic duct lumen. In the less severe case, the bile is normal in color but reduced in amount. The extensive inflammation and fibrosis that occur in the submucosal fibrous connective tissue of the duct wall may extend into the surrounding tissues of the hepatoduodenal ligament, making identification of the duct itself extremely difficult. Enlarged, succulent regional lymph nodes are usually present. The basic histopathologic features of the disease in the liver are bile stasis, parenchymal injury, and portal tract involvement. In the earlier stages, focal necrosis, broadened portal tracts and acute and chronic inflammatory cell infiltrates are the characteristic changes. In the later stages, periportal fibrosis and bile stasis predominate. ~ CLINICAL COURSE

The disease has an insidious onset and, in general, follows a chronic progressive course with intermittent episodes of remission and exacerbation. At the onset, general indications of illness, such as fatigue, weakness and loss of weight, are common; fever is an unusual feature. Most patients complain of vague abdominal distress, b u t acute abdominal pain is uncommon. Pruritus and jaundice are the most common prominent symptoms. Pruritis frequently precedes the awareness of jaundice, at times by months. Enlargement of the liver and spleen was present in two thirds of our patients. The serum bilirubin and alkaline phosphatase determinations are elevated. The clinical course of these patients varies widely, and the general well-being of some during the protracted period of illness has been commented upon by several authors. 6, 8. ,2 However, other patients, particularly if the biliary tract becomes secondarily infected and bacterial cholangitis is superimposed upon the chronic process, experience a progressive decline of hepatic function and die within 2 - 3 years from the time of diagnosis. 39

DIAGNOSIS AND TREATMENT

The basic clinical problem is that of an insidious, recurrent, progressive obstructive jaundice with elevated serum bilirubin and alkaline phosphatase levels but generally well-preserved hepatocellular function. Special diagnostic tests for obstructive jaundice have been of limited value in the preoperative diagnosis of this disease. Radioactive isotope liver scans and ultrasound scans are nondiagnostic. Percutaneous transhepatic cholangiography is usually unsuccessful, due to the small size of the ducts and the thickening of their walls. One third of the patients reported by Fee e t al. 4 with SC had liver biopsy reports that were consistent with PBC. Endoscopic retrograde cholangiography has been reported to be of value in (1) suggesting the diagnosis of the disease before operation by demonstration of an extensively steo nosed and irregular ductal system without proximal dilatation and (2) documenting through repeated periodic examinations the course of the disease and the effectiveness of therapy. ~, '~ A cho!angiogram, performed either as a transhepatic preoperative study or as an intraoperative dye injection with complete filling of the intra- and extrahepatic ductal system, is one of the most reliable diagnostic procedures in identifying this disease process and differentiating it from the ever-present possibility of a sclerosing bile duct carcinoma. The typical diffuse narrowing of the ductal system with alternating areas of constriction and dilatation can Usually be readily differentiated from the obstruction of bile duct carcinoma with diffuse uniform dilatation of the proximal ductal system (Fig. 16). The final diagnosis, and particularly its differentiation from Fig 16.-T-tube cholangiogram and artist's sketch of alternating areas of constriction and dilatation of intra~- and extrahepatic ducts characteristic of sclerosing cholangitis.

40

sclerosing bile duct carcinoma, is dependent upon operative visualization of the duct with biopsy of the duct wall and detailed operative cholangiograms of high quality, with complete filling of the intra- and extrahepatic ducts. In some doubtful instances, the final diagnosis must await follow-up of the clinical course over a period of years. After a presumptive diagnosis of SC has been established, the time-honored mainstays of treatment have been prolonged T-tube drainage of the common bile duct and steroid therapy. Our experience with prolonged T-tube drainage has been far less satisfactory than that reported by others. The beneficial effects of bile drainage are thought to result from an interruption of the hepatoenteric cycle, which reduces the stimulus to bile production and lessens the bile pool in the body, thereby alleviating the jaundice and, most importantly, the pruritus. Oviedo et al. '~ reported upon 3 patients successfully treated with prolonged T-tube drainage. The disease process was of only a few days' or a week's duration in these cases. Although biopsy of the duct wall disclosed chronic inflammation and fibrosis, it might be questioned whether these were truly cases of chronic sclerosing cholangitis. In our experience, little bile flow occurs through the T tube in the cases where relief is most urgently needed, as the process extends into the intrahepatic system well above the T tube. The same interruption of the hepatoenteric cycle may usually be achieved with oral administration of cholestyramine. Finally, and quite important, the presence of the T tube will inevitably lead to bhcterial contamination of the biliary system which, once established, can never be eradicated, because of the underlying disease process. It has been our custom to insert a T tube at the time of duct exploration but to remove it at the end ofthe 2d week unless clamping of the tube over a 2-day period produces untoward results. Operative cholangiograms may indicate areas of localized stenosis, such as those described by Caroli and Rosnera and Grodsinsky et al., 5 in the extrahepatic ductal system with areas of dilatation above. Such patients may benefit sometimes for months or even for a few years from the creation of a duct-enteric anastomosis above the strictured area. The most important objective of operation, however, is to distinguish the true case of SC from the sclerosing carcinoma. As Altemeier et a l ? have stressed, many patients thought to have shown the changes characteristic of SC have, at operation, been found to have a malignant duct tumor. PROGNOSIS

The evaluation of therapy is exceedingly difficult due to the rarity of these cases and the capricious nature of their clinical 41

course. I n o u r p r e v i o u s l y r e v i e w e d cases, we could not d e m o n s t r a t e a n y c l e a r - c u t e v i d e n c e of cortisone effectiveness; h o w e v e r , t h e r e is a c o n s e n s u s t h a t cortisone will lessen the i n f l a m m a t o r y r e a c t i o n , a s s i s t in o p e n i n g . t h e b i l i a r y t r a c t a n d .provoke r e m i s sions, p a r t i c u l a r l y in " e a r l y cases." R e g a r d l e s s of t h e i r d e b a t a b l e efficacy, steroids a r e a l m o s t u n i v e r s a l l y a d m i n i s t e r e d d u r i n g a n a c u t e r e a c t i v a t i o n of t h e disease, a n d in s o m e p a t i e n t s , one h a s t h e clinical i m p r e s s i o n t h a t t h e y "do b e t t e r " on l o n g - t e r m s t e r o i d t h e r a p y . C h o l e s t y r a m i n e is h e l p f u l in r e d u c i n g p r u r i t u s . A n t i biotics a r e i n d i c a t e d w h e n t h e r e is e v i d e n c e of s u p e r i m p o s e d bact e r i a l cholangitis. I m m u n o s u p p r e s s i v e a g e n t s , s u c h as a z a t h i o p r i n e ( I m u r a n ) , h a v e also b e e n tried, w i t h q u e s t i o n a b l e effectiveness. T h e course of t h e b i l i a r y disease in p a t i e n t s w i t h o t h e r assoc i a t e d illnesses, s u c h as u l c e r a t i v e colitis a n d r e g i o n a l ileitis, is not b e n e f i t e d b y excision of t h e i n v o l v e d bowel. Most all w h o h a v e h a d occasion to follow t h e s e p a t i e n t s for long periods of t i m e h a v e come to t h e conclusion t h a t , r e g a r d l e s s of t h e t h e r a p y e m p l o y e d , s o o n e r or l a t e r t h e d i s e a s e is fatal, in m o s t p a t i e n t s w i t h i n 2 - 3 y e a r s . I n o t h e r p a t i e n t s , t h e d i s e a s e is m u c h m o r e b e n i g n , a n d p a t i e n t s w i t h c h r o n i c j a u n d i c e , e n l a r g e d livers, e l e v a t e d s e r u m lipids a n d c h o l e s t e r o l a n d m u l t i p l e s k i n x a n t h o m a s m a y occas i o n a l l y live 1 5 - 2 0 y e a r s in a r e s t r i c t e d b u t a c c e p t a b l e mode. REFERENCES 1. Abbruzzese, A. A.: Retrograde cholangiogTaphy and sclerosing eholangitis, Dig. Dis. 19:571, 1974. 2. Altemeier, W. A., Gall, E. A., Culbertson, W. R., and Inge, W. W.: Sclerosing carcinoma of the intrahepatic (hilar) bile ducts, Surgery 60:191, 1966. 3. Caroli, J., and Rosner, D.: Cholangitis, in Backus, H. L. (ed.): Gastroenterology (Philadelphia: W. B. Saunders Company, 1976), Vol. 3, p. 865. 4. Fee, H. J., Gewirtz, H., Schiller, J., and Longmire, W. P., Jr.: Sclerosing cholangitis and primary biliary cirrhosis-A disease spectrum? (Accepted for publication, Ann. Surg.) 5. Grodsinsky, C., Block, M. A., and Brush, B. E.: Varieties of sclerosing cholangitis, surgical significance, Bull. Soc. Int. Chir. 33:487, 1974. 6. Holubitsky, I. B., and McKenzie, A. D.: Primary sclerosing cholangitis of the extrahepatic bile ducts, Can. J. Surg. 7:277, 1964. 7. Levin, P., Joseph, W., Longmire, W. P., Jr., and Mellinkoff, S. M.: Choledochostomy in the treatment of intrahepatic cholangiolitic hepatitis, Am. Surg. 31:683, 1965. 8. Longmire, W. P., Jr., Joseph, W. L., Levin, P. hi., and Mellinkoff, S. hi.: Diagnosis and treatment of cholangiolitic hepatitis, Ann. Surg. 162:356, 1965. 9. Meyers, R. N., Cooper, J. H., and Padis, N.: Primary sclerosing cholangitis, Am. J. Gastroenterol. 53:327, 1970. 1O. Oviedo, M. A., Volkmer, D., and Scanlon, E. F.: Primary sclerosing cholangitis, Arch. Surg. 109;747, 1974. 11. Ruskin, R. B., Katon, R. M., Bilbao, hi. K., and Smith, F.: Evaluation of sclerosing cholangitis by endoscopic retrograde cholangiopancreatography, Arch. Intern. Med. 136:232, 1976. 12. Schwartz, S. I.: Primary sclerosing cholangitis: A disease revisited, Surg. Clin. North Am. 53:1161, 1973. 42

13. Sherlock, S.: Chronic cholangitides; etiology, diagnosis, and treatment, Br. Med. J. 3:515, 1968. 14. Thorpe, hi. E. C., Scheuer, P. J., and Sherlock, S.: Primary sclerosing cholangitis, the biliary tree, and ulcerative colitis, Gut 8:435, 1967.

IATROGENIC TRAUMA Benign stricture of the bile ducts most often arises from inadvertent injury of the duct during cholecystectomy. These injuries occur rarely, when one considers that cholecystectomy is the second most common intra-abdominal operation performed in this country, with 350,000 such procedures done each year. However, even occasional injuries, when totaled countrywide, involve a sizable number of patients. Almost a thousand such cases have been treated in one clinic in this country during a 27-year period. '7 In t h e majority of cases the cause of bile duct injury is not known. In 648 of the 958 cases reported by Warren and Jefferson 17 no cause of injury could be determined. Some of the known causes were massive bleeding (58 cases), difficult cholecystectomy (37 cases), and difficult gastrectomy (8 cases). In a series of 24 cases where details of the operation at the time of injury were known and the ~ubsequent course evaluated, G the injury was recognized in 9 patients at operation and unrecognized in 15. When the injury was recognized, abnormalities of the extrahepatic ducts were thought to have led to the injqry in 6 patients. In recent years at our clinic we have noted that the majorit y of injuries of the extrahepatic ducts that occurred at cholecystectomy have been associated with the frequently occurring anomalies of the system. In a series of 200 dissections of the extrahepatic biliary system, Flint s found only 69 that conformed to the arrangement of vessels and ducts as given in textbooks of anatomy (Fig. 17). The loss of the usual anatomical landmarks and the resultant confusion of the surgeon probably lead more frequently to excisions of sizable segments of the ductal system rather than to mere divisions or ligatures. Such excisions account for the large number of strictures that occur high in the hilus of the liver and involve the hepatic or common hepatic ducts. Injuries that would seem attributable to poor anesthesia, inadequate exposure and assistance, as well as to such time-honored causes as hemorrhage from cystic artery or tenting of the common duct, have been rare. I t m u s t be recognized that the diameter of the normal common duct m a y vary widely (from 2 mm to 1.5 cm) and that merely its small size cannot be considered in deciding whether or not the duct in question is the common bile duct. Such anomalies as low confluence of the hepatic ducts, drainage of the cystic duct into the right hepatic duct and confluence of the main right segmental 43

COMMONANOMALIESOFEXTRAHEPATISEGMENTAL C ANDLOBAR DUCTSOFSURGICALIMPORTANCE .~', R. segmental d u c t ~ ~ ~ _ /~X, \ . . lob= h+pot~c~o~t Segrnentol duct

+,,c ooc, ?:

•] f~_~

.x~ R. hepatic duct

-]

<,,,,c ooo,

.h~

L. hepatic duct

;/

c,,,,c'+

Low confluence of hep0tic ducts with cystic duct droining into common bite duct

Fig 17.--Variations in bile duct anatomy. The frequently occurring anatomical variations of the extrahepatic ducts lead to confusion of landmarks during cholecystectomy with resulting injury of the ductal system. One such anomaly that may confuse the surgeon is an extraparenchymal course of the dorsal caudal segmental duct from the right lobe to join the remainder of the right hepatic, the common hepatic or the cystic ducts. (From Longmire, W. P., Jr., and Tompkins, R. K.: Ann. Surg. 182:478, 1975.)

hepatic ducts outside of the liver are the most commonly encountered anomalies with which every abdominal surgeon should be thoroughly acquainted. Undoubtedly, the safest technique for elective cholecystectomy is that advocated by Glenn 4 a number of years ago. It involves identification of the cystic duct and artery, occlusion of these structures with a single throw of a ligature tied down sufficiently to occlude but not to crush and necrotize the tissue should placement of the ligature be in error, and then dissection of the gallbladder from its bed from the fundus down44

ward. Finally, the ligature of the duct and artery is completed after positive identification. One further condition that is likely to lead to injury occurs in the obese female whose panniculus and abdominal viscera tend to fall backward on either side of the vertebral column and midline structures when the patient is placed in the supine position on the operating table. Because of this the hepatoduodenal ligament and bile ducts are nearer the anterior abdominal wall than might be anticipated. Under such circumstances, the common duct is encountered earlier in the dissection .than planned and m a y be divided under the impression that it represents a part of the cystic duct. If an injury occurs and the mishap is recognized, how should the surgeon proceed? First, it is probably advisable for him to seek additional experienced surgical assistance for the completion of the operation because of the unanticipated judgmental and technical decisions to be made under particularly stressful circumstances. If less than 1 cm of the duct is missing, a meticulous end-to-end repair should be performed with a T tube placed through the anastomosis from above. If more than 1 cm of duct is missing, as is so often the case (in 15 of 20 cases, more than 1.5 cm of duct were found to be missing), G a duct-enteric-duodenal or jejunal anastomosis should be performed. In our review of 10 years ago, a successful result was achieved in only 1 of 8 immediate repairs. Delayed primary repairs were performed in 16 patients. Choledocho- or hepaticojejunostomy was successful in 8 patients and failed in 1. End-to-end duct repair was successful in 1 patient b u t failed in 4 other patients. Choledochoduodenostomy was successful in 2 patients. Thus, in the entire group a successful result was achieved with the initial repair in one-half the cases. A carefully stitched mucosa-to-mucosa anastomosis constructed without tension is the generally recommended method of bile duct repair. When a good repair has been achieved, the transanastomotic drainage tube remains in place for 1 - 3 months. For the difficult inaccurate anastomosis, the tube splinting the anastomotic line should be left in place for a year or more. This very important principle in bile duct repair was identified by McGoon and Clagett 12 in their 1958 review of 52 patients from the Mayo Clinic in whom Vitallium tubes had been inserted into the biliary ducts during stricture repair and reestablishment of end-to-end duct continuity. When the tubes were originally inserted, it was hoped that they would remain in place and continue to function indefinitely. However, with the passage of time, a substantial number of these patients required removal of their heavily encrusted and occluded tubes. McGoon and Clagett made the interesting observation; however, that at the time of removal of the Vitallium tube (a period of yehrs after its insertion), the inflam45

matory reaction about the site of the stricture had subsided, scarring and induration in the wall of the duct had abated and the mucosa had regenerated and had formed a normal-appearing unbroken ductal lining. They proposed that the Vitallium tube method of repair, when used, should be planned as a 2-stage operation, the first stage for insertion of the Vitallium tube and the second, at a date determined by the occurrence of occlusion of the tube, for its removal. In their series 12 patients remained well after the second-stage removal of the tube. The interval between insertion and removal of the tube ranged from 13 months to 8 years. The important principle emphasized by this series was the value of long-term intubation of the anastomosis. This was found to be associated with complete healing and absorption of surrounding scar tissue, even in the difficult cases where the upper end of the tube was inserted into a small orifice in the undersurface of the liver surrounded by scar tissue and the lower end of the duct was sutured in the fibrotic ring. The importance of such prolonged intubation after repair o f t h e duct has been challenged by Madden and McCann 1~who believe, based on experimental and clinical observations, that the use of a T tube should be selective and that primary reconstruction of the common bile duct by end-to-end anastomosis without an internal splint or stent support and without tension on the suture line is a logical and feasible operation. Madden and McCann were able to perform end-to-end anastomoses in all of the 8 p a t i e n t s o n whom tl/~y reported. They have not, however, discussed the use or omission of tube stents in duct-enteric anastomoses. William 21 has also emphasized the disadvantages of splinting biliary anastomoses. On the other hand, the extensive experience in the treatment of biliary strictures at the Lahey is and Mayo ~3 clinics supports the concept of prolonged stenting of difficult biliary reconstructions. Hoag, 5 in 1937, pointing out the unusual redundance and flexibility of the mucosa and submucosa of the gastric wall, described what he considered to be an original technique for performing a hepaticogastrostomy for bile duct reconstruction. Through a small opening in the stomach wall, a cuff of mucosa and submucosa 1 - 2.5 cm in length was drawn up and sutured to the edges of a segment of the flared end of a rubber catheter. The tube covered with the mucous membrane was then pulled out as far as possible without undue tension, and the muscular coat of the stomach was sutured to the base of the tube to maintain the projection of the mucosa. This intubated projecting cuff of gastric mucosa was then pushed into the opened end of the hepatic duct, and the surrounding stomach w a l l w a s sutured snugly about the opening in the scarred hilus of the liver. Cole 2 11 years later described a similar procedure that utilized the mucosa of the end of a Roux-en-Y 46

jejunal limb that was sutured to the flared end of a rubber catheter and inserted into the hepatic ducts at the hilus after the excision of an obstructing core of scarred hilar tissue. Four cases were reported, with good results in 3. A similar procedure was described by Smith, 15 utilizing a specially designed Y tube, the ends of which were introduced into the left and right hepatic ducts. A subsequent report of Wexler and Smith 19 indicated that these tubes had been abandoned because of their tendency to become occluded by biliary debris and sludge, leading to cholangitis and necessitating another operation for their removal. Instead, a transhepatic splint described by Smith ~6 in 1964 is now recommended for drawing the attached mucosal patch up into the hilus and into contact with the hepatic duct mucosa (Fig. 18). The tube is irrigated regularly during the 3 - 4month period it remains in place. Of their 61 repairs in 50 seriously ill patients, 85% have had an ultimately successful result. A further modification of the transhepatic intubation technique by Saypol and Kurian ~4 utilizes a U tube that passes through the abdominal wall transhepatically through a mucosato-mucosa hepaticojejunostomy down the Roux-en-Y limb and to the outside. They recommend keeping a tube stent in place 2 years, if possible. If necessary, their U tube can be replaced by attaching a new tube to the end of the old one and drawing it into place as the old tube is removed, a procedure which has been named "railroading:" Long-term stenting has also been advocated by Cameron et al. ~ They have reported on 10 patients with high hilar hepatic duct Fig 18.-Hepaticojejunostomy with transhepatic intubation as proposed by Smith. Mucosa of Roux-en-Y jejunal limb is fixed to the transhepatic tube and drawn up into the hepatic duct orifice. The jejunal wall is sutured to surrounding hilar tissues. To abdominal wall

47

strictures treated by the passage of a large caliber (1 cm diameter) Silastic stent with multiple side holes through the anterior abdominal wall transhepatically into the biliary tree through the duct-jejunal anastomosis into the Roux-en-Y jejunal limb. The stents are usually left in place for 12 months. During this period the patients irrigate the tube daily with saline. Should the tube become partially occluded with biliary debris, it may be replaced by first passing a flexible guide wire through the stent into the jejunal loop under fluoroscopic control. The old stent is then removed and a new one inserted over the guide wire. There have been no failures during an average follow-up period of 36 months. This method has the advantage of providing an effective means of irrigation during prolonged intubation. Straight tubes passed in. the opposite direction up through the anastomosis into the intrahepatic ducts will usually be extruded in 3 - 6 months, even though securely-sutured in place. Complications of the transhepatic intubation procedure include persistent hematobilia, recurrent cholangitis and subphrenic accumulations of bile with or without infection. The majority of these difficulties will resolve spontaneously, but early removal of the stent may be required. In 1948 the anastomosis of a left lateral intrahepatic segmental duct to a Roux-en-Yjejunal limb (cholangiojejunostomy)8 was proposed for the treatment of the high, difficult biliary stricture after multiple attempts at hilar repair had failed. In this procedure the left lateral segment of the liver is mobilized by diviSion of the left triangular ligament (Fig. 19, A). With the liver elevated into the wound, an incision is made in the capsule and extended into the parenchyma until an intrahepatic duct of sufficient size to accept a no. 14 or 16 catheter is encountered. Bleeding vessels are clipped or ligated. The small hepatic arterial branch that is intimately attached to the duct wall is suture-ligated with 5-0 arterial silk. The mesenteric border of a Roux-enY jejunal limb is sutured to the inferior capsule of the cut liver surface. After completion of a direct suture anastomosis of the duct to the intestine with multiple sutures of fine silk, the jejunum is sutured over the raw surface to the superior capsule of the liver (Fig. 19, B). A catheter is kept through the anastomosis, brought down the jejunal limb and through the anterior abdominal wall to the outside. The 3 conditions we have felt essential for a successful result El0 19.-Intrahepatic cholangiojejunostomy. A, mobilization of left lateral hepatic segment by division of left triangular ligament. B, mesenteric border of jejunum is. sutured to inferior capsule. Duct will be sutured to jejunal opening with fine silk. The antim~senteric border of jejunum is sutured to the superior capsule of the liver. (From Longmire, W. P., Jr., and Sanford, M. C.: Surgery 24:264, 1948.) 48

49

are: (1) Dilatation of the intrahepatic ductal system, (2) free communication between the left and right hepatic ducts and (3) complete removal of all stones or debris in the intrahepatic ducts. Although the obstructed biliary system is usually approached at the hilus, special problem cases continue to appear in which the approach to the left lateral intrahepatic duct system is indicated. 1~The results of the procedure when utilized in the palliative treatment of bile duct carcinomas occurring at the confluence of the left and right hepatic ducts have been unsatisfactory. The technique has been helpful in certain selected patients with malignant duct obstruction when there is free communication of the left and right hepatic ducts. 9 In summary, the importance of bile duct anomalies in provoking injuries of the ducts during cholecystectomy is emphasized. Initial end-to-end duct repair and duct-enteric anastomosis for more extensive injuries both tend to fail, probably due in part to the small caliber of the duct and the thinness of the wall with Fig 20.-Interposition of a jejunal segment between the hepatic ducts and duodenum delivers bile into the duodenum to prevent gastric hyperacidity while preventing reflux of the duodenal contents into the biliary system. (From Wheeler, E. S., and Longmire, W. P., Jr.: Repair of benign strictures of the common bile duct by jejunal interposition choledochoduodenostomy. [In press.])

Interposed jejunal segr

50

l e a k a g e of bile and increased s u r r o u n d i n g i n f l a m m a t i o n a n d scarring. T h e o p t i m u m t i m e to o b t a i n a successful r e p a i r is on theoccasion of the 2d o p e r a t i o n , w h e n the d u c t still r e t a i n s its maxim u m e x t r a p a r e n c h y m a l length, its l u m e n is dilated and the wall is thickened. W i t h each successive a t t e m p t , t h e r e will be additional s u r r o u n d i n g scar and less duct l e n g t h outside the liver. P r o l o n g e d t r a n s h e p a t i c i n t u b a t i o n is indicated for t h e p r e c a r i o u s high, difficult anastomosis. Duct-jejunal (Roux-en-Y) a n a s t o m o sis h a s the a d v a n t a g e of b e i n g outside the a l i m e n t a r y s t r e a m if l e a k a g e should occur, and t h e lack of r e g u r g i t a t i o n into the d u c t aids in p r e v e n t i n g l o n g - t e r m s c a r r i n g and stenosis. Duct-duodenal anastomosis is a d v a n t a g e o u s b e c a u s e it is a less complicated p r o c e d u r e a n d one t h a t is not ulcerogenic. We h a v e r e c e n t l y conv e r t e d t h e Roux-en-Y j e j u n a l limb into a j e j u n a l s e g m e n t w i t h anastomosis of the lower end into the d u o d e n u m . 2~ B r i n g i n g bile back into the d u o d e n u m reduces the t e n d e n c y t o w a r d gastric h y p e r a c i d i t y t h a t occurs w h e n bile is totally d i v e r t e d into a jejunal limb. T h e s e g m e n t acts to p r e v e n t reflux of d u o d e n a l contents into the b i l i a r y s y s t e m , a p r o b l e m o c c u r r i n g w i t h a direct ductd u o d e n a l anastomosis (Fig. 20). Injuries t h a t involve s m a l l segm e n t a l ducts ( u s u a l l y the r i g h t s e g m e n t a l h e p a t i c duct to t h e dorsal c a u d a l s e g m e n t ) should be ligated a n d well drained, unless a n end-to-end r e p a i r can be performed. In u n u s u a l situations, it m a y be n e c e s s a r y to accept d r a i n a g e of only 1 h e p a t i c duct. H o w e v e r , if the liver is n o r m a l and t h e o t h e r h e p a t i c duct is completely, o b s t r u c t e d a n d uninfected, t h e d r a i n e d lobe will hypertrophj~ as t h e o b s t r u c t e d lobe u n d e r g o e s a n a s y m p t o m a t i c progressive atrophy. 7 REFERENCES 1. Cameron, J. L., Skinner, D. B., and Zuidema, G. D.: Long-term transhepatic intubation for hilar hepatic duct strictures, Ann. Surg. 183:488, 1976. 2. Cole,W. H.: Strictures of the common duct, Can. Med. Assoc. J. 58:582, 1948. 3. Flint, E. R.: Abnormalities of the right hepatic, cystic and gastroduodenal arteries and ofthc bile ducts, Br. J. Surg. 10:509, 1922. 4. Glenn, F.: Atlus of Biliary Tract Surgery (New York: The Macmillan Company, 1963), p. 28. 5. Hoag, C. L.: Reconstruction Of the bile ducts, a new method of anastomosis, Surg. Gynecol. Obstet. 64:1051, 1937. 6. Longmire, W. P., Jr.: The early management of injury to the extrahepatic biliary tract. J.A.M.A. 195:623, 1966. 7. Longmire, W. P., Jr., and Tompkins, R. K.: Lesions ofthe segmental and lobar hepatic ducts, Ann. Surg. 182:478, 1975. 8. Longmire, W. P., Jr., and Sanford, hi. C.: Intrahepatic cholangiojejunostomy with partial hepatectomy for biliary obstruction, Surgery 24:264, 1948. 9. Longmire, W. P., Jr., McArthur, M. S., Bastounis, E. A., and Hiatt, J.: Carcinoma of the extrahepatic biliary tract, Ann. Surg. 178:333, 1973. 10. Madden, J. L., and McCann, W. J.: Reconstruction of the common bile duct by end-to-end anastomosis without the use of an internal splinter stent support, Surg. Gynecol. Obstet. 112:305, 1961. 11. McArthur, M. S., and Longmire, W. P., Jr.: Further indications for intrahepatic cholangiojejunostomy, Ann. Surg. 175:190, 1972. 51

12. McGoon, D. C., and Clagett, O. T.: Vitallium tube method for repair of strictures of the bile ducts, Surg. Gynecol. Obstet. 106:409, 1958. 13. Remine, W. H., and Ferris, D. G.: Surgery for biliary strictures, Surg. Clin. North Am. 47:877, 1967. 14. Saypol, G. M., and Kurian, G.: A technique of repair of stricture of the bile duct, Surg. Gynecol. Obstet. 128:1071, 1969. 15. Smith, R.: Surgery of the bile ducts, stricture of the bile ducts, Proc. R. Soc. Med. 62:131, 1969. 16. Smith, R.: Hepaticojejunostomy with transhepatic intubation. A technique for very high strictures ofthe hepatic ducts, Br. J. Surg. 51:186, 1964. 17. Warren, K. W., and Jefferson, M. F.: Prevention and repair of strictures of the extrahepatic bile ducts, Surg. Clin. North Am. 53:1169, 1973. 18. Warren, K. W., Mountain, S. C., and Midell, A. I.: Management of strictures of the biliary tract, Surg. Clin. North Am. 51:711, 1971. 19. Wexler, hl. J., and Smith, R.: Jejunal mucosal graft, Am. J. Surg. 129:204, 1975. 20. Wheeler, I., and Longmire, W. P., Jr.: Biliary duodenostomy with segmental jejunal interposition. Surg. Gynecol. Obstet. (In press.) 21. William, T. R.: Experiences with surgical reconstruction of the hepatic ducts, Ann. Surg. 179:540, 1973.

CONGENITAL BILIARY ATRESIA In 1953 Gross 3 reported on 198 patients with obstructive jaundice in the early months of life. Fifteen patients were successfully treated medically. Of the 183 treated surgically, 26 with inspissated mucus or bile in the ducts were successfully treated by manipulation or irrigation of the duct. A historically important footnote to these cases stated, "At operation, 3 of these babies were thought to have obliteration of the extrahepatic ducts, but subsequent to operation had passage of bile and clearance of jaundice. They m u s t have had exceedingly tiny ducts which, subsequent to the regional manipulations, did open up." In only 12 of the 183 surgically treated patients was adequate biliary drainage obtained by duct-enteric anastomosis. We must remember, therefore, that of the total series of 198 patients with neonatal jaundice, 53 (26%) were relieved by medical or surgical management and were reported as cured a year or more after treatment. Pointing out that the extrahepatic bile ducts are patent in early fetal life, then lose their lumina by epithelial concrescence and finally become patent, again at some later time, Gross expressed a theory, accepted for many years, that the highly probable cause of all types of biliary atresia was arrested development during the solid stage, a view that seemed at the time to explain the pathologic findings far better than any other theory. The term "hypoplasia" was used by us in 1964 in reporting on 5 children with varying degrees of extrahepatic biliary obstruction2 Unlike atresia, hypoplasia might vary in degree, we said. "It may be associated with normal or near normal biliary drainage or with almost complete absence of biliary drainage, and thereby the clinical course may be similar to that of an irremedia52

ble biliary atresia." The cause of the hypoplasia was not commented upon at the time, but it was considered to be another variety of congenital or developmental anomaly. For a number of years, pathologists and clinicians have been puzzled by their inability to distinguish clearly hepatitis from congenital atresia in neonatal jaundice. Results of liver function tests and even the histologic changes seen on liver biopsy present many findings that seem to overlap, making it impossible in many cases to differentiate between the two supposedly quite separate and distinct clinical entities. TREATMENT

For many years the possible detrimental effects of anesthesia and operation on the hepatitic liver were so feared that delay of operation for 3 - 4 months was widely recommended to allow possible spontaneous resolution should the jaundice be caused by hepatitis. The possible aggravation of hepatic scarring and fibrosis by prolonged atretic obstruction was also recognized, so the 3month waiting period before surgical exploration was generally accepted as the interval that would allow hepatitis, if present, to show evidence of clearing without causing irreversible intrahepatic changes should the obstruction be due to a correctable atresia. With the introduction of the Kasai procedure4 of9 portoenterost0mY in 1959 (Fig. 21), emphasis was placed on early operation, if possible even before the child was 1 month old, a time when, in most instances, it is still impossible to differentiate neonatal hepatitis from congenital biliary atresia. Further clinical experience with early operation, as well as the findings on pathologic examination of liver tissue and portal structures removed at operation, have encouraged the adoption of the so-called unified concept of neonatal jaundice to replace the former idea of two totally separate disease processes. Landing 7 suggested that neonatal hepatitis and extrahepatic atresia are different results of a single basic dynamic cholangiopathic process occurring postnatally. He suggested that the process is inflammatory and probably viral in origin with varying degrees of liver cell and duct epithelial injury followed by obliteration of the biliary tree lumina and later sclerosis that determine the various possible outcomes. It has been proposed that this progressive sclerosing obliteration of the ductal system may, in some instances, be halted or even reversed by excision of the extrahepatic ducts combined with a bile drainage procedure (the Kasai operation). The possibility that many of these cases are the result of an obstructive inflammatory condition that may vary in intensity, rather than a fixed, unalterhble, congenital, developmental anomaly, gives quite a different outlook to the possible treatment 53

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Fill 2 1 . - M o d i l i c a l i o n of Kasai's original operation of porloenleroslom7 to prevent regurgitation of jejunal limb conlenls inlo and about the hilar bile ducls in an effort to decrease recurrent bouts of cholangitis.

and outcome. However, such a theory cannot exclude the occurrence also of congenital atresia as a developmental defect. The Kasai procedure attempts to enhance biliary drainage by removing essentially all of the obstructed or potentially obst{ucted extrahepatic ductal system and draining the hepatic ducts into a defunctionalized segment ofjejunum. Lilly,8 Bill' and many other surgeons throughout the world have confirmed the presence of bile drainage from the liver following a portoenterostomy for neonatal jaundice, and Kasai's first patient is living without hepatic dysfunction 18 years after operation. 5 However, the presence of bile in the anastomosed intestine after operation, or even the disappearance of jaundice, does not insure a successful long-term result. LONG-TERM RESULTS

Kasai e t al. s have recently reported upon 80 patients with "noncorrectable biliary atresia" operated upon during the past 18 years. (The number of patients with correctable atresia operated upon during this period is not stated.) Twenty were alive without jaundice, 14 for more than 2 years after operation. The authors commented that when advanced changes are present in the liver before operation, varying degrees of fibrosis may remain afterward. A variety of mo.difications to the original Kasai operation have been devised in an attempt to reduce the incidence ofpostop54

erative cholangitis, a frequent complication that may lead to cirrhosis and portal hypertension, even in patients in whom jaundice has been relieved (Fig. 22). Kasai e t al. 5 have stated that early operation, before 2 months of age, and the prevention of postoperative cholangitis are the key factors in improving the long-term results following hepatic portoenterostomy. Jaundice disappeared completely in 15 of 51 patients reported by Miyata e t a l . 1~ after hepatic portoenterostomy; however, in 7 of the 15, critical portal hypertension developed, and 4 had died at the time of their follow-up report. Of their patients whose jaundice cleared postoperatively, 71% subsequently showed transient episodes of cholangitis. Miyata and associates pointed out that a substantial number of patients who have supposedly been '
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55

TABLE 4.-COMPARISON OF RESULTS OBTAINED IN PATIENTS WITH BILIARY ATRESIA GROSS 3

Total number of patients Recovered with medical treatment Surgical cases Inspissated bilecured with irrigation

AUTIIORS MIYATA E T AL. I~

KASAI E T AL. ~

198

51

80

15

-

-

51 -

80 -

183 35 26 living & well 9 late deaths 12

Duct-enteric anastomosis Portoenterostomy 0 51 80 Number of patients 61 (31%)* 15 (29%)t 20 (25%)~; jaundice relieved Total patients "cured" 52 (26%) 8 (15.7%) 14 (17.5%) *Includes 9 patients with inspissated bile who subsequently died. tIncludes 7 patients in whom portal hypertension developed; 4 subsequently died. $Includes 6 patients living without jaundice more than 2 years.

s o m e w h a t c o m p a r a b l e to t h e follow-up r e s u l t s of the J a p a n e s e series.

SUMMARY

Koop ~ h a s followed this subject w i t h g r e a t interest, since K a s a i s e r v e d as a r e s i d e n t on his service 18 y e a r s ago, s h o r t l y a f t e r the first successful K a s a i o p e r a t i o n had b e e n performed. H e insists t h a t t r u e e x t r a h e p a t i c a t r e s i a does exist, t h a t it is a c o n g e n i t a l d e v e l o p m e n t a l defect r e s u l t i n g in i n t r a h e p a t i c c h a n g e s c o m p a r a ble to a c q u i r e d b i l i a r y o b s t r u c t i o n in a n older p e r s o n and t h a t it is s u r g i c a l l y correctable. A second condition, b i l i a r y hypoplasia, is c h a r a c t e r i z e d b y d i m i n u t i v e b u t grossly visible ducts w i t h v a r y ing d e g r e e s of r e d u c t i o n in t h e i r caliber a n d b y i n t r a h e p a t i c cirrhosis. W h e n t h e diagnosis of h y p o p l a s i a is e s t a b l i s h e d b y operative c h o l a n g i o g r a m , t h e decision m u s t be m a d e w h e t h e r to proceed w i t h a p o r t o e n t e r o s t o m y or to close a n d observe the p a t i e n t for signs of d e c r e a s i n g jaundice. I f i m p r o v e m e n t does not occur in 1 m o n t h , the K a s a i p r o c e d u r e is r e c o m m e n d e d . F i n a l l y , t h e r e are the cases in which e x t r a h e p a t i c ducts are not visible to the n a k e d eye, a l t h o u g h t h e r e m a y indeed be small ducts with microscopic l u m i n a from which bile e x c r e t i o n m a y b e e n h a n c e d , e i t h e r b y r e d u c i n g the r e s i s t a n c e to bile flow from t h e liver or, t h r o u g h some m e c h a n i s m as y e t not understood, b y excising the e x t r a h e 56

Fi9 23.--Variations in neonatal ductal obstruction. A, congenital atresia of the extrahepatic bile duct with dilated intrahepatic ducts. Treated by hepaticoduodenostomy. B, gross hypoplasia of the ductal system. Note the alternating areas of constriction and dilatation, somewhat similar to that seen with sclerosing cholangitis. The patient was treated with choledochoduodenostomy. Excision of the lateral segment of the left lobe did not reveal any grossly identifiable intrahepatic ducts. The patient was last seen at age 8 with advanced hepatic cirrhosis and splenic enlargement, but he was well enough to attend school. C, microhypoplasia. Via a normal gallbladder the minute ducts were identified both intra- and extrahepatically. After 3V2 months of observation failed to demonstrate any improvement in the child's liver function, a cholecystoduodenostomy was performed, but without benefit. (From Longmire', W. P., Jr.: Ann. Surg. 159:335, 1964.)

patic ductal system and allowing the intraparenchymal hilar ducts to drain directly into the intestinal tract. In the absence of microscopically identifiable patent hilar ducts, Koop believes pertoenterostomy will be ineffective (Figs. 23 and 24). Whether or not the Kasai procedure will truly result in an overall improvement of the outcome of neonatal jaundice remains open for further evaluation. The concept that most cases of neonatal jaundice (excluding the rare, truly developmental anomaly) are the result of an ongoing or dynamic process, possibly similar to sclerosing cholangitis in the adult as suggested by Fonkalsrud, 2 seems more logical t h a n trying to categorize all cases into one of two distinct types. The frequent overlapping of the hepatobiliary pathology and of the clinical course of neonatal jaundice m a k e it extremely difficult clearly to categorize the disease into one of two distinct types based on its suspected etiology, i.e., an inflammatory neonatal hepatitis vs a congenital atresia malformation. 57

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Correctable CongenitalAtresia (extrohepatlcl F~: Hepotico-jejunostomy

Gross Hypoplasia t~: Observe

M]croscoplc Hypopiosio ~ : Kasai Operotion

Absence of ducts ~ : Noncorrectoble

Fig 24.-Condition of ductal system in four types of neonatal jaundice. A, congenital extrahepatic'atresia with dilatation of the intrahepatic system, a correctable condition. B, gross hypoplasia. Miniature but grossly visible ductal system, observed for improvement followed by portoenterostomy if necessary. C, microhypoplasia. Extrahepatic ductal system identifiable on histologic section, treated by portoenterostomy. D, complete atresia. Absent intra- and extrahepatic ducts on microscopic examination of the liver and hilar tissue. No effective treatment is available today. Hepatic transplantation has been utilized, but the results have been less satisfactory than when indications for liver replacement other than atresia are present. REFERENCES 1. Bill, A. H. J.: Discussion of paper by Lilly, J. R.: Biliary lipid excretion after hepatic portoenterostomy, Trans. Am. Surg. Assoc. 94:369, 1976. 2. Fonkalsrud, E. W.: Discussion of paper by Lilly, J. R.: Biliary lipid excretion after hepatic pertoenterostomy, Trans. Am. Surg. Assoc. 94:375, 1976. 3. Gross, R.: The Surgery of Infancy and Childhood (Philadelphia: W. B. Saunders Company, 1953), p. 521. 4. Kasai, M., and Suzuki, S.: A new operation for noncorrectable biliary atresia; hepatic portoenterostomy, Shujutsu (Operation) 13:733, 1959 (in Japanese). 5. Kasai, hi., Watanabe, I., and Ohi, R.: Follow-up studies of long term survivors after hepat!c portoenterostomy for "noncorrectable" biliary atresia, J. Pediatr. Surg. 10:173, 1975. . I 6. Koop, C. E.: Progressive extrahepatm biliary obstruction of the newborn, J. Pediatr. Surg. 10:169, 1975. 58

7. Landing, B. H.: Changing approach to neonatal hepatitis and biliary atresia, Pediatrics 53:647, 1974. 8. Lilly,J. R.: The Japanese operation for biliary atresia. Remedyor mischief, Pediatrics 55:12, 1975. 9. Longmire, W. P., Jr.: Congenital biliary hypoplasia, Ann. Surg. 159:335, 1964. 10. Miyata, M., Satani, M., Ueda, T., and Okamoto, E.: Long-termresults of hepatic portoenterostomyforbiliary atresia, Surgery 76:234, 1974.

SUMMARY The diverse causes of extrahepatic biliary obstruction discussed in this monograph are relatively rare clinical conditions, but each represents an important pathologic entity t h a t occasionally needs to be differentiated from the more common causes of biliary obstruction. All surgeons who perform operations on the biliary tract should be familiar with these pathologic conditions, their gross recognition, differential diagnosis, modes of t r e a t m e n t and prognosis. Most of these rare conditions have an unfavorable prognosis, but they are areas of intense clinical and laboratory investigation, and ideas concerning etiology and concepts of m a n a g e m e n t are undergoing modificatiofi and change. This discussion of obstructive biliary diseases obviously cannot be all-inclusive. The subjects selected are representative of an important dynamic field in which today's concepts and recommendations m a y well represent tomorrow's discarded theories and outmoded remedies.

59