Henry Ford Hospital Medical Journal

Volume 22 | Number 4 Article 5

12-1974 Sclerosing Cholangitis: Surgical Significance C. Grodsinsky

M. A. Block

B. E. Brush

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Recommended Citation Grodsinsky, C.; Block, M. A.; and Brush, B. E. (1974) "Sclerosing Cholangitis: Surgical Significance," Henry Ford Hospital Medical Journal : Vol. 22 : No. 4 , 211-216. Available at: https://scholarlycommons.henryford.com/hfhmedjournal/vol22/iss4/5

This Article is brought to you for free and open access by Henry Ford Health System Scholarly Commons. It has been accepted for inclusion in Henry Ford Hospital Medical Journal by an authorized editor of Henry Ford Health System Scholarly Commons. Henry Ford Hosp. Med. Journal Vol. 22, No. 4, 1974

Sclerosing Cholangitis: Surgical Significance

C. Grodsinsky, MD, M.A. Block, MD and B.E. Brush, MD*

A COMMON duct stone or carcinoma of the head of the pancreas is usually suspected in a patient with jaundice. When the surgeon operates, a thickened gallbladder is found but no stones are palpable. The wall of the common duct is very thick, and the thickness en­ croaches on the lumen so that it is dif­ The authors' experience with the rare entity ficult to introduce instruments or a of sclerosing cholangitis suggests that several T-tube. Operative cholangiography re­ anatomical patterns of involvement of the ex­ trahepatic biliary tract may occur. The in­ veals narrowing of the extrahepatic volvement may include the intraluminal ducts biliary system and the intrahepatic ducts segmentally or diffusely, or the process may may also be involved. There is no evi­ primarily be external to the ducts as dence of calculous disease, and malig­ pericholedochitis. It may or may not represent one component of a systemic disease, particu- nancy of the pancreas or bile ducts lady autoimmune disease. seems unlikely.

Although involvement may be diffuse, In such a patient, the tentative diag­ progression may be slow and compatible with nosis of "primary sclerosing cholangitis" many years of life. is justified. The entity was well described Long time followup is essential before any by Schwartz and Dale in 1958' although value can be assigned to a given modality of the honor of having described the first treatment. Carcinoma of the ductal system case is frequently given to the French may be found after a more exhaustive exami­ surgeon Delbet.^ Obviously, it is not im­ nation or at autopsy. portant whether there have been 100 or All efforts should be provided operatively, only 50 cases reported to the present however, to relieve biliary tract obstruction. If time. The important consideration is this is not possible or provides only incom­ whether or not the surgeon of the 1970s plete relief, steroid therapy should be ad­ is apt to encounter a "bona fide" case of ministered. sclerosing cholangitis and whether or not he will render optimal treatment.

Most authors consider the following criteria necessary for inclusion of a case:'""

1. Absence of previous biliary tract operations *A11 from the Department of Surgery 2. Absence of gallstones Address reprint requests to Dr. Grodsinsky at Henry Ford Hospital, 2799 West Grand 3. Diffuse, generalized involvement Boulevard, Detroit Ml 48202 of the extrahepatic biliary ducts

211 Crodsinsky, Block and Brush

4. Exclusion of cholangiocarcinoma months previously showed microscopic evi­ dence of a marked cellular infiltration in the Warren and his groups admitted that portal spaces but the hepatic parenchyma ap­ their criteria for classification were much peared normal except for an increase of pig­ less rigid than others. ment in the hepatic cells. When the patient was seen a few weeks after his last episode of cholangitis, his physical examination showed Although this entity is rare, we en­ a liver enlarged to two fingers below the right countered among 369 patients six for costal margin. He recalled a brief period of whom the diagnosis of sclerosing painless jaundice approximately 40 years be­ cholangitis was initially given. All had fore. been treated for extrahepatic obstructive At the time of his examination, the patient's lesions. In two of the six the intraopera­ total serum bilirubin varied from 2.08 to 5.45 tive findings were similar but the pa­ mg % and the serum alkaline phospha­ tients eventually died. Absence of post­ tase varied from 9.9 to 17.2 Bodansky units mortem examinations made confirma­ % (normal 1.5 - 4.0). The serum antinu­ clear factor test was positive (weak to tion of the intraoperative diagnosis im­ medium homogeneous). The prothrombin possible. Our experience suggests that time, SCOT, and serum immunoglobulin de­ sclerosing cholangitis may be charac­ terminations were all within normal limits. terized by at least three patterns of The intravenous cholangiogram showed the faint outline of a slightly dilated proximal anatomic involvement of the bile ducts. common bile duct. 1. Segmental extrahepatic, intralumi­ At operation, the liver appeared normal but nal obstructive sclerosing the gallbladder appeared distended. No 2. Diffuse sclerosing, commonly as­ stones were found in the biliary tract. The pancreas appeared normal. The segment of sociated with chronic ulcerative the common bile duct within the pancreas colitis was stenotic with dilation present proximal to 3. Diffuse, primarily extrahepatic this (Figure 1). A silver wire probe could not sclerosing pericholedochitis

Segmental Sclerosing Cholangitis This variety of sclerosing cholangitis results in recurrent bouts of cholangitis which are relieved if operative proce­ dures to by-pass the obstruction are feasible. At operation, stenosis is limited to a segment, usually located distally, at the extrahepatic bile ducts. No other disease process has been evident and the biliary tract sclerosis has not ap­ peared to be progressive in the patient whose case illustrates this clinical pat­ tern. This permits a good outlook for his future.

Case 1: A man, aged 64 years, had had five episodes of chills, fever, and jaundice during the 21 months preceding his examina­ tion. Each of these bouts lasted several weeks. He had been taking only thyroid and Figure 1 vitamin medications and consumed no al­ Operative cholangiogram showing obstruc­ cohol. He was initially treated for suspected tion in distal common bile duct due to seg­ infectious hepatitis. A liver biopsy done six mental sclerosing cholangitis.

212 Sclerosing Cholangitis: Surgical Significance

be passed through the stenotic segment. Biopsies from the stenotic portion showed evidence only of acute and chronic inflamma­ tion with fibrosis (Figure 2). A side-to-side choledochoduodenostomy and a cholecys- toduodenostomy were performed.

Postoperatively, the patient's serum al­ kaline phosphatase and serum bilirubin re­ turned to normal levels. The patient has re­ mained asymptomatic six years later.

Diffuse Sclerosing Cholangitis Asso­ ciated with Chronic Ulcerative Colitis

Sclerosing cholangitis involving the entire biliary tract, both intrahepatic and extrahepatic, is associated with other diseases, particularly chronic ulcerative colitis.' This clinical variety is also only slowly progressive in many patients who remain asymptomatic for many years.'

Case 2: Two cholesystograms done in 1968 revealed a nonfunctioning bladder in a Figure 2 40-year-old female who had recurrent Photomicrograph of biopsy from bile duct in­ episodes of right upper abdominal pain volved by segmental sclerosing cholangitis. radiating to the back. A total colectomy and The endothelial lining is covered by a thick ileorectostomy had been performed 11 years band of tissue. Ft and E x115. previously (in 1957) because of disabling man­ ifestations of chronic ulcerative colitis for the preceding eight years. Following this opera­ tion the patient had been relatively asymp­ tomatic except for an anal fistula requiring a fistulectomy in 1963.

At operation for the biliary tract difficulty the gallbladder was found to be thick-walled, the cystic duct thick and scarred, and the ex­ trahepatic bile ducts also thickened and nodular to palpation. An operative cholan­ giogram showed an irregular small lumen throughout the entire extrahepatic biliarv tract (Figure 3). One fleck of a pigment debris was found in the wall of the excised gallblad­ der but no calculi otherwise. Microscopically, the gallbladder wall was involved by a heavy infiltrate of inflammatory cells. Fibrosis around bile ducts, but normal hepatic cells, were seen in a liver biopsy.

Postoperatively, the patient had two episodes of mild pain in the right upper ab­ Figure 3 domen during the first year but no further Operative cholangiogram from patient having biliary tract symptoms during the subsequent diffuse sclerosing cholangitis associated with five years. However, she has required exci­ chronic ulcerative colitis. The duct is nar­ sion of the rectal stump with an ileostomy be­ rowed by intraluminal irregularities and bead­ cause of a rectovaginal fistula. ing.

213 Grodsinsky, Block and Brush

Primary Fibrosing Pericholedochitis Postoperatively, the patient's jaundice cleared spontaneously, and although the This variety of chronic inflammation serum bilirubin returned to normal levels, the and fibrosis, involving the extrahepatic serum alkaline phosphatase remained ele­ biliary tract, is difficult to distinguish vated. However, painless jaundice recurred from manifestations of carcinoma or the five months later. The ANF test remained positive and the serum immunoglobulins IgG consequences of biliary lithiasis or other and IgM remained elevated. After instituting lesions predisposing to chronic inflam­ steroid therapy the jaundice again rapidly dis­ mation in this region, such as duodenal appeared. She has remained asymptomatic ulcer disease and pancreatitis. Biopsies for the subsequent four years as steroid therapy was continued. The alkaline phos­ which show only scarring and chronic in­ phatase was 8.9 units after receiving steroids flammation are not reliable for excluding for 18 months. an underlying carcinoma. The subse­ quent clinical course after operative Discussion findings must be reviewed to establish the diagnosis. By applying indiscriminately the first three diagnostic criteria listed previ­ Case 3: A woman, aged 47 years, was ously, many cases of primary sclerosing seen in 1968 because she had had pruritis cholangitis, such as the segmental vari­ for four months and jaundice for one month. ety, will be excluded. Of course sclerosis The only medication she had taken was Don- of the extrahepatic ducts may be secon­ natal for abdominal gaseousness during the preceding year. She did not drink alcohol and dary to trauma, including blunt, pene­ had no pain. There was no history of abdomi­ trating and operative. It may also result nal operations. Her liver was slightly enlarged from inflammatory processes arising but not tender. from adjacent structures. Laboratory studies showed the total serum bilirubin ranged from 12.2 to 19.8 mg % and In certain situations, it may be difficult the alkaline phosphatase 7.6 to 46 Bodansky to be certain whether sclerosis of the ex­ units.The SCOT varied from 135 to 700 units. trahepatic ducts is primary or secondary The IgM component of the serum immuno­ to other disease processes or of iatro­ globulins was elevated to 161 units (normal 30-120) and the IgG was elevated to 1556 units genic origin. On occasion it may be dif­ (normal 600-1400). The ANF test was positive, ficult even at operation to identify showing medium speckle-like threads. The sclerosing cholangitis as primary or sec­ direct and Coombs' test, serum amylase and ondary. lipase as well as other usual laboratory studies were negative or within normal limits. A liver scan using 131-1 Rose Bengal failed to show Case 4: A 25-year-old female was first excretion from the liver into the intestinal seen in 1971 because of jaundice. Two years tract. previously she had been operated on in another hospital for evidence of biliary tract At operation, the region of the porta symptoms. No stones were found in the hepatis and gallbladder, as well as adjacent biliary tract, a cholecystectomy was per­ structures including the duodenum, hepatic formed, and evidence of sclerosis of the ex­ flexure and associated mesocolon were in­ trahepatic ducts was noted. Two months volved in an inflammatory process with much postoperatively the patient developed jaun­ fibrosis. The liver was dark but had a smooth dice and exploration of the common bile duct surface and showed none of the features of was performed. Evidently diffuse sclerosis of primary biliary cirrhosis. No fluid could be the bile ducts prevented the T-tube, placed in obtained by aspirating the gallbladder and no the common bile duct, from providing relief stones were palpable. A segment of the of jaundice. No evidence of primary liver dis­ common bile duct was isolated but found to ease was observed. be entirely incorporated in a fibrotic process. Biopsies from the gallbladder and portal At the time of our examination in 1971, the hepatis and a lymph node adjacent to the cys­ patient demonstrated features of obstructive tic duct showed only chronic inflammation jaundice with a serum bilirubin of 11 mg%. and fibrosis. A definitive operative procedure At operation, a lumen for the extrahepatic did not appear feasible. biliary tract could not be demonstrated. The

214 Sclerosing Cholangitis: Surgical Significance

entire tract was involved with dense scarring and chronic inflammation. Also, chronic in­ flammation surrounding cholangioles was evident during a liver biopsy. Drains were placed to the divided scar at the hilum of the liver.

The patient developed a biliary cutaneous fistula postoperatively. Injection of radio- opaque media showed the presence of a nar­ row duct entering the liver with narrow in­ trahepatic radicals (Figure 4). Because of re­ current bouts of cholangitis, the patient again underwent operation. A duct within the hilum of the liver was identified and a Roux-en-y hepaticojejunostomy accomplished (Figure 5). The patient subsequently improved, and except for several episodes of fever, re­ mained asymptomatic for 18 months. She then became jaundiced and febrile. Sus­ pected obstruction at the anastomosis led to a revision of the hepaticojejunostomy. She re­ covered following this operation and has been asymptomatic for the last six months.

It is never possible to identify sclero­ sing cholangitis preoperatively. The manifestations do not readily distin­ guish this entity from more common va-

Figure 5 Cholangiogram performed through a Catell tube shows adequate opening of hepaticoje­ junostomy needed to relieve biliary obstruc­ tion.

rieties of chronic disease of the ex­ trahepatic biliary tract. Percutaneous hepatic cholangiography may be helpful, but not specifically diagnostic. Present use of transduodenal endoscopy has be­ come useful in suggesting lesions of the pancreatic duct or in carcinomas of the bile ducts (Figure 6). In time, its con­ tinuous application may provide certain features suggesting the possibility of sclerosing cholangitis. At operation the thickening of the extrahepatic tract is usually diffuse, but in some patients it is more pronounced in segments. The ex­ ternal appearance of the ducts is not in­ creased. Operative cholangiography is Figure 4 of real value in the assessment of the Radio-opaque media in biliary cutaneous fis­ anatomic situation. In a few patients tula showing narrow intra- and extrahepatic duct system in patient with probably diffuse stones can be expected since obstructive sclerosing cholangitis. factors predispose to stone formation.

215 Grodsinsky, Block and Brush

resent one manifestation of a systemic disease process."'*"-' As demonstrated by two of our patients, serum immuno­ globulin and anti-nuclear factor deter­ minations indicate the presence of an auto-immune response by the patient. Perhaps the rare condition of stenosis of the sphincter of Odd! should be indi­ cated as a component of sclerosing cholangitis.•*

The diffuse involvement of the ex­ trahepatic biliary tract, as well as in­ trahepatic involvement, make the ulti­ mate outlook poor for the sclerosing cholangitis patient. However, our ex­ perience, as well as that reported by Figure 6 Transduodenal pancreatic duct cannulation others, indicates that in many instances showing also marked stenosis of lower end of the disease is compatible with many common bile duct. At surgery, cholangiocar­ years of life.'' Therefore, all possible av­ cinoma. enues of mechanical relief of obstruc­ tion by operative procedures should be But, strict criteria for the diagnosis re­ provided. In addition, steroids should quire absence of stones. be administered when operative aid is not possible or is only incompletely ef­ The etiology of the lesion is not fective. known. It is evident that many cases rep­

References

1. Schwartz SI and Dale WA: Primary 6. Toader C: Benign stenosis of the hepatic sclerosing cholangitis. A review and re­ duct: Report of 17 cases. Bull Soc Int Chir port of 6 cases . Arch Surg 77:439, 1958 27:299-304, 1968

2. Cutler B and Donaldson GA: Primary 7. Bartholomew LG, Cain JC, Woolner LB, sclerosing cholangitis and obliterative Utz DC and Ferris DO: Sclerosing cholangitis. Amer y Surg 117:502-11, 1969 cholangitis. Its possible association with Ridel's struma and fibrous retro- 3. Thorpe MEC, Scheuer PJ and Sherlock S: peritonitis. Report of two cases. New Primary sclerosing cholangitis, the biliary Eng J Med 269:8-12, 1963 tract, and ulcerative colitis. Cut 8:435-48, 8. Dillard BM and Black WC: Polyarteritis 1967 nodosa of the gallbladder and bile ducts. Amer Surg 36:423-7, 1970 4. Wenger J, Gingrich GW and Mendeloff J: Sclerosing cholangitis — a manifestation 9. Sotgiu G: Involvement of the biliary tract of systemic disease. Arch Intern Med in acute hepatitis. Postgrad Med J 116:509-14, 1965 41:234-42, 1965 5. Warren DW, Athanassiades S and Monge 10. Block MA, Brush BE, Ponka JL and Cre- Jl: Primary sclerosing cholangitis: A study peau A: Stenosis of the sphincter of Oddi of forty-two cases. Amer J Surg 111:23-38, as a cause of jaundice. Arch Surg 1966 76:888-94, 1958

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