CASE REPORTS
Segmental Primary Sclerosing Cholangitis Mimicking Bile Duct CancermReport of a Case and Review of the Japanese Literature T s u k a s a TSUNODA 1, Toshifumi ETO 1, Masafumi YAMADA1, Yoshitsugu TAJ!MA~, Shigetoshi MATSUOx, Ryoichi TSUCHIYA1, Tsuneo SHIOZAWA2 and
Takeshi MATSUOa ABSTRACT: A rare case of the segmental type of primary sclerosing cholangitis (PSC) is reported herein. A 27 year old Japanese m a n with obstructive jaundice was referred to our hospital with a provisional diagnosis of bile duct cancer at the hepatic hilum. A PTCD tube h a d been inserted f r o m the right anterior segmental duct and direct cholangiograms showed complete obstruction of the bile duct at the confluence of the right and left hepatic ducts. Resection of the extrahepafic bile duct a n d partial hepatectomy were thus performed, however, th e postoperative histological examination revealed no malignant features. Instead, the lesion was shown to be compatible with PSC microscopically. A diagnosis of PSC was finally established on the basis of clinical features, cholangiographic appearance and the pathological report. KEY WORDS:
primary sclerosing cholangitis, segmental type
INTRODUCTION
P r i m a r y sclerosing cholangitis (PSC), a rare disease o f u n k n o w n etiology, is characterized by chronic fibrosing inflammation of the bile ducts, usually affecting both the extrahepatic and intrahepatic ductal systems. In some instances~ the disease involves the extrahepatic bile duct segmentally, thereby creating symptoms mimicking bile duct cancer. We recently treated a patient with a 1The Second Department of Surgery, Nagasaki University School of Medicine, 2theDepartment of lnternal Medicine, SaseboSenju Hospital, and 3theFirstDepartment of Pathology, Nagasaki University School of Medicine, Nagasaki,Japan Reprint requests to: Tsukasa Tsunoda, MD, The Second Department of Surgery, Nagasaki University School of Medicine, 7-1 Sakamoto-machi, Nagasaki 852, Japan
localized stricture of the bile duct at the hepatic hilum. The lesion was managed by resection of the extrahepatic bile d u c t a n d partial hepatectomy following an initial diagnosis of bile duct cancer. As it was found to be microscopically compatible with PSC, the lesion was subsequently diagnosed as the segmental type of PSC on the basis of clinical features, cholangiographic appearance and, the pathological report. We report this case herein and discuss the problems associated with the diagnosis and treatment of-this disease, reviewing the literature on similar cases reported previously in Japan. CASE REPORT
A 27 year old Japanese man was referred to our hospital on 15 April, 1987 ~ i t h a suspected diagnosis of bile duct cancer at the hepatic hilum. He had been well until de-
JAPANESEJOURNALOF SURGERY,VOL.21, No. 3 pp. 329-334, 1991
330
Tsunoda et al.
veloping jaundice, 2 months prior to his admission. The disease had been diagnosed as obstructive jaundice on March 19, 1987, and he had u n d e r g o n e percutaneous transhepatic biliary drainage (PTBD) at another hospital. There were no other symptoms or signs and he had never undergone any abdominal surgery. His past and family histories were essentially negative apart from the fact that his mother hadcirrhosis of the liver. Physical examination revealed him to be in a good nutritional state. The palpebral conjunctiva was slightly icteric. A PTBD t u b e had been inserted f r o m below the right costal margin and, apart from the jaundice, there were no abnormal findings. Laboratory tests revealed a total bilirubin value of 2.8 mg/dl, the direct being 2.0 mg/dl and an alkaline phosphatase value of 441 IU/1, the normal being 75-266, Serum electrolytes and amylase levels were within normal limits. Tests for antimitochondrial antibody and anti-smooth-muscle antibody were done postoperatively and both proved negative. CT scanning revealed dilatation of the intrahepatic bile ducts but failed to demonstrate any mass lesions at the hepatic hilum (Fig. 1). Endoscopic retrograde cholangiography (ERC)showed complete obstruction of the common hepatic duct (Fig. 2). There were no stones in the gallbladder or bile
Fig. 1. A C T ' showing dilatation of the intrahepatic bile ducts.
1991
Fig. 2. An ERCP showing the normalsized extrahepatic bile duct with complete obstruction at the hepatic hilum. duct. C h o l a n g i o g r a p h y was p e r f o r m e d through the PTBD tube which had been inserted at the other hospital, but the left intrahepatic ducts were not visualized. Therefore, percutaneous transhepatic cholangiography (PTC) was performed through the left segmental duct, and cholangiography through the PTBD at the same time. The cholangiogram showed complete obstruction of the bile duct at the confluence of the right and left hepatic ducts and marked dilatation of the left intrahepatic bile ducts (Fig. 3). Celiac angiography showed n o abnormal findings. Cytology of the bile drainage specimen was done 3 times with negative results for malignancy. On 27 April, 1987, the patient underwent abdominal exploration, based on the initial diagnosis of bile duct cancer at the hepatic hilum. The liver, though not enlarged was slightly greenish while the gallbladder was unremarkable. The hepatoduodenal ligament was slightly involved by fibrosis and no
Volume 21 Number 3
Primary sclerosing cholangitis
331
Fig. 3. Simultaneous direct cholangiogram from both the anterior and lateral segmental ducts of the liver. V-shaped complete obstruction of the right hepatic duct and U-shaped complete obstruction of the left hepatic duct with marked dilatation are demonstrated. mass lesion was palpable along the bile duct, which was felt normally. After dissection o f the hepatoduodenal ligament, a small firm mass was f o u n d in the confluence o f the hepadc ducts, extending slightly into the fight hepatic duct. T h e r e were a few large soft lymphnodes along the c o m m o n bile duct, the largest of which was 1 )< 3 cm in diameter. Frozen secdons o f the lymphnode proved negative for malignancy. Resection o f the extrahepadc bile ducts, dissection o f l y m p h n o d e s i n the h e p a t o d u o d e n a l ligament and partial hepatect0my of Couinaud's segment 4 and 5 were performed. C h o l e cystectomy was also done. The intestinal continuity was reconstructed by anastomoses o f the two fight intrahepadc bile ducts and a left hepatic duct to the p-looped jejunal limb in the Roux-en-Y fashion. Macroscopic examination of the resected specimen revealed the bile duct wall of the affected area to be thick and fibrolas with a tiny lumen (Fig. 4). Microscopy of the stricture site showed wall thickening by the fibrous tissue occupying all layers o f the bile ducts except the mucosa. Chronic inflammatory cells such as lymphocytes, histiocytes a n d eosin0phils infiltrated the t h i c k e n e d bile duct Wall (Fig. 5). Malignant features
a
b
Fig. 4. a. Resected specimen, b. An illustration of the resected specimen: The bile duct wall is thickened at the confluence of the hepatic ducts. Fibrosis is most severe in the fight hepatic bile duct. RHD: right hepatic duct, L H D : left. hepatic duct, PSD: posterior segmental duct.
332
Tsunoda et al.
Fig. 5. Microphotograph showing wall thickening of the bile duct by fibrous tissue (HE, X40). The mucosa is virtually intact.
were not demonstrated a n d reactive lymp h o a d e n o p a t h y was shown in the regional lymphnodes. T h e patient's postoperative course was uneventful and he has b e e n well for the last 2 a n d a half years without any symptoms. DISCUSSION
T h e n u m b e r o f reports o f PSC has b e e n increasing in the USA a n d E u r o p e a n countries, x,~but it is still a rare disease in Japan. In fact, Furukawa et al. in 1984, collected only 76 cases from the J a p a n e s e literature? T h e r e is no a g r e e m e n t concerning the diagnostic criteria o f PSC but at present, it seems to require the exclusion o f choledocholithiasis, previous biliary surgery, and bile duct carcinoma in patients with sclerosis o f the bile duct. A purely anatomic classification o f PSC has b e e n p r o p o s e d by Caroli a n d R o s n e r 4 w h o divide the c o n d i t i o n i n t o diffuse involvement of the biliary tree and segmental involvement. T h e latter is further divided into three subgroups namely: a) fibroinflammatory involvement of the hepatic duct junction; b) selective involvem e n t o f the c o m m o n hepatic duct; and c) selective involvement of the c o m m o n bile duct. However, in some instances it is difficult to decide which of Caroli a n d Rosner's
Jpn. J. Surg. May 1991
groups a particular patient belongs. T h e Furukawa report o f 76 cases revealed that diffuse involvement was seen in 77 per cen t of the cases, while the segmental type was seen in 23 p e r cent, a m o n g which subgroups a) a n d b ) were s e e n in 16 per cent a n d subgroup c) in 7 p e r cent? T h e exact pre- a n d intraoperative diagnoses o f segmental PSC are extremely difficult to establish because o f its rarity and the resemblance of the clinical features and cholangiographic a p p e a r a n c e to those o f bile duct cancer. T o elucidate the problems of this obscure disease, 11 cases reported in detail in the J a p a n e s e literature, including our case, were analyzed (Table 1). 5-14 T h e r e was no history o f previous surgery in any o f the patients, n o r any association with gallstone disease or ulcerative colitis. T h e m e a n age was 51.9 years, with a r a n g e o f 24 to 80 years, a n d there was no sex preponderance. Jaundice and pruritus, occasionally in association with pain or fever, were the two most c o m m o n symptoms. T h e preoperative diagnosis was bile duct cancer or suspected bile duct cancer in all 11 patients. According to Caroli a n d Rosner's subgroup division, 3 patients b e l o n g e d to the a) subgroup, 5 to b) and 3 to c). T h e proximal bile duct was often affected with the m e a n strictured length o f the bile duct in 8 cases b e i n g 1.6 cm, on cholangiograms or resected specimens. A review o f the results of direct cholangiography (ERC, PTC) revealed that contrast material passed though the strictured site in 6 patients (55 per cent), while complete obstruction was demonstrated in 5 (45 p e r cent). In 2 o f these 5 patients (Cases8 and 9), however, the contrast material started to pass through the strictured site during a preoperative PTBD. T h e passage o f contrast material occurs m o r e often in PSC than bile duct cancer. Although L o n g m i r C ~ stressed that there is generally n o dilatation o f the proximal bile d u c t to the strictured site in PSC, proximal dilatation o f the bile duct in our collected cases was demonstrated in all patients with segmental PSC.
Volume 21 Number 3
333
Primary sclerosing cholangitis
Table 1. Summary of Cases of"Localized" Primary Sclerosing Cholangitis Reported in Japan Case Age/Sex Author (Year)
Location
Length
Symptoms
Treatment
Prognosis
1. 53/F Sugita (1973)
CHD
3 cm
aundice aruritus
T-tube drainage
operative death (biliary infection)
2: 33/M Beppu (1976)
CBD
2 cm
jaundice pain
pancreatoduodenectomy
good (1 yr 3 mos follow-up)
3. 56/M Kawamura (1977)
CHD
"localized"
jaundice pruritus
cholecystectomy stenting of BD
good (7 yrs follow up)
4. 6zt/M Yoshikawa (1982)
CHD
1.5 cm
jaundice pruritus
left hepatic lobectomy, res. of EHBD
good (1 yr 10 mos follow up)
5. 24/M Narai (1982)
CHD
0.5 cm
jaundice pruritus
res. of EHBD
good (1 yr follow up)
6. 80/F Ito (1982)
confluence of hepatic ducts
2.0 cm
jaundice pain
external drainage of BD
dead (11 mos, cholangitis)
7. 55/M Nara (1982)
hepatic hilum
?
jaundice pain
right hepatic lobectomy res. of EHBD
operative death (pneumonia)
8. 56/F Suzuki (1984)
CBD
"localized"
jaundice fever
res. of EHBD
good (3 yrs follow up)
9. 48/F Hirata (1986)
CBD
0.5 cm
jaundice
res. of EHBD
good (2 yrs 10 mos follow up)
CBD
1.2 cm
jaundice pruritus
pancreatoduodenectomy
dead (1 yr, sepsis)
hepatic hilum
1.7cm
jaundice
partial hepatectomy res. of EHBD
good (2 yrs 6 mos follow up)
10. 75/F Nakagawa (1988) 11. 27/M Tsunoda (1989)
CHD, common hepatic duct; CBD, common bile duct; res., resection; EH, extrahepatic; BD, bile duct
T h e operative findings s e e m to vary with the process o f the disease. T h e h e p a t o d u o denal ligament was diffusely involved by fibrosis in different densities and a firm mass or cord-like mass o f the bile duct was felt between e x a m i n i n g fingers. T h e p r e s e n c e o f
enlarged soft lymph nodes was described in 5 patients which may be helpful for diagnosing this inflammatory disease intraoperatively. T h e interpretation o f tissue frozen sections is also quite difficult a n d to rule out bile duct cancer completely, we must resect
Tsunoda et al.
334
the lesion or take multiple biopsies from the fibrosing bile duct and submit these for pathological examination. With regard to the surgical procedure, resection of the lesion was done in 8 patients, drainage o f the bile duct in 2 and stenting of the bile duct in 1. T h e r e were 2 operative deaths, resulting from failure to ameliorate severe cholangitis. Surgical resection was not inferior to drainage or stenting of the bile duct in terms of follow-up results. Current investigations on such etiological factors as immune complexes and immunologic injury directed at the bile duct epithelium, may pave the way for a rational treatment program in the future. At present, however, we believe that a patient with extrahepatic segmental PSC mimicking bile duct cancer should be treated by resection and followed UP Over a long period to reveal whether or n o t r e s e c t i o n influences its progress. (Received for publication on Jan. 19, 1990)
7.
8.
9.
10.
11.
REFERENCES 1. Helzberg JH, Petersen JM, Boyer JL. Improved survival with primary sclerosing cholangitis. Gastroenterology 1987; 92: 1869-1875. 2. Schrnmpf E, Fausa O, Kolmannskog F, Elg~o K, Ritland S, Gjone E. Sclerosing cholangitis in ulcerative colids: a follow-up study. Scand J Gastroenterol 1982; 17: 33-39~ 3. Furukawa H; Kikuchi T, Taniguchi T, Hara S, Ryu M. A case report of primary sclerosing cholangitis--A review of Japanese literature. Nippon Shokaki Gel(a Gakkai Zasshi (jpn J Gastroenterol Surg) 1984; 17: 1883-1886. (in Japanese) 4. Caroli J, Rosner D. Chronic sclerosing cholangitis. In: Bockus HK, ed. Gastroenterology, Vol 3. Philadelphia: WB Saunders 1976; 868-873. 5. Sugita T, Hatano H, Hanaoka M, Tominaga S, Sakaguchi S, Kitami Y, Ishida T. Two cases of primary sclerosing cholangitis. Geka Shinryo (Geka Shinryo) 1973; 15: 737-742. (in Japanese) 6. Beppu M, Horikawa S, Hikita K, Kurita K, Hirai T,
12.
13.
14.
15.
JPn~l~y Surg. 1991
Murai N, Taniguchi S, Yoshimoto S, Doi Y, Uehara N, Ohta T. Primary sclerosing cholangitisA case report. Nippon Shokaldbyo Gakkai Zasshi (Jpn J Gastroent) 1976; 73: 1590-1598. (in Japanese) Kawamura S. Nagadomi Y, Harada T, Fuji T, Shimizu M, Kodama T, Okamoto Y,-Noda K, Mizuta M, Takemoto T. The interesting retrograde cholangiographic appearance of primary sclerosing cholangitis and other similar diseases. Nippon Shokaki Naishikyo Gakkai Zasshi (Gastroenterol Endoscopy) 1977; 19: 140-148. (inJapanese~ Yoshikawa S, Nakaba H, Sasako Y, Nishigaki K, Maeda G, Saya M. A case of primary sclerosing cholangitis treated by left hepatic lobectomy and right intrahepaticojejunostomy. Geka Shinryo (Geka Shinryo) 1982; 24: 223-227. (in Japanese) Narai S, Otsuka T, Sato T, Imaizumi S, Kuribayashi K. Sato Y, Yoshida K, Yamagiwa I. A case of segmental sclerosing cholangitis. Nippon Rinsho Geka Igakkai Zasshi (Journal of Japanese Society for Clinical Surgery) 1982; 12: 1356-1361. (in Japanese) Ito N, Oishi A, Tameda Y, Kosaka Y, Takezawa H, Yatani R, Miyake T, Hamaguchi K. Primary sclerosmg cholangitis: Reports of two autopsied cases and review of fifty-one cases reported in Japan. Kanzo (Acta HepatologicaJaponica) 1982; 23: 1184-1192. (in Japanese) Nara N, Bizen R. Uemura S, Sousetsu T, Kudoh T. A case of primary sclerosing cholangitis mimicking bile duct cancer. Naika (Internal Medicine) 1982; 49: 179-182. (in Japanese) Suzuki N, Machi Y, Hirohashi K, Kinoshita H, Sakai K, Kobayashi Y. A case report of primary sclerosing cholangitis. Shokaki Geka (Gastroenterological Surgery) 1984; 7: 621-624. (in Japanese) Hirata S, Sasaki N, Inoue K, Kawaguchi M, Mutou H, Orihashi K, Gomyo Y, Ohe K. A case report of localized primary sclerosing cholangitis mimicking choledochal cancer. Nippon Rinsho Geka Igakkai Zasshi (Journal of Japanese Society for Clinical Surgery) 1986; 47: 1334-1340. (in Japanese) Nakagawa H, Nakagou R, Ohyama M, Yoshida E, Kobayashi T, Kurokawa T, Manabe T, Kobayashi S. A case of localized, primary sclerosing cholangitis difficult to differentiate from cholangiocarcinoma. Nippon Rinsho Geka Igakkai Zasshi (Journal of Japanese Society for Clinical Surgery) 1988; 49: 892-898. (in Japanese) Thompson HH, Pitt HA, Tompkins RK, Longmire WP Jr. Primary sclerosing cholangitis--A heterogeneous disease. Ann Surg 1982; 196: 127-136.
Segmental Primary Sclerosing Cholangitis Mimicking Bile Duct CancermReport of a Case and Review of the Japanese Literature T s u k a s a TSUNODA 1, Toshifumi ETO 1, Masafumi YAMADA1, Yoshitsugu TAJ!MA~, Shigetoshi MATSUOx, Ryoichi TSUCHIYA1, Tsuneo SHIOZAWA2 and
Takeshi MATSUOa ABSTRACT: A rare case of the segmental type of primary sclerosing cholangitis (PSC) is reported herein. A 27 year old Japanese m a n with obstructive jaundice was referred to our hospital with a provisional diagnosis of bile duct cancer at the hepatic hilum. A PTCD tube h a d been inserted f r o m the right anterior segmental duct and direct cholangiograms showed complete obstruction of the bile duct at the confluence of the right and left hepatic ducts. Resection of the extrahepafic bile duct a n d partial hepatectomy were thus performed, however, th e postoperative histological examination revealed no malignant features. Instead, the lesion was shown to be compatible with PSC microscopically. A diagnosis of PSC was finally established on the basis of clinical features, cholangiographic appearance and the pathological report. KEY WORDS:
primary sclerosing cholangitis, segmental type
INTRODUCTION
P r i m a r y sclerosing cholangitis (PSC), a rare disease o f u n k n o w n etiology, is characterized by chronic fibrosing inflammation of the bile ducts, usually affecting both the extrahepatic and intrahepatic ductal systems. In some instances~ the disease involves the extrahepatic bile duct segmentally, thereby creating symptoms mimicking bile duct cancer. We recently treated a patient with a 1The Second Department of Surgery, Nagasaki University School of Medicine, 2theDepartment of lnternal Medicine, SaseboSenju Hospital, and 3theFirstDepartment of Pathology, Nagasaki University School of Medicine, Nagasaki,Japan Reprint requests to: Tsukasa Tsunoda, MD, The Second Department of Surgery, Nagasaki University School of Medicine, 7-1 Sakamoto-machi, Nagasaki 852, Japan
localized stricture of the bile duct at the hepatic hilum. The lesion was managed by resection of the extrahepatic bile d u c t a n d partial hepatectomy following an initial diagnosis of bile duct cancer. As it was found to be microscopically compatible with PSC, the lesion was subsequently diagnosed as the segmental type of PSC on the basis of clinical features, cholangiographic appearance and, the pathological report. We report this case herein and discuss the problems associated with the diagnosis and treatment of-this disease, reviewing the literature on similar cases reported previously in Japan. CASE REPORT
A 27 year old Japanese man was referred to our hospital on 15 April, 1987 ~ i t h a suspected diagnosis of bile duct cancer at the hepatic hilum. He had been well until de-
JAPANESEJOURNALOF SURGERY,VOL.21, No. 3 pp. 329-334, 1991
330
Tsunoda et al.
veloping jaundice, 2 months prior to his admission. The disease had been diagnosed as obstructive jaundice on March 19, 1987, and he had u n d e r g o n e percutaneous transhepatic biliary drainage (PTBD) at another hospital. There were no other symptoms or signs and he had never undergone any abdominal surgery. His past and family histories were essentially negative apart from the fact that his mother hadcirrhosis of the liver. Physical examination revealed him to be in a good nutritional state. The palpebral conjunctiva was slightly icteric. A PTBD t u b e had been inserted f r o m below the right costal margin and, apart from the jaundice, there were no abnormal findings. Laboratory tests revealed a total bilirubin value of 2.8 mg/dl, the direct being 2.0 mg/dl and an alkaline phosphatase value of 441 IU/1, the normal being 75-266, Serum electrolytes and amylase levels were within normal limits. Tests for antimitochondrial antibody and anti-smooth-muscle antibody were done postoperatively and both proved negative. CT scanning revealed dilatation of the intrahepatic bile ducts but failed to demonstrate any mass lesions at the hepatic hilum (Fig. 1). Endoscopic retrograde cholangiography (ERC)showed complete obstruction of the common hepatic duct (Fig. 2). There were no stones in the gallbladder or bile
Fig. 1. A C T ' showing dilatation of the intrahepatic bile ducts.
1991
Fig. 2. An ERCP showing the normalsized extrahepatic bile duct with complete obstruction at the hepatic hilum. duct. C h o l a n g i o g r a p h y was p e r f o r m e d through the PTBD tube which had been inserted at the other hospital, but the left intrahepatic ducts were not visualized. Therefore, percutaneous transhepatic cholangiography (PTC) was performed through the left segmental duct, and cholangiography through the PTBD at the same time. The cholangiogram showed complete obstruction of the bile duct at the confluence of the right and left hepatic ducts and marked dilatation of the left intrahepatic bile ducts (Fig. 3). Celiac angiography showed n o abnormal findings. Cytology of the bile drainage specimen was done 3 times with negative results for malignancy. On 27 April, 1987, the patient underwent abdominal exploration, based on the initial diagnosis of bile duct cancer at the hepatic hilum. The liver, though not enlarged was slightly greenish while the gallbladder was unremarkable. The hepatoduodenal ligament was slightly involved by fibrosis and no
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Primary sclerosing cholangitis
331
Fig. 3. Simultaneous direct cholangiogram from both the anterior and lateral segmental ducts of the liver. V-shaped complete obstruction of the right hepatic duct and U-shaped complete obstruction of the left hepatic duct with marked dilatation are demonstrated. mass lesion was palpable along the bile duct, which was felt normally. After dissection o f the hepatoduodenal ligament, a small firm mass was f o u n d in the confluence o f the hepadc ducts, extending slightly into the fight hepatic duct. T h e r e were a few large soft lymphnodes along the c o m m o n bile duct, the largest of which was 1 )< 3 cm in diameter. Frozen secdons o f the lymphnode proved negative for malignancy. Resection o f the extrahepadc bile ducts, dissection o f l y m p h n o d e s i n the h e p a t o d u o d e n a l ligament and partial hepatect0my of Couinaud's segment 4 and 5 were performed. C h o l e cystectomy was also done. The intestinal continuity was reconstructed by anastomoses o f the two fight intrahepadc bile ducts and a left hepatic duct to the p-looped jejunal limb in the Roux-en-Y fashion. Macroscopic examination of the resected specimen revealed the bile duct wall of the affected area to be thick and fibrolas with a tiny lumen (Fig. 4). Microscopy of the stricture site showed wall thickening by the fibrous tissue occupying all layers o f the bile ducts except the mucosa. Chronic inflammatory cells such as lymphocytes, histiocytes a n d eosin0phils infiltrated the t h i c k e n e d bile duct Wall (Fig. 5). Malignant features
a
b
Fig. 4. a. Resected specimen, b. An illustration of the resected specimen: The bile duct wall is thickened at the confluence of the hepatic ducts. Fibrosis is most severe in the fight hepatic bile duct. RHD: right hepatic duct, L H D : left. hepatic duct, PSD: posterior segmental duct.
332
Tsunoda et al.
Fig. 5. Microphotograph showing wall thickening of the bile duct by fibrous tissue (HE, X40). The mucosa is virtually intact.
were not demonstrated a n d reactive lymp h o a d e n o p a t h y was shown in the regional lymphnodes. T h e patient's postoperative course was uneventful and he has b e e n well for the last 2 a n d a half years without any symptoms. DISCUSSION
T h e n u m b e r o f reports o f PSC has b e e n increasing in the USA a n d E u r o p e a n countries, x,~but it is still a rare disease in Japan. In fact, Furukawa et al. in 1984, collected only 76 cases from the J a p a n e s e literature? T h e r e is no a g r e e m e n t concerning the diagnostic criteria o f PSC but at present, it seems to require the exclusion o f choledocholithiasis, previous biliary surgery, and bile duct carcinoma in patients with sclerosis o f the bile duct. A purely anatomic classification o f PSC has b e e n p r o p o s e d by Caroli a n d R o s n e r 4 w h o divide the c o n d i t i o n i n t o diffuse involvement of the biliary tree and segmental involvement. T h e latter is further divided into three subgroups namely: a) fibroinflammatory involvement of the hepatic duct junction; b) selective involvem e n t o f the c o m m o n hepatic duct; and c) selective involvement of the c o m m o n bile duct. However, in some instances it is difficult to decide which of Caroli a n d Rosner's
Jpn. J. Surg. May 1991
groups a particular patient belongs. T h e Furukawa report o f 76 cases revealed that diffuse involvement was seen in 77 per cen t of the cases, while the segmental type was seen in 23 p e r cent, a m o n g which subgroups a) a n d b ) were s e e n in 16 per cent a n d subgroup c) in 7 p e r cent? T h e exact pre- a n d intraoperative diagnoses o f segmental PSC are extremely difficult to establish because o f its rarity and the resemblance of the clinical features and cholangiographic a p p e a r a n c e to those o f bile duct cancer. T o elucidate the problems of this obscure disease, 11 cases reported in detail in the J a p a n e s e literature, including our case, were analyzed (Table 1). 5-14 T h e r e was no history o f previous surgery in any o f the patients, n o r any association with gallstone disease or ulcerative colitis. T h e m e a n age was 51.9 years, with a r a n g e o f 24 to 80 years, a n d there was no sex preponderance. Jaundice and pruritus, occasionally in association with pain or fever, were the two most c o m m o n symptoms. T h e preoperative diagnosis was bile duct cancer or suspected bile duct cancer in all 11 patients. According to Caroli a n d Rosner's subgroup division, 3 patients b e l o n g e d to the a) subgroup, 5 to b) and 3 to c). T h e proximal bile duct was often affected with the m e a n strictured length o f the bile duct in 8 cases b e i n g 1.6 cm, on cholangiograms or resected specimens. A review o f the results of direct cholangiography (ERC, PTC) revealed that contrast material passed though the strictured site in 6 patients (55 per cent), while complete obstruction was demonstrated in 5 (45 p e r cent). In 2 o f these 5 patients (Cases8 and 9), however, the contrast material started to pass through the strictured site during a preoperative PTBD. T h e passage o f contrast material occurs m o r e often in PSC than bile duct cancer. Although L o n g m i r C ~ stressed that there is generally n o dilatation o f the proximal bile d u c t to the strictured site in PSC, proximal dilatation o f the bile duct in our collected cases was demonstrated in all patients with segmental PSC.
Volume 21 Number 3
333
Primary sclerosing cholangitis
Table 1. Summary of Cases of"Localized" Primary Sclerosing Cholangitis Reported in Japan Case Age/Sex Author (Year)
Location
Length
Symptoms
Treatment
Prognosis
1. 53/F Sugita (1973)
CHD
3 cm
aundice aruritus
T-tube drainage
operative death (biliary infection)
2: 33/M Beppu (1976)
CBD
2 cm
jaundice pain
pancreatoduodenectomy
good (1 yr 3 mos follow-up)
3. 56/M Kawamura (1977)
CHD
"localized"
jaundice pruritus
cholecystectomy stenting of BD
good (7 yrs follow up)
4. 6zt/M Yoshikawa (1982)
CHD
1.5 cm
jaundice pruritus
left hepatic lobectomy, res. of EHBD
good (1 yr 10 mos follow up)
5. 24/M Narai (1982)
CHD
0.5 cm
jaundice pruritus
res. of EHBD
good (1 yr follow up)
6. 80/F Ito (1982)
confluence of hepatic ducts
2.0 cm
jaundice pain
external drainage of BD
dead (11 mos, cholangitis)
7. 55/M Nara (1982)
hepatic hilum
?
jaundice pain
right hepatic lobectomy res. of EHBD
operative death (pneumonia)
8. 56/F Suzuki (1984)
CBD
"localized"
jaundice fever
res. of EHBD
good (3 yrs follow up)
9. 48/F Hirata (1986)
CBD
0.5 cm
jaundice
res. of EHBD
good (2 yrs 10 mos follow up)
CBD
1.2 cm
jaundice pruritus
pancreatoduodenectomy
dead (1 yr, sepsis)
hepatic hilum
1.7cm
jaundice
partial hepatectomy res. of EHBD
good (2 yrs 6 mos follow up)
10. 75/F Nakagawa (1988) 11. 27/M Tsunoda (1989)
CHD, common hepatic duct; CBD, common bile duct; res., resection; EH, extrahepatic; BD, bile duct
T h e operative findings s e e m to vary with the process o f the disease. T h e h e p a t o d u o denal ligament was diffusely involved by fibrosis in different densities and a firm mass or cord-like mass o f the bile duct was felt between e x a m i n i n g fingers. T h e p r e s e n c e o f
enlarged soft lymph nodes was described in 5 patients which may be helpful for diagnosing this inflammatory disease intraoperatively. T h e interpretation o f tissue frozen sections is also quite difficult a n d to rule out bile duct cancer completely, we must resect
Tsunoda et al.
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the lesion or take multiple biopsies from the fibrosing bile duct and submit these for pathological examination. With regard to the surgical procedure, resection of the lesion was done in 8 patients, drainage o f the bile duct in 2 and stenting of the bile duct in 1. T h e r e were 2 operative deaths, resulting from failure to ameliorate severe cholangitis. Surgical resection was not inferior to drainage or stenting of the bile duct in terms of follow-up results. Current investigations on such etiological factors as immune complexes and immunologic injury directed at the bile duct epithelium, may pave the way for a rational treatment program in the future. At present, however, we believe that a patient with extrahepatic segmental PSC mimicking bile duct cancer should be treated by resection and followed UP Over a long period to reveal whether or n o t r e s e c t i o n influences its progress. (Received for publication on Jan. 19, 1990)
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