Surgical audit: G.
10.
11.
12.
P. Copeland et al.
and complications in non-cardiac surgery. Medicine 1978; 57: 357-70. Goldman L, Caldera DL, Nussbaum SB et al. Multifactorial index of cardiac risk in non-cardiac surgical procedures. N Engl J Med 1971; 297: 845250. LeGall J, Loirat P, Alperovitch A et ul. A simplified acute physiology score for ITU patients. Crit Cure Med 1984; 12: 975-7. Knaus WA, Draper EA, Wagner DP et ul. Apache 11: a severity
13. 14.
ofdisease classification system. Crit Cure Med 1985; 13:818-29. Ramsay G, MacGregor JR, Murray GD, Neithercut D, Ledingham IMcA, George WD. Prediction of surgical risk in adults. Surg Res Comm 1988; 3 : 95-103. Overall JE, Williams CM. Models for medical diagnosis. Behuv Sci 1961; 6 : 13441.
Paper accepted 8 August 1990
Case report Br. J. Surg. 1991, Vol. 78, March, 360-361
Hepatocellular carci noma cornpl icat ing primary scl e rosi ng cholangitis T. Ismail, L. Angrisani, S. Hubscher* and P. McMaster The Liver Unit, Queen Elizabeth Hospital and *Department of Pathology, University of Birmingham,.Birmingham, UK Correspondence to; Mr T. Ismail, Liver Research Laboratories, Queen Elizabeth Hospital, Birmingham 815 2TH. U K Primary sclerosing cholangitis is a condition of unknown aetiology and ill-defined progression. Although a rare cause of chronic liver disease, it is the third most common indication for orthotopic liver transplantation'. The incidence of malignancy complicating primary sclerosing cholangitis is high'; nearly all tumours that occur with the condition are cholangiocellular carcinomas. T h e association of hepatocellular carcinoma occurring in a patient with chronic liver disease secondary t o primary .sclerosing cholangitis has not previously been well documented. We describe a case of well differentiated hepatocellular carcinoma arising in a cirrhotic liver associated with primary sclerosing cholangitis.
antigen and autoantibodies were negative and an isotope liver scan shovted general reduction of uptake but no focal lesion. Percutaneous needle liver biopsy showed fibrous expansion of portal tracts with a lymphoid infiltrate and concentric fibrosis around medium-sized bile ducts. After evaluation, the patient was accepted for the liver transplant programme. At transplantation in October 1987, a cirrhotic liver with gross portal hypertension and massive gastric varices were found. Total hepatectomy and liver replacement with a gallbladder conduit to a Roux-en-Y small bowel loop were performed. After operation the patient developed cardiorespiratory failure and septicaemia, and he died 17 days after transplantation. Gross examination revealed a green cirrhotic liver weighing 1060 g. On slicing, there were several discrete, pale brown nodules, the largest measuring 4 cm in diameter. Histological examination confirmed the presence of established cirrhosis with a mixed pattern of nodularity. There were occasional fibro-obliterative duct lesions and cystic dilations of intrahepatic bile ducts consistent with primary sclerosing cholangitis. The nodules seen macroscopically showed neoplastic transformation in the form of a well differentiated hepatocellular carcinoma with permeation of small vascular channels in surrounding fibrous septa. In addition, there was widespread liver cell dysplasia. Excision was complete with tumour negative nodes in the porta hepatis.
Discussion As far as we are aware, this is the first case of classical hepatocellular carcinoma occurring in a cirrhotic liver
A 39-year-old male Caucasian cabinet-maker presented in 1972 with pruritus and progressive jaundice. There was no history of alcohol abuse, blood transfusion or previous jaundice. At subsequent laparotomy, an operative cholangiogram was consistent with primary sclerosing cholangitis. Cholecystectomy and sphincterotomy were performed to improve biliary drainage. The patient then remained well until 1981 when he retired from his work because of increasing tiredness, fatigue and intermittent diarrhoea. Biopsies at colonoscopy confirmed ulcerative colitis throughout the colon. He settled on treatment with steroids and Salazopyrins (Pharmacia, Milton Keynes, UK). His general health gradually deteriorated and endoscopic retrograde cholangiopancreatography in 1984 confirmed changes consistent with primary sclerosing cholangitis with a major stricture at the hilum. This was initially managed by nasobiliary intubation and saline lavage without success. In 1987, the patient presented with hepatosplenomegaly, gross ascites and variceal bleeding. This precipitated encephalopathy and he was referred to our unit for further assessment. On admission, he was thin, wasted, and had a soft irregular enlarged liver. Investigations included serum bilirubin (47 pmol/l; normal range 5-20 pmol/l); alkaline phosphatase (1260 units/l; normal range 7C290 units/l); aspartate transaminase (91 units/l; normal range 5-20 units/l); haemoglobin (12.5 g/dl); prothrombin time (17/16 s); cc-fetoprotein (58 kilounits/l; normal range < 5 kilounits/l). Hepatitis B surface
complicating primary sclerosing cholangitis. The fibrolamellar variant of hepatocellular carcinoma associated with ulcerative colitis and primary sclerosing cholangitis has recently been reported3. T h e fibrolamellar variant of hepatoma generally occurs in young adults with non-cirrhotic livers and is said to have a relatively favourable prognosis4. One other case of hepatocellular carcinoma in a 26-year-old patient with primary sclerosing cholangitis has been reported5. Although no comment was made on the histological type, it may also have been a fibrolamellar variant as the liver parenchyma was non-cirrhotic with normal carcinoembryonic antigen and a-fetoprotein levels. The authors made particular reference to tumour recurrence in the graft following retransplantation for chronic rejection rather than the uniqueness of this occurrence. Combined hepatocellular a n d cholangiocellular carcinoma was found in one of eight patients with hepatobiliary tumours associated with primary sclerosing cholangitis and ulcerative colitis in a M a y o series'. The finding of hepatocellular carcinoma complicating primary sclerosing cholangitis has potentially important implications in the management of the condition. Management by means of reconstructive surgery or endoscopic manipulation is controversial. For end-stage liver disease due to primary sclerosing cholangitis, liver transplantation is now an accepted treatment option. Although cholangiocellular carcinoma is well recognized t o occur in association with the condition, there appears t o be a risk of hepatocellular carcinoma developing. Investigations should therefore include abdominal computed tomography and estimation of serum tumour markers (a-fetoprotein, neurotensiri and vitamin B, binding protein levels) in addition to routine screening. Intraoperative ultrasonography should be employed routinely a t the time of hepatobiliary surgery.
360
0007-1323/91/0303$02
Case report
0 1991 Butterworth-Heinemann
Ltd
Case report
Hepatocellular carcinoma associated with primary sclerosing cholangitis and cirrhosis limits the role of potentially curative liver transplantation because of the risk of tumour recurrence. However, incidental primary liver tumours have a relatively good outcome6. This case report is the first to associate hepatocellular carcinoma with primary sclerosing cholangitis and cirrhosis, and highlights the importance of routine screening before and during operation for a parenchymal tumour mass.
Acknowledgements
3. 4.
5.
T. Ismail is supported by a Sheldon Fellowship from the West Midland Regional Health Authority.
References 1.
2.
European Liver Transplant Registry. Hospital Paul Brousse,
6.
Villejuif, France. December 1988. Wee A, Ludwig J, Coffey RJ, La Russo NF, Weisner RH. Hepatobiliary carcinoma associated with primary sclerosing cholangitis and chronic ulcerative colitis. Hum Patholl985; 16(7): 719-26. Snook JA, Kelly P, Chapman RW, Jewel1 D P . Fibrolamellar hepatocellular carcinoma complicating ulcerative colitis with primary sclerosing cholangitis. Gut 1989; 30: 243-5. Starzl TE, Iwatsuki S, Shaw BW Jr, Nalesnik MA, Farhi DC, van Theil DH. Treatment of fibrolamellar hepatoma with partial or total hepatectomy and transplantation of the liver. Surg Gynecol Obstet 1986; 162: 145-8. Klompmaker IJ, de Bruijn K M , Gouw AH, Barns JL, Slooff MJH. Recurrence of hepatocellular carcinoma after liver retransplantation. Br Med J 1988; 296: 1445. Iwatsuki S, Gordon RD, Shaw BW, Starzl TE. Role of liver transplantation in cancer therapy. Ann Surg 1985; 202: 401-7.
Paper accepted 18 September 1990
Surgical workshop Br. J. Surg. 1991, Vol. 78, March, 361-362
Patients and methods
Use of a resorbable mesh graft t o obtain haemostasis from the cut surface of the liver after hepatic resection
The data were derived from a review of the records of 153 patients undergoing hepatic resection (1983-89) in the Clinic of Digestive Surgery at Geneva University Hospital. In this series, 13 patients (8 per cent) had persistent bleeding of the cut surface of the liver despite selective suturing of bleeding points. These 13 patients received a mesh graft of polyglycolic acid to cover the cut surface of the liver and are the subject of this report. There were seven right and three left hepatectomies, two right bisegmentectomies (one in a cirrhotic patient and one in a transplanted liver) and one right lobectomy (Couinaud's classification). The
R . Prgtre, G. Mentha and A. Rohner Department of Surgery, Clinic of Digestive Surgery, H6pital Cantonal Universitaire, Geneva, Switzerland Correspondence to: Professor A. Rohner, Dbparternent de Chirurgie, Clinique de Chirurgie Digestive, HBpital 'Cantonal Universitaire, 1211 -Geneva 4, Switzerland
Haemostasis following hepatic resection may be difficult to achieve. Individual bleeding points are best treated by selective ligation or by sutures carefully placed in the parenchyma, while residual oozing is usually controlled by electrocautery and local tamponade. Various topical haemostatic agents are also frequently applied. It is customary then to cover the raw surface of the liver to maintain a tamponading effect, to minimize any leakage of bile and, perhaps, also to prevent subsequent visceral adhesions. The omentum and the falciform ligament have long been recognized as the best materials for this purpose'. The omentum in particular serves as both a source of tissue thromboplastin to promote local coagulation and as a large source of macrophages and lymphatic vessels to carry away dead cells and tissue debris. However, the omentum is not always available, while the falciform ligament is frequently too short or has already been destroyed. Construction of another autologous flap from Gerota's fascia has been described'. Although it creates a communication between the abdominal cavity and the retroperitoneum, no related complications have been reported. In circumstances when an autologous flap was not readily available, we have employed a resorbable mesh graft of polyglycolic acid, anchored at the hepatic capsule, to achieve haemostasis of the liver surface (Figure I). Results of this technique are briefly reported.
Figure 1 Resorbable mesh graft sutured on to the resected margin of the liver. This 30-year-old patient had undergone urgent orthotopic liver transplantation for acute hepatic failure. He was reoperated on on the 23rd day after surgery for resection of infected necrosis of segments V I and VII. Haemostasis had been dgfficult to obtain and the mesh was applied to help eliminate residual oozing. Bleeding stopped as assessed both cliniially and by a Second look' on the following day. Sepsis did not persist even with immunosuppressive therapy. The patient was discharged home soon afterwards, and is currently well
.~
0007-1323/9 1/03036 1 4 2
0 199 1 Butterworth-Heinemann
Ltd
361
10.
11.
12.
P. Copeland et al.
and complications in non-cardiac surgery. Medicine 1978; 57: 357-70. Goldman L, Caldera DL, Nussbaum SB et al. Multifactorial index of cardiac risk in non-cardiac surgical procedures. N Engl J Med 1971; 297: 845250. LeGall J, Loirat P, Alperovitch A et ul. A simplified acute physiology score for ITU patients. Crit Cure Med 1984; 12: 975-7. Knaus WA, Draper EA, Wagner DP et ul. Apache 11: a severity
13. 14.
ofdisease classification system. Crit Cure Med 1985; 13:818-29. Ramsay G, MacGregor JR, Murray GD, Neithercut D, Ledingham IMcA, George WD. Prediction of surgical risk in adults. Surg Res Comm 1988; 3 : 95-103. Overall JE, Williams CM. Models for medical diagnosis. Behuv Sci 1961; 6 : 13441.
Paper accepted 8 August 1990
Case report Br. J. Surg. 1991, Vol. 78, March, 360-361
Hepatocellular carci noma cornpl icat ing primary scl e rosi ng cholangitis T. Ismail, L. Angrisani, S. Hubscher* and P. McMaster The Liver Unit, Queen Elizabeth Hospital and *Department of Pathology, University of Birmingham,.Birmingham, UK Correspondence to; Mr T. Ismail, Liver Research Laboratories, Queen Elizabeth Hospital, Birmingham 815 2TH. U K Primary sclerosing cholangitis is a condition of unknown aetiology and ill-defined progression. Although a rare cause of chronic liver disease, it is the third most common indication for orthotopic liver transplantation'. The incidence of malignancy complicating primary sclerosing cholangitis is high'; nearly all tumours that occur with the condition are cholangiocellular carcinomas. T h e association of hepatocellular carcinoma occurring in a patient with chronic liver disease secondary t o primary .sclerosing cholangitis has not previously been well documented. We describe a case of well differentiated hepatocellular carcinoma arising in a cirrhotic liver associated with primary sclerosing cholangitis.
antigen and autoantibodies were negative and an isotope liver scan shovted general reduction of uptake but no focal lesion. Percutaneous needle liver biopsy showed fibrous expansion of portal tracts with a lymphoid infiltrate and concentric fibrosis around medium-sized bile ducts. After evaluation, the patient was accepted for the liver transplant programme. At transplantation in October 1987, a cirrhotic liver with gross portal hypertension and massive gastric varices were found. Total hepatectomy and liver replacement with a gallbladder conduit to a Roux-en-Y small bowel loop were performed. After operation the patient developed cardiorespiratory failure and septicaemia, and he died 17 days after transplantation. Gross examination revealed a green cirrhotic liver weighing 1060 g. On slicing, there were several discrete, pale brown nodules, the largest measuring 4 cm in diameter. Histological examination confirmed the presence of established cirrhosis with a mixed pattern of nodularity. There were occasional fibro-obliterative duct lesions and cystic dilations of intrahepatic bile ducts consistent with primary sclerosing cholangitis. The nodules seen macroscopically showed neoplastic transformation in the form of a well differentiated hepatocellular carcinoma with permeation of small vascular channels in surrounding fibrous septa. In addition, there was widespread liver cell dysplasia. Excision was complete with tumour negative nodes in the porta hepatis.
Discussion As far as we are aware, this is the first case of classical hepatocellular carcinoma occurring in a cirrhotic liver
A 39-year-old male Caucasian cabinet-maker presented in 1972 with pruritus and progressive jaundice. There was no history of alcohol abuse, blood transfusion or previous jaundice. At subsequent laparotomy, an operative cholangiogram was consistent with primary sclerosing cholangitis. Cholecystectomy and sphincterotomy were performed to improve biliary drainage. The patient then remained well until 1981 when he retired from his work because of increasing tiredness, fatigue and intermittent diarrhoea. Biopsies at colonoscopy confirmed ulcerative colitis throughout the colon. He settled on treatment with steroids and Salazopyrins (Pharmacia, Milton Keynes, UK). His general health gradually deteriorated and endoscopic retrograde cholangiopancreatography in 1984 confirmed changes consistent with primary sclerosing cholangitis with a major stricture at the hilum. This was initially managed by nasobiliary intubation and saline lavage without success. In 1987, the patient presented with hepatosplenomegaly, gross ascites and variceal bleeding. This precipitated encephalopathy and he was referred to our unit for further assessment. On admission, he was thin, wasted, and had a soft irregular enlarged liver. Investigations included serum bilirubin (47 pmol/l; normal range 5-20 pmol/l); alkaline phosphatase (1260 units/l; normal range 7C290 units/l); aspartate transaminase (91 units/l; normal range 5-20 units/l); haemoglobin (12.5 g/dl); prothrombin time (17/16 s); cc-fetoprotein (58 kilounits/l; normal range < 5 kilounits/l). Hepatitis B surface
complicating primary sclerosing cholangitis. The fibrolamellar variant of hepatocellular carcinoma associated with ulcerative colitis and primary sclerosing cholangitis has recently been reported3. T h e fibrolamellar variant of hepatoma generally occurs in young adults with non-cirrhotic livers and is said to have a relatively favourable prognosis4. One other case of hepatocellular carcinoma in a 26-year-old patient with primary sclerosing cholangitis has been reported5. Although no comment was made on the histological type, it may also have been a fibrolamellar variant as the liver parenchyma was non-cirrhotic with normal carcinoembryonic antigen and a-fetoprotein levels. The authors made particular reference to tumour recurrence in the graft following retransplantation for chronic rejection rather than the uniqueness of this occurrence. Combined hepatocellular a n d cholangiocellular carcinoma was found in one of eight patients with hepatobiliary tumours associated with primary sclerosing cholangitis and ulcerative colitis in a M a y o series'. The finding of hepatocellular carcinoma complicating primary sclerosing cholangitis has potentially important implications in the management of the condition. Management by means of reconstructive surgery or endoscopic manipulation is controversial. For end-stage liver disease due to primary sclerosing cholangitis, liver transplantation is now an accepted treatment option. Although cholangiocellular carcinoma is well recognized t o occur in association with the condition, there appears t o be a risk of hepatocellular carcinoma developing. Investigations should therefore include abdominal computed tomography and estimation of serum tumour markers (a-fetoprotein, neurotensiri and vitamin B, binding protein levels) in addition to routine screening. Intraoperative ultrasonography should be employed routinely a t the time of hepatobiliary surgery.
360
0007-1323/91/0303$02
Case report
0 1991 Butterworth-Heinemann
Ltd
Case report
Hepatocellular carcinoma associated with primary sclerosing cholangitis and cirrhosis limits the role of potentially curative liver transplantation because of the risk of tumour recurrence. However, incidental primary liver tumours have a relatively good outcome6. This case report is the first to associate hepatocellular carcinoma with primary sclerosing cholangitis and cirrhosis, and highlights the importance of routine screening before and during operation for a parenchymal tumour mass.
Acknowledgements
3. 4.
5.
T. Ismail is supported by a Sheldon Fellowship from the West Midland Regional Health Authority.
References 1.
2.
European Liver Transplant Registry. Hospital Paul Brousse,
6.
Villejuif, France. December 1988. Wee A, Ludwig J, Coffey RJ, La Russo NF, Weisner RH. Hepatobiliary carcinoma associated with primary sclerosing cholangitis and chronic ulcerative colitis. Hum Patholl985; 16(7): 719-26. Snook JA, Kelly P, Chapman RW, Jewel1 D P . Fibrolamellar hepatocellular carcinoma complicating ulcerative colitis with primary sclerosing cholangitis. Gut 1989; 30: 243-5. Starzl TE, Iwatsuki S, Shaw BW Jr, Nalesnik MA, Farhi DC, van Theil DH. Treatment of fibrolamellar hepatoma with partial or total hepatectomy and transplantation of the liver. Surg Gynecol Obstet 1986; 162: 145-8. Klompmaker IJ, de Bruijn K M , Gouw AH, Barns JL, Slooff MJH. Recurrence of hepatocellular carcinoma after liver retransplantation. Br Med J 1988; 296: 1445. Iwatsuki S, Gordon RD, Shaw BW, Starzl TE. Role of liver transplantation in cancer therapy. Ann Surg 1985; 202: 401-7.
Paper accepted 18 September 1990
Surgical workshop Br. J. Surg. 1991, Vol. 78, March, 361-362
Patients and methods
Use of a resorbable mesh graft t o obtain haemostasis from the cut surface of the liver after hepatic resection
The data were derived from a review of the records of 153 patients undergoing hepatic resection (1983-89) in the Clinic of Digestive Surgery at Geneva University Hospital. In this series, 13 patients (8 per cent) had persistent bleeding of the cut surface of the liver despite selective suturing of bleeding points. These 13 patients received a mesh graft of polyglycolic acid to cover the cut surface of the liver and are the subject of this report. There were seven right and three left hepatectomies, two right bisegmentectomies (one in a cirrhotic patient and one in a transplanted liver) and one right lobectomy (Couinaud's classification). The
R . Prgtre, G. Mentha and A. Rohner Department of Surgery, Clinic of Digestive Surgery, H6pital Cantonal Universitaire, Geneva, Switzerland Correspondence to: Professor A. Rohner, Dbparternent de Chirurgie, Clinique de Chirurgie Digestive, HBpital 'Cantonal Universitaire, 1211 -Geneva 4, Switzerland
Haemostasis following hepatic resection may be difficult to achieve. Individual bleeding points are best treated by selective ligation or by sutures carefully placed in the parenchyma, while residual oozing is usually controlled by electrocautery and local tamponade. Various topical haemostatic agents are also frequently applied. It is customary then to cover the raw surface of the liver to maintain a tamponading effect, to minimize any leakage of bile and, perhaps, also to prevent subsequent visceral adhesions. The omentum and the falciform ligament have long been recognized as the best materials for this purpose'. The omentum in particular serves as both a source of tissue thromboplastin to promote local coagulation and as a large source of macrophages and lymphatic vessels to carry away dead cells and tissue debris. However, the omentum is not always available, while the falciform ligament is frequently too short or has already been destroyed. Construction of another autologous flap from Gerota's fascia has been described'. Although it creates a communication between the abdominal cavity and the retroperitoneum, no related complications have been reported. In circumstances when an autologous flap was not readily available, we have employed a resorbable mesh graft of polyglycolic acid, anchored at the hepatic capsule, to achieve haemostasis of the liver surface (Figure I). Results of this technique are briefly reported.
Figure 1 Resorbable mesh graft sutured on to the resected margin of the liver. This 30-year-old patient had undergone urgent orthotopic liver transplantation for acute hepatic failure. He was reoperated on on the 23rd day after surgery for resection of infected necrosis of segments V I and VII. Haemostasis had been dgfficult to obtain and the mesh was applied to help eliminate residual oozing. Bleeding stopped as assessed both cliniially and by a Second look' on the following day. Sepsis did not persist even with immunosuppressive therapy. The patient was discharged home soon afterwards, and is currently well
.~
0007-1323/9 1/03036 1 4 2
0 199 1 Butterworth-Heinemann
Ltd
361
