1
HEPATOLOGY Vol. 22, No. 4, Pt. 2, 1995 AASLD ABSTRACTS 145A 153 SURGICAL PORTO-SYSTEMIC SHUNT IN CHILDREN WTI'H VARICEAL BLEEDING. N Jabbour, ] Reves. S Todo. C Ramirez, "rE Starzl Pittsburgh Transplantation Institute, Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania. Varieeal bleeding is a major complication in children with portal hypertension, with or without cirrhosis. Sclerotherapy is considered the treatment of choice in these patients, however, porto-systemic shunting plays a major role in children who fail sclerotherapy; and in those with variceal disease extending into the stomach making sclerotherepy difficult and dangerous. We report our experience in 15 children who underwent porto-systemic shunting, between 1989 to 1995:1 H type porto-tarsi shunt, 1 side-to-side splenorenal shunt, and 13 distal splenorenal shunts. The mean age was 8.6 years (range 3-14 years). The primary diagnosis was biliary atresia (n=6), congenital hepatic fibrosis (n=2), portal vein thrombosis without cirrhosis (n =4) and portal vein thrombosis after liver transplantation (LTX) (n =3). All patients had multiple episodes of variceal bleeding that failed repeated sclerotherapy. Shunt procedure was performed semi-eleetively in all patients. Doppler ultrasound was used to document shunt patancy at 6 months intervals. The operation was performed suecessfaUy in all children, without any operative mortality or morbidity. During follow-up period of 4 years (3 months - 6 years) recurrent variceal bleeding was observed in 2 patients: 1 developed shunt thrombosis, that required LTX 4 mouths after shunt procedure, and the other is awaiting liver re-transplantation due to progressive liver disease 4 years post-shunt procedure. Eseephalopathy was observed in 1 patient in the early post-operative period that responded to conservative treatment. All patients are presently functional and with normal growth. Shunt patency was documented in all but 1 patient throughout the follow-up period. We conclude that surgical shunts play a major role in children with variceal bleeding and preserved liver fimction who fail sclerotherapy. This procedure is technically feasible even in small size patients, has a low incidence of encephalopathy, and a high shunt patensy rate. 154 SPIRAL COMPUTED ANGIOGRAPHY FOR THE EVALUATION OF VESSEL ENCASEMENT FOR PANCREATIC CANCER. J Cunninqharn. N. Glaichen*. S. Brewer. Departments of Surgery and Radiology*, Mount Sinai Medical Center, New York, NY Introduction: Preoperative imaging of patients with pancreatic cancer is crucial in determining resectability and planning treatment. Purpose: To determine the role spiral computed angiography (SCA) plays in the evaluation of vessel involvement in patients with pancreatic cancer. Materials and Methods: SCA was obtained in six cases to evaluate the mesenteric vessels for encasement SCA images are obtained by a single 30 second breath hold for each of the mesenteric arterial and venous images. 3D images were generated with the helical data sets in any plane desired to evaluate vessel encasement. Results: In case #1, the SCA revealed complete obstruction of the portal vein (PV) at the superior mesenteric vein (SMV)-splenic vein (SV) confluence not seen on dynamic CT. In case #2 and #5, the vessels were normal. In case #3, the tumor was compressing the inferior vena cava but the mesenteric vessels were normal. In case #4, the CT was read as encasement of the PV at the SMV confluence but the SCA showed no evidence of this. In the last case, there was no arterial encasement and the venous phase was shot too late and images could not be obtained. The dynamic CT in this case showed PV encasement. The operative findings correlated with the SCA images in all five cases explored. In case #3, distant metastatic disease was found on laparoscopy and the patient was not explored. The finding of PV encasement seen on two CT scans was not found at the time of surgery. In no case was celiac or superior mesenteric artery involvement detected. The sensitivity (SENS), specificity (SPEC), positive predictive value (PPV), negative predictive value (NPV), and accuracy (ACC) were calculated for the four vessels evaluated: CT SCA CONCLUSION: SCA allows for a non- SENS 50% 100% invasive evaluation of the mesenteric SPEC 86% 100% vessels. This test is quicker, less ACC 72% 100% expensive and uses less of a dye toad PPV 33% 100% than angiography. The preliminary NPV 92% 100% results appear to be very reliable. 155 IMAGE ANALYSIS OF LESIONS OF THE GAI/3LADDERAND BILIARY SYSTEM, E.M.B~t, ~ . Saint LouisUniverslty and St. John's Mercy Medical Center, St. Louis, MO. DMA ploidy analysis of lesions arising from the intra- and extrahepatie billary system and gallbladder was performed utilizingFeulgen-stalned 5#m sections from archival paraffln-embedded tissue. The Cell Analysis System Image Analyzer (CAS-200) was utilized for the study. Cases Included: cholangioearclnome (CCa), extra- hepatic bile duct cancer (BDCa), carcinoma of the gallbladder (GBCa), bile duct adenome (BDA), adenomyoma (Ad), and normal gallbladder (NPD) as controls. The number of cells analyzed ranged from 113-240, average 184. CV's ranged from 1.96 to 16.3, average 9.00. DNA INDEX (Main Peak) DX (n) DIPLOID ANEUPL TETRAPL Aneup with (0.9-1.10)(1.11-1.79) (1.80-2.20) extension into hypertetrapl region CCa 6 3 2 l BDCa 5 3 1 1 GBCa ii 1 6 2 2 BDA 2 2 Ad 3 3 NPD 5 5 CONCLUSIONS: I) All benign lesions (NPD, Ad, A, BDA) showed predominantly diploid populations. 2) Aneuploidy, tetraploidy, and hypertetraploldy were seen in 10/11 GBCa, 3/6 CCa, 2/5 BDCa. 3) All 3 well-differentlated Ca's (2 CCa, I BDCa) had >67Z diploid populations. Moderately or poorly differentiated Ca's with dlploid peaks (1 CCa, 2 BDCa) contained 40-50Z populations in aneuplold or tetraplold regions; 1 moderately differentiated GB Ca contained 80g of cells in the diploid region. 4) Image analysis is useful for studying DNA ploldy of lesions of billary origin, and may be applied when small t~or vol~,~es are present in tissue sections. 156 p53 AND K-RAS GENE MUTATIONS IN CHOLANGIOCARCINOMA. A Nakeeb. RJC Slebos#. RH Hruban*. WC Doolev. GJA Offerhaust. HA Pil~, Depts. of Surgery and Pathology*, The Johns Hopkins Medical Institutions, Baltimore, Maryland and Dept. of Pathologyt, Academic Medical Center, Amsterdam, The Netherlands We have documented an increased incidence of patients with cholangiocarcinoma in West Virginia. A subsequent health survey also suggests that environmental exposure to chemicals and/or radionuclides may play a role in the pathogenesis of biliary malignancies and that cigarette smoking is not a risk factor. K-ras gene mutations have been associated with smoking, but neither ras oocogene nor p53 suppressor genes have been associated with environmental toxins. Therefore, we analyzed the incidence of p53 suppressor gene expression and codon 12 K-ras oncogene mutations in patients with perihilar cholangiocarcinoma. Paraffin embedded specimens from 27 patients, 12 from West Virginia and 15 from other states were studied. Immunohistochemical detection of intranuclear p53 gene product was performed with the D07 monoclonal antibody with target unmasking fluid for antigen retrieval. DNA sequences at codon 12 of the K-ras gene were amplified by polymerase chain reaction. The percentage of patients with p53 over-expression or ras gene mutations were: pS3 K-ras West Virginia 67 %* 17 % Other States 21% 27 % Total 46 % 22 % * p<0.05 vs Other States, chi-square These data suggest that patients from West Virginia with perihilar cholangiocarcinoma have 1) a high incidence of p53 suppressor gene expression and 2) an equal incidence ofras gene mutations. Further studies will be required to determine whether a direct link exists between p53 suppressor gene mutations and environmental exposure to chemicals and/or radionuclides. However, we conclude that p53 suppressor gene mutations may play a role in the increased incidence of cholangiocarcinoma in West Virginia.

Surgical porto-systemic shunt in children with variceal bleeding Pittsburgh Transplantation Institute, Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania

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Page 1: Surgical porto-systemic shunt in children with variceal bleeding Pittsburgh Transplantation Institute, Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania

HEPATOLOGY Vol. 22, No. 4, Pt. 2, 1995 AASLD ABSTRACTS 145A

153 SURGICAL PORTO-SYSTEMIC SHUNT IN CHILDREN WTI'H VARICEAL BLEEDING. N Jabbour, ] Reves. S Todo. C Ramirez, "rE Starzl Pittsburgh Transplantation Institute, Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania.

Varieeal bleeding is a major complication in children with portal hypertension, with or without cirrhosis. Sclerotherapy is considered the treatment of choice in these patients, however, porto-systemic shunting plays a major role in children who fail sclerotherapy; and in those with variceal disease extending into the stomach making sclerotherepy difficult and dangerous. We report our experience in 15 children who underwent porto-systemic shunting, between 1989 to 1995:1 H type porto-tarsi shunt, 1 side-to-side splenorenal shunt, and 13 distal splenorenal shunts. The mean age was 8.6 years (range 3-14 years). The primary diagnosis was biliary atresia (n=6), congenital hepatic fibrosis (n=2), portal vein thrombosis without cirrhosis (n =4) and portal vein thrombosis after liver transplantation (LTX) (n =3). All patients had multiple episodes of variceal bleeding that failed repeated sclerotherapy. Shunt procedure was performed semi-eleetively in all patients. Doppler ultrasound was used to document shunt patancy at 6 months intervals. The operation was performed suecessfaUy in all children, without any operative mortality or morbidity. During follow-up period of 4 years (3 months - 6 years) recurrent variceal bleeding was observed in 2 patients: 1 developed shunt thrombosis, that required LTX 4 mouths after shunt procedure, and the other is awaiting liver re-transplantation due to progressive liver disease 4 years post-shunt procedure. Eseephalopathy was observed in 1 patient in the early post-operative period that responded to conservative treatment. All patients are presently functional and with normal growth. Shunt patency was documented in all but 1 patient throughout the follow-up period. We conclude that surgical shunts play a major role in children with variceal bleeding and preserved liver fimction who fail sclerotherapy. This procedure is technically feasible even in small size patients, has a low incidence of encephalopathy, and a high shunt patensy rate.

154 SPIRAL COMPUTED ANGIOGRAPHY FOR THE EVALUATION OF VESSEL ENCASEMENT FOR PANCREATIC CANCER. J Cunninqharn. N. Glaichen*. S. Brewer. Departments of Surgery and Radiology*, Mount Sinai Medical Center, New York, NY

Introduction: Preoperative imaging of patients with pancreatic cancer is crucial in determining resectability and planning treatment. Purpose: To determine the role spiral computed angiography (SCA) plays in the evaluation of vessel involvement in patients with pancreatic cancer. Materials and Methods: SCA was obtained in six cases to evaluate the mesenteric vessels for encasement SCA images are obtained by a single 30 second breath hold for each of the mesenteric arterial and venous images. 3D images were generated with the helical data sets in any plane desired to evaluate vessel encasement. Results: In case #1, the SCA revealed complete obstruction of the portal vein (PV) at the superior mesenteric vein (SMV)-splenic vein (SV) confluence not seen on dynamic CT. In case #2 and #5, the vessels were normal. In case #3, the tumor was compressing the inferior vena cava but the mesenteric vessels were normal. In case #4, the CT was read as encasement of the PV at the SMV confluence but the SCA showed no evidence of this. In the last case, there was no arterial encasement and the venous phase was shot too late and images could not be obtained. The dynamic CT in this case showed PV encasement. The operative findings correlated with the SCA images in all five cases explored. In case #3, distant metastatic disease was found on laparoscopy and the patient was not explored. The finding of PV encasement seen on two CT scans was not found at the time of surgery. In no case was celiac or superior mesenteric artery involvement detected. The sensitivity (SENS), specificity (SPEC), positive predictive value (PPV), negative predictive value (NPV), and accuracy (ACC) were calculated for the four vessels evaluated:

CT SCA CONCLUSION: SCA allows for a non- SENS 50% 100% invasive evaluation of the mesenteric SPEC 86% 100% vessels. This test is quicker, less ACC 72% 1 0 0 % expensive and uses less of a dye toad PPV 33% 100% than angiography. The preliminary NPV 92% 100% results appear to be very reliable.

155 IMAGE ANALYSIS OF LESIONS OF THE GAI/3LADDERAND BILIARY SYSTEM, E.M.B~t, ~ . Saint LouisUniverslty and St. John's Mercy Medical Center, St. Louis, MO.

DMA ploidy analysis of lesions arising from the intra- and extrahepatie billary system and gallbladder was performed utilizingFeulgen-stalned 5#m sections from archival paraffln-embedded tissue. The Cell Analysis System Image Analyzer (CAS-200) was utilized for the study. Cases Included: cholangioearclnome (CCa), extra- hepatic bile duct cancer (BDCa), carcinoma of the gallbladder (GBCa), bile duct adenome (BDA), adenomyoma (Ad), and normal gallbladder (NPD) as controls. The number of cells analyzed ranged from 113-240, average 184. CV's ranged from 1.96 to 16.3, average 9.00.

DNA INDEX (Main Peak) DX (n) DIPLOID ANEUPL TETRAPL Aneup with

(0.9-1.10)(1.11-1.79) (1.80-2.20) extension into hypertetrapl region

CCa 6 3 2 l BDCa 5 3 1 1 GBCa ii 1 6 2 2 BDA 2 2 Ad 3 3 NPD 5 5 CONCLUSIONS: I) All benign lesions (NPD, Ad, A, BDA) showed

predominantly diploid populations. 2) Aneuploidy, tetraploidy, and hypertetraploldy were

seen in 10/11 GBCa, 3/6 CCa, 2/5 BDCa. 3) All 3 well-differentlated Ca's (2 CCa, I BDCa) had

>67Z diploid populations. Moderately or poorly differentiated Ca's with dlploid peaks (1 CCa, 2 BDCa) contained 40-50Z populations in aneuplold or tetraplold regions; 1 moderately differentiated GB Ca contained 80g of cells in the diploid region.

4) Image analysis is useful for studying DNA ploldy of lesions of billary origin, and may be applied when small t~or vol~,~es are present in tissue sections.

156 p53 AND K-RAS GENE MUTATIONS IN CHOLANGIOCARCINOMA. A Nakeeb. RJC Slebos#. RH Hruban*. WC Doolev. GJA Offerhaust. HA Pil~, Depts. of Surgery and Pathology*, The Johns Hopkins Medical Institutions, Baltimore, Maryland and Dept. of Pathologyt, Academic Medical Center, Amsterdam, The Netherlands

We have documented an increased incidence of patients with cholangiocarcinoma in West Virginia. A subsequent health survey also suggests that environmental exposure to chemicals and/or radionuclides may play a role in the pathogenesis of biliary malignancies and that cigarette smoking is not a risk factor. K-ras gene mutations have been associated with smoking, but neither ras oocogene nor p53 suppressor genes have been associated with environmental toxins. Therefore, we analyzed the incidence of p53 suppressor gene expression and codon 12 K-ras oncogene mutations in patients with perihilar cholangiocarcinoma. Paraffin embedded specimens from 27 patients, 12 from West Virginia and 15 from other states were studied. Immunohistochemical detection of intranuclear p53 gene product was performed with the D07 monoclonal antibody with target unmasking fluid for antigen retrieval. DNA sequences at codon 12 of the K-ras gene were amplified by polymerase chain reaction. The percentage of patients with p53 over-expression or ras gene mutations were:

pS3 K-ras

West Virginia 67 % * 17 %

Other States 21% 27 %

Total 46 % 22 % * p<0 .05 vs Other States, chi-square

These data suggest that patients from West Virginia with perihilar cholangiocarcinoma have 1) a high incidence of p53 suppressor gene expression and 2) an equal incidence ofras gene mutations. Further studies will be required to determine whether a direct link exists between p53 suppressor gene mutations and environmental exposure to chemicals and/or radionuclides. However, we conclude that p53 suppressor gene mutations may play a role in the increased incidence of cholangiocarcinoma in West Virginia.