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Mesenchymal hamartoma of the liver: Report of 30 cases and review of 30 cases and review of the literature
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technical factors. The other two died on postoperative day 1 and 7. One was a death ascribed to liver failure; the other was a complication of cardiac tamponade secondary to erosion of a central venous line through the superior vena cava. The technical details of the operation are described. The immunosuppression protocol is also discussed and centers upon the use of Cyclosporine. The Minnesota group utilizes percutaneous transplant biopsies on a weekly basis or more frequently if indicated. This allows for early determination of acute rejection. In the immediate post-transplant period, liberal use of exchange transfusion is particularly helpful for control of abnormal clotting parameters. Exchange transfusion is also used prior to transplantation if there is significant alteration in coagulation secondary to hepatic decompensation. The authors conclude that if the child survives 24 hours after transplantation, the chance of subsequent survival are excellent.-- Thomas V. Whalen, Jr. Liver Transplantation for Biliary Atresia. S. lwatsuki, B. Shaw, and T. Starzl. World J Surg 8:51-56 (February), 1984.
The experience over a nearly 20-year period with liver transplantation for biliary atresia is reviewed. Three-hundred liver transplants have been performed, first at the University of Colorado and since 1981 at the University of Pittsburgh. One-hundred and seventy of these patients were treated before March 1980 and received conventional immunosuppression with Azathioprine, Prednisone, and usually antilymphocyte globulin. Since March 1980, Cyclosporine with Prednisone has been used. The steroid dose with Cyclosporine is considerably lower than that which is used with the conventional regime. Among 170 patients given the conventional therapy, 86 were pediatric recipients aged 18 or under. Of 100 patients with Cyclosporine therapy since March 1980, 40 have been pediatric patients. These 126 pediatric patients with at least an 18-month follow-up comprise the body of the report. Of the 126 patients, 71 had biliary atresia and/or hypoplasia as their basic disease. The next most common indication was inborn metabolic errors (23/126). Finally, chronic aggressive hepatitis was an indication in 16 patients. The authors describe the technique that they utilize for orthotopic liver transplantation and refer to several previous publications for more detailed analysis and technical steps. Of the 86 patients treated with conventional immunosuppression, 53 died within a year of transplantation. The causes of death were frequently multiple, but a single predominant factor was infection (20/86). The second most common reason for death was a surgical technical complication (15/ 86). In the Cyclosporine era, 14 of 40 patients died within one year of transplantation. Seven of these were primarily due to a surgical complication with no patient succumbing predominantly to infection. Graft rejection occurred in 9 of the 86 patients pre-Cyclosporine and in 4 of 40 patients in the Cyclosporine era. The one year survival after transplantation improved from 40% to 65% with the addition of Cyclosporine as the principle immunosuppressant. The authors have found that survival of children with biliary atresia is substantially lower than the overall survival and, in particular, lower than the survival for children transplanted for an inborn metabolic error. There was no substantial impact of age on survival or perioperative complications. The authors feel that the technical difficulties of transplantation in the small sized infant
ABSTRACTS
has, at least in their hands, been overcome and that age should not be a consideration in accepting a candidate for transplantation. They do note, however, that obtaining a small donor liver can have an impact on this p r o b l e m . Thomas V. Whalen, Jr. Mesenchymal Hamartoma Of The Liver: Report of 30 Cases and Review of 30 Cases and Review of the Literature. J. T.
Stocker, and K. G. Ishak. Pediatr Pathol 1:245-267 (JulySeptember), 1983. Thirty cases of mesenchymal hamartoma of the liver are presented. The average age is 15 months and 69% are male. Most present as progressive abdominal enlargement. Physical examination and radiologic findings are nonspecific and consistent only with a liver mass. Operative management is usually resection although internal drainage has been reported. The mass arises either from disordered mesenchymal growth or degeneration of mesenchyme. This is not a malignancy, and mortality is associated with operative complications. Recurrence and malignant transformation has not been reported.--Charles Stolar The Collateral Arterial Network of the Spleen Following Ligation of the Splenic Artery in Traumatic Rupture of the Spleen: an Arteriographic Study. D. C. Keramidas, D.
Kelekis, Th. Dolatzas, et al. Z Kinderchir 39:50-51 (February), 1984. Organ-preserving procedures are favored in splenic trauma since the immunologic competence of this organ has been fully recognized. The authors ligated the splenic artery combined with splenorhaphy successfully in eight children following splenic trauma. Arteriographies were done on the 20th day, the 1st, 3rd, and 4th month following surgery. A collateral arterial network was detected originating mainly from the short gastric arteries with retrograde filling of the ligated splenic artery. It is concluded that collateral splenic blood supply develops rapidly and its pattern is rather uniform.--Thomas A. Angerpointner The Surgical Treatment of Hyperinsulinism in Infancy and
Childhood. M. H. Gough. Br J Surg 71:75 78 (January), 1984. Hyperinsulinemic hypoglycemia in infancy may be transient or persistent. The latter is a rarer and more dangerous condition which has been previously underdiagnosed. It is caused by pancreatic nesidioblastosis or adenorna. Criteria for diagnosis include (1) inappropriately elevated plasma insulin levels, (2) an IV glucose infusion rate of more than 15 m g / k g / m i n to maintain a blood glucose level over 2 mmol/L, (3) low blood ketones, and (4) a glycemic response to glucagon. It is not possible to differentiate preoperatively between nesidioblastosis and adenoma in most cases. Those patients with adenoma and most with nesidioblastosis will require pancreatectomy but the extent of the first resection remains controversial. The author describes 7 patients: 5 neonates with nesidioblastosis, 1 neonate, and 1 9 year-old girl with pancreatic adenoma. Each neonate with nesidioblastosis underwent 75% pancreatectomy (to the superior mesenteric vessels), which failed to control the hyperinsulinism in
technical factors. The other two died on postoperative day 1 and 7. One was a death ascribed to liver failure; the other was a complication of cardiac tamponade secondary to erosion of a central venous line through the superior vena cava. The technical details of the operation are described. The immunosuppression protocol is also discussed and centers upon the use of Cyclosporine. The Minnesota group utilizes percutaneous transplant biopsies on a weekly basis or more frequently if indicated. This allows for early determination of acute rejection. In the immediate post-transplant period, liberal use of exchange transfusion is particularly helpful for control of abnormal clotting parameters. Exchange transfusion is also used prior to transplantation if there is significant alteration in coagulation secondary to hepatic decompensation. The authors conclude that if the child survives 24 hours after transplantation, the chance of subsequent survival are excellent.-- Thomas V. Whalen, Jr. Liver Transplantation for Biliary Atresia. S. lwatsuki, B. Shaw, and T. Starzl. World J Surg 8:51-56 (February), 1984.
The experience over a nearly 20-year period with liver transplantation for biliary atresia is reviewed. Three-hundred liver transplants have been performed, first at the University of Colorado and since 1981 at the University of Pittsburgh. One-hundred and seventy of these patients were treated before March 1980 and received conventional immunosuppression with Azathioprine, Prednisone, and usually antilymphocyte globulin. Since March 1980, Cyclosporine with Prednisone has been used. The steroid dose with Cyclosporine is considerably lower than that which is used with the conventional regime. Among 170 patients given the conventional therapy, 86 were pediatric recipients aged 18 or under. Of 100 patients with Cyclosporine therapy since March 1980, 40 have been pediatric patients. These 126 pediatric patients with at least an 18-month follow-up comprise the body of the report. Of the 126 patients, 71 had biliary atresia and/or hypoplasia as their basic disease. The next most common indication was inborn metabolic errors (23/126). Finally, chronic aggressive hepatitis was an indication in 16 patients. The authors describe the technique that they utilize for orthotopic liver transplantation and refer to several previous publications for more detailed analysis and technical steps. Of the 86 patients treated with conventional immunosuppression, 53 died within a year of transplantation. The causes of death were frequently multiple, but a single predominant factor was infection (20/86). The second most common reason for death was a surgical technical complication (15/ 86). In the Cyclosporine era, 14 of 40 patients died within one year of transplantation. Seven of these were primarily due to a surgical complication with no patient succumbing predominantly to infection. Graft rejection occurred in 9 of the 86 patients pre-Cyclosporine and in 4 of 40 patients in the Cyclosporine era. The one year survival after transplantation improved from 40% to 65% with the addition of Cyclosporine as the principle immunosuppressant. The authors have found that survival of children with biliary atresia is substantially lower than the overall survival and, in particular, lower than the survival for children transplanted for an inborn metabolic error. There was no substantial impact of age on survival or perioperative complications. The authors feel that the technical difficulties of transplantation in the small sized infant
ABSTRACTS
has, at least in their hands, been overcome and that age should not be a consideration in accepting a candidate for transplantation. They do note, however, that obtaining a small donor liver can have an impact on this p r o b l e m . Thomas V. Whalen, Jr. Mesenchymal Hamartoma Of The Liver: Report of 30 Cases and Review of 30 Cases and Review of the Literature. J. T.
Stocker, and K. G. Ishak. Pediatr Pathol 1:245-267 (JulySeptember), 1983. Thirty cases of mesenchymal hamartoma of the liver are presented. The average age is 15 months and 69% are male. Most present as progressive abdominal enlargement. Physical examination and radiologic findings are nonspecific and consistent only with a liver mass. Operative management is usually resection although internal drainage has been reported. The mass arises either from disordered mesenchymal growth or degeneration of mesenchyme. This is not a malignancy, and mortality is associated with operative complications. Recurrence and malignant transformation has not been reported.--Charles Stolar The Collateral Arterial Network of the Spleen Following Ligation of the Splenic Artery in Traumatic Rupture of the Spleen: an Arteriographic Study. D. C. Keramidas, D.
Kelekis, Th. Dolatzas, et al. Z Kinderchir 39:50-51 (February), 1984. Organ-preserving procedures are favored in splenic trauma since the immunologic competence of this organ has been fully recognized. The authors ligated the splenic artery combined with splenorhaphy successfully in eight children following splenic trauma. Arteriographies were done on the 20th day, the 1st, 3rd, and 4th month following surgery. A collateral arterial network was detected originating mainly from the short gastric arteries with retrograde filling of the ligated splenic artery. It is concluded that collateral splenic blood supply develops rapidly and its pattern is rather uniform.--Thomas A. Angerpointner The Surgical Treatment of Hyperinsulinism in Infancy and
Childhood. M. H. Gough. Br J Surg 71:75 78 (January), 1984. Hyperinsulinemic hypoglycemia in infancy may be transient or persistent. The latter is a rarer and more dangerous condition which has been previously underdiagnosed. It is caused by pancreatic nesidioblastosis or adenorna. Criteria for diagnosis include (1) inappropriately elevated plasma insulin levels, (2) an IV glucose infusion rate of more than 15 m g / k g / m i n to maintain a blood glucose level over 2 mmol/L, (3) low blood ketones, and (4) a glycemic response to glucagon. It is not possible to differentiate preoperatively between nesidioblastosis and adenoma in most cases. Those patients with adenoma and most with nesidioblastosis will require pancreatectomy but the extent of the first resection remains controversial. The author describes 7 patients: 5 neonates with nesidioblastosis, 1 neonate, and 1 9 year-old girl with pancreatic adenoma. Each neonate with nesidioblastosis underwent 75% pancreatectomy (to the superior mesenteric vessels), which failed to control the hyperinsulinism in