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Biliary atresia—a 25-year survey. et al
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chronic form found in children 6 months to 6 years of age with constipation and megacolon. The infant reported became acutely ill, and was managed without surgery using normal saline colonic irrigations. Sequential biopsies demonstrated early hyperganglionosis. However, by 9 months of age the number of ganglion cells per high power field returned to normal. Although the current literature seems to point to a need for surgical intervention in NID, this case suggests a more conservative therapeutic approach may be effective, in which colonic irrigations, parenteral nutrition, and antibiotics for secondary inflammatory changes may allow for successful resolution of a temporary condition.-Thomas F. Truq Jr
ABDOMEN Biliary Atresia-A 25-Year Survey. et al. R. Engelskirchen, A.M. Holschneider, M. Gharib. Eur J Pediatr Surg 1:154-160, (June), 1991. Between 1963 and 1988, 90 children were treated for biliary atresia or hypoplasia. There were 45 boys and 45 girls. Forty-seven of these children had purely extrahepatic lesions (atresia 42, hypoplasia 5), 21 had purely intrahepatic bile duct lesions, and 22 had both extrahepatic and intrahepatic lesions. Eleven of the children did not undergo surgical treatment. Seventy-nine of the 90 patients underwent laparotomy. Of these, diagnostic laparotomy only without operative bile drainage was carried out in 34 cases. Forty-five children underwent a drainage procedure. Until 1966, hepatojejunostomy with implantation of artificial bile ducts was conducted in I2 cases. Later, cholecystoduodenostomy was performed 4 times and Kasai-Kimura hepatoportojejunostomy in 29 cases. Twentyseven patients survived (30%). If the children with intrahepatic bile duct hypoplasia are considered only, the survival rate was 80% (12115). Eight children of the 29 with hepatoportojejunostomy (27.5%) are still alive today. However, permanent bile drainage could be achieved in only 5 of these 8 children. Only one child can be regarded completely healthy as far as his liver is concerned. These results demonstrate that, even though a 5-year survival rate of 25% to 30% can be achieved by means of hepatoportojejunostomy, complete recovery from the underlying disease is rarely possible because even in long-term survivors, health complications such as progressive liver transformation, portal hypertension, bleeding from esophageal varices, and other phenomena may occur. Liver transplantation is the method of choice for saving these children.-Thomas A. Angerpointner The Use of Reduced-Size Liver Transplants in Children, Including Split Livers and Living Related Liver Transplants. C.E. Brtilsch, L.H. Stevens, and P.F. willington. Eur J Pediatr Surg 1:166-171, (June), 1991. The shortage of suitable donor organs is a crucial factor limiting the application of liver transplantation. Reduced-size liver transplantation (RLT). split liver transplantation (SLT), and livingrelated liver transplantation (LRLT) represent surgical efforts to deal with the donor supply. RLT allows a recipient to receive a graft from a donor several times their own size, thus increasing the proportion of the donor pool available to the small recipient. SLT and LRLT provide more organs. SLT distributes one cadaveric liver among two recipients, whereas LRLT provides a previously unused source of organs. This report examines the results achieved with RLT. SLT, and LRLT at the University of Chicago and compares these results with those achieved by standard, full-size orthotopic liver transplantation (OLT). Between November 1984 and February 1991, 457 liver transplantations were performed at the University of Chicago. Two hundred and fourteen of these transplants were placed into recipients less than 13 years of age. One hundred three were full-size livers and 111 were reduced grafts (RLT, SLT, LRLT). Mean age of the recipients was 1.9 ? 1.8
ABSTRACTS
years in reduced-size grafts and 3.5 ? 3.4 years in full-size grafts. The most common indication for liver transplantation was biliary atresia (105 patients). Overall patient survival after full-size transplantation was 71.8%; with reduced size transplantation it was 72.6%. Patient survivals for primary transplants with specific types of reduced-size grafts were: RLT 76.5%, SLT 66.7%, and LRLT 89.5%. Particularly, LRLT provided excellent results with no donor mortality and acceptable morbidity. It is concluded that reducedsized livers should be used for transplantation in the pediatric age group.-Thomas A. Angerpointner Biliary Infusion Therapy in the lnspissated Bile Syndrome of Cystic Fibrosis. J.S. Evans, D.E. George and D. Mollit. J Pediatr Gastroenterol Nutr 12:131-135, (January), 1991. This is a case report of a 3.5-month-old white boy born with meconium ileus, peritonitis, and jejunal atresia from cystic fibrosis. Persistent jaundice in the newborn period prompted phenobarbital therapy. “Tc-IDA radionuclide scan failed to demonstrate biliary excretion. His stools became acholic at 3 months of age, and the total bilirubin increased to 9.7. A percutaneous liver biopsy showed bile duct proliferation and early fibrosis. Intraoperative cholangiography demonstrated normal gallbladder and biliary tree. A cholecystostomy tube was left in placed, through which a 2% solution of N-acetylcysteine was irrigated into the biliary tract. Biliary lavage was carried out for a total of 14 days. Synthetic cholecystokinin was also administered early in the course of biliary lavage. Cholecystography demonstrated a patent duct on the passage of green-color stools. Biliary lavage for inspissated bile syndrome may prove beneficial in children with cystic fibrosis.-Thomas F. Trac,v, Jr Complete Spontaneous Disruption of the Common Bile Duct: A Late Complication of Necrotizing Enterocolitis? S.E. Do&. R.L. Levine, K.I. Norton, et al. J Pediatr Gastroenterol Nutr 12:379382, (April), 1991. This report describes a 2.3-kg baby who presented at 8 days of age with necrotizing enterocolitis. The baby initially responded to medical management, and on the third day of conservative management a small amount of free interperitoneal air was noted on plain abdominal films. However, clinically, the child had improved to the extent that exploration was not undertaken. At 1 month after treatment, he was admitted to the hospital for partial bowel obstruction, and a transverse colon stricture was resected. At 20 months of age he was readmitted to the hospital with fever and nonbilious vomiting. Clinical examination showed peritonitis, which prompted laparotomy. A complete rupture of the distal common bile duct was found. In the absence of a history of trauma or any other significant findings of congenital biliary obstruction, the authors contend that this was an unusual case of spontaneous disruption secondary to a severe neonatal necrotizing enterocolitis that most probably involved the duodenum.-Thomas F. Tracy, Jr Gastric Perforation as a Complication of Splenectomy: Report of Five Cases and Review of the Literature. J.H. McClenuthan. Can J Surg 34:175-178, (April), 1991. The authors report five cases (aged 18 to 70 years) with gastric perforation as a complication of spienectomy and review the literature, finding 10 more cases (aged 9 to 67 years). Splenectomy was done for trauma (6) hematology (6), and malignancy (3). None of the patients was suspected of having had a gastric injury. However, because they had been drained, the most common presentation was an increased volume of drainage during the first postoperative week. Other patients presented later with left subphrenic abscess. The injury took 2 days to 3 months to diagnose and was confirmed by radiological studies. The perforation typically occurred in the gastric fundus near the site where the short gastric
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chronic form found in children 6 months to 6 years of age with constipation and megacolon. The infant reported became acutely ill, and was managed without surgery using normal saline colonic irrigations. Sequential biopsies demonstrated early hyperganglionosis. However, by 9 months of age the number of ganglion cells per high power field returned to normal. Although the current literature seems to point to a need for surgical intervention in NID, this case suggests a more conservative therapeutic approach may be effective, in which colonic irrigations, parenteral nutrition, and antibiotics for secondary inflammatory changes may allow for successful resolution of a temporary condition.-Thomas F. Truq Jr
ABDOMEN Biliary Atresia-A 25-Year Survey. et al. R. Engelskirchen, A.M. Holschneider, M. Gharib. Eur J Pediatr Surg 1:154-160, (June), 1991. Between 1963 and 1988, 90 children were treated for biliary atresia or hypoplasia. There were 45 boys and 45 girls. Forty-seven of these children had purely extrahepatic lesions (atresia 42, hypoplasia 5), 21 had purely intrahepatic bile duct lesions, and 22 had both extrahepatic and intrahepatic lesions. Eleven of the children did not undergo surgical treatment. Seventy-nine of the 90 patients underwent laparotomy. Of these, diagnostic laparotomy only without operative bile drainage was carried out in 34 cases. Forty-five children underwent a drainage procedure. Until 1966, hepatojejunostomy with implantation of artificial bile ducts was conducted in I2 cases. Later, cholecystoduodenostomy was performed 4 times and Kasai-Kimura hepatoportojejunostomy in 29 cases. Twentyseven patients survived (30%). If the children with intrahepatic bile duct hypoplasia are considered only, the survival rate was 80% (12115). Eight children of the 29 with hepatoportojejunostomy (27.5%) are still alive today. However, permanent bile drainage could be achieved in only 5 of these 8 children. Only one child can be regarded completely healthy as far as his liver is concerned. These results demonstrate that, even though a 5-year survival rate of 25% to 30% can be achieved by means of hepatoportojejunostomy, complete recovery from the underlying disease is rarely possible because even in long-term survivors, health complications such as progressive liver transformation, portal hypertension, bleeding from esophageal varices, and other phenomena may occur. Liver transplantation is the method of choice for saving these children.-Thomas A. Angerpointner The Use of Reduced-Size Liver Transplants in Children, Including Split Livers and Living Related Liver Transplants. C.E. Brtilsch, L.H. Stevens, and P.F. willington. Eur J Pediatr Surg 1:166-171, (June), 1991. The shortage of suitable donor organs is a crucial factor limiting the application of liver transplantation. Reduced-size liver transplantation (RLT). split liver transplantation (SLT), and livingrelated liver transplantation (LRLT) represent surgical efforts to deal with the donor supply. RLT allows a recipient to receive a graft from a donor several times their own size, thus increasing the proportion of the donor pool available to the small recipient. SLT and LRLT provide more organs. SLT distributes one cadaveric liver among two recipients, whereas LRLT provides a previously unused source of organs. This report examines the results achieved with RLT. SLT, and LRLT at the University of Chicago and compares these results with those achieved by standard, full-size orthotopic liver transplantation (OLT). Between November 1984 and February 1991, 457 liver transplantations were performed at the University of Chicago. Two hundred and fourteen of these transplants were placed into recipients less than 13 years of age. One hundred three were full-size livers and 111 were reduced grafts (RLT, SLT, LRLT). Mean age of the recipients was 1.9 ? 1.8
ABSTRACTS
years in reduced-size grafts and 3.5 ? 3.4 years in full-size grafts. The most common indication for liver transplantation was biliary atresia (105 patients). Overall patient survival after full-size transplantation was 71.8%; with reduced size transplantation it was 72.6%. Patient survivals for primary transplants with specific types of reduced-size grafts were: RLT 76.5%, SLT 66.7%, and LRLT 89.5%. Particularly, LRLT provided excellent results with no donor mortality and acceptable morbidity. It is concluded that reducedsized livers should be used for transplantation in the pediatric age group.-Thomas A. Angerpointner Biliary Infusion Therapy in the lnspissated Bile Syndrome of Cystic Fibrosis. J.S. Evans, D.E. George and D. Mollit. J Pediatr Gastroenterol Nutr 12:131-135, (January), 1991. This is a case report of a 3.5-month-old white boy born with meconium ileus, peritonitis, and jejunal atresia from cystic fibrosis. Persistent jaundice in the newborn period prompted phenobarbital therapy. “Tc-IDA radionuclide scan failed to demonstrate biliary excretion. His stools became acholic at 3 months of age, and the total bilirubin increased to 9.7. A percutaneous liver biopsy showed bile duct proliferation and early fibrosis. Intraoperative cholangiography demonstrated normal gallbladder and biliary tree. A cholecystostomy tube was left in placed, through which a 2% solution of N-acetylcysteine was irrigated into the biliary tract. Biliary lavage was carried out for a total of 14 days. Synthetic cholecystokinin was also administered early in the course of biliary lavage. Cholecystography demonstrated a patent duct on the passage of green-color stools. Biliary lavage for inspissated bile syndrome may prove beneficial in children with cystic fibrosis.-Thomas F. Trac,v, Jr Complete Spontaneous Disruption of the Common Bile Duct: A Late Complication of Necrotizing Enterocolitis? S.E. Do&. R.L. Levine, K.I. Norton, et al. J Pediatr Gastroenterol Nutr 12:379382, (April), 1991. This report describes a 2.3-kg baby who presented at 8 days of age with necrotizing enterocolitis. The baby initially responded to medical management, and on the third day of conservative management a small amount of free interperitoneal air was noted on plain abdominal films. However, clinically, the child had improved to the extent that exploration was not undertaken. At 1 month after treatment, he was admitted to the hospital for partial bowel obstruction, and a transverse colon stricture was resected. At 20 months of age he was readmitted to the hospital with fever and nonbilious vomiting. Clinical examination showed peritonitis, which prompted laparotomy. A complete rupture of the distal common bile duct was found. In the absence of a history of trauma or any other significant findings of congenital biliary obstruction, the authors contend that this was an unusual case of spontaneous disruption secondary to a severe neonatal necrotizing enterocolitis that most probably involved the duodenum.-Thomas F. Tracy, Jr Gastric Perforation as a Complication of Splenectomy: Report of Five Cases and Review of the Literature. J.H. McClenuthan. Can J Surg 34:175-178, (April), 1991. The authors report five cases (aged 18 to 70 years) with gastric perforation as a complication of spienectomy and review the literature, finding 10 more cases (aged 9 to 67 years). Splenectomy was done for trauma (6) hematology (6), and malignancy (3). None of the patients was suspected of having had a gastric injury. However, because they had been drained, the most common presentation was an increased volume of drainage during the first postoperative week. Other patients presented later with left subphrenic abscess. The injury took 2 days to 3 months to diagnose and was confirmed by radiological studies. The perforation typically occurred in the gastric fundus near the site where the short gastric