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Orthotopic liver transplantation for graft-versus-host disease following bone marrow transplantation
INTERNATIONAL
871
ABSTRACTS
tion to technically demanding ERCP and sphincterotomy. Both of these may be alternatives to laparotomy and common duct exploration.--Thomas F. Tracy, Jr Congenital
Biliary Tract Disease. FM
Ferrer, R.J. Hall, B.A.
Stewart, et al. Surg Clin North Am 70:1403-1418, (December),
1990.
This is an overview of the most common types of congenital biliary tract anomalies. It is a good review of the current status of biliary atresia. The authors recommend frozen section examination of the portal mass prior to performing the portoenterostomy. They currently temporarily exteriorize the bilioenteric conduit, primarily in order to monitor postoperative bile flow, and construct an intussusception valve into the distal Roux-en-Y limb. Eighty percent to 90% of infants with biliary atresia who are operated on when less than 10 weeks of age drain bile. Portal hypertension, as manifested by variceal hemorrhage, ascites, or hypersplenism, occurs in 20% to 25% of patients. Sclerotherapy is used to control variceal bleeding. In this series, long-term survival was obtained in 46% of patients operated on when younger than 66 days of age and in 24% of patients when operated on after that time. Twenty-eight percent of the patients in the series are long-term survivors without the need for liver transplantation. Choledochal cyst seems to be a lesion resulting from development faults of the PancreaticobiliaIy system. A common channel etiology has been advanced in the past to account for the etiology of choledochal cysts. Recently, a bilialy autonomic dysfunction (decreased ganglion cells) has been incriminated. Cyst enterostomy leads to a 50% reoperative rate and is no longer recommended. In addition, biliary carcinomas develop in 2.5% to 4.7% of patients with choledocal cysts and half of these develop in patients who have had internal drainage procedures. Cyst excision with biliary enteric anastomosis is now the operative procedure of choice and currently has a less than 10% morbidity rate secondary to cholangitis, anastomotic stricture, or pancreatic duct injury. Idiopathic perforation of the common bile duct is probably a congenital defect because of the characteristic location of the perforation at the junction of the cystic and common bile ducts. It is manifested by the insidious onset of ascites, low-grade jaundice, and clay-colored stools. In most cases, simple peritoneal drainage of the perforation site and biliary tract decompression are the procedures of choice.-Richard R. Rickeffs Long-Term
Outcome After Surgery for Biliary Atresia. J. Lau-
rent, F. Gauthier, 0. Bernard, et al. Gastroenterology
Heterotopic Liver Transplantation for Fulminant Wilson’s Disease. D.A. Stamp)?, S.J. Munoz, Ml. Moritz, et al. Gastroenterology
99:1834-1836, (December), 1990. Wilson’s disease may present as fulminant hepatocellular failure with intravascular hemolysis, renal failure, and life-threatening cerebral edema. Orthotopic liver transplantation has been shown to correct the basic metabolic defect in Wilson’s disease. It is rapidly becoming an important modality for the therapy of irreversible fulminant hepatocellular failure. This report describes a 15year-old girl with Wilson’s disease whose mental status deteriorated to stage IV coma within 48 hours of admission to the hospital. An attempt was made to perform an orthotopic liver transplantation with an unmatched donor because of the patient’s critical status. However, manipulation of the patient’s liver resulted in significant elevation of the intracranial pressure leading to the risk of imminent uncal herniation. Therefore, it was decided to perform a heterotopic liver transplantation using the right lobe of the donor liver. The patient recovered full neurological function within 10 days postoperatively. She subsequently had an orthotopic liver transplant on the 27th postoperative day because of antibodymediated graft injury secondary to ABO incompatibility in the first graft. Liver function tests returned to near-normal levels within 5 weeks of the second transplant.-Richard R. Ricketts Orthotopic Liver Transplantation for Graft-Versus-Host Disease Following Bone Marrow Transplantation. D.F. Rhodes, WM. Lee, J.R. Wingard, et al. Gastroenterology 99:536-538, (Au-
gust), 1990. Chronic graft-versus-host disease (GVHD) is seen in 30% to 50% of long-term survivors of allogeneic bone marrow transplants, 90% of whom demonstrate hepatic involvement. The liver disease occasionally progresses to cirrhosis. In this report, a 19-year-old woman underwent a bone marrow transplant for acute myelogenous leukemia. One year later a liver bipsy demonstrated changes consistent with ongoing GVHD. Two and a half years later she developed esophageal variceal bleeding from hepatic cirrhosis, which was not controllable by many sessions of sclerotherapy. Therefore, she underwent an orthotopic liver transplant with success. Two years after the liver transplantation she is well with normal liver function and without biochemical evidence of rejection or recurrence of GVHD. As bone marrow transplantation becomes more widely available, the incidence of GVHD-induced end-stage liver disease will likely increase. This appears to be another indication for liver transplantation.-Richard R. Ricketts
99:1793-1797,
(December), 1990. The authors reviewed the status of 40 of 122 children (32.7%) who were alive 10 years following a Kasai-type procedure. Of the 82 children who died before 10 years, 72 died as a direct complication of the liver disease and 10 died of refractory bacterial cholangitis. Of the survivors, most were within 21.5 standard deviations with respect to weight and height. All but three attended school in normal grades for their age. Features of cirrhosis were present in all but two long-term survivors. Of the 40 long-term survivors, 21 have normal total serum bilirubin levels, 11 have normal liver function tests, 11 do not have signs of portal hypertension, and 2 have no cirrhosis. Overall, normal liver function tests and absence of portal hypertension were observed in 11 of 122 children (9%). These long-term results indicate that the Kasai procedures are helpful in a significant portion of children with biliary atresia but that improvement is not equivalent to cure. Approximately 80% of children who initially undergo surgery with a Kasai procedure will eventually require liver transplantation.-Richard R. Rickefts
Benign Pneumatosis Intestinalis With Subcutaneous Emphysema in a Liver Transplant Recipient. R.E. Suchse, G. W. Burke, M Jonas, et al. Am J Gastroenterol85:876-879, (July), 1990.
Pneumatosis intestinalis can be associated with intraabdominal catastrophes, particularly in neonates, but it may also appear in an innocuous form in kidney transplant recipients. A 5-year-old girl underwent an orthotopic liver transplant following a failed Kasai procedure for biliary atresia. She developed chronic rejection and vanishing bile duct syndrome. Fourteen months after the liver transplant she presented with complaints of nausea, vomiting, and weakness. She was found to have diffuse pneumatosis intestinalis but no free air. She developed subcutaneous air in the perineum and along the lateral abdominal wall bilaterally. She was treated with antibiotics, nasogastric suction, and her usual immunosuppressive drugs. She improved within 3 days. After 1 week the antibiotics were discontinued, and the patient was started on an elemental diet. The pneumatosis resolved radiographically within 2 weeks. She had no further episodes of pneumatosis intestinalis up to 8
871
ABSTRACTS
tion to technically demanding ERCP and sphincterotomy. Both of these may be alternatives to laparotomy and common duct exploration.--Thomas F. Tracy, Jr Congenital
Biliary Tract Disease. FM
Ferrer, R.J. Hall, B.A.
Stewart, et al. Surg Clin North Am 70:1403-1418, (December),
1990.
This is an overview of the most common types of congenital biliary tract anomalies. It is a good review of the current status of biliary atresia. The authors recommend frozen section examination of the portal mass prior to performing the portoenterostomy. They currently temporarily exteriorize the bilioenteric conduit, primarily in order to monitor postoperative bile flow, and construct an intussusception valve into the distal Roux-en-Y limb. Eighty percent to 90% of infants with biliary atresia who are operated on when less than 10 weeks of age drain bile. Portal hypertension, as manifested by variceal hemorrhage, ascites, or hypersplenism, occurs in 20% to 25% of patients. Sclerotherapy is used to control variceal bleeding. In this series, long-term survival was obtained in 46% of patients operated on when younger than 66 days of age and in 24% of patients when operated on after that time. Twenty-eight percent of the patients in the series are long-term survivors without the need for liver transplantation. Choledochal cyst seems to be a lesion resulting from development faults of the PancreaticobiliaIy system. A common channel etiology has been advanced in the past to account for the etiology of choledochal cysts. Recently, a bilialy autonomic dysfunction (decreased ganglion cells) has been incriminated. Cyst enterostomy leads to a 50% reoperative rate and is no longer recommended. In addition, biliary carcinomas develop in 2.5% to 4.7% of patients with choledocal cysts and half of these develop in patients who have had internal drainage procedures. Cyst excision with biliary enteric anastomosis is now the operative procedure of choice and currently has a less than 10% morbidity rate secondary to cholangitis, anastomotic stricture, or pancreatic duct injury. Idiopathic perforation of the common bile duct is probably a congenital defect because of the characteristic location of the perforation at the junction of the cystic and common bile ducts. It is manifested by the insidious onset of ascites, low-grade jaundice, and clay-colored stools. In most cases, simple peritoneal drainage of the perforation site and biliary tract decompression are the procedures of choice.-Richard R. Rickeffs Long-Term
Outcome After Surgery for Biliary Atresia. J. Lau-
rent, F. Gauthier, 0. Bernard, et al. Gastroenterology
Heterotopic Liver Transplantation for Fulminant Wilson’s Disease. D.A. Stamp)?, S.J. Munoz, Ml. Moritz, et al. Gastroenterology
99:1834-1836, (December), 1990. Wilson’s disease may present as fulminant hepatocellular failure with intravascular hemolysis, renal failure, and life-threatening cerebral edema. Orthotopic liver transplantation has been shown to correct the basic metabolic defect in Wilson’s disease. It is rapidly becoming an important modality for the therapy of irreversible fulminant hepatocellular failure. This report describes a 15year-old girl with Wilson’s disease whose mental status deteriorated to stage IV coma within 48 hours of admission to the hospital. An attempt was made to perform an orthotopic liver transplantation with an unmatched donor because of the patient’s critical status. However, manipulation of the patient’s liver resulted in significant elevation of the intracranial pressure leading to the risk of imminent uncal herniation. Therefore, it was decided to perform a heterotopic liver transplantation using the right lobe of the donor liver. The patient recovered full neurological function within 10 days postoperatively. She subsequently had an orthotopic liver transplant on the 27th postoperative day because of antibodymediated graft injury secondary to ABO incompatibility in the first graft. Liver function tests returned to near-normal levels within 5 weeks of the second transplant.-Richard R. Ricketts Orthotopic Liver Transplantation for Graft-Versus-Host Disease Following Bone Marrow Transplantation. D.F. Rhodes, WM. Lee, J.R. Wingard, et al. Gastroenterology 99:536-538, (Au-
gust), 1990. Chronic graft-versus-host disease (GVHD) is seen in 30% to 50% of long-term survivors of allogeneic bone marrow transplants, 90% of whom demonstrate hepatic involvement. The liver disease occasionally progresses to cirrhosis. In this report, a 19-year-old woman underwent a bone marrow transplant for acute myelogenous leukemia. One year later a liver bipsy demonstrated changes consistent with ongoing GVHD. Two and a half years later she developed esophageal variceal bleeding from hepatic cirrhosis, which was not controllable by many sessions of sclerotherapy. Therefore, she underwent an orthotopic liver transplant with success. Two years after the liver transplantation she is well with normal liver function and without biochemical evidence of rejection or recurrence of GVHD. As bone marrow transplantation becomes more widely available, the incidence of GVHD-induced end-stage liver disease will likely increase. This appears to be another indication for liver transplantation.-Richard R. Ricketts
99:1793-1797,
(December), 1990. The authors reviewed the status of 40 of 122 children (32.7%) who were alive 10 years following a Kasai-type procedure. Of the 82 children who died before 10 years, 72 died as a direct complication of the liver disease and 10 died of refractory bacterial cholangitis. Of the survivors, most were within 21.5 standard deviations with respect to weight and height. All but three attended school in normal grades for their age. Features of cirrhosis were present in all but two long-term survivors. Of the 40 long-term survivors, 21 have normal total serum bilirubin levels, 11 have normal liver function tests, 11 do not have signs of portal hypertension, and 2 have no cirrhosis. Overall, normal liver function tests and absence of portal hypertension were observed in 11 of 122 children (9%). These long-term results indicate that the Kasai procedures are helpful in a significant portion of children with biliary atresia but that improvement is not equivalent to cure. Approximately 80% of children who initially undergo surgery with a Kasai procedure will eventually require liver transplantation.-Richard R. Rickefts
Benign Pneumatosis Intestinalis With Subcutaneous Emphysema in a Liver Transplant Recipient. R.E. Suchse, G. W. Burke, M Jonas, et al. Am J Gastroenterol85:876-879, (July), 1990.
Pneumatosis intestinalis can be associated with intraabdominal catastrophes, particularly in neonates, but it may also appear in an innocuous form in kidney transplant recipients. A 5-year-old girl underwent an orthotopic liver transplant following a failed Kasai procedure for biliary atresia. She developed chronic rejection and vanishing bile duct syndrome. Fourteen months after the liver transplant she presented with complaints of nausea, vomiting, and weakness. She was found to have diffuse pneumatosis intestinalis but no free air. She developed subcutaneous air in the perineum and along the lateral abdominal wall bilaterally. She was treated with antibiotics, nasogastric suction, and her usual immunosuppressive drugs. She improved within 3 days. After 1 week the antibiotics were discontinued, and the patient was started on an elemental diet. The pneumatosis resolved radiographically within 2 weeks. She had no further episodes of pneumatosis intestinalis up to 8