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Selective distal splenorenal shunts
102
Liver Transplantation With Use of Cyclosporin A and PredT.E. Starzl, G.B.G. Klintmalm, K.A. Porter, S. lwatsuki, and G.P.J. Schroter. N Engl J Med 305:266-269, nisone.
(July), 1981. The authors describe a trial of orthotopic liver transplantation in which cyclosporin A and low dose prednisone was used for immunosuppression in 14 patients ages 8-41 yr. Two patients died during operation. Ten (83%) of the 12 patients who survived surgery are living 8-14.5 me following transplantation. One child died 19 days postoperatively following thrombosis of the homograft hepatic artery. Another patient lived for I yr before succumbing to recurrence of cholangiocarcinoma. Episodes of infection were easily controlled with small increases in prednisone and complications were relatively easily managed when they occurred. These factors were not present in prior trials. There were no occurrences of de novo neoplasms (including lymphomas) in the short period of follow up. The authors ascribe the exceptional improvement in survival to the use of this new immunosuppressant agent. Eugene S. Wiener Hepatic Haemangiomata: Diagnosis and Management. V.F. Larcher, E.R. Howard, and Ah'x P. Mowat. Arch Dis Child
56:7 14, (January), 1981. Six cases of hemangioma of the liver are described in detail. Presentation was within the first weeks of life in live cases: hepatomegaly, cardiac failure, and anemia were the main features. In three cases, cutaneous hemangiomata substantiated the diagnosis of hepatic hcmangioma, but in the other three cases biopsy was necessary to exclude hepatoblastoma. Technetium scan and ultrasound helped in identifying and localizing the hemangioma but celiac axis angiography was needed to assess the probable benefit from arterial ligation or the feasibility of resection. Control of cardiac failure by digoxin, diuretics and corticosteroids was unsuccessful in three cases but hepatic artery ligation produced immediate and lasting improvement in each instance. Problems with portal hypertension and intrahepatic obstructive jaundice were ultimately fatal in one case. One case resolved spontaneously and one improved following radiotherapy (3000 rad) given, with reservations, because of intractable disseminated intravascular coagulation and hemorrhagic ascites. A scheme of management based on the conclusions from these cases is: (i) expectant treatment in the absence of complications, (ii) digoxin and diuretics for cardiac failure but, if unsuccessful, (iii) hepatic artery ligation in patients under 6 wk of age or (iv) trial of Prednisolone ( 2 m g / k g / d a y ) before recourse to hepatic artery ligation in patients over 6 wk of age. P.A.M. Raine Extrahepatic Biliary Atresia Associated With Cyanotic Congenital Heart Disease: Three Case Reports and a Review. D.D. Zukin, R.R. Liberthson, and A.M. Lake. Clin
Pediatr 20:64-66, (January), 1981. Three infants with coexisting biliary atresia and cyanotic congenital heart d i s e a s e a r e presented in detail. All three had extrahepatic biliary atresia and the heart conditions w e r e Tetralogy of Fallot (2) and transposition of the great arteries. Two patients presented with a greenish hue to t h e skin due to the combination of jaundice and cyanosis. Two of the patients failed to develop biliary drainage after portoen-
ABSTRACTS
terostomy and succumbed to hepatic failure. The other infant had a successful portoenterostomy and is awaiting repair of her cardiac defect. The authors reviewed five patients with the coexisting anomalies with only one survivor. The atypical skin coloration may lead to delay in diagnosis of anomalies which are potentially correctable. Randall W. Powell Congenital Spherocytosis Is Often Not Hereditary. R.F. Stevens and D.I.K. Evans. Clin Pediatr 20:47-60, (January), 1981.
A retrospective review of 50 patients from 41 families with hereditary spherocytosis revealed 19 (38%) with a negative family history. In three patients a parent was discovered on laboratory examination to have the disease while four families were not tested. In 12 patients' families with a negative history laboratory investigations were negative in the parents and all siblings. The authors feel this can be best explained by spontaneous mutation with the remote possibility of a carrier state or recessively inherited mutation explaining some atypical cases. Of importance is the absence of a positive family histury in 38% of the patients in this series. Randall W. Powell Spontaneous Splenic Rupture in Infectious Mononucleosis: Sonographic Diagnosis and Follow-Up. M. A. Johnson, P. L. Cooperberg, J. Boisvert. J. L. Stoller, and tt. Winrob. Am J
Roentgenol 136:111 l14,(.lanuary), 1981. Three patients, ages 14, 14, and 23 yr, developed abdominal pain during a coursc ot" mononucleosis. Sonographic examinations revealed findings consistent with splenic rupture including splenomegaly (1), subcapsular and pericapsular fluid collections (3), and intraperitoneal fluid (1). In two patients the findings regressed on serial sonographic studies. In the 23 yr old male a persistent perisplenic fluid collection was aspirated (500 cc) and did not recur on subsequent scans. One patient required transfusion and one underwent surgery. -Randall W. Powell Selective Distal Splenorenal Shunts. ,4. L. Maehado, J. E. B. Filho, A. B. Campos, J. B. Pires de Paula Pessoa, and R. A. Correia. Am J Surg 142:281 289, 1981.
Twenty patients underwent elective distal splenorenal shunts between 1974 and 1978. They ranged in age from 9 to 52 yr. Portal hypertension was present in 16 because of hepatic schistosomiasis, Laennec's cirrhosis in 2, congenital hepatic fibrosis in 1, and extrahepatic portal thrombosis in 1. The surgical technique is described. Evaluation of the immediate results included pressure measurements before and after anastomosis, the evaluation of transient postoperative ascites, the presence of jaundice and hemorrhage, and mortality. Evaluation of the intermediate results up to 4 yr postoperatively included the presence of encephalopathy (no patients), the recurrence of hemorrhage, and the occurrence of hypersplenism (none). The authors concur that this procedure is excellent for decompression of portal hypertension due to schistosomiasis, an epidemic in Brazil. Because of minimal ascites, excellent liver function, improvement in hypersplenism, and decompression of congested short gastric veins, they conclude that this procedure is far better than standard portacaval shunts for patients with hepatosplenic schistosomiasis.--Jane F. Goldthorn
Liver Transplantation With Use of Cyclosporin A and PredT.E. Starzl, G.B.G. Klintmalm, K.A. Porter, S. lwatsuki, and G.P.J. Schroter. N Engl J Med 305:266-269, nisone.
(July), 1981. The authors describe a trial of orthotopic liver transplantation in which cyclosporin A and low dose prednisone was used for immunosuppression in 14 patients ages 8-41 yr. Two patients died during operation. Ten (83%) of the 12 patients who survived surgery are living 8-14.5 me following transplantation. One child died 19 days postoperatively following thrombosis of the homograft hepatic artery. Another patient lived for I yr before succumbing to recurrence of cholangiocarcinoma. Episodes of infection were easily controlled with small increases in prednisone and complications were relatively easily managed when they occurred. These factors were not present in prior trials. There were no occurrences of de novo neoplasms (including lymphomas) in the short period of follow up. The authors ascribe the exceptional improvement in survival to the use of this new immunosuppressant agent. Eugene S. Wiener Hepatic Haemangiomata: Diagnosis and Management. V.F. Larcher, E.R. Howard, and Ah'x P. Mowat. Arch Dis Child
56:7 14, (January), 1981. Six cases of hemangioma of the liver are described in detail. Presentation was within the first weeks of life in live cases: hepatomegaly, cardiac failure, and anemia were the main features. In three cases, cutaneous hemangiomata substantiated the diagnosis of hepatic hcmangioma, but in the other three cases biopsy was necessary to exclude hepatoblastoma. Technetium scan and ultrasound helped in identifying and localizing the hemangioma but celiac axis angiography was needed to assess the probable benefit from arterial ligation or the feasibility of resection. Control of cardiac failure by digoxin, diuretics and corticosteroids was unsuccessful in three cases but hepatic artery ligation produced immediate and lasting improvement in each instance. Problems with portal hypertension and intrahepatic obstructive jaundice were ultimately fatal in one case. One case resolved spontaneously and one improved following radiotherapy (3000 rad) given, with reservations, because of intractable disseminated intravascular coagulation and hemorrhagic ascites. A scheme of management based on the conclusions from these cases is: (i) expectant treatment in the absence of complications, (ii) digoxin and diuretics for cardiac failure but, if unsuccessful, (iii) hepatic artery ligation in patients under 6 wk of age or (iv) trial of Prednisolone ( 2 m g / k g / d a y ) before recourse to hepatic artery ligation in patients over 6 wk of age. P.A.M. Raine Extrahepatic Biliary Atresia Associated With Cyanotic Congenital Heart Disease: Three Case Reports and a Review. D.D. Zukin, R.R. Liberthson, and A.M. Lake. Clin
Pediatr 20:64-66, (January), 1981. Three infants with coexisting biliary atresia and cyanotic congenital heart d i s e a s e a r e presented in detail. All three had extrahepatic biliary atresia and the heart conditions w e r e Tetralogy of Fallot (2) and transposition of the great arteries. Two patients presented with a greenish hue to t h e skin due to the combination of jaundice and cyanosis. Two of the patients failed to develop biliary drainage after portoen-
ABSTRACTS
terostomy and succumbed to hepatic failure. The other infant had a successful portoenterostomy and is awaiting repair of her cardiac defect. The authors reviewed five patients with the coexisting anomalies with only one survivor. The atypical skin coloration may lead to delay in diagnosis of anomalies which are potentially correctable. Randall W. Powell Congenital Spherocytosis Is Often Not Hereditary. R.F. Stevens and D.I.K. Evans. Clin Pediatr 20:47-60, (January), 1981.
A retrospective review of 50 patients from 41 families with hereditary spherocytosis revealed 19 (38%) with a negative family history. In three patients a parent was discovered on laboratory examination to have the disease while four families were not tested. In 12 patients' families with a negative history laboratory investigations were negative in the parents and all siblings. The authors feel this can be best explained by spontaneous mutation with the remote possibility of a carrier state or recessively inherited mutation explaining some atypical cases. Of importance is the absence of a positive family histury in 38% of the patients in this series. Randall W. Powell Spontaneous Splenic Rupture in Infectious Mononucleosis: Sonographic Diagnosis and Follow-Up. M. A. Johnson, P. L. Cooperberg, J. Boisvert. J. L. Stoller, and tt. Winrob. Am J
Roentgenol 136:111 l14,(.lanuary), 1981. Three patients, ages 14, 14, and 23 yr, developed abdominal pain during a coursc ot" mononucleosis. Sonographic examinations revealed findings consistent with splenic rupture including splenomegaly (1), subcapsular and pericapsular fluid collections (3), and intraperitoneal fluid (1). In two patients the findings regressed on serial sonographic studies. In the 23 yr old male a persistent perisplenic fluid collection was aspirated (500 cc) and did not recur on subsequent scans. One patient required transfusion and one underwent surgery. -Randall W. Powell Selective Distal Splenorenal Shunts. ,4. L. Maehado, J. E. B. Filho, A. B. Campos, J. B. Pires de Paula Pessoa, and R. A. Correia. Am J Surg 142:281 289, 1981.
Twenty patients underwent elective distal splenorenal shunts between 1974 and 1978. They ranged in age from 9 to 52 yr. Portal hypertension was present in 16 because of hepatic schistosomiasis, Laennec's cirrhosis in 2, congenital hepatic fibrosis in 1, and extrahepatic portal thrombosis in 1. The surgical technique is described. Evaluation of the immediate results included pressure measurements before and after anastomosis, the evaluation of transient postoperative ascites, the presence of jaundice and hemorrhage, and mortality. Evaluation of the intermediate results up to 4 yr postoperatively included the presence of encephalopathy (no patients), the recurrence of hemorrhage, and the occurrence of hypersplenism (none). The authors concur that this procedure is excellent for decompression of portal hypertension due to schistosomiasis, an epidemic in Brazil. Because of minimal ascites, excellent liver function, improvement in hypersplenism, and decompression of congested short gastric veins, they conclude that this procedure is far better than standard portacaval shunts for patients with hepatosplenic schistosomiasis.--Jane F. Goldthorn