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Congenital Biliary Atresia: Analysis of 97 Cases With Reference to Prognosis After Hepatic Portoenterostomy

Congenital Biliary Atresia: Analysis of 97 Cases With Reference to Prognosis After Hepatic... Abstract • The prognosis of 97 patients with congenital biliary atresia treated by hepatic portoenterostomy was studied. In 37 of 97 patients (38%), good bile excretion was observed after surgery, and jaundice cleared. Sixteen of the 37 patients developed ascending cholangitis; 11, esophageal varices; and nine died. Seven died of ascending cholangitis and two of hepatic failure. Fourteen patients had an uneventful course. In the remaining 60 patients, bile excretion was scanty, and the disease progressed to biliary cirrhosis; 52 died. Causes of death were hepatic failure (53%), intracranial hemorrhage (16%), rupture of esophageal varices (12%), severe dehydration (9%), and postoperative complications (9%). The age at death ranged from 2 to 52 months, with an average of 16 months. (Am J Dis Child, 130:830-833, 1976) References 1. Holmes JB: Congenital obliteration of the bile ducts: Diagnosis and suggestions for treatment . Am J Dis Child 11:405-431, 1916. 2. Gerrish EW, Cole JW: Surgical jaundice in infants and children . Arch Surg 63:529-535, 1951.Crossref 3. Gross RE: The Surgery of Infancy and Childhood . Philadelphia, WB Saunders Co, 1953, p 508. 4. Silverberg M, Craig J, Gellis SS: Problems in the diagnosis of biliary atresia: A review and consideration of histological criteria . Am J Dis Child 99:574-584, 1960.Crossref 5. Moore TC: Congenital atresia of the extrahepatic bile ducts: Report of 31 proved cases . Surg Gynecol Obstet 96:215-225, 1953. 6. Longmire WP Jr, Sanford MC: Intrahepatic cholangiojejunostomy with partial hepatectomy for biliary obstruction . Surgery 24:264-276, 1948. 7. Sterling JA: Total bile duct atresia . Pediatr Clin North Am 4:769-780, 1957. 8. Sterling JA, Lowemburg H: Observations of infants with hepatic duct atresia and use of artificial duct prosthesis . Pediatr Clin North Am 9:485-504, 1962. 9. Kasai M, Kimura S, Wagatsuma M, et al: Die chirurgischen Behandlungen der angeborenen Missbildungen des Gallengangs . Therapiewoche 12:710-715, 1963. 10. Kasai M, Kimura S, Asakura Y, et al: Surgical treatment of biliary atresia . J Pediatr Surg 3:665-675, 1968.Crossref 11. Sawaguchi S, Nakajo T, Hori T, et al: Two stage operation for congenital biliary atresia: A temporary external biliary fistula with a jejunal segment. Read before the fourth annual meeting of the Pacific Association of Pediatric Surgeons, Vancouver and Harrison Hot Springs, Canada, 1971. 12. Sawaguchi S, Akiyama H, Hori T, et al: Surgical treatment of congenital biliary atresia: Hepatic porto-jejunostomy. Read before the fifth annual meeting of the Pacific Association of Pediatric Surgeons, Tokyo, 1972. 13. Redo SF: Congenital atresia of extrahepatic bile ducts . Arch Surg 69:886-897, 1954.Crossref 14. Schnug GE: Importance of early operation in congenital atresia of the extrahepatic bile ducts: Report of ten proved cases . Ann Surg 148:931-936, 1958.Crossref 15. Krovetz LJ: Congenital biliary atresia: II. Analysis of the therapeutic problem . Surgery 47:468-489, 1960. 16. Danks DM, Campbell PE, Clarke AM, et al: Extrahepatic biliary atresia: The frequency of potentially operable cases . Am J Dis Child 128:684-686, 1974.Crossref 17. Kobayashi A, Utsunomiya T, Ohbe Y, et al: Ascending cholangitis after successful surgical repair of biliary atresia . Arch Dis Child 48:697-703, 1973.Crossref 18. Kobayashi A, Utsunomiya T, Kawai S, et al: Long-term antibiotic therapy for prevention of ascending cholangitis after successful surgical repair of biliary atresia. Read before the 11th annual meeting of the Japanese Society of Pediatric Surgery, Tokyo, 1974. 19. Thaler MM, Gellis SS: Studies in neonatal hepatitis and biliary atresia: III. Progression and regression of cirrhosis in biliary atresia . Am J Dis Child 116:271-279, 1968.Crossref 20. Cameron R, Bunton GL: Congenital biliary atresia . Br Med J 2:1253-1257, 1960.Crossref 21. Rickham PP, Lee EYC: Neonatal jaundice: Surgical aspects . Clin Pediatr 3:197-208, 1964.Crossref 22. Myers RL, Baggenstoss AH, Logan GB, et al: Congenital atresia of the extrahepatic biliary tract: A clinical and pathologic study . Pediatrics 18:767-781, 1956. 23. Cohen MI, Gartner LM: The use of medium-chain triglycerides in the management of biliary atresia . J Pediatr 79:379-384, 1971.Crossref 24. Weber A, Roy CC: The malabsorption associated with chronic liver disease in children . Pediatrics 50:73-83, 1972. 25. Kobayashi A, Kawai S, Utsunomiya T, et al: Bone disease in infants and children with hepatobiliary disease . Arch Dis Child 49:641-646, 1974.Crossref 26. Kobayashi A, Utsunomiya T, Ohbe Y, et al: Intestinal absorption of calcium and magnesium in hepatobiliary disease in infancy . Arch Dis Child 49:90-96, 1974.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Congenital Biliary Atresia: Analysis of 97 Cases With Reference to Prognosis After Hepatic Portoenterostomy

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References (28)

Publisher
American Medical Association
Copyright
Copyright © 1976 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1976.02120090040007
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Abstract

Abstract • The prognosis of 97 patients with congenital biliary atresia treated by hepatic portoenterostomy was studied. In 37 of 97 patients (38%), good bile excretion was observed after surgery, and jaundice cleared. Sixteen of the 37 patients developed ascending cholangitis; 11, esophageal varices; and nine died. Seven died of ascending cholangitis and two of hepatic failure. Fourteen patients had an uneventful course. In the remaining 60 patients, bile excretion was scanty, and the disease progressed to biliary cirrhosis; 52 died. Causes of death were hepatic failure (53%), intracranial hemorrhage (16%), rupture of esophageal varices (12%), severe dehydration (9%), and postoperative complications (9%). The age at death ranged from 2 to 52 months, with an average of 16 months. (Am J Dis Child, 130:830-833, 1976) References 1. Holmes JB: Congenital obliteration of the bile ducts: Diagnosis and suggestions for treatment . Am J Dis Child 11:405-431, 1916. 2. Gerrish EW, Cole JW: Surgical jaundice in infants and children . Arch Surg 63:529-535, 1951.Crossref 3. Gross RE: The Surgery of Infancy and Childhood . Philadelphia, WB Saunders Co, 1953, p 508. 4. Silverberg M, Craig J, Gellis SS: Problems in the diagnosis of biliary atresia: A review and consideration of histological criteria . Am J Dis Child 99:574-584, 1960.Crossref 5. Moore TC: Congenital atresia of the extrahepatic bile ducts: Report of 31 proved cases . Surg Gynecol Obstet 96:215-225, 1953. 6. Longmire WP Jr, Sanford MC: Intrahepatic cholangiojejunostomy with partial hepatectomy for biliary obstruction . Surgery 24:264-276, 1948. 7. Sterling JA: Total bile duct atresia . Pediatr Clin North Am 4:769-780, 1957. 8. Sterling JA, Lowemburg H: Observations of infants with hepatic duct atresia and use of artificial duct prosthesis . Pediatr Clin North Am 9:485-504, 1962. 9. Kasai M, Kimura S, Wagatsuma M, et al: Die chirurgischen Behandlungen der angeborenen Missbildungen des Gallengangs . Therapiewoche 12:710-715, 1963. 10. Kasai M, Kimura S, Asakura Y, et al: Surgical treatment of biliary atresia . J Pediatr Surg 3:665-675, 1968.Crossref 11. Sawaguchi S, Nakajo T, Hori T, et al: Two stage operation for congenital biliary atresia: A temporary external biliary fistula with a jejunal segment. Read before the fourth annual meeting of the Pacific Association of Pediatric Surgeons, Vancouver and Harrison Hot Springs, Canada, 1971. 12. Sawaguchi S, Akiyama H, Hori T, et al: Surgical treatment of congenital biliary atresia: Hepatic porto-jejunostomy. Read before the fifth annual meeting of the Pacific Association of Pediatric Surgeons, Tokyo, 1972. 13. Redo SF: Congenital atresia of extrahepatic bile ducts . Arch Surg 69:886-897, 1954.Crossref 14. Schnug GE: Importance of early operation in congenital atresia of the extrahepatic bile ducts: Report of ten proved cases . Ann Surg 148:931-936, 1958.Crossref 15. Krovetz LJ: Congenital biliary atresia: II. Analysis of the therapeutic problem . Surgery 47:468-489, 1960. 16. Danks DM, Campbell PE, Clarke AM, et al: Extrahepatic biliary atresia: The frequency of potentially operable cases . Am J Dis Child 128:684-686, 1974.Crossref 17. Kobayashi A, Utsunomiya T, Ohbe Y, et al: Ascending cholangitis after successful surgical repair of biliary atresia . Arch Dis Child 48:697-703, 1973.Crossref 18. Kobayashi A, Utsunomiya T, Kawai S, et al: Long-term antibiotic therapy for prevention of ascending cholangitis after successful surgical repair of biliary atresia. Read before the 11th annual meeting of the Japanese Society of Pediatric Surgery, Tokyo, 1974. 19. Thaler MM, Gellis SS: Studies in neonatal hepatitis and biliary atresia: III. Progression and regression of cirrhosis in biliary atresia . Am J Dis Child 116:271-279, 1968.Crossref 20. Cameron R, Bunton GL: Congenital biliary atresia . Br Med J 2:1253-1257, 1960.Crossref 21. Rickham PP, Lee EYC: Neonatal jaundice: Surgical aspects . Clin Pediatr 3:197-208, 1964.Crossref 22. Myers RL, Baggenstoss AH, Logan GB, et al: Congenital atresia of the extrahepatic biliary tract: A clinical and pathologic study . Pediatrics 18:767-781, 1956. 23. Cohen MI, Gartner LM: The use of medium-chain triglycerides in the management of biliary atresia . J Pediatr 79:379-384, 1971.Crossref 24. Weber A, Roy CC: The malabsorption associated with chronic liver disease in children . Pediatrics 50:73-83, 1972. 25. Kobayashi A, Kawai S, Utsunomiya T, et al: Bone disease in infants and children with hepatobiliary disease . Arch Dis Child 49:641-646, 1974.Crossref 26. Kobayashi A, Utsunomiya T, Ohbe Y, et al: Intestinal absorption of calcium and magnesium in hepatobiliary disease in infancy . Arch Dis Child 49:90-96, 1974.Crossref

Journal

American Journal of Diseases of Children – American Medical Association

Published: Aug 1, 1976

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