Book contents
- Frontmatter
- Contents
- List of contributors
- Acknowledgments
- Preface
- Part I General issues
- Part II Head and neck
- Part III Thorax
- Part IV Abdomen
- 20 Abdominal surgery: general aspects
- 21 Abdominal wall defects
- 22 Inguinal and umbilical hernias
- 23 Infantile hypertrophic pyloric stenosis
- 24 Small bowel disorders
- 25 Cystic fibrosis
- 26 Necrotizing enterocolitis
- 27 Inflammatory bowel disease in children
- 28 Intestinal failure
- 29 Appendicitis
- 30 Hirschsprung's disease
- 31 Anorectal malformations: experience with the posterior sagittal approach
- 32 Gastrointestinal motility disorders
- 33 The Malone antegrade continence enema (MACE) procedure
- 34 Splenectomy
- 35 Biliary atresia
- 36 Choledochal cyst
- 37 Biliary stone disease
- 38 Portal hypertension
- 39 Persistent hyperinsulinemic hypoglycemia in infancy
- 40 Acute and chronic pancreatitis in children
- Part V Urology
- Part VI Oncology
- Part VII Transplantation
- Part VIII Trauma
- Part IX Miscellaneous
- Index
- Plate section
- References
35 - Biliary atresia
from Part IV - Abdomen
Published online by Cambridge University Press: 08 January 2010
- Frontmatter
- Contents
- List of contributors
- Acknowledgments
- Preface
- Part I General issues
- Part II Head and neck
- Part III Thorax
- Part IV Abdomen
- 20 Abdominal surgery: general aspects
- 21 Abdominal wall defects
- 22 Inguinal and umbilical hernias
- 23 Infantile hypertrophic pyloric stenosis
- 24 Small bowel disorders
- 25 Cystic fibrosis
- 26 Necrotizing enterocolitis
- 27 Inflammatory bowel disease in children
- 28 Intestinal failure
- 29 Appendicitis
- 30 Hirschsprung's disease
- 31 Anorectal malformations: experience with the posterior sagittal approach
- 32 Gastrointestinal motility disorders
- 33 The Malone antegrade continence enema (MACE) procedure
- 34 Splenectomy
- 35 Biliary atresia
- 36 Choledochal cyst
- 37 Biliary stone disease
- 38 Portal hypertension
- 39 Persistent hyperinsulinemic hypoglycemia in infancy
- 40 Acute and chronic pancreatitis in children
- Part V Urology
- Part VI Oncology
- Part VII Transplantation
- Part VIII Trauma
- Part IX Miscellaneous
- Index
- Plate section
- References
Summary
Introduction
Biliary atresia presents in the neonatal period, occurs with a frequency of between 1 in 8000 and 1 in 16000 live births and accounts for more than 50% of pediatric liver transplantation. The cause of the disease remains obscure but current evidence suggests that there is more than one etiologic factor. Both the intra- and extrahepatic bile ducts are affected and affected infants present with jaundice and pale stools within the first few weeks of life. The intrahepatic pathology, which has been likened to sclerosing cholangitis, is accompanied by an inflammatory sclerosing lesion of the extrahepatic bile ducts, which results in obstruction of the lumen and, in some cases, complete disappearance of segments of the biliary tract. Death from cirrhotic liver failure occurs within 2 years in untreated cases and biliary atresia represents the most frequent reason for liver transplantation in childhood.
Historical issues
Thomson3 published the first major review in 1892. He collected 49 cases from the literature and added a further case of his own. He recorded that “the children themselves are either jaundiced at birth, or they become so within the first week or so of life; otherwise they are healthy and well nourished.” He concluded that, whatever the etiology of the condition, it was characterized by a progressive destructive inflammatory lesion of the biliary tract.
- Type
- Chapter
- Information
- Pediatric Surgery and UrologyLong-Term Outcomes, pp. 446 - 464Publisher: Cambridge University PressPrint publication year: 2006