Biliary atresia & choledochal malformation--Embryological and anatomical considerations.
Semin Pediatr Surg
; 31(6): 151235, 2022 Dec.
Article
em En
| MEDLINE
| ID: mdl-36442454
ABSTRACT
The two main biliary pathologies in paediatric practice, biliary atresia and choledochal malformations (CM), have their origins within prenatal life. Nevertheless, the actual mechanisms remain elusive with many unanswered questions. The extrahepatic bile duct develops as a funnel-like structure emerging from the foregut from about 3-4 weeks of gestation into the mesenchyme of the septum transversum. The cranial elements of this contain hepatoblasts - the precursors to the two key cell lines that will become hepatocytes and biliary epithelial cells. The intrahepatic bile ducts develop separately and emerge from a complex process involving the ductal plate surrounding the in-growing portal venous system from about the 7-8th week of gestation. A developmental defect at some point(s) in this process may be the cause of at least some variants of BA - the Biliary Atresia Splenic Malformation syndrome particularly - though evidence in the more common isolated BA is much more circumstantial. Similarly, some types of choledochal malformation, specifically the cystic type of CM, are invariably present during prenatal life although again an actual aetiological mechanism remains elusive.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Temas:
Geral
Base de dados:
MEDLINE
Assunto principal:
Atresia Biliar
/
Ductos Biliares Extra-Hepáticos
Limite:
Child
/
Female
/
Humans
/
Pregnancy
Idioma:
En
Revista:
Semin Pediatr Surg
Assunto da revista:
PEDIATRIA
Ano de publicação:
2022
Tipo de documento:
Article