TY - JOUR
T1 - Endoscopic retrograde cholangiopancreatography in adult patients with biliary atresia
AU - Hyun, Jong Jin
AU - Irani, Shayan S.
AU - Kozarek, Richard A.
N1 - Publisher Copyright:
Copyright © 2018 the Author(s). Published by Wolters Kluwer Health, Inc.
PY - 2018/5/1
Y1 - 2018/5/1
N2 - Introduction: Biliary atresia is a progressive inflammatory disease of the bile duct that eventually results in biliary cirrhosis. It is a rare neonatal disease that mandates treatment within the first 2 years of life in order for the infant to survive. Patients usually undergo palliative Kasai portoenterostomy. Even when Kasai portoenterostomy has been performed in a timely manner, progression is still inevitable. In fact, the majority of patients require curative liver transplantation at a later stage before reaching adulthood. Methods: Two jaundiced biliary atresia patients who have lived well beyond 20 years with their native liver after undergoing Kasai portoenterostomy and underwent endoscopic retrograde cholangiopancreatography (ERCP) were identified. The data on patients' clinical information, procedures performed, and outcomes were retrospectively collected by chart review. Results: Presence of a long Roux limb and acute angulation from external adhesions along with ductal anomaly from disease itself rendered ERCP challenging, and intraoperative ERCP had to be performed in 1 patient. As enteroscopes had to be used, availability of accessory devices was limited. Conclusion: Management of adult biliary atresia patients with biliary obstruction with ERCP is feasible, at times, through multidisciplinary means.
AB - Introduction: Biliary atresia is a progressive inflammatory disease of the bile duct that eventually results in biliary cirrhosis. It is a rare neonatal disease that mandates treatment within the first 2 years of life in order for the infant to survive. Patients usually undergo palliative Kasai portoenterostomy. Even when Kasai portoenterostomy has been performed in a timely manner, progression is still inevitable. In fact, the majority of patients require curative liver transplantation at a later stage before reaching adulthood. Methods: Two jaundiced biliary atresia patients who have lived well beyond 20 years with their native liver after undergoing Kasai portoenterostomy and underwent endoscopic retrograde cholangiopancreatography (ERCP) were identified. The data on patients' clinical information, procedures performed, and outcomes were retrospectively collected by chart review. Results: Presence of a long Roux limb and acute angulation from external adhesions along with ductal anomaly from disease itself rendered ERCP challenging, and intraoperative ERCP had to be performed in 1 patient. As enteroscopes had to be used, availability of accessory devices was limited. Conclusion: Management of adult biliary atresia patients with biliary obstruction with ERCP is feasible, at times, through multidisciplinary means.
KW - Kasai portoenterostomy
KW - ascending cholangitis
KW - biliary atresia
KW - endoscopic retrograde cholangiopancreatography
UR - http://www.scopus.com/inward/record.url?scp=85046505432&partnerID=8YFLogxK
U2 - 10.1097/MD.0000000000010603
DO - 10.1097/MD.0000000000010603
M3 - Article
C2 - 29718863
AN - SCOPUS:85046505432
SN - 0025-7974
VL - 97
JO - Medicine; analytical reviews of general medicine, neurology, psychiatry, dermatology, and pediatries
JF - Medicine; analytical reviews of general medicine, neurology, psychiatry, dermatology, and pediatries
IS - 18
M1 - e0603
ER -