Send to

Choose Destination
Pediatr Dev Pathol. 2018 Jan-Feb;21(1):29-40. doi: 10.1177/1093526617707851. Epub 2017 May 5.

Hepatic Hilar Lymph Node Reactivity at Kasai Portoenterostomy for Biliary Atresia: Correlations With Age, Outcome, and Histology of Proximal Biliary Remnant.

Author information

1 Division of Pathology and Laboratory Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
2 Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
3 Department of Pathology, Johns Hopkins School of Medicine, Baltimore, Maryland, USA.
4 Division of Pathology, The Hospital for Sick Children, Toronto, Canada.
5 Department of Pathology, Indiana University School of Medicine, Indianapolis, Indiana.
6 Department of Pathology, Texas Children's Hospital, Houston, Texas, USA.
7 Department of Pathology, Seattle Children's Hospital, Seattle, Washington, USA.
8 Department of Pathology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, USA.
9 Department of Anatomic Pathology, University of California, San Francisco, California, USA.
10 Department of Pathology, Children's Hospital Colorado, Aurora, Colorado, USA.
11 Department of Pathology, Kravis Children's Hospital, Mount Sinai Medical Center, New York, New York, USA.
12 Department of Pathology, Ann & Robert H. Lurie Children's Hospital, Chicago, Illinois, USA.
13 Department of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
14 Department of Pathology, Children's Healthcare of Atlanta, Atlanta, Georgia, USA.
15 Department of Pathology, Children's Hospital Los Angeles, Los Angeles, California, USA.
16 Department of Pathology and Immunology, Washington University School of Medicine, St Louis, Missouri, USA.
17 Quest Diagnostics, Health Informatics, Madison, New Jersey, USA.
18 Department of Biostatistics, University of Michigan, Ann Arbor, Michigan, USA.
19 Department of Surgery, University of Michigan, Ann Arbor, Michigan, USA.


We hypothesized that if infection is the proximate cause of congenital biliary atresia, an appropriate response to antigen would occur in lymph nodes contiguous with the biliary remnant. We compared the number of follicular germinal centers (GC) in 79 surgically excised hilar lymph nodes (LN) and 27 incidentally discovered cystic duct LNs in 84 subjects at the time of hepatic portoenterostomy (HPE) for biliary atresia (BA) to autopsy controls from the pancreaticobiliary region of non-septic infants >3 months old at death. All 27 control LN lacked GC, a sign in infants of a primary response to antigenic stimulation. GC were found in 53% of 106 LN in 56 of 84 subjects. Visible surgically excised LN contiguous with the most proximal biliary remnants had 1 or more well-formed reactive GC in only 26/51 subjects. Presence of GC and number of GC/LN was unrelated to age at onset of jaundice or to active fibroplasia in the biliary remnant but was related to older age at HPE. Absent GC in visible and incidentally removed cystic duct LNs predicted survival with the native liver at 2 and 3 years after HPE, Pā€‰=ā€‰.03, but significance was lost at longer intervals. The uncommon inflammatory lesions occasionally found in remnants could be secondary either to bile-induced injury or secondary infection established as obstruction evolves. The absence of consistent evidence of antigenic stimulation in LN contiguous with the biliary remnant supports existence of at least 1 major alternative to infection in the etiology of biliary atresia.


Hilar lymph node; Kasai procedure; biliary atesia; biliary remnant; germinal center reaction; outcome


Supplemental Content

Full text links

Icon for Atypon
Loading ...
Support Center