1. Long term Consequences of Hepaticojejunostomy in Patient with Choledochal Cyst

2. History
24 Year old woman
6 Months of age “severe cholangitis’’ emergently decompressed via cholecystostomy tube choledochal cyst noted
Definitive surgery: resection of choledochal cyst with roux-en-y reconstuction in the retrocolic position

3. History
Revised 1 year later 2° to obstruction: resection of residual cuff of choledochal cyst with hepaticojejunostomy subsequently developed atrophy of the left lateral segment
Liver BX 2yrs old - ? Liver cirrhosis
Labs July/01: ALP-235, AST-47, ALT-86, T.B 1.7, ALB-3.5, PLATS-112, PT-12, HB-12.5

4. History
July/01: Esophageal variceal bleeding 2° to portal vein thrombosis managed with endoscopic band ligation until obliterated
Jan/02: recurrent episodes of cholangitis
ALP ALB T.B AST-202
ALT WCC HB PLATS INR
CA (< 37) CEA ND

5. Imaging
Transhepatic cholangiography reveals stricturing of the right main hepatic duct and complete obstruction of the left main hepatic duct above the level of the main hepatic duct
No definite lesion at the biliary enteric anastomosis

7. TRANSHEPATIC INTERNAL/EXTERNAL CATHETERS (12FR) PLACED IN THE RIGHT AND LEFT MAIN HEPATIC DUCTS POST DILATION WITH 7MM ANGIOPLASTY BALLOONS WITH GOOD DRAINAGE FROM THE BILIARY SYSTEM INTO THE JEJUNUM

8. CT-Liver-Jan/02 Atrophic left lobe. Prominent intrahepatic ducts.
ATROPHIC LEFT LOBE OF LIVER
R-LOBE APPEARS REGENERATED
PROMINENT INTRAHEPATIC BILE DUCTS
SEPARATION OF THE HILUM WITH
AREA OF LOW ATTENUATION IN RIGHT
HEPATIC LOBE NEAR THE HILUM
ENLARGED LIVER
SPLENOMEGALY
Atrophic left lobe. Prominent intrahepatic ducts.
Separation of the hilum with an area of low attenuation
in the right hepatic lobe near the hilum.
Splenomegaly.

9. Liver Biopsy
PORTAL BILE DUCTULAR PROLIFERATION WITH FEW PERIDUCTULAR NEUTROPHILS AND CHRONIC INFLAM-MATORY CELLS C/W EARLY CHRONIC BILE STATSIS. NO EVIDENCE OF MALIGNANCY/CIRRHOSIS

10. History
Repeated dilations and catheter exchanges with resolution of strictures jan/02-sept/02 transhepatic catheters removed
Prophylactic ciprofloxacin 500mg PO BID x2 weeks every month
Ursodiol 500 MGS PO BID
Nadolol 40 MGS PO QD

12. Labs OCT/02 ALB-3.7 T.B-0.7 ALP-317 AST-70 ALT-41
FEB/03 ALB T.B-0.5 ALP-236 AST ALT-43 CA

13. ERCP