Biliary Obstruction – post transplant
Patient Presentation:
J.B., a 1 year old girl presents to inpatient transplant unit with fever, mild jaundice, pruritus, and pale colored stools. Patient is known to the transplant unit, she previously received a living related liver transplant (donor was her father) secondary to complications from a urea cycle defect. Post-transplant course was complicated by thrombosis of the hepatic artery and portal vein on post op days 3 and 5. Patient has no central line, no nausea/vomiting/diarrhea, no respiratory symptoms, and no sick contacts.
Labs as follows:
CBC- normal
Hepatic panel:ALT 198, AST 293, ALK Phos 780, GGT 1864, Total Bili 6.9, Direct Bili 5.8, Albumin: 3.6
Prograf- 7.9 (Goal is 5-8) [Prograf is an immunosuppressive drug]
Anti-Xa- 0.64 (Goal 0.5-1) [Xa is activated factor X]
Blood culture- no growth x 3 days
Urine culture- no growth x 3 days
Differential list:
Viral infection, Rejection, Cholangitis, Biliary obstruction
Diagnosis/Treatment:
Viral studies sent all came back negative. As the patient received a liver from her parent, the risk of rejection is considerably lower than it would be with a patient who received a transplant from an unrelated donor. However, rejection is always a fear, especially when the patient has a fever and shows signs of organ malfunction (lab values, acholic stools, jaundice, itching). Therefore, the team determined a liver biopsy was necessary. At the same time the patient was sedated for biopsy, she also underwent a PTC. PTC is performed in Interventional Radiology, and enables the radiologist to visualize an obstruction in the bile ducts, if there is one, and either balloon it open or place a temporary drain to facilitate emptying of the duct.
The liver biopsy came back normal. PTC showed a dilated and blocked bile duct. Old bile was drained from the duct and sent for culture, which later grew back pseudomonas. This was likely the cause of her fevers. A biliary drainage tube was inserted into the duct to re-open it, but was unable to be pushed all the way into the bowel due to the severity of the stricture. The tube was instead placed to external drainage to collect the bile outside her body.
Outcome:
A 10 day course of IV antibiotics was given for pseudomonas. Patient returned for repeat cholangiograms every 1-2 weeks for several months, each time inserting a larger tube into the duct in order to create scar tissue to keep the duct open and be able to remove the tube. Patient still has biliary tube in place, but it is capped and no longer to external drainage. The radiologists hope to be able to pull the drain when she returns for her next cholangiogram.