Anesthetic management of a patient with progressive familial intrahepatic cholestasis type III
Progressive familial intrahepatic cholestasis (PFIC) type III is caused by a mutation in the ABCB4 gene, which encodes multidrug resistance protein 3 (MDR3). Dysfunctional MDR3 activity results in an imbalance in bile compositionwhich ultimately causes solubilization of biliary epithelial membranes, cell death and inflammation.1
A 32-year-old G2P1, known heterozygous carrier of a missense variant of ABCB4 gene, presented to an outside facility at 20 weeks and 2 days with hematemesis. Initial workup revealed grade III esophageal varices with active bleeding and hepatic portal hypertension. Blood work revealed elevated liver enzymes, INR of 1.6 and negative hepatitis serologies. Cardiac workup was unremarkable. The varices were banded to achieve hemostasis and hemodynamic stability. She was then transferred to our institution for further evaluation.
PFIC type III was confirmed given elevated bile acids, liver cirrhosis on biopsy, and genetic test. A transjugular intrahepatic portosystemic shunt (TIPS) was performed at 21 weeks. The patient was admitted for delivery at 36 weeks due to risk of fetal demise in the setting of elevated bile acids. Given the possibility of fetal cholestatic phenotype (potentially transient), the final delivery method was to be determined pending fetal coagulation profile after percutaneous umbilical blood sampling (PUBS). Given the normalized coagulation profile in the patient (Table 1) an epidural was deemed safe and was performed at the L3-L4 level. The PUBS was performed after confirming surgical level. Despite normal fetal coagulation profile, the patient elected for a cesarean delivery. The epidural was extended with a total of 15 ml of Lidocaine 2% with epinephrine and 100 mg of fentanyl to provide a T4 surgical level. The surgery and post-operative course were unremarkable. The patient was discharged home on post-operative day 4. Currently, she is undergoing testing for liver transplant candidacy.
Increased maternal blood volume, compression of the inferior vena cava favors esophageal varices bleeding. The use of TIPS during pregnancy is controversial, but may favor some patients.2When caring for parturient with PFIC, the anesthetic plan should be individualized based on liver function and comorbidities. A multidisciplinary approach including gastroenterology, maternal fetal medicine and anesthesiology is imperative.
1.Best Pract Res Clin Gastroenterol 2010;24:541–53.
2.Liver Transpl 2008;14:1081–91.