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Anesthetic management of a parturient with congenital hepatic fibrosis
Abstract Number: FCH-338
Abstract Type: Case Report Case Series
Congenital hepatic fibrosis (CHFib) is a rare disease of autosomal dominant inheritance with variable penetrance and expression, hence, diverse clinical presentations. The course of this disease leads to portal hypertension which is associated with increased risks of gastrointestinal hemorrhage, and impaired liver function.1
A 37-year-old G0 woman was incidentally diagnosed with congenital hepatic fibrosis, chronic portal vein stenosis/hypertension, portal vein thrombosis, thrombocytopenia and esophageal varices. In addition, her known past medical history included Factor V Leiden heterozygosity and protein S deficiency. In pre-pregnancy consultation, she was recommended to have a splenectomy and to consider achieving parentage with a gestational carrier or adoption. She declined splenectomy and conceived spontaneously. Antenatally, she received multidisciplinary care with maternal-fetal medicine, gastroenterology, hematology and anesthesiology. Portal hypertension was treated with carvedilol, and portal vein thrombosis, Factor V Leiden heterozygosity and protein S deficiency with enoxaparin. At 36 weeks, she was transitioned to unfractionated heparin 15,000 units twice daily. At 38 weeks, she was admitted for fetal growth restriction with abnormal umbilical artery Doppler. The obstetric plan was to commence induction and to perform an assisted second stage of delivery to minimize the impact of Valsalva on portal pressure.
The patient coagulation profile is summarized in table 1. Heparin was discontinued for 24 h, and given a normal coagulation profile and ROTEM, an epidural was deemed safe. An epidural was performed at the L3-L4 level. The patient tolerated the procedure well. During the second stage, a forceps-assisted vaginal delivery of a 2185 g female infant was performed. Postpartum, enoxaparin 40 mg daily was resumed. Her post-partum period was uneventful and was discharged home on postpartum day 3.
When caring for a parturient with CHFib, it is important to consider that esophageal varices bleeding is more common during pregnancy given increased blood volume and inferior vena cava compression. Up to 50% of parturients with known portal hypertension may experience esophageal bleeding.2 Liver function should be closely monitored as it may impact the anesthetic plan. A multidisciplinary approach is imperative when caring for patients with CHfib.
1. Int J Gynaecol Obstet 2005;88:142–3.
2. Liver Transpl 2008;14:1081–91.