Benign Recurrent Intrahepatic Cholestasis in Pregnancy: Fetal Death at 36 Weeks of Gestation.

Mariam Ayyash,Ashina Singh,Nicolina Smith,Madhurima Keerthy,Majid Shaman,Daniel Martin

doi:10.1155/2021/5086846

Mariam Ayyash, Ashina Singh + Show 4 more

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https://doi.org/10.1155/2021/5086846

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Abstract

Introduction Benign recurrent intrahepatic cholestasis is a rare hepatologic disorder characterized by recurrent, self-limited episodes of severe pruritus, jaundice, and elevated bile acids. While there are guidelines for the management of intrahepatic cholestasis of pregnancy, the literature regarding benign recurrent intrahepatic cholestasis and pregnancy is limited. Case A 29-year-old G1P0 woman, with history of liver toxicity, had elevated total serum bile acid levels and liver enzymes documented at 8 weeks of gestation and throughout her pregnancy. She had a reactive nonstress test just 3 days prior to her induction. Fetal demise was noted when she presented at 36 weeks for her induction. Conclusion We recommend that women with elevated total serum bile acid early in pregnancy due to a separate entity relative to intrahepatic cholestasis of pregnancy be managed in a more individualized approach.

Highlights

Benign recurrent intrahepatic cholestasis is a rare hepatologic disorder characterized by recurrent, self-limited episodes of severe pruritus, jaundice, and elevated bile acids
Our patient had a unique presentation of cholestasis during pregnancy, which was later confirmed as Benign recurrent intrahepatic cholestasis (BRIC)
Her total serum bile acids (TSBA) levels had remained above 200 μmol/L after the first trimester and went under 100 μmol/L only when she presented with the fetal death at the time of her scheduled labor induction

Summary

Introduction

Benign recurrent intrahepatic cholestasis (BRIC) was first described in 1959 [1]. It can present any time in life, from infancy to late adulthood, and is characterized by recurrent self-limited episodes of severe pruritus and jaundice that can last from several weeks to months [2]. Given the risk of fetal death within the context of ICP, the Society for Maternal-Fetal Medicine recommends induction of labor for women with ICP in the late preterm/early term period, between 36 weeks and 39 weeks if TSBA is under 100 μmol/L and at 36 weeks if TSBA is more than or equal to 100 μmol/L [8]. Because such recommendations are for ICP, there are no particular guidelines for labor induction within the setting of BRIC or other cholestatic liver disorders, when bile acid levels may be elevated within the first trimester. Upon presenting at her 36-week gestation mark for induction of labor, the patient had fetal death

Case Presentation

Discussion