Recommendations for management of pregnancy complicated by Caroli disease: A case report and literature review

We investigated the clinical and genetic characteristics of a 28-year-old pregnant Chinese woman with Caroli disease (CD) and systematically reviewed the published literature on pregnancy complicated by CD. We reviewed our case and 8 patients with 9 pregnancies included in 6 reports. The main clinical characteristics of pregnancy complicated by CD are cholestasis, liver and renal insufficiency, portal hypertension, and cirrhosis. In severe cases, there may be an increased risk of disease progression during pregnancy. Management of pregnancy complicated by CD is primarily supportive. Antimicrobial therapy and prevention of cholestasis and portal hypertension are recommended. Patients may benefit from multidisciplinary care.