Pregnancy complicated by portal hypertension secondary to Noncirrhotic portal fibrosis

Abstract

Noncirrhotic portal fibrosis (NCPF) is a liver disorder characterized by periportal fibrosis of small and medium branches of portal vein resulting in portal hypertension with the sequelae of variceal bleed requiring immediate attention. We report a case of 27-years-old primigravida with NCPF presented for her booking visit with 3 months of amenorrhea. Clinical examination and ultrasound revealed 12 weeks of intrauterine gestation with moderate splenomegaly, dilated portal vein, and splenic vein. The patient was referred to medical gastroenterologist. Hematological investigations were found normal except for borderline thrombocytopenia. Upper gastrointestinal endoscopy revealed grade I esophageal varices. She was monitored bimonthly by obstetrician and hepatologist as she was potential for variceal bleed, anemia, liver derangement, and coagulation abnormalities during antenatal and intrapartum period. She had well-preserved liver function throughout pregnancy and no variceal bleed. Fetal surveillance was done and she received prophylactic corticosteroids at 28 weeks of gestation for fetal lung maturation. Antenatal period was uneventful. In view of breech presentation, elective cesarean section was performed and a healthy male baby was delivered weighing 2.9 kg, with good Apgar score. Intrapartum and postpartum period was uneventful. Surveillance by a multidisciplinary team is important for such high-risk pregnancy for optimizing obstetric and neonatal care.