Refractory Intrahepatic Cholestasis of Pregnancy in Twin Gestation Managed With Ursodeoxycholic Acid and Adjunctive...

Intrahepatic cholestasis of pregnancy (ICP) is a liver disorder which is characterized by pruritus and elevated bile acids during pregnancy and is further coupled with higher maternal and fetal risks. We report a rare case of early-onset, refractory ICP with elevated liver enzymes in a 25-year-old primigravida with a monochorionic diamniotic twin gestation complicated by selective fetal growth restriction (sFGR). At 24 weeks of gestation, the patient presented with severe generalized pruritus, mainly on the palms and soles, impairing her quality of life. The patient with insignificant medical history of liver diseases, but has a significant family history of ICP. On examination, she was afebrile, hemodynamically stable, and without jaundice or rash. Laboratory findings revealed moderately raised bile acids (40.98 µmol/L) fulfilling criteria for moderate ICP but coupled with elevated liver enzymes. Viral hepatitis serologies, autoimmune profile, and abdominal ultrasound were all unremarkable. Treatment with ursodeoxycholic acid (UDCA) 500 mg twice daily was initiated. Given worsening biochemical (doubling of bile acids) and clinical findings, gastroenterologists were consulted after a week of treatment initiation, and their recommendation was to add cholestyramine 4 g once daily as an adjunctive. Afterwards, with persistently elevated bile acids, the UDCA dose increased to 500 mg three times daily, and cholestyramine was increased to 4 g twice daily. Phytonadione (vitamin K) 10 mg orally daily was added to reduce the risk of bleeding due to reduced absorption of fat-soluble vitamins caused by cholestyramine. At 32 weeks of gestation, she developed vomiting, coffee-ground emesis, vaginal spotting, and abdominal pain, progressing to preterm labor with non-reassuring fetal heart tracings. An emergency category 1 cesarean section was performed, delivering two viable female infants (1600 g and 1160 g), both admitted to the neonatal intensive care unit (NICU). Postnatally, the maternal condition was stable, and biochemical parameters normalized rapidly within 72 hours postpartum. Pruritus resolved completely, confirming the diagnosis of ICP.