BACKGROUND: Portal vein cavernoma (PVC) is a rare disease resulting from extrahepatic portal vein thrombosis and development of collateral venous circulation. The management of pregnancy and delivery in woman with PVC has rarely been described. CASES: Two primiparous women are presented to illustrate the management of PVC during pregnancy and discuss the delivery route according to the symptoms and the PVC complications. The first patient presented PVC associated with large jejunal varices and high anticardiolipid antibodies. She was treated with beta-blocker therapy and low molecular weight heparin during pregnancy, and delivered by cesarean section. The second patient presented protein S deficiency complicated by PVC and thrombocytopenia and delivered vaginally without complications. CONCLUSION: Many issues should be considered when counseling women with PVC, including the management before and during pregnancy according to symptoms and PVC complications. A multidisciplinary approach seems to be key to the management of delivery. Our advice to caregivers is that elective cesarean section seems necessary in cases with digestive varices. Vaginal delivery, with a passive second stage, seems to be relatively safe and less morbid in women without digestive varices, when maternal and fetal tolerance permits.
BACKGROUND: Portal vein cavernoma (PVC) is a rare disease resulting from extrahepatic portal vein thrombosis and development of collateral venous circulation. The management of pregnancy and delivery in woman with PVC has rarely been described. CASES: Two primiparous women are presented to illustrate the management of PVC during pregnancy and discuss the delivery route according to the symptoms and the PVC complications. The first patient presented PVC associated with large jejunal varices and high anticardiolipid antibodies. She was treated with beta-blocker therapy and low molecular weight heparin during pregnancy, and delivered by cesarean section. The second patient presented protein S deficiency complicated by PVC and thrombocytopenia and delivered vaginally without complications. CONCLUSION: Many issues should be considered when counseling women with PVC, including the management before and during pregnancy according to symptoms and PVC complications. A multidisciplinary approach seems to be key to the management of delivery. Our advice to caregivers is that elective cesarean section seems necessary in cases with digestive varices. Vaginal delivery, with a passive second stage, seems to be relatively safe and less morbid in women without digestive varices, when maternal and fetal tolerance permits.