von Hippel-Lindau disease is an autosomal dominant genetic disorder that presents with a variable constellation of symptoms and signs due to formation of hemangioblastomas and cysts. We address the management of pregnancies associated with this disorder from an obstetric perspective.
A 26-year-old known to have type 2A von Hippel-Lindau disease with a significant surgical history secondary to disease manifestations was managed from early pregnancy to delivery. A literature review to determine the best evidence-based care from an obstetric perspective, addressing patient management preconceptionally, in the antepartum period (including antepartum surgical care), during labor and delivery (including type of analgesia/anesthesia, mode of delivery), and the postpartum period, as well as contraception, was performed.
A pregnant woman with von Hippel-Lindau disease should be managed in conjunction with a multidisciplinary team. Routine clinical and radiological imaging should be continued within safe parameters of pregnancy. Mode of delivery, analgesia/anesthesia, and postpartum care (including birth control) should be determined based on each patient’s disease phenotype and individual risk-to-benefit ratio on a case-by-case basis.
Obstetricians and gynecologists, family physicians
After completing this CME activity, physicians should be better able to identify the clinical manifestation, the genetic manifestation, and the clinical diagnosis of von Hippel-Lindau disease and monitor von-Hippel-Lindau disease in pregnancy and care for the pregnant woman with von Hippel-Lindau disease as it pertains to the preconceptional, antepartum, intrapartum, and postpartum periods (including contraception).