Background: 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.
Case: 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.
Conclusions: 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.