title: von Hippel-Lindau disease and pregnancy: what an obstetrician should know.
abstract: 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.
  
  
  METHODS
  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.
authors.affiliation: Clinical Instructor, Hutzel Hospital/Wayne State University, Detroit, MI.
authors.forename: Henry
authors.initials: H
authors.lastname: Adekola
authors.affiliation: Professor, Division of Maternal and Fetal Medicine, Department of Obstetrics and Gynecology, Hutzel Hospital/Wayne State University, Detroit, MI.
authors.forename: Eleazar
authors.initials: E
authors.lastname: Soto
authors.affiliation: Clinical Instructor, Hutzel Hospital/Wayne State University, Detroit, MI.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Lam
authors.affiliation: Clinical Instructor, Hutzel Hospital/Wayne State University, Detroit, MI.
authors.forename: Elena
authors.initials: E
authors.lastname: Bronshtein
authors.affiliation: Professor, Division of Maternal and Fetal Medicine, Department of Obstetrics and Gynecology, Hutzel Hospital/Wayne State University, Detroit, MI.
authors.forename: Tinnakorn
authors.initials: T
authors.lastname: Chaiworapongsa
authors.affiliation: Professor, Division of Maternal and Fetal Medicine, Department of Obstetrics and Gynecology, Hutzel Hospital/Wayne State University, Detroit, MI.
authors.forename: Yoram
authors.initials: Y
authors.lastname: Sorokin
mesh_terms: D000328:Adult; D000773:Anesthesia, Obstetrical; D036861:Delivery, Obstetric; D005260:Female; D005817:Genetic Counseling; D006801:Humans; D018743:Perinatal Care; D011247:Pregnancy; D011248:Pregnancy Complications / Q000175:diagnosis / Q000235:genetics / Q000628:therapy*; D011295:Prenatal Care; D006623:von Hippel-Lindau Disease / Q000175:diagnosis / Q000235:genetics / Q000628:therapy*