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Pregnancy complicated by von Hippel-Lindau disease
K K Ogasawara1, E M Ogasawara, G Hirata
1Department of Obstetrics and Gynecology, University of Hawaii School of Medicine, Honolulu, USA.
Background:
Von Hippel-Lindau disease is an autosomal-dominant genetic disorder with variable penetrance characterized by multiorgan hemangioblastomas and a predisposition to carcinoma.
Case:
A 23-year-old pregnant woman at 35 weeks' gestation, with a family history of von Hippel-Lindau disease, presented with paraplegia caused by an acute intramedullary hemorrhage from a spinal hemangioma at the thoracic (T) 4-5 level. An unruptured hemangioblastoma was noted at the T7-8 level. A T3-6 laminectomy resulted in the improvement of symptoms. The postoperative period was complicated by autonomic dysreflexia and preterm labor. The woman was delivered by cesarean under epidural anesthesia.
Conclusion:
Pregnant patients with von Hippel-Lindau disease present problems related to hemangioblastomas of the central nervous system. Imaging studies of the central nervous system are mandatory for prompt recognition and treatment of complications related to intramedullary hemorrhage. If spinal hemangioblastomas are identified, cesarean delivery may be the most sensible choice.