Postpartum cerebellar herniation in von Hippel-Lindau syndrome

PURPOSE: To describe exacerbation during pregnancy of cerebellar hemangiobl astoma in von Hippel-Lindau syndrome. METHOD: Case-report. A 21-year-old woman with von Hippel-Lindau syndrome wa s found on routine ocular examination to have severe papilledema 1 week aft er giving birth. RESULTS: Immediate magnetic resonance imaging disclosed a large cerebellar cyst from hemangioblastoma causing cerebellar tonsillar herniation. Immedia te neurosurgical intervention was life saving. CONCLUSION: Worsening of intracranial hemangioblastoma during pregnancy in cases of von Hippel-Lindau syndrome should be realized and periodic neurolo gic and ophthalmologic observation is warranted. (Am J Ophthalmol 1999;128: 387-389. (C) 1999 by Elsevier Science Inc. All rights reserved.).