Neuropediatrics 2009; 40(4): 201-204
DOI: 10.1055/s-0029-1243164
Short Communication

© Georg Thieme Verlag KG Stuttgart · New York

Postural Headache in Marfan Syndrome Associated with Spinal Cysts and Liquor Hypotension

N. C. Voermans1 , K. G. J. van Dijk2 , M. M. Bos3 , L.-F. de Geus-Oei4 , A. Verrips5 , E. J. van Lindert6
  • 1Department of Neurology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
  • 2Department of Pediatrics, Rijnstate Hospital, Arnhem, The Netherlands
  • 3Department of Neurology, Elkerliek Hospital, Helmond, The Netherlands
  • 4Department of Nuclear Medicine, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
  • 5Department of Pediatric Neurology, Canisius Wilhelmina Hospital, Nijmegen, The Netherlands
  • 6Department of Neurosurgery, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
Further Information

Publication History

received 19.08.2009

accepted 01.10.2009

Publication Date:
04 February 2010 (online)


We here report a 13-year-old Marfan patient who suffered from severe, medication-resistant, intermittent headache, which was provoked when getting into an upright position and immediately relieved by lying down or after intravenous rehydration. The postural benefit and the sudden relief after intravenous hydration suggested (intermittent) intracranial hypotension, although a normal opening pressure on lumbar punction was observed and no cerebrospinal fluid (CSF) leakage was identified. Imaging studies revealed severe dural ectasia at lumbosacral level, and two intradural cysts and two extradural presacral cysts were detected. Most likely, altered hydrodynamics in intra- and extracranial spinal meningeal cysts caused intermittent CSF hypotension above these cysts, resulting in intermittent intracranial hypotension. Surgical marsupialisation of the intradural cysts proved to be effective. This resulted in a significant reduction of the headache during the clinical follow-up of eight years.



Nicol C. Voermans, MD 

Department of Neurology

Radboud University

Nijmegen Medical Center

P.O. Box 9101

6500 HB Nijmegen

The Netherlands

Phone: +31/24/361 3396

Fax: +31/24/354 1122