Clinical Study Venous sinus stenting is a valuable treatment for fulminant idiopathic intracranial hypertension Benjamin D. Elder a,⇑ , C. Rory Goodwin a , Thomas A. Kosztowski a , Martin G. Radvany b , Philippe Gailloud b , Abhay Moghekar c , Prem S. Subramanian d , Neil R. Miller d , Daniele Rigamonti a a Department of Neurosurgery, The Johns Hopkins Hospital, Zayed Tower, 1800 Orleans Street, Baltimore, MD 21287, USA b Department of Radiology, The Johns Hopkins Hospital, Baltimore, MD, USA c Department of Neurology, The Johns Hopkins Hospital, Baltimore, MD, USA d Department of Ophthalmology, The Johns Hopkins Hospital, Baltimore, MD, USA article info Article history: Received 3 October 2014 Accepted 17 October 2014 Keywords: Hydrocephalus Idiopathic intracranial hypertension Pseudotumor cerebri Transverse sinus stenting Vision loss abstract Over the past 10 years, transverse sinus stenting has grown in popularity as a treatment for idiopathic intracranial hypertension. Although promising results have been demonstrated in several reported series, the vast majority of patients in these series have been treated on an elective basis rather than in the set- ting of fulminant disease with acute visual deterioration. We identified four patients who presented with severe acute vision loss between 2008 and 2012 who were treated with urgent transverse sinus stenting with temporary cerebrospinal fluid (CSF) diversion with lumbar puncture or lumbar drain as a bridge to therapy. All patients presented with headache, and this was stable or had improved at last follow-up. Three patients had improvement in some or all visual parameters following stenting, whereas one patient who presented with severe acute vision loss and optic disc pallor progressed to blindness despite suc- cessful stenting. We hypothesize that she presented too late in the course of the disease for improvement to occur. Although the management of fulminant idiopathic intracranial hypertension remains challeng- ing, we believe that transverse sinus stenting, in conjunction with temporary CSF diversion, represents a viable treatment option in the acute and appropriate setting. Published by Elsevier Ltd. 1. Introduction Pseudotumor cerebri (PTC), known as idiopathic intracranial hypertension (IIH) if no underlying cause is identified, is an uncom- mon disease characterized by the modified Dandy criteria, which include elevated intracranial pressure (ICP) (>25 cm H 2 O on lumbar puncture [LP] in lateral decubitus position in adults) with a normal cerebrospinal fluid (CSF) profile, and typically with papilledema [1]. Although the most common presenting symptom is generalized headache, the symptom with the highest morbidity is vision loss, believed to result from axoplasmic flow stasis with high optic nerve head pressures and eventual optic nerve ischemia, as reviewed in detail recently [2]. Other frequently reported symptoms and signs include pulsatile tinnitus, nausea or vomiting, and double vision, most often from unilateral or bilateral sixth nerve palsies [3]. The most common initial treatment of IIH is medical manage- ment with acetazolamide, furosemide, or topiramate; however, many patients either have trouble tolerating the side effects of these medications and their ICP remains poorly controlled despite this treatment. These patients often are treated with optic nerve sheath fenestration (ONSF) or permanent CSF diversion. Although ONSF is effective in many patients, it primarily helps to treat papilledema and often is ineffective in alleviating headaches [3]. Additionally, it is not without complications as up to a 40% complication rate has been reported following this procedure, including the risk of central retinal artery occlusion resulting in permanent, severe visual loss [4]. CSF diversion with lumbar or ventricular shunting is highly effective at treating both headaches and papilledema to prevent vision loss, but requires a more invasive surgical procedure. Also, shunting in PTC rarely requires only a single procedure, as the failure rate of ventriculoperitoneal (VP) shunts has been reported to be as high as 75% within 2 years of surgery, and the failure rate of lumboperitoneal shunts was reported at 86% within 18 months at our institution [5]. Given the problems associated with these proce- dures, other treatments are clearly needed. Transverse sinus stenting has grown in popularity as a treat- ment for PTC since its initial description over 10 years ago [6]. http://dx.doi.org/10.1016/j.jocn.2014.10.012 0967-5868/Published by Elsevier Ltd. ⇑ Corresponding author. E-mail address: belder4@jhmi.edu (B.D. Elder). Journal of Clinical Neuroscience 22 (2015) 685–689 Contents lists available at ScienceDirect Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn