Styloidogenic jugular venous compression syndrome: a case report and review of the literature
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CASE REPORT
Styloidogenic jugular venous compression syndrome: a case report and review of the literature James Mooney 1 & Jacob Lepard 1 & S. Hassan A. Akbari 1 & James M. Johnston 1 Received: 16 January 2020 / Accepted: 13 April 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Background Styloidogenic jugular venous compression syndrome (SJVCS) has been shown to present with a similar symptomatology to idiopathic intracranial hypertension (IIH) and is caused by compression of the internal jugular vein (IJV) between the lateral tubercle of C1 and the styloid process. Treatments including venous stenting and styloidectomy have been reported with good outcomes; however, treatment of a pediatric patient with SJVCS with styloidectomy has not previously been reported in the literature. Case Report A 12-year-old male presented with refractory positional headaches, nausea, and vomiting, and after, workup including lumbar puncture (LP) and intracranial pressure (ICP) monitoring was found to have intracranial hypertension associated with contralateral neck turning. Computed tomography venogram (CTV) revealed severe bilateral compression of the IJV’s between the styloid processes and C1 tubercle. The patient was successfully treated with unilateral right-sided styloidectomy with symptomatic relief. Conclusions This is the first reported pediatric case of SJVCS treated successfully with styloidectomy to our knowledge and adds to the limited literature that styloidectomy is a durable treatment option for SJVCS. Keywords Styloidogenic jugular venous compression syndrome . Idiopathic intracranial hypertension . Pseudotumor cerebri
Introduction Idiopathic intracranial hypertension (IIH) is a neurologic condition characterized by increased intracranial pressure without a detectable cause [6]. IIH typically occurs in obese women and is associated with headaches and visual changes [13]; although occasionally, the condition has been associated with venous sinus stenosis, most commonly of the transverse sinus [5, 10]. Venous sinus stenting for the treatment of IIH associated with venous outflow obstruction has been previously reported [1–3, 7]. Rarely, an elongated styloid process has been shown to cause subcranial compression of the internal jugular vein (IJV) between the lateral tubercle of C1 and the styloid process, a condition termed styloidogenic jugular venous compression syndrome (SJVCS), resulting in the same symptoms as IIH [4]. Eagle syndrome (ES), characterized by * James Mooney [email protected] 1
Department of Neurosurgery, University of Alabama at Birmingham, 1802 6th Avenue South, FOT 1001, Birmingham, AL 35233, USA
symptoms associated with an elongated styloid process, has been classified into several subtypes [8, 14], and authors have proposed categorizing SJVCS as a subtype of ES [12]. We report the first pediatric case of SJVCS treated successfully with styloidectomy to our knowledge.
Case presentation A 12-year-old male presented with 5 days of positional headaches with nausea,
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