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.
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.
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.
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Ahmed RM, Wilkinson M, Parker GD, Thurtell MJ, Macdonald J, McCluskey PJ, Allan R, Dunne V, Hanlon M, Owler BK, Halmagyi GM (2011) Transverse sinus stenting for idiopathic intracranial hypertension: a review of 52 patients and of model predictions. AJNR Am J Neuroradiol 32:1408–1414. https://doi.org/10.3174/ajnr.A2575
Albuquerque FC, Dashti SR, Hu YC, Newman CB, Teleb M, McDougall CG, Rekate HL (2011) Intracranial venous sinus stenting for benign intracranial hypertension: clinical indications, technique, and preliminary results. World Neurosurg 75:648–652; discussion 592-645. https://doi.org/10.1016/j.wneu.2010.11.012
Asif H, Craven CL, Siddiqui AH, Shah SN, Matloob SA, Thorne L, Robertson F, Watkins LD, Toma AK (2018) Idiopathic intracranial hypertension: 120-day clinical, radiological, and manometric outcomes after stent insertion into the dural venous sinus. J Neurosurg 129:723–731. https://doi.org/10.3171/2017.4.jns162871
Dashti SR, Nakaji P, Hu YC, Frei DF, Abla AA, Yao T, Fiorella D (2012) Styloidogenic jugular venous compression syndrome: diagnosis and treatment: case report. Neurosurgery 70:E795–E799. https://doi.org/10.1227/NEU.0b013e3182333859
Farb RI, Vanek I, Scott JN, Mikulis DJ, Willinsky RA, Tomlinson G, terBrugge KG (2003) Idiopathic intracranial hypertension: the prevalence and morphology of sinovenous stenosis. Neurology 60:1418–1424. https://doi.org/10.1212/01.wnl.0000066683.34093.e2
Friedman DI, Jacobson DM (2002) Diagnostic criteria for idiopathic intracranial hypertension. Neurology 59:1492–1495. https://doi.org/10.1212/01.wnl.0000029570.69134.1b
Gross BA, Albuquerque FC, Moon K, McDougall CG (2016) New frontiers in venous sinus stenting: illustrative cases. J Clin Neurosci 33:241–244. https://doi.org/10.1016/j.jocn.2016.05.032
Han K, Chao AC, Chang FC, Chung CP, Hsu HY, Sheng WY, Wu J, Hu HH (2015) Obstruction of venous drainage linked to transient global amnesia. PLoS One 10:e0132893. https://doi.org/10.1371/journal.pone.0132893
Higgins JN, Garnett MR, Pickard JD, Axon PR (2017) An evaluation of styloidectomy as an adjunct or alternative to jugular stenting in idiopathic intracranial hypertension and disturbances of cranial venous outflow. J Neurol Surg B Skull Base 78:158–163. https://doi.org/10.1055/s-0036-1594238
Johnston I, Kollar C, Dunkley S, Assaad N, Parker G (2002) Cranial venous outflow obstruction in the pseudotumour syndrome: incidence, nature and relevance. J Clin Neurosci 9:273–278. https://doi.org/10.1054/jocn.2001.0986
Li M, Gao X, Rajah GB, Liang J, Chen J, Yan F, Bao Y, Jiao L, Zhang H, Ding Y, Ji X, Meng R (2019) Styloidectomy and venous stenting for treatment of styloid-induced internal jugular vein stenosis: a case report and literature review. World Neurosurg 130:129–132. https://doi.org/10.1016/j.wneu.2019.06.100
Li M, Sun Y, Chan CC, Fan C, Ji X, Meng R (2019) Internal jugular vein stenosis associated with elongated styloid process: five case reports and literature review. BMC Neurol 19:112. https://doi.org/10.1186/s12883-019-1344-0
Rowe FJ, Sarkies NJ (1999) The relationship between obesity and idiopathic intracranial hypertension. Int J Obes Relat Metab Disord 23:54–59. https://doi.org/10.1038/sj.ijo.0800758
Saha MK, Hamieh T, Larkin B, McMillan W (2012) Cerebral hemorrhage due to internal jugular vein stenosis in a hemodialysis patient. Clin Exp Nephrol 16:345–349. https://doi.org/10.1007/s10157-011-0548-4
Zhao X, Cavallo C, Hlubek RJ, Mooney MA, Belykh E, Gandhi S, Moreira LB, Lei T, Albuquerque FC, Preul MC, Nakaji P (2019) Styloidogenic jugular venous compression syndrome: clinical features and case series. Oper Neurosurg (Hagerstown) 17:554–561. https://doi.org/10.1093/ons/opz012
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Mooney, J., Lepard, J., Akbari, S.H.A. et al. Styloidogenic jugular venous compression syndrome: a case report and review of the literature. Childs Nerv Syst 36, 3135–3139 (2020). https://doi.org/10.1007/s00381-020-04622-6
- Styloidogenic jugular venous compression syndrome
- Idiopathic intracranial hypertension
- Pseudotumor cerebri