Intracranial hypertension is rarely associated with peripheral hemodialysis shunts, presumably in association with central venous stenosis.1,2 Hemodialysis Reliable Outflow (HeRO) grafts (CryoLife, Inc., Kennesaw, GA) are designed to bypass preexisting
central venous stenosis by connecting the brachial artery with the venous circulation
through the ipsilateral internal jugular vein (IJV) (figure, C and D).3 We report a case of
intracranial hypertension immediately after placement of a HeR

Biousse, Valérie

Mackay, Devin D.

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Cases Neurology® Clinical PracticeHemodialysisgraft-induced intracranialhypertensionDevin D. Mackay, MDValérie Biousse, MDIntracranial hypertension is rarely associated with peripheral hemodialysis shunts, pre-sumably in association with central venous stenosis.1,2 Hemodialysis Reliable Outflow(HeRO) grafts (CryoLife, Inc., Kennesaw, GA) are designed to bypass preexistingcentral venous stenosis by connecting the brachial artery with the venous circulationthrough the ipsilateral internal jugular vein (IJV) (figure, C and D).3 We report a case ofintracranial hypertension immediately after placement of a HeRO graft, review similar casesin the medical literature, and discuss possible pathophysiology.Case reportA 60-year-old woman with a nonfunctioning right arm hemodialysis arteriovenous (AV) shuntdeveloped blurred vision, headaches, and optic disc edema 3 days after placement of a left HeROgraft. She had a history of proliferative diabetic retinopathy treated with laser and surgery with apoor visual outcome in the left eye and relatively good vision (20/50) in the right eye. She had ahistory of stent placement in the proximal right subclavian vein for central venous stenosis.At presentation, body mass index was 43.5 kg/m2 and blood pressure was 178/80 mm Hg.Blood urea nitrogen was 20 mg/dL. Visual acuity was unchanged from baseline: 20/50 in theright eye and count fingers at 1 foot in the left eye. Color vision was 5/14 color plates correctin the right eye, decreased from her baseline. There was a left relative afferent pupillarydefect related to her previous failed left eye surgeries. Extraocular movements were full.There was severe optic disc edema with peripapillary hemorrhages, suggesting papilledemafrom raised intracranial pressure in the right eye (figure, A). The left ocular fundus could notbe visualized secondary to previous surgeries. There was severe constriction of the right eyevisual field on Goldmann visual field testing, consistent with severe papilledema. MRI andmagnetic resonance venography of the head and neck demonstrated right optic disc eleva-tion, a partially empty sella, dilated optic nerve sheaths, and bilateral distal transverse sinusstenosis without venous sinus thrombosis, suggesting intracranial hypertension. A lumbarpuncture in the prone position under fluoroscopy demonstrated an opening pressure of38 cm of water with normal CSF contents. We suspected increased central venous pressure,presumably from superior vena cava syndrome related to impaired venous return in thechest. Venography was attempted, but the guide wire could not be passed into either IJV. Aright subclavian vein stent with 60%–70% midstent stenosis was found overlying the ostiumPracticalImplicationsPeripheral hemodialysis graftsmay precipitate intracranialhypertension, particularly inpatients with central venousstenosis, and should promptconsideration of central venousimaging. Patientsmay be treatedby graft ligation, venoplasty6stent placement, or CSFdiversion.Departments of Neurology, Ophthalmology, and Neurosurgery (DDM), Indiana University School of Medicine,Indiana University Neuroscience Center, Indianapolis, IN; and Departments of Ophthalmology and Neurology(VB), Emory University School of Medicine, Atlanta, GA.Funding information and disclosures are provided at the end of the article. Full disclosure form informationprovided by the authors is available with the full text of this article at Neurology.org/cp.Correspondence to: vbiouss@emory.edu494 © 2015 American Academy of Neurologyª 2015 American Academy of Neurology. Unauthorized reproduction of this article is prohibited.of the right IJV (figure, D). A CT venogram of the chest confirmed focal stenosis of a rightsubclavian vein stent overlying the ostium of the right IJV and a patent left-sided HeROgraft. A vascular surgery consultant recommended against HeRO graft removal. A ventricu-loperitoneal shunt was placed (opening pressure 46 cm of water), and 4 weeks after surgery,her visual function was improved in the right eye with dramatic improvement of the rightoptic disc edema (figure, B).DISCUSSIONA review of the English literature yielded at least 12 cases of presumed intracranial hyperten-sion from central venous stenosis and hemodialysis AV shunts, none involving a HeRO graft(table). All cases involved brachiocephalic vein stenosis or occlusion (10 of 12) or IJVocclusion (2 of 12). All were treated with shunt ligation with or without venoplasty andFigure Increased venous flow with central venous stenosis may be associated with intracranial hypertension(A) View of the right ocular fundus demonstrating optic disc edema with peripapillary hemorrhages. Dark scars from panretinal photocoagulation are visiblein the periphery of the retina. (B) Improved papilledema and peripapillary hemorrhages 4 weeks after a CSF shunting procedure. (C) Suggested treatmentalgorithm for patients with failing arteriovenous grafts (AVGs) or arteriovenous fistulas (AVFs) due to central venous stenosis, with the Hemodialysis Re-liable Outflow (HeRO) AVG demonstrating the bypassing of central venous stenosis to access the venous circulation at the internal jugular vein (IJV) (usedwith the permission of CryoLife, Inc.). (D) Radiograph of right subclavian vein stent stenosis overlying the ostium of the right IJV. Arrow indicates right sub-clavian vein stent stenosis. (E) Radiograph of the chest showing the path of the HeRO AVG from arterial anastamosis in the left brachial artery to the pointof venous access at the left IJV and extension to the superior cavoatrial junction. The top arrow indicates where graft enters left IJV, right arrow indicatesHeRO graft arterial access site, and left arrow indicates termination of graft with central venous access.Neurology: Clinical Practice ||| December 2015 Neurology.org/cp 495Hemodialysis graft-induced intracranial hypertensionª 2015 American Academy of Neurology. Unauthorized reproduction of this article is prohibited.1 of 12 was additionally treated with a lumboperitoneal shunt, with resolution of intra-cranial hypertension in 11 of 12 cases (complete resolution in 10 and partial in 1). Onecase progressed to superior sagittal sinus thrombosis, cerebral infarction, and death.2Intracranial hypertension is a rare complication of hemodialysis graft placement, and a re-cently proposed “2 hit” hypothesis suggests that both high venous flow and venous obstruc-tion are required risk factors, which presumably overwhelm intracranial venous outflowchannels leading to elevated intracranial venous pressure, increased resistance to CSF drain-age, and intracranial hypertension.1,2 The incidence of unsuspected central venous stenosis inpatients with functioning AV hemodialysis grafts is approximately 29%, but surprisingly onlya small number of cases of intracranial hypertension associated with central venous stenosisand an AV hemodialysis shunt have been described.2,5–8 Furthermore, intracranial hyperten-sion has not been reported in association with HeRO grafts, despite increasing venous flowspecifically in patients with central venous stenosis, suggesting the contribution of additionalfactors to the pathogenesis of hemodialysis graft-induced intracranial hypertension. Althoughusually treated by graft ligation or venoplasty with or without a stent, CSF shunting withoutgraft ligation is also a treatment option in patients with isolated intracranial hypertensionwhose venous anatomy is seriously compromised. Venous imaging of chest/neck veins inhemodialysis patients with unexplained intracranial hypertension may expedite diagnosis andappropriate treatment and should be considered in cases with previous AV shunts.REFERENCES1. Taban M, Taban M, Lee MS, Smith SD, Heyka R, Kosmorsky GS. Prevalence of optic nerve edema inpatients on peripheral hemodialysis. Ophthalmology 2007;114:1580–1583.Table Literature review of intracranial hypertension from central venous stenosis and hemodialysis shuntReference AV shunt type Central venous disease Treatment Outcome2 R arm AVF R BCV thrombosis AVF ligation Resolution of IH2 R arm Gor-tex AVgraftBilateral BCV thrombosis Graft ligation Partial resolution of IH4 L arm AVF L BCV stenosis AVF ligation Resolution of IH2 L arm AVF L BCV stenosis Venoplasty of L BCV Resolution of IH5 L arm AVF L BCV stenosis L AVF ligation, R AVF placed Resolution of IH2 R brachial artery toIJV graftR axillary, SCV, BCV, and IJVocclusionUnilateral optic nerve sheathfenestration, then graftocclusionResolution of IH2 L brachiobasilic AVF L SCV and bilateral BCVocclusion, calcified R SCVstent, SVC stenosisVenoplasty of SVC, followedby LP shunt, followed by AVFligationSuperior sagittal sinusthrombosis, brain infarction,and death6 L arm AVF L BCV occlusion Failed L BCV recannulation,AVF ligationResolution of IH6 AVF L BCV stenosis Venoplasty 1 stent in L BCV Resolution of IH, recurrence 7mo later, resolved with repeatvenoplasty7 R arm AV graft R BCV occlusion AV graft repair Resolution of IH2 Chronic R IJVcatheter, history offailed L arm AVFR SCV and BCV stenosis R BCV venoplasty, removalof right IJV catheterResolution of IH8 L arm AVF L BCV occlusion L BCV venoplasty and stent Resolution of IHAbbreviations: AV 5 arteriovenous; AVF 5 arteriovenous fistula; BCV 5 brachiocephalic vein; IH 5 intracranial hypertension; IJV 5internal jugular vein; LP 5 lumboperitoneal; SCV 5 subclavian vein; SVC 5 superior vena cava.496 © 2015 American Academy of NeurologyDevin D. Mackay and Valérie Biousseª 2015 American Academy of Neurology. Unauthorized reproduction of this article is prohibited.2. Simon MA, Duffis EJ, Curi MA, Turbin RE, Prestigiacomo CJ, Frohman LP. Papilledema due to apermanent catheter for renal dialysis and an arteriovenous fistula: a “two hit” hypothesis.J Neuroophthalmol 2014;34:29–33.3. Katzman HE, McLafferty RB, Ross JR, Glickman MH, Peden EK, Lawson JH. Initial experience andoutcome of a new hemodialysis access device for catheter-dependent patients. J Vasc Surg 2009;50:600–607.4. Hartmann A, Mast H, Stapf C, Koch HC, Marx P. Peripheral hemodialysis shunt with intracranialvenous congestion. Stroke 2001;32:2945–2946.5. Nishimoto H, Ogasawara K, Miura K, Ohmama S, Kashimura H, Ogawa A. Acute intracranial hy-pertension due to occlusion of the brachiocephalic vein in a patient undergoing hemodialysis. Cere-brovasc Dis 2005;20:207–208.6. Herzig DW, Stemer AB, Bell RS, Liu AH, Armonda RA, Bank WO. Neurological sequelae frombrachiocephalic vein stenosis. J Neurosurg 2013;118:1058–1062.7. Samaniego EA, Abrams KJ, Dabus G, Starr R, Linfante I. Severe venous congestive encephalopathysecondary to a dialysis arteriovenous graft. J Neurointerv Surg 2013;5:e37.8. Prasad V, Baghai S, Gandhi D, Moeslein F, Jindal G. Cerebral infarction due to Central vein occlusionin a hemodialysis patient. J Neuroimaging doi: 10.1111/jon.12152. Epub 2014 Jul 23.ACKNOWLEDGMENTFinancial support was provided in part by an unrestricted departmental grant (Department of Ophthal-mology, Emory University) from Research to Prevent Blindness, Inc., New York, and by the NIH/NEIcore grant P30-EY06360 (Department of Ophthalmology, Emory University).STUDY FUNDINGNo targeted funding reported.DISCLOSURESD.D. Mackay reports no disclosures. V. Biousse serves on the editorial boards of American Journal ofOphthalmology and Journal of Neuro-ophthalmology; is a consultant for GenSight (Paris, France); receivespublishing royalties for Walsh and Hoyt’s Clinical Neuro-Ophthalmology (Lippincott Williams &Wilkins, 2007), Neuro-Ophthalmology Illustrated (Thieme, 2009), and Up-to-Date; and receives re-search support from Research to Prevent Blindness. Full disclosure form information provided by theauthors is available with the full text of this article at Neurology.org/cp.Neurology: Clinical Practice ||| December 2015 Neurology.org/cp 497Hemodialysis graft-induced intracranial hypertensionª 2015 American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

Hemodialysis graft-induced intracranial hypertension

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Devin D. Mackay

Valérie Biousse

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Neurology Clinical Practice

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Hemodialysis graft-induced intracranial hypertension

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