Papilledema Due to a Permanent Catheter for Renal Dialysis and an Arteriovenous Fistula: a “Two Hit” Hypothesis

Original Contribution

Papilledema Due to a Permanent Catheter for Renal Dialysis and an Arteriovenous Fistula: A “Two Hit” Hypothesis

Melissa A. Simon, MD, Ennis J. Dufﬁs, MD, Michael A. Curi, MD, Roger E. Turbin, MD, Charles J. Prestigiacomo, MD, Larry P. Frohman, MD

Abstract: Elevated intracranial pressure in patients with included sevelamer carbonate, benzonatate, enalapril, chronic renal failure has several potential causes. Its rare fexofenadine, isosorbide, hydralazine, amlodipine, nifedi- occurrence secondary to the hemodynamic effects of pine, and phenytoin. hemodialysis is described and the findings support a multifactorial etiology (“two hits”). A failed AVF placed in the left arm required ligation and led to placement of a permanent catheter in the right IJV Journal of Neuro-Ophthalmology 2014;34:29–33 and a new AVF was created in his right arm. Because the doi: 10.1097/WNO.0000000000000063 © 2013 by North American Neuro-Ophthalmology Society patient developed a steal syndrome affecting his right hand, the distal radial artery was treated with coil embolization. The right AVF continued to mature and the patient continued to rely on the central catheter for dialysis. variety of factors may lead to elevated intracranial pres- Three weeks later, visual acuity was 20/50 in each eye, A sure (ICP) in patients with chronic renal failure. Our with normal pupillary reactions, color vision, and ocular patient had a permanent central catheter for hemodialysis motility. Bilateral cataracts were present on slit-lamp and, after multiple procedures for dialysis access, presented examination. Confrontation visual fields were intact, and with headaches and bilateral papilledema caused by elevated fundoscopy revealed bilateral optic disc edema. Neurolog- ICP. We propose a multifactorial etiology to explain our ical examination was normal. patient’s clinical findings. Magnetic resonance imaging (MRI) studies could not be performed because of the patient’s pacemaker. Computed tomography (CT) of the brain without contrast (due to renal CASE REPORT failure) showed only microvascular ischemic changes and mild pansinusitis. Opening pressure on lumbar puncture A 65-year-old man complained of headaches, tinnitus, and was 30 cm H O with mild elevation of cerebrospinal fluid slowly progressive blurring of vision for several months. 2 (CSF) protein of 51 mg/dL (normal, 15–45 mg/dL). CSF He had end-stage renal disease and had undergone glucose and cell count were normal. Because of his renal multiple angioplasties and revisions of arteriovenous failure, the patient could not be treated with acetazolamide. fistulas (AVF) for dialysis. At the time of presentation, Three days after the lumbar puncture, acuity was 20/30 he had a permanent dialysis catheter in the right internal bilaterally. Automated visual fields revealed enlargement of jugular vein (IJV). The patient was obese, with a history of the blind spots and there was bilateral optic disc edema on hypertension, hypercholesterolemia, and seizures and had funduscopy (Fig. 1). a pacemaker for episodes of bradycardia. Medications An intracranial venogram demonstrated elevated intra- Departments of Ophthalmology and Visual Science (MAS, RET, LPF), cranial venous pressures throughout the cerebral venous Neurosurgery (EJD), Vascular Surgery (MAC), Neurology and system with measurements ranging from 40 to 50 mm Hg. Neurosciences (LPF), Rutgers-New Jersey Medical School, Newark, New Jersey. Although the venous system was patent, there was reduced fi The authors report no conflicts of interest. opaci cation of the transverse sinuses and the IJVs. In Address correspondence to Melissa A. Simon, MD, 90 Bergen Street, addition, the transit times from the cerebral arterial through Suite 6100, Newark, NJ 07103; E-mail: [email protected] venous phases was increased.

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FIG. 1. Bilateral papilledema is present.

Six days later, a fistulogram of the right dialysis shunt Over the following year, the patient experienced recur- showed a patent AVF of the right forearm. A central rent subclavian vein stenosis with right arm swelling. venogram demonstrated a patent right subclavian vein and However, he had no recurrence of headaches or visual superior vena cava, the permanent catheter in the right IJV, complaints. and high-grade stenosis of the proximal right subclavian vein extending into the brachiocephalic vein (Fig. 2). Angioplasty DISCUSSION of the right subclavian vein was performed and the right IJV catheter was removed. A repeat venogram immediately after There are 5 reported cases of patients with increased ICP these procedures demonstrated improved flow from the sub- linked to dialysis catheters or grafts, all with a combination clavian vein into the superior vena cava (Fig. 3). of increased flow from the AVF and obstruction from The next day, the patient reported that his headaches venous stenosis or thrombosis (1–5). In 3 patients, papil- had resolved, and 5 months later, visual acuity was 20/25 ledema resolved after ligation of the graft, 1 after balloon bilaterally, with normal visual fields and fundi. The patient dilation to improve stenosis, and 1 patient’s symptoms did noted resolution of headaches and tinnitus. not resolve before her illness progressed to brain infarction

FIG. 2. Venogram with high-grade stenosis of the right subclavian vein extending into the brachiocephalic vein.

30 Simon et al: J Neuro-Ophthalmol 2014; 34: 29-33

FIG. 3. Venogram after removal of catheter from the right internal jugular vein with improved ﬂow from the right subclavian vein into the superior vena cava.

and death (Table 1). All cases, including ours, are unified by because he had no alternative pathways for drainage; he similar etiologies: high venous flow in conjunction with had a chronic left innominate vein occlusion because of venous obstruction. Retrograde venous flow from AVFs the pacemaker wire restricting outflow from the left side of into the ipsilateral IJV led to impaired cerebral venous the head and a fistula on the right with the subclavian drainage. In addition, compromised venous outflow by an stenosis. The result was high pressure in the right subcla- obstructed central vessel caused increased flow elsewhere in vian vein and restricted drainage of the external jugular the cerebral venous network. Both factors led to elevated vein, which could have provided an alternative drainage ICP. pathway. No vascular collaterals were apparent on his Our patient had both an AVF-causing high flow and fistulogram. a central venous obstruction caused by ipsilateral permanent Dialysis catheters have been associated with venous catheter and extended pacemaker wire. Our case is unique, stenosis. Surratt et al (6) evaluated 43 patients for new in that the permanent catheter was still in place at the onset fistula placement. In preoperative evaluation, 17 patients of elevated ICP. Because the symptoms improved dramat- with previous or existing temporary dialysis catheters in ically after removal of the catheter and the AVF remained in the subclavian vein had moderate or severe subclavian vein place, the increased ICP cannot be explained solely by the stenosis. No stenoses were found in patients without a his- AVF. Rather, the central catheter caused decreased venous tory of dialysis catheters in the subclavian vein. Wilkin et al return from the intracranial venous system, being partially (7) used ultrasound to evaluate the IJV of 143 patients with obstructive in the IJV, the brachiocephalic vein, and a history of dialysis catheter placement and found right IJV superior vena cava. Increased venous flow from the thrombosis in 25.9% of patients and 62% of these were arteriovenous graft combined with outflow obstruction occluded. Neither study assessed visual symptoms or head- resulted in elevated intracranial venous pressure. ache related to stenosis or occlusion. Given the lack of reflux of dye into the jugular system The question remains whether more aggressive screening after catheter removal (Fig. 3), we do not think that the measures for papilledema should be instituted for patients fistula alone or the right subclavian stenosis alone caused with hemodialysis catheters or AVFs to detect possible our patient’s symptoms. Additionally, our patient devel- elevated ICP. A cross-sectional observational case series of oped right arm swelling and subclavian vein stenosis in the 44 patients with peripheral arteriovenous shunts found that same location over a year after catheter removal, requiring none had optic disc edema or symptoms of elevated ICP repeat angioplasty. It is interesting that this did not lead to (8). This low prevalence supports our theory that “two-hits” headache and papilledema. This suggests that the catheter are required in these patients if they are to develop elevated was restricting outflow from the right IJV, either directly ICP: 1) the presence of a patent arteriovenous graft poten- or by increasing IJV pressure with compromised superior tially increasing venous pressures and/or flow and 2) vena cava outflow. Our patient’s condition was unusual a thrombotic occlusion, as in the other reported cases, or

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TABLE 1. Cases of increased intracranial pressure linked to patient with dialysis catheters or grafts Report Summary of Findings Proposed Mechanism of Increased ICP Intervention and Outcome

Lal et al (1) Patient with right AVF, right BCV thrombosis Retrograde blood flow in the cerebral venous Arteriovenous graft ligation with resolution of after repeat subclavian vein system elevated ICP catheterizations, developed papilledema Molina et al (2) Patient with both right and left BCV thrombosis Intracranial venous outflow obstruction and Graft was ligated with only partial resolution of after repeat subclavian and IJV retrograde flow elevated ICP catheterizations developed ICP with bilateral papilledema after placement of right forearm Gor-tex graft Chang et al (5) Patient with left HD shunt and left BCV Retrograde flow in left IJV and cerebral venous Balloon dilation of left BCV, with improvement stenosis developed right optic disc edema, system and outflow obstruction of stenosis and retrograde flow; resolution of left-sided headache, left face and neck symptoms and optic disc edema swelling Cuadra et al (3) Patient with right brachial to IJV graft remaining Cerebral venous hypertension attributed to Permanent graft occlusion with resolution of after successful renal transplant and combination of internal jugular occlusion cerebral venous hypertension and occlusion of the right axillary, subclavian, causing retrograde flow into cerebral and papilledema and IJVs with bilateral papilledema jugular circulation and functioning hemoaccess graft causing increased blow fl io tal: et Simon ow Cleper et al (4) Patient with failed kidney transplants, Retrograde flow caused by high flow in left AVF Balloon dilation of SVC without resolution. occlusion of the left subclavian and left and contributed to impaired cerebral venous Lumboperitoneal shunt with resolution of right BCVs, calcified right subclavian stent, outflow and elevated ICP elevated ICP and disc edema. Patient then

er-ptaml2014 Neuro-Ophthalmol J and stenosed SVC had left brachiobasilic developed recurrent elevated intracranial AVF placed and developed facial edema, pressure and superior sagittal sinus bilateral optic disc edema, and elevated thrombosis and brain infarction. The AVF opening pressure on lumbar puncture was closed, but patient ultimately expired.

AVF, arteriovenous ﬁstula; BCV, brachiocephalic vein; ICP, intracranial pressure; IJV, internal jugular vein; HD, hemodialysis; SVC, superior vena cava. 4 29-33 34: ;

occlusion from a permanent central catheter as a mechanism 4. Cleper R, Goldenberg-Cohen N, Kornreich L, Krause I, Davidovits M. Neurologic and ophthalmologic complications of for compromising venous drainage from the jugular system. vascular access in a hemodialysis patient. Pediatr Nephrol. 2007;22:1377–1382. 5. Chang S, Masaryk TJ, Lee MS. Optic nerve edema: complication REFERENCES of peripheral hemodialysis shunt. Semin Ophthalmol. 1. Lal SM, Twardowski ZJ, Van Stone J, Keniston D, Scott WJ, 2004;19:88–90. Berg G, Kirt Nichols W. Benign intracranial hypertension: 6. Surratt RS, Picus D, Hicks ME, Darcy MD, Kleinhoffer M, a complication of subclavian vein catheterization and Jendrisak M. The importance of preoperative evaluation of the arteriovenous ﬁstula. Am J Kidney Dis. 1986;8:262–264. subclavian vein in dialysis access planning. AJR. 2. Molina JC, Martinez-Vea A, Riu S, Callizo J, Barbod A, Garcia C, 1991;156:623–625. Peralta C, Oliver JA. Pseudotumor cerebri: an unusual 7. Wilkin TD, Kraus MA, Lane KA, Trerotola SO. Internal jugular complication of brachiocephalic vein thrombosis associated vein thrombosis associated with hemodialysis catheters. with hemodialysis catheters. Am J Kidney Dis. 1998;31:E3. Radiology. 2003;228:697–700. 3. Cauda SA, Padberg FT, Turbin RE, Farkas J, Frohman LP. 8. Taban M, Taban M, Lee MS, Smith SD, Heyka R, Kosmorsky GS. Cerebral venous hypertension and blindness: a reversible Prevalence of optic nerve edema in patients on peripheral complication. J Vasc Surg. 2005;42:792–795. hemodialysis. Ophthalmology. 2007;114:1580–1583.

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