Fig. 1. Gadolinium-enhanced breath-hold MRA shows focal stenosis of the mid right renal artery (arrow) as well as early filling of a renal vein, suggesting AV fistula (arrowhead).

 

Fig. 2. Digital subtraction abdominal aortogram confirms the findings of the MRA.

 

Case Reference No. CC-0298-01

A 40-year-old woman presented to an ophthalmologist with episodic headaches. She was noted to have papilledema and severe hypertension (which was sustained on serial measurements). Biochemical screening for pheochromocytoma was undertaken; although there was elevation of serum VMA and metanephrines as well as urinary norepinephrine, the elevations were mild and it was thought they might be due to stress. Doppler screening of the renal arteries suggested right renal artery stenosis. MRA of the renal arteries confirmed a mid right renal artery stenosis and revealed the unexpected suggestion of an intrarenal arteriovenous fistula (AVF) (Fig. 1). Aortography performed prior to anticipated therapy confirmed the renal artery stenosis (believed most likely the result of fibromuscular dysplasia) and the fistula (Fig. 2); it also showed an enhancing left paraaortic mass which, in retrospect, was present on the MRA (Fig. 3). Percutaneous intervention was deferred pending work-up of the paraaortic mass which was felt most likely to represent a pheochromocytoma.

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Case Reference No. CC-0298-01

Update 2/23/98

 

 

 

Fig. 3. Late arterial phase of the aortogram shows an enhancing left paraaortic mass (arrow). In retrospect, this is present (overlapping the lower pole of the kidney) on the MRA and was seen on oblique views.

Questions

1) Has anyone encountered renal AVF in association with fibromuscular dysplasia? There is literature which suggests an association (there is also literature which describes association of pheochromocytoma and fibromuscular dysplasia, although it postulates only a coincidental association). Has anyone treated this combination?

2) The patient has three lesions which are potential sources for her hypertension. In what order and manner should these be managed?