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Fraley Syndrome: A Case ReportClinical History: A 35 year old woman with history of nephrolithiasis presented with worsening right flank pain, nausea and vomiting for 3 days. She had passed a stone 4 days prior. She denied fevers or chills. Upon initial exam, vital signs were stable. Her exam was significant for costovertebral angle tenderness on the right side. Hematology revealed a white count of 9.6 thousand. Her chemistry was within normal limits with a creatinine of 0.8. An initial UA revealed a specific gravity of 1.001 and was otherwise normal. A CT scan of her abdomen revealed moderate to severe hydronephrosis of her right kidney with no evidence of stones in kidney or collecting system (Figure 1). Crossing vessels at the ureteropelvic junction were seen (Figure 2,3). Findings: Figure 1: CT cross sectional image shows marked hydronephrosis of right kidney. Figure 2: CT image in the coronal plane demonstrates dilatation of upper renal calyces. Figure 3: CT cross sectional image demonstrates faint linear hyperdensity at level of obstruction corresponding to crossing vessels at the superior infundibulum. Diagnosis: Fraley Syndrome Discussion: Fraley syndrome was initially described in 1966 where extrinsic compression of the renal calyces by aberrant renal artery configurations caused symptoms of recurrent flank pain and renal obstruction in four cases. In all these cases, the upper segment of the renal artery caused the obstruction, either by compressing against another arterial branch or in one case, a vein. The mechanism by which this obstruction evolves is theorized to be due to ptotic positioning of the kidney within the pelvis, which rotates the kidney anteriorly and may worsen the degree of compression [1]. Fraley syndrome can be demonstrated on various radiographical studies. The resultant hydronephrosis is best seen on CT and ultrasound. Initial tests for demonstrating the renovascular lesion include intravenous and retrograde pyelography, which result in a filling defect in the infundibulum and caliectasis in the obstructed calyx [1]. The use of intravenous pyelography and angiography for diagnosis of this condition has given way to newer modalities such as CT angiography and MR angiography [2]. CT angiography is particularly useful as it is less invasive than intravascular imaging and provides accurate information about vessels as small as 1mm in diameter [2]. MR angiography is also a useful tool as it can accurately depict the lesion without the use of contrast medium or radiation, the former important due to the occurrence of renal disease in these patients. Zuckier et al described that renal scintiscanning using labeled hippuran and DTPA augmented by diuretic administration is an excellent functional exam for follow up due to its low radiation dose [3]. Pre-operative planning of endovascular repair is best accomplished using helical CT angiography with 3D reconstructions [4]. Treatment approaches range from open surgical to minimally invasive and are guided by the degree of disease. Open surgical approaches were first described by Fraley himself in 1966 and included variations on transection of the infundibulum at the level of the lesion with reanastomosis either ventral or posterior to the vessels. Partial nephrectomy is also an option, reserved for patients with difficult anatomy, nephrolithiasis, or repeated infections [3]. Endopyelotomy is minimally invasive option that can also treat this lesion although various reports put the success rate between 33-96% if there are crossing vessels at the uretropelvic junction[4]. In summary, we present a case of Fraley syndrome in a young women who presented with recurrent flank pain and had pronounced hydronephrosis on abdominal CT scan. References / Suggested Reading:
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Special thanks to Patricia Miller for securing documents in preparation of this case.
Sarel Gaur
University of Rochester School of Medicine 2011