Robot-Assisted Laparoscopic Calyceo-Pyelostomy for Vascular Compression of the Upper Calyx (Fraley Syndrome)

Fraley's Syndrome is a rare anatomic vascular malformation described in 1966 where an aberrant crossing vessel compresses the upper infundibulum and leads to upper calyx massive dilation. It is mostly asymptomatic and the diagnosis often missed; however, surgery is usually required for symptomatic cases. Open surgery is still frequently used while minimally invasive management is anecdotal in the literature. We report the first robot-assisted calyceo-pyelostomy. An 18-year-old female patient presented with intermittent, recurring, severe left flank pain leading to insomnia, evolving in the last 3 months. A 3-phases computerized tomography scan (CT-scan) showed a rotated left kidney, with upper calyx massive dilation and thinning of the upper renal parenchyma without any evident obstacle. Cystocopy and retrograde pyelography were performed. It confirmed a single ureteral meatus, a single ureter, and a narrowed upper calyx with upper calyx dilation. An ureteral catheter (JJ stent) was inserted in the upper calyx and the patient was planned for surgical exploration. During robotic surgery, an abnormal crossing vessel was identified and the diagnosis of Fraley's syndrome was made intraoperatively. According to previous literature, a calyceo-pyelostomy with uncrossing of the upper major calyx and resection of the narrowed upper infundibulum was performed. Total operative time was 114 min, pneumoperitoneum time was 96 min, blood loss less than 10 ml, and patient was released on post-operative day 2. The ureteral stent was removed 4 weeks after the intervention. At 12 postoperative weeks, the patient had complete regression of pain and the 12 postoperative week CT-scan showed a reduction of the dilation and a patent anastomosis. Fraley's syndrome is a rare entity. In our case, the diagnosis of Fraley syndrome was made intraoperatively after an extensive previous workup, underlining the difficulty to make this diagnosis. We report and provide a video of the first robot-assisted procedure for Fraley's syndrome in a nephron-sparing technique. Patient was pain-free at the 3-month.