In duplicated renal systems, it is the lower pole that is typically obstructed at the UPJ. Bilateral UPJ obstruction has been commonly reported, while bilateral upper pole UPJ has not been specifically reported in the literature. A case is presented with a discussion as to the therapeutic options and clinical management. A 16-year-old Caucasian girl presented with intermittent bilateral back pain aggravated by activity. She had no clinically significant medical or surgical

history. A bone scan demonstrated delayed excretion and retention of radioisotope in the upper poles of both kidneys suggesting renal obstruction AUY-922 concentration (Fig. 1A,B). Ultrasonography revealed bilateral symmetric upper pole hydronephrosis (Fig. 2). Magnetic resonance urogram (Fig. 3) and mercaptoacetyltriglycine diuretic renogram (Fig. 4) revealed bilateral complete duplication and bilateral upper pole ureteropelvic junction (UPJ) obstructions. The lower poles appeared normal. Surgical repair was recommended, and the patient underwent bilateral robotically assisted upper pole pyeloplasties using a Y-to-V advancement repair with upper pole double-J ureteral stent placement. Postoperatively, the right ureteral stent became obstructed, requiring replacement on postoperative day 3 because of urinary ascites and pain. She did well and was discharged on postoperative day 8 on prophylactic antibiotics. The stents were removed

6 weeks postoperatively. The patient showed complete TSA HDAC research buy resolution of her symptoms despite vigorous activity. She suffered 2 minor episodes of cystitis, which resolved with treatment. Follow-up imaging showed persisting hydronephrosis, which appeared improved with more parenchyma visible between the calyces (Fig. 5). The family has deferred obtaining subsequent mercaptoacetyltriglycine scan because of her clinical improvement. At the most recent follow-up 3 years postoperatively, she is attending college and is asymptomatic. Unilateral upper pole UPJ obstruction is extremely rare1, 2, 3, 4, 5, 6 and 7; bilateral upper pole UPJ obstruction has not been reported to date. Common presentation is with flank pain,2 and 8 as well as infection, and through

prenatal detection of hydronephrosis.4 Vascular occlusion is considered a common cause, although the specific details ADAMTS5 are not well defined in the literature.1 and 2 This may have some similarity to the so-called Fraley syndrome of vascular upper infundibular obstruction.9 This patient’s diagnosis was delayed because of confusion with musculoskeletal pain in the absence of lateralizing symptoms. Modern imaging can adequately define the anatomy, but optimal treatment is not well defined. Bilateral upper pole partial nephrectomies could be a viable option. However, renal preservation seemed to be a worthwhile goal. The renal pelvises were not markedly dilated making an upper-to-lower pyelopyelostomy less likely to be feasible.