Bilateral ureteral obstruction after open ureteral reimplantation in a 3-year-old patient with Williams Beuren syndrome.

Vesicoureteral reflux (VUR) is a common urological problem in the pediatric population and can be corrected by ureteral reimplantation in severe or persistent cases. This procedure is generally well tolerated, although complications, including ureteral obstruction, may occur in the postoperative period. We present a rare case of a 3-year-old with Williams Beuren syndrome who underwent bilateral ureteral reimplantation for VUR and subsequently developed bilateral ureteral obstruction with acute renal failure requiring nephrostomy tube placement within 48 hours of surgery.

Pediatric genitourinary tumors.

Each year advances are made in the clinical evaluation and treatment of genitourinary tumors in children. Understanding of cellular, molecular, and genetic processes in tumorigenesis is evolving rapidly. In addition, information is accumulating about the long-term outcome and complications associated with treatment modalities. This article reviews the 2001 literature on pediatric Wilms tumor, other renal tumors, rhabdomyosarcoma of the pelvis, paratesticular rhabdomyosarcoma, and testicular tumors. The emphasis in molecular biology is to identify molecular or genetic markers that predict outcome. The National Wilms' Tumor Study Group reported on the complications of surgery and the decreased complication rate when these procedures are performed by pediatric surgical specialists. Long-term complications of treatment are also discussed, including short stature and leukemia. The Intergroup Rhabdomyosarcoma Study Group reported on the results of treatment for nonmetastatic disease and the goals of the upcoming Study V, reduction of chemotherapy and radiotherapy. They also presented data from Study IV and discussed the findings on improved prognosis.

Lower urinary tract reconstruction for duplicated renal units with ureterocele. Is excision of the ureterocele with reconstruction of the bladder base necessary?

PURPOSE: Current practice in reconstruction of the lower urinary tract for duplicated renal systems with an associated ureterocele is excision of the ureterocele with reconstruction of the bladder and a common sheath ureteroneocystostomy. For a nonfunctioning upper pole treatment is partial nephroureterectomy. We postulate that lower urinary tract reconstruction can be performed successfully through an extravesical approach without excision of the ureterocele or reconstruction of the bladder base. We present our experience with that approach. MATERIALS AND METHODS: Between 1996 and 2001, 60 patients presented with the diagnosis of ureterocele and obstruction of the upper pole ureter. Partial nephrectomy was performed in 12 cases of which 4 had reflux to the lower pole moiety. Upper pole only dismembered ureteroneocystostomy was performed in 7 of 15 cases reconstructed using the extravesical approach. RESULTS: Average postoperative stay was 3.7 days. The Foley catheter was removed within 24 to 48 hours. Postoperative ultrasound showed decompression of the obstructed system and the ureterocele. Reflux was corrected in all patients. Flow rate with measurement of post-void residual 6 weeks postoperatively in toilet trained children showed complete bladder emptying. CONCLUSIONS: Lower urinary tract reconstruction for duplicated renal systems with obstruction of the upper pole can be accomplished safely with decreased morbidity through the extravesical approach without excision of the ureterocele or reconstruction of the bladder base. Moreover, in instances when there is no reflux to the lower pole moiety, upper pole only extravesical ureteroneocystostomy can be performed.

Efficacy of continuous epidural analgesia versus single dose caudal analgesia in children after intravesical ureteroneocystostomy.

PURPOSE: We compared the effects of single dose caudal injection and continuous epidural infusion of bupivacaine on postoperative pain intensity and supplemental opioid analgesic requirements in children undergoing intravesical ureteroneocystostomy. MATERIALS AND METHODS: Children 6 to 18 years old scheduled for ureteroneocystostomy were recruited for the study. Patients were randomized to group 1--caudal injection of 0.25% bupivacaine before approximately surgical incision and group 2--placement of an epidural catheter with injection of 0.25% bupivacaine, followed by a continuous epidural infusion upon completion of surgery. All patients received intravenous morphine patient controlled analgesic (PCA) as a rescue analgesic, and ketorolac and oxybutynin postoperatively. The epidural catheter was discontinued 48 hours after surgery, with removal of the urinary drainage catheter 4 hours later. Outcome measures included pain intensity rating, supplemental morphine requirements, presence and pain intensity of bladder spasms, analgesia related side effects, time to tolerating a regular diet, and patient and parent satisfaction. RESULTS: There was no statistically significant difference in average daily pain scores between the 2 groups. In the postanesthesia care unit. Significantly more patients in the caudal group required morphine than in the epidural group (56% versus 11%). The total PCA demand was significantly greater in the caudal group on days 1 and 2 postoperatively. Patients in the caudal group took significantly longer to tolerate a regular diet than those in the epidural group. CONCLUSIONS: Continuous epidural analgesia and single dose caudal injection of bupivacaine in conjunction with intravenous morphine PCA and ketorolac provide adequate pain control following intravesical ureteroneocystostomy. Continuous epidural analgesia reduces the need for supplemental intravenous morphine and allows children to tolerate a regular diet earlier.

Nitric oxide in obstructive uropathy: role of endothelial nitric oxide synthase.

PURPOSE: Nitric oxide (NO) ameliorates fibrosis in experimental obstructive uropathy. Previously, we demonstrated that renal fibrosis was decreased after 2 weeks of unilateral ureteral obstruction in inducible nitric oxide synthase (iNOS) knock-out mice given L-arginine supplemented drinking water. We proposed that the 2 constitutive isoforms of nitric oxide synthase (NOS) mediated down-regulation of renal fibrosis in response to prolonged ureteral obstruction. To determine the specific role of endothelial NOS (eNOS) versus neuronal NOS in modulating renal fibrosis due to obstructive uropathy, we evaluated renal injury following unilateral ureteral obstruction in C57BL/6J mice subjected to biochemical inhibition of the constitutive isoforms of NOS and in eNOS knockout mice. MATERIALS AND METHODS: Four groups of C57BL/6J mice were studied. Complete unilateral ureteral obstruction was created by ligating the right ureter at age 8 weeks. A single daily intraperitoneal injection of 30 mg./kg. S-methyl-L-thiocitrulline (SMLT), a selective neuronal and endothelial NOS inhibitor was started 24 hours before ureteral obstruction and administered to half of the study animals. SMLT treated mice and control animals were further subdivided to receive either regular tap water or 1% L-arginine (weight/volume) supplemented water after unilateral ureteral obstruction. Animals were sacrificed on postoperative day 3, 7 or 14. In addition, eNOS knockout mice with unilateral ureteral obstruction were given tap water or L-arginine supplemented water to drink and sacrificed after 14 days. Urine specimens from the bladder and the obstructed renal pelvis along with serum were collected. Nitrite level in each fluid was determined. Renal morphology and cortical thickness were assessed in the normal and obstructed kidneys. Interstitial fibrosis was evaluated using trichrome stain. RESULTS: SMLT was well tolerated by C57BL/6J mice. Serum nitrite levels and nitrite excretion in bladder urine were similar in all SMLT treated groups throughout the duration of unilateral ureteral obstruction. A reduction of pelvic urine nitrite levels by 89%, 68% and 48% versus bladder urine nitrite levels was observed after 3, 7 and 14 days of unilateral ureteral obstruction (p <0.05). Administration of SMLT resulted in a significant increase in bladder urine nitrite level at 7 days and in pelvic urine nitrite levels at 14 days. No significant histological differences in the obstructed kidney were seen after 3, 7 or 14 days of unilateral ureteral obstruction in SMLT treated versus control mice regardless of whether they received tap water or L-arginine supplemented drinking water. In eNOS knockout mice with unilateral ureteral obstruction for 14 days L-arginine supplementation had no effect on pelvic urine nitrite levels and did not alter renal histopathology or cortical thickness. CONCLUSIONS: NO production is decreased in the obstructed kidney in mice with unilateral ureteral obstruction. Biochemical inhibition of constitutive NOS did not modulate renal injury after 14 days of unilateral ureteral obstruction. In contrast to previous findings with iNOS knockout mice, dietary supplementation with L-arginine had no effect on the degree of fibrosis in the obstructed kidney in SMLT treated C57BL/6J or eNOS knockout mice. We conclude that NO derived from eNOS within the kidney has a pivotal role in protecting against renal fibrosis in response to unilateral ureteral obstruction.