Holding Water: Congenital Anomalies of the Kidney and Urinary Tract, CKD, and the Ongoing Role of Excellence in Plumbing.

Congenital anomalies of the kidneys and urinary tracts can result in diminished natal kidney function, possibly through common embryologic pathway disruption or as a result of development taking place in the face of disordered 'post-renal' drainage. Impaired conduit and reservoir function present potential for an ongoing assault leading to further deterioration and progression of chronic kidney disease, a risk that extends to adults with these conditions, even after "correction". The drainage and storage aspects of the urinary system that can impact kidney function are reviewed with attention to correctable or manageable problems including: Bladder dysfunction wherein the low pressure storage of urine is compromised requiring the kidney to work against a pressure gradient, the classic post renal failure problem. The kidney in the aftermath of obstruction which may have lost concentrating capacity leading to a tendency to dehydration ('pre-renal' failure) and through polyuria which exacerbates bladder pressure problems. Further there is an added challenge in evaluation for ongoing or reemergent obstruction in a significantly dilated system where the capacious system leads to slow turnover of urine often requiring a ureteral stent or nephrostomy to clearly establish clinical significance of delayed drainage. Stasis where slow urine flow leads to buildup of debris (stone) or potentiates infection. Vessicoureteral reflux which allows for introduction of lower urinary tract bacteria to the kidney and can lead to pyelonephritis. Conditions which combine problems such as posterior urethral valves where the bladder outlet obstruction compromises kidney function potentially impairing concentrating ability, creates bladder compromise often reducing emptying efficiency or elevating bladder storage pressures, as well as dilating the system potentially promoting stasis. Cognizance of the potential for plumbing problems to further kidney deterioration as patients with congenital urinary tract anomalies, even after they have been repaired is incumbent on those caring for these patients as they age. Thoughtful evaluation of those patients in whom kidney compromise maybe aggravated by drainage and storage disorder will optimize native renal function.

Intermittent testicular torsion in the pediatric patient: sonographic indicators of a difficult diagnosis.

OBJECTIVE: The purpose of this study was to determine whether intermittent testicular torsion, defined as the sudden onset of unilateral scrotal pain of short duration with spontaneous resolution, has sonographic indicators that can be used to establish the diagnosis. MATERIALS AND METHODS: A retrospective search for pediatric patients presenting with suspected intermittent testicular torsion over a 2-year period was performed. The sonographic findings, medical records for clinical presentation, surgical outcomes, and comorbidities relevant to intermittent testicular torsion were reviewed. RESULTS: Analysis was performed on two separate categories for intermittent testicular torsion: total patient episodes (n = 19) and surgical cases (n = 10), with a total of 15 patients with 1.26 episodes per patient. Of the 19 episodes of intermittent testicular torsion, 63% (12) had either absent flow followed by normal to increased flow (n = 6, p = 0.18) or increased testicular flow (n = 6), 26% (5) had normal flow, and 10.5% (2) had decreased flow. Of the total episodes of intermittent testicular torsion, 79% (15) had a whirlpool sign or pseudomass, 10.5% (2) had neither a whirlpool sign or pseudomass but a "boggy" thickened cord, and 10.5% (2) had a normal appearance of the spermatic cord with no pseudomass. CONCLUSION: The whirlpool sign or an abnormal boggy cord and pseudomass formation below the twisted spermatic cord were both significant findings to support a diagnosis of intermittent testicular torsion. Although change from no perfusion to perfusion during the examination was not statistically significant in our cases, when present it led to the correct diagnosis in each case.

Outcomes of fetuses with lower urinary tract obstruction treated with vesicoamniotic shunt: a single-institution experience.

PURPOSE: The purpose of this manuscript was to examine the outcomes of patients with lower urinary tract obstruction (LUTO) treated with vesicoamniotic shunt (VAS) to improve the quality of prenatal consultation and therapy. METHODS: The medical records of all patients diagnosed with LUTO at our center between January 2004 and March 2012 were reviewed retrospectively. RESULTS: Of 14 male fetuses with LUTO, all with characteristic ultrasound findings, 11 underwent intervention. One patient received vesicocentesis alone, while 10 had VAS. Two fetuses additionally underwent cystoscopy (one with attempted valve ablation), and two had peritoneoamniotic shunts. Of 16 total VAS, 13 were placed successfully, 8 dislodged (median 7 days), and 1 obstructed (84 days). Two fetuses suffered in utero demise, and two have unknown outcomes. LUTO was confirmed in six of eight live-born fetuses. One patient died in the neonatal period, while seven survived. All six available at follow-up (median 3.7 years), had significant genitourinary morbidity. Five patients had chronic kidney disease, but only one has required dialysis and transplant. Three had respiratory insufficiency, and one required a tracheostomy. CONCLUSION: Despite significant perinatal and long-term morbidity, VAS offers patients faced with a poor prognosis an improved chance of survival. Our results underscore the need for further research into the diagnosis and treatment of LUTO.

Single-center experience with pediatric laparoscopic extravesical reimplantation: safe and effective in simple and complex anatomy.

PURPOSE: Minimally invasive approaches to the surgical management of vesicoureteric reflux (VUR) have become more prominent over the last 10 years with progress in both endoscopic and laparoscopic/robotic surgery. We hypothesized that laparoscopic extravesical detrussoraphy (LED) for the management of VUR in children with complex bladders and/or bilateral VUR was safe and effective. SUBJECTS AND METHODS: Under institutional review board approval we evaluated the charts of all patients seen at our institution over the last 8 years who had undergone LED for the management of VUR. We evaluated demographic variables, surgical variables, and postoperative results. Postoperative bladder function was examined in the patients as well as need for secondary procedures. Patients with complex bladders included all patients who had previous surgery on the affected side, neurogenic bladders, and duplex or complex anatomy. RESULTS: Ninety-eight patients with 144 ureters were treated during this time period. The overall VUR resolution by voiding cystourethrogram was 95.2%. The average age was 6.74 years, with 13 children over the age of 12 years old. Average length of stay (LOS) was 1.7 days for children 5 years and older and 1.0 days for children less than 5 years old (P=.004). LOS was not affected by body mass index or complexity of the procedure. There were 46 bilateral procedures, and the incidence of urinary retention was 6.5% versus 0% in the unilateral group (P=.09). Of our patients, 27.6% had complex bladders, including 9 patients with complete ureteral duplications, 10 with periureteral diverticula, and 8 with prior surgery on the affected side. There were two complications requiring a second procedure in this group (7%). No patient with a complex bladder had persistent VUR. CONCLUSION: LED for the management of children with complex bladders and VUR is safe and effective. This technique is versatile and achieves high VUR resolution rates with minimal morbidity.

The impact of surgical approach and urinary diversion on patient outcomes in pediatric pyeloplasty.

PURPOSE: Recent comparisons of the impact of the surgical approach on pediatric pyeloplasty outcomes have generally incorporated a form of internal ureteral drainage. We hypothesized that the surgical approach does not affect outcomes in children who undergo unstented pyeloplasty and stenting offers no long-term benefit in those with pediatric pyeloplasty. MATERIALS AND METHODS: After receiving institutional review board approval we examined the records of all children who underwent initial pyeloplasty from December 2001 to December 2009. We compared unstented and stented pyeloplasties, and each surgical approach in the unstented group. RESULTS: During the study period 367 pyeloplasties were performed, including 231 unstented and 136 stented procedures. When comparing unstented to stented pyeloplasties, there was no difference in the complication or failure rate. Of unstented pyeloplasties 71, 67 and 93 were done using a transperitoneal laparoscopic approach, a flank approach and dorsal lumbotomy, respectively. There were 5 failures, of which 2 were laparoscopic, 2 used a flank approach and 1 used dorsal lumbotomy (p = 0.51). A total of 31 patients, including 10 treated with a laparoscopic approach, 3 with a flank approach and 18 with dorsal lumbotomy (p = 0.02), required second procedures, of which 24 were temporary drainage for a urine leak. Univariate analysis of factors associated with secondary procedures in the unstented pyeloplasty group showed that only surgical approach was significant (p = 0.05). CONCLUSIONS: In pediatric pyeloplasty there is no significant difference in outcome between stented and unstented repairs. In unstented repairs complications may vary by surgical approach. Regardless of the approach unstented pyeloplasty is safe and effective in the pediatric population.

A single-center experience with prone retroperitoneoscopic versus open renal surgery in children: an age- and procedure-matched comparison.

PURPOSE: We determined how surgical outcomes differ among children undergoing prone retroperitoneoscopic (RP) and open total and partial nephrectomies for benign renal disease. PATIENTS AND METHODS: A retrospective analysis was performed, using medical records of children who were undergoing benign renal surgery at a single institution from 2001 to 2010. Patients were divided into open or RP cohorts, then subdivided into those who were undergoing total or partial nephrectomies. A further breakdown by age (<3 years and ≥3 years) was performed. Bilateral nephrectomies were separately evaluated. Surgical outcomes were compared across groups. RESULTS: There were 308 (333 renal units) children who underwent RP (n=154) or open (n=154) total or partial nephrectomies, 25 of which were bilateral (20 RP, 5 open); 199 patients underwent total nephrectomies (RP n=118, open n=81), and 109 underwent partial nephrectomies (RP n=36, open n=73). After controlling for age and concomitant procedures, operative times were similar for the RP total and bilateral nephrectomy groups, but longer for the RP partial nephrectomy group, compared with their open counterparts. In both total and partial nephrectomies, the RP group had a shorter hospital stay. There were no open conversions in any of the RP groups and no differences in complications in the total, partial, and bilateral analyses. There were 13 patients who previously received peritoneal dialysis (8 bilateral RP, 3 unilateral RP, and 2 unilateral open), all of whom resumed dialysis in a mean of 1.11 days. CONCLUSIONS: This is the largest series to date that compares RP and open renal surgeries in children. The RP and open approaches were comparably safe and efficacious. Hospital stays were significantly shorter in the RP total and partial groups, although operative times were significantly longer in the RP partial nephrectomy group. Prone retroperitoneoscopic surgery should be considered a viable option for renal surgery at any age.

Ex-premature infant boys with hypospadias are similar in size to age-matched, ex-premature infant boys without hypospadias.

OBJECTIVE: Studies have postulated that hypospadias, prematurity, and low birth weight are linked by defects in androgen signaling. To determine whether premature, hypospadiac boys are small and remain so, we compared their size at birth and at hypospadias repair to premature boys who underwent post-neonatal circumcision. METHODS: We identified premature boys admitted to Texas Children's Hospital who underwent either hypospadias repair or circumcision after 4 months of age. Age, weight, and height at birth and surgery were recorded. RESULTS: Fifty-four boys had hypospadias and 34 did not. For hypospadiac boys, the mean birth weight and age, height, and weight at surgery were lower than for boys without hypospadias. More importantly, length-for-age and weight-for-age percentiles were also lower for hypospadiac boys. When subset analysis was performed on boys younger than 2 years at surgery, however, there were no significant differences in height or weight between hypospadiac and non-hypospadiac boys. CONCLUSION: Our series suggests that premature, hypospadiac boys are born smaller than age-matched, non-hypospadiac controls. However, there were no age-corrected size differences between hypospadiac and non-hypospadiac boys at surgery. This implies that hypospadiac boys exhibit post-neonatal 'rebound' growth. Global growth deficits, if any, do not persist in hypospadiac boys.

Cross-sectional evaluation of parental decision making factors for vesicoureteral reflux management in children.

PURPOSE: Parental decision making in children with vesicoureteral reflux has potentially become more complex with the evolution of ethnic diversity in the United States, the Internet, the publication of contradictory clinical data and the emergence of minimally invasive surgery. We performed a cross-sectional study of parental management for pediatric vesicoureteral reflux. MATERIALS AND METHODS: We administered a 26-item questionnaire to parents of children with vesicoureteral reflux seen at Texas Children's Hospital urology offices or undergoing antireflux surgery at that institution. Univariate and multivariate analysis was done on patient disease characteristics, demographics, predicted reflux duration, surgery success rate, antibiotic cessation, complication risk, financial considerations, urologist recommendations, Internet information, friend recommendations, and postoperative voiding cystourethrography, renal ultrasound and recovery. RESULTS: Enrolled in the study were 15 boys and 49 girls with a mean age of 3.5 years and a mean reflux grade of 2.8. Of the cases 37 were bilateral. Parents chose endoscopic treatment in 38 children, open ureteroneocystostomy in 9, antibiotic prophylaxis in 14 and observation without antibiotics in 3. Univariate analysis suggested that Hispanic parents rated ultrasound and financial considerations as more important than white parents (p <0.05). Multivariate analysis revealed that differences seen on univariate analysis may have been due to an association between race and income. Finally, 93.6% of parents rated urologist opinion as very or extremely important. CONCLUSIONS: Data indicate that the parents of our patients highly value the opinion of the pediatric urologist when choosing treatment for their children with vesicoureteral reflux. Despite social changes the physician-parental relationship remains critical. Differences in parental decision making may be linked to associations between race and income.

Factors associated with delayed treatment of acute testicular torsion-do demographics or interhospital transfer matter?

PURPOSE: Testicular torsion is a true urological emergency. We determined whether a delay in treatment due to hospital transfer or socioeconomic factors would impact the orchiectomy rate in children with this condition. MATERIALS AND METHODS: We retrospectively evaluated the records of boys seen at a single institution emergency department who proceeded to surgery for a diagnosis of acute testicular torsion from 2003 to 2008. Charts were reviewed for transfer status, symptom duration, race, insurance presence or absence and distance from the hospital. Orchiectomy specimens were evaluated for histological confirmation of nonviability. RESULTS: We reviewed 97 records. The orchiectomy rate in patients who were vs were not transferred to the emergency department was 47.8% vs 68.9%, respectively (p = 0.07). Symptom duration was greater in the orchiectomy group with a mean difference of 47.9 hours (p <0.01). The mean transfer delay was 1 hour 15 minutes longer in the orchiectomy group (p = 0.01). Boys who underwent orchiectomy were 2.2 years younger than those who avoided orchiectomy (p = 0.01). Multivariate analysis showed that symptom duration and distance from the hospital were the strongest predictors of orchiectomy. CONCLUSIONS: Data suggest that torsion is a time dependent event and factors that delay time to treatment lead to poorer outcomes. These factors include distance from the hospital and the time delay associated with hospital transfer.

Endoscopic treatment of symptomatic refluxing renal transplant ureteroneocystostomies in children.

To present a multi-center experience with the use of Dx/HA copolymer for treatment of symptomatic refluxing renal transplant UNC in children. A multi-center, retrospective chart review was performed. Eleven patients with a mean age of eight yr underwent renal transplantation with an anti-refluxing UNC. Data were collected to determine the safety and effectiveness of the procedure and to identify possible predictors of success. Endoscopic treatment was successful in one of five males and five of six females, for an overall success rate of 54.5%. The etiology of renal failure was associated with success of treatment, with 4/6 (67%) patients with upper tract pathology demonstrating resolution of the VUR, as compared with one of three (33%) patients with lower tract pathology. Male patients had a higher incidence of lower tract pathology. No complications were associated with the endoscopic procedure. Endoscopic injection of Dx/HA remains a safe option for the treatment of symptomatic refluxing transplant UNC in children. Although the success rate is lower than that seen in the treatment of primary VUR, the minimally invasive nature and safety of this technique may offer advantages over open reconstruction of the refluxing transplant ureter.

Bladder injuries during laparoscopic orchiopexy: incidence and lessons learned.

PURPOSE: Laparoscopic orchiopexy is a safe operation. However, the bladder can be injured during creation of the transperitoneal tunnel for the cryptorchid testis. We reviewed our experience with this complication. MATERIALS AND METHODS: We searched the operative notes of patients who had undergone laparoscopic orchiopexy between August 15, 2002 and October 1, 2008, and identified bladder injuries and their treatment. RESULTS: A total of 93 patients underwent laparoscopic orchiopexies for 101 undescended testes during the study interval, with 3 procedures resulting in bladder injuries. The 3 operations varied with regard to whether the injury was recognized intraoperatively or postoperatively, and repaired in an open or laparoscopic fashion. CONCLUSIONS: Bladder injury during laparoscopic orchiopexy is a rare but serious complication that can be managed by an open or laparoscopic approach. We recommend placement of a urethral catheter and syringe assisted drainage of all urine from the bladder at the beginning of the operation, careful perivesical dissection particularly in children with prior inguinal surgery, filling and emptying of the bladder during the procedure, and maintaining a high index of suspicion especially when hematuria is observed.

Experimental congenital vesicoureteral reflux in sheep is associated with reduced renal expression levels of aquaporin 1 and 2.

PURPOSE: Impaired concentrating capacity has been demonstrated in human refluxing kidneys and in kidneys of growing animals with experimental congenital vesicoureteral reflux. Aquaporin water channel proteins are the principal mediators of water homeostasis in the kidney. We examined the impact of experimental congenital vesicoureteral reflux on renal aquaporin protein expression. MATERIALS AND METHODS: Vesicoureteral reflux was surgically induced bilaterally in male fetal sheep at 95 days of gestation (term 140 days). Following birth animals received antibiotic prophylaxis, and reflux grades were assessed by fluoroscopic voiding cystogram. After sacrifice at age 6 months 1 kidney of each animal (5 refluxing kidneys, and 3 from normal age and sex matched controls) was retrieved, frozen in liquid nitrogen and used for immunoblotting. The second kidney was perfusion fixed and processed for immunohistochemical analysis. RESULTS: Semiquantitative immunoblotting of inner medulla showed that vesicoureteral reflux was associated with a marked down-regulation in expression of aquaporin 1 (arbitrary units 7.0 +/- 4.3 vs 22.5 +/- 2.8, p <0.05) and aquaporin 2 (5.7 +/- 5.1 vs 24.8 +/- 3.8, p <0.05), which was confirmed by immunocytochemical analysis. Dot blot analysis revealed a homogeneous down-regulation of aquaporin 2 expression throughout the refluxing kidney to 0.029 vs 0.1 in normal kidneys (p = 0.026). CONCLUSIONS: Progressively impaired renal concentrating capacity induced by experimental fetal reflux is associated with decreased levels of renal aquaporin 1 and 2 expression. This long-term down-regulation of aquaporin 1 and 2 provides important molecular evidence for a defect in resorptive capacity of water induced by vesicoureteral reflux.