Pediatric metanephric adenoma with Fanconi-Bickel syndrome: a case report and review of literature.

BACKGROUND: Metanephric adenoma (MA) is a rare benign renal tumor that resembles renal cell carcinoma and Wilms' tumor in radiological as well as pathological appearance. It can present at any age or gender, and it is extremely rare in the pediatric age group with less than 50 reported cases. Fanconi-Bickel syndrome (FBS) is a rare autosomal recessive disorder of carbohydrate metabolism. Herein, we report a rare incidence of MA in a boy with a genetically confirmed FBS who underwent a nephron-sparing surgery. CASE PRESENTATION: A 21-month-old boy was referred to the pediatric urology clinic for further evaluation of an incidentally discovered left renal mass. His laboratory investigations showed normal renal function, hypophosphatemia, high blood glucose level, markedly elevated serum alkaline phosphatase, and low serum vitamin D. Blood picture showed signs of polycythemia and urinalysis showed glucosuria and aminoaciduria. Genetic testing was positive for Fanconi-Bickel syndrome. Radiological investigations were carried out with abdominal ultrasound and computerized tomography (CT) with intravenous contrast documented a sharply marginated peripheral hypoechoic hypovascular homogeneously enhancing mass at the upper pole of the left kidney measuring 2.0 × 1.8 × 2.0 cm. The child was admitted and started on supportive treatment until his medical condition was stabilized, then underwent elective open left partial nephrectomy via a left upper transverse abdominal transperitoneal incision. The excised renal mass was sent for histopathological assessment and was found to be a tumor composed of tightly packed tubules with no mitotic figures or necrosis and scanty cytoplasm consistent with MA. After good hydration and tumor resection, his polycythemia gradually improved. The patient was discharged home in a good condition with his proper replacement therapies. His follow-up abdominal ultrasound after 12 months showed no signs of recurrence. CONCLUSIONS: Metanephric adenoma is extremely rare in the pediatric age group, especially in those who have a FBS. The only way to diagnose and treat this tumor is by surgical resection as most patients are asymptomatic. A nephron-sparing surgery is better for this age group in which the future renal function is considered.

Bladder function in children with posterior urethral valves: impact of antenatal versus postnatal diagnosis

ABSTRACT Purpose: Posterior urethral valves (PUVs) are the most common cause of congenital bladder obstruction in boys. Our aim was to assess the impact of early diagnosis and fulguration of PUVs on bladder function and compare their functional and urodynamic outcome with children who underwent delayed intervention. Materials and Methods: We retrospectively evaluated 153 patients who underwent primary valve ablation from two tertiary hospitals between 2001 and 2018. Patients have been divided into 2 groups, group 1 included 69 patients who were detected antenatally and underwent early fulguration of PUVs while group 2 included 84 children presented postnatally and underwent delayed valve ablation. The recorded data throughout follow-up in renal function tests, urodynamics and changes in the upper urinary tracts were evaluated and compared. Results: Median age at time of valve ablation was 10 days in group 1 and 7 months in group 2. The median follow-up period was 6.5 and 7 years in group 1 and 2, respectively. Chronic kidney disease (CKD) developed in 15 (22%) boys in group 1 while in group 2 it was observed in 31 (37%), p=0.04. While Q-max, mean bladder capacity and post-void residual (PVR) volumes were comparable in both groups, percent PVR was significantly higher in group 2 (3.27 vs. 1.44, p=0.002). Detrusor overactivity was slightly different in both groups (p = 0.07). Conclusions: Compared to delayed intervention, primary ablation of PUVs during the early neonatal life possibly provides the optimum chance to have optimum renal function without impact on bladder function.

Bladder function in children with posterior urethral valves: impact of antenatal versus postnatal diagnosis.

PURPOSE: Posterior urethral valves (PUVs) are the most common cause of congenital bladder obstruction in boys. Our aim was to assess the impact of early diagnosis and fulguration of PUVs on bladder function and compare their functional and urodynamic outcome with children who underwent delayed intervention. MATERIALS AND METHODS: We retrospectively evaluated 153 patients who underwent primary valve ablation from two tertiary hospitals between 2001 and 2018. Patients have been divided into 2 groups, group 1 included 69 patients who were detected antenatally and underwent early fulguration of PUVs while group 2 included 84 children presented postnatally and underwent delayed valve ablation. The recorded data throughout follow-up in renal function tests, urodynamics and changes in the upper urinary tracts were evaluated and compared. RESULTS: Median age at time of valve ablation was 10 days in group 1 and 7 months in group 2. The median follow-up period was 6.5 and 7 years in group 1 and 2, respectively. Chronic kidney disease (CKD) developed in 15 (22%) boys in group 1 while in group 2 it was observed in 31 (37%), p=0.04. While Q-max, mean bladder capacity and post-void residual (PVR) volumes were comparable in both groups, percent PVR was significantly higher in group 2 (3.27 vs. 1.44, p=0.002). Detrusor overactivity was slightly different in both groups (p = 0.07). CONCLUSIONS: Compared to delayed intervention, primary ablation of PUVs during the early neonatal life possibly provides the optimum chance to have optimum renal function without impact on bladder function.

Laparoscopic nephrectomy in children: Does the approach matter?

INTRODUCTION AND OBJECTIVE: Laparoscopic nephrectomy (LN) is an established technique for the management of benign non-functioning kidneys in children. It can be performed either by the transperitoneal (TP) or retroperitoneal (RP) approach. The aim of this study was to compare safety and efficacy of TP with RP approach for LN in children. STUDY DESIGN: Between 2007 and 2019, 79 consecutive pediatric patients with benign non-functioning kidneys underwent laparoscopic nephrectomy by a single surgeon. Patients were categorized into two groups according to the approach; Group 1 included 41 patients who underwent transperitoneal laparoscopic nephrectomy (TPLN), while Group 2 included 38 patients who underwent retroperitoneal laparoscopic nephrectomy (RPLN). Comparison was done between the two groups regarding the mean operative time, complications, time to resume oral intake, need for postoperative analgesia and postoperative hospital stay. RESULTS: Laparoscopic nephrectomy was successfully completed in 75 of the 79 cases. Four cases were converted to open surgery: 3 from the TPLN group and one from the RPLN group (p = 0.34). There was statistically significant difference between the two groups in terms of estimated blood loss (p = 0.013), time to resume oral intake (p = 0.001) and postoperative hospital stay (p = 0.024). Overall, there was no statistically significant difference as regard mean operative time, overall complication rate, and postoperative analgesia need. DISCUSSION: LN is now an established technique in children and surgeon experience is a limiting factor. This experience should be gained progressively with respect to both TP and RP approaches to deal with different indications by optimal approach. Both TP and RP approaches are excellent approaches and it is up to individual surgeons, based on their experience, which approach they select. In this study, a comparative study was made between both TP and RP approaches for pediatric LN. There was no statistically significant difference between the two approaches as regard success rate, mean operative time, need for postoperative analgesia and overall complication rate. In the meantime, RPLN had the advantage of lower intraoperative blood loss, shorter time to resume oral intake and shorter hospital stay. CONCLUSIONS: Laparoscopic nephrectomy in children may be performed by transperitoneal or retroperitoneal approaches, obtaining equal safety and efficacy. The retroperitoneal approach seems to be advantageous in terms of lower intraoperative blood loss, time to resume oral intake and shorter postoperative hospital stay.

Unusual Presentation, Relapse, and Metastasis of a Pediatric Testicular Yolk Sac Tumor: Case Report.

Testicular tumors are not uncommon in children and represent 1%-2% of all pediatric malignancies. Prepubertal testicular yolk sac tumor is the most common childhood testicular cancer, accounting for 70%-80% of all cases. The clinical presentation varies from one patient to another; most common presentation is painless scrotal mass. Herein, we present a case of pediatric patient with a testicular yolk sac tumor who had unusual presentation followed by a local relapse and metastasis and continued to have high markers while he was on chemotherapy, then underwent retroperitoneal lymph node dissection and local recurrence excision.

Ginkgo biloba Extract Attenuates Methotrexate-Induced Testicular Injury in Rats: Cross-talk Between Oxidative Stress, Inflammation, Apoptosis, and miRNA-29a Expression.

Ginkgo biloba leaf extract (GIN) is a popular Chinese herbal medicine. It has a nephroprotective effect against the nephrotoxicity induced by the chemotherapeutic agent methotrexate (MTX). This work was designed to explore the testicular protective role of GIN on MTX-induced testicular injury in a rat model. The experimental protocol lasted for 10 days for the 4 studied groups. First group: received saline (normal control, NC group). The second group was administered GIN (100 mg/kg/day) orally for 10 days (GIN C). Third group: injected with MTX (20 mg/kg ip) only on the fifth day (MTX group). Fourth group: administered GIN for 10 days with MTX injection on the fifth day (GIN+MTX group). MTX induced testicular injury as evident by a marked rise in the malondialdehyde (MDA) content, interleukin-6 (IL-6) and IL-1ß protein levels, nuclear factor kappa-B (NF-κB) protein expression, bcl-2 associated × protein (Bax) mRNA expression, p53 mRNA and protein expressions, and miRNA29-a expression along with a marked decline in the serum level of testosterone and superoxide dismutase (SOD) content in testicular tissue in relation to the NC group. Moreover, histopathological testicular damage with a notable decrease in the Johnsen score together with a significant elevation in the testicular injury score was observed in the MTX group in comparison to the NC group. The administration of GIN ameliorated the biochemical changes as well as the testicular histopathological findings and scores. GIN could protect against MTX-induced gonadotoxicity by its antioxidant, anti-inflammatory, antiapoptotic activities plus the regulation of the miRNA-29a testicular expression.

Impact Effect of Methyl Tertiary-Butyl Ether "Twelve Months Vapor Inhalation Study in Rats".

We investigated the early risk of developing cancer by inhalation of low doses (60 µL/day) of methyl tertiary butyl ether (MTBE) vapors using protein SDS-PAGE and LC-MS/MS analysis of rat sera. Furthermore, histological alterations were assessed in the trachea and lungs of 60 adult male Wistar rats. SDS-PAGE of blood sera showed three protein bands corresponding to 29, 28, and 21 kDa. Mass spectroscopy was used to identify these three bands. The upper and middle protein bands showed homology to carbonic anhydrase 2 (CA II), whereas the lower protein band showed homology with peroxiredoxin 2. We found that exposure to MTBE resulted in histopathological alterations in the trachea and the lungs. The histological anomalies of trachea and lung showed that the lumen of trachea, bronchi, and air alveoli packed with free and necrotic epithelial cells (epithelialization). The tracheal lamina propria of lung demonstrated aggregation of lymphoid cells, lymphoid hyperplasia, hemorrhage, adenomas, fibroid degeneration, steatosis, foam cells, severe inflammatory cells with monocytic infiltration, edema, hemorrhage. Occluded, congested, and hypertrophied lung arteries in addition, degenerated thyroid follicles, were observed. The hyaline cartilage displayed degeneration, deformation, and abnormal protrusion. In conclusion, our results suggest that inhalation of very low concentrations of the gasoline additive MTBE could induce an increase in protein levels and resulted in histopathological alterations of the trachea and the lungs.

Candesartan in a rat model of testicular toxicity: New insight on its protective mechanism.

IMPACT STATEMENT: Cisplatin is a commonly used drug in the treatment of solid tumors and its application is associated with testicular toxicity. The effect of candesartan in cisplatin-induced testicular toxicity and its fundamental mechanism of action were investigated. Candesartan had certainly repaired the testicular injury and ameliorated both biochemical and histopathological changes. Candesartan mitigated the gonadotoxicity induced by cisplatin via antioxidative, anti-inflammatory, and antiapoptotic actions.

Isolated low grade prenatally detected unilateral hydronephrosis: do we need long term follow-up?

ABSTRACT Purpose: To assess the need for postnatal evaluation and the medium term outcome in patients with isolated unilateral low grade prenatally detected hydronephrosis. Materials and Methods: We prospectively selected 424 patients (690 kidney units) with a prenatal diagnosis of urinary tract dilatation between 2010 and 2013. We included only those patients with isolated unilateral low-grade hydronephrosis who underwent at least 2 postnatal ultrasound examinations. The Society for Fetal Urology (SFU) grading system was utilized for assessment of the hydronephrosis. We excluded patients with bilateral dilation or other urological abnormalities. The fate of hydronephrosis including resolution, stability or worsening was documented. Results: A total of 66 infants (44 boys and 22 girls) with antenatally diagnosed unilateral urinary tract dilation (23 right and 43 left) were identified. Ultrasounds showed SFU grade 1 hydronephrosis in 32 patients (48%) and SFU grade 2 hydronephrosis in 34 (52%). After a mean follow-up period of 32 months (range 12 to 60), 37 patients (56%) had complete resolution of hydronephrosis while the remaining 29 were stable (44%). None of our patients developed UTIs during follow-up and none required surgical intervention. Conclusions: Prenatally detected, isolated unilateral low-grade hydronephrosis usually have a favorable prognosis. All cases in our cohort showed either stability or resolution of hydronephrosis without any harmful consequences. Based on our findings on medium-term in this category of patients, long-term follow-up is not warranted.

The role of mesenchymal stem cells in chemotherapy-induced gonadotoxicity.

BACKGROUND: The therapeutic potential of bone marrow-derived mesenchymal stem cells (BM-MSCs) against cisplatin-induced nephrotoxicity has been reported, however, its efficacy in gonadotoxicity still has not been addressed. Herein, we investigated the effect of BM-MSCs in cisplatin-induced testicular toxicity and its underlying mechanism of action. METHODS: Thirty male Sprague-Dawley rats were divided into a control group: injected with phosphate-buffered saline (PBS) intraperitoneal (ip), a cisplatin group: injected with a single dose of 7 mg/kg cisplatin ip to induce gonadotoxicity and a BM-MSCs group: received cisplatin ip followed by BM-MSCs injection 1 day after cisplatin. In testicular tissues, malondialdehyde (MDA), superoxide dismutase (SOD), and reduced glutathione (GSH) levels were assessed. Additionally, gene expressions of inducible nitric oxide synthase (iNOS), caspase-3, and p38 mitogen-activated protein kinase (MAPK) were measured. The testicular tumor necrosis factor alpha (TNF-α) protein contents and Bcl-2 associated X protein (BAX) expression were determined. Histopathology of testicular tissues was examined. RESULTS: Cisplatin injection showed a significant decrease in GSH and SOD testicular levels besides a significant increase of MDA and TNF-α testicular levels and upregulation of testicular gene expressions of iNOS, caspase-3, and p38-MAPK in comparison to the control group. Moreover, a marked increase in BAX protein expression was observed in the cisplatin group when compared with the control one. Histopathological examination exhibited significant seminiferous tubules atrophy in cisplatin-treated rats. CONCLUSIONS: The BM-MSCs injection significantly repaired the testicular injury and improved both biochemical and histopathological changes. The MSCs mitigated the gonadotoxicity induced by cisplatin through antioxidative, anti-inflammatory, and antiapoptotic mechanisms.

Isolated low grade prenatally detected unilateral hydronephrosis: do we need long term follow-up?

PURPOSE: To assess the need for postnatal evaluation and the medium term outcome in patients with isolated unilateral low grade prenatally detected hydronephrosis. MATERIALS AND METHODS: We prospectively selected 424 patients (690 kidney units) with a prenatal diagnosis of urinary tract dilatation between 2010 and 2013. We included only those patients with isolated unilateral low-grade hydronephrosis who underwent at least 2 postnatal ultrasound examinations. The Society for Fetal Urology (SFU) grading system was utilized for assessment of the hydronephrosis. We excluded patients with bilateral dilation or other urological abnormalities. The fate of hydronephrosis including resolution, stability or worsening was documented. RESULTS: A total of 66 infants (44 boys and 22 girls) with antenatally diagnosed unilateral urinary tract dilation (23 right and 43 left) were identified. Ultrasounds showed SFU grade 1 hydronephrosis in 32 patients (48%) and SFU grade 2 hydronephrosis in 34 (52%). After a mean follow-up period of 32 months (range 12 to 60), 37 patients (56%) had complete resolution of hydronephrosis while the remaining 29 were stable (44%). None of our patients developed UTIs during follow-up and none required surgical intervention. CONCLUSIONS: Prenatally detected, isolated unilateral low-grade hydronephrosis usually have a favorable prognosis. All cases in our cohort showed either stability or resolution of hydronephrosis without any harmful consequences. Based on our findings on medium-term in this category of patients, long-term follow-up is not warranted.

Prenatally detected, unilateral, high-grade hydronephrosis: Can we predict the natural history?

INTRODUCTION: Fetal hydronephrosis (HN) occurs in approximately 5% of pregnancies and its prognosis depends mainly on the grade of the dilation. We attempted to determine the fate of isolated, unilateral, high-grade HN in children with antenatal diagnosis, emphasizing the risk factors for progression. METHODS: We retrospectively evaluated 424 children (690 kidney units) with antenatal HN in the period between 2010 and 2014. We included only those patients with isolated high-grade HN (Society for Fetal Urology [SFU] Grade 3 or 4). Patients with bilateral HN or unilateral HN associated with dilated ureter or reflux and patients with missed followup were excluded. The prognosis of HN (whether improved, stabilized, or progressed) and the need for surgical intervention in this subset of patients was evaluated. RESULTS: A total of 44 children (34 boys and 10 girls) were identified. Ultrasounds showed SFU Grade 3 HN in 24 (54%) and SFU Grade 4 HN in 20 (46%). After a mean followup of three years (range 1-5), 10 children (23%) needed surgical intervention; four Grade 3 HN (16%) and six Grade 4 HN (30%). The majority of children with differential renal function (DRF) ≥40% (69.5%) were stable or improved. Five girls (50%) and five boys (17%) progressed and required surgical intervention. No patient with a renal pelvis anteroposterior diameter (APD) <1.5 cm needed surgical intervention. CONCLUSIONS: Infants with isolated, unilateral, high-grade HN might be managed conservatively. Male gender, DRF ≥40%, SFU Grade 3 HN, and APD <1.5 cm were favourable prognostic factors.

Posterior urethral valves: Impact of low birth weight and preterm delivery on the final renal outcome.

OBJECTIVE: To investigate the relationship between low birth weight (LBW; <2.5 kg) and preterm delivery (<37 weeks gestational age) and final renal outcome in infants with posterior urethral valves (PUVs), emphasising the risk factors for the development of chronic kidney disease (CKD). PATIENTS AND METHODS: A retrospective review was performed for all infants with PUVs who were treated between 1990 and 2010. In all, 52 infants were identified to have LBW and/or delivered preterm (Group 1). Infants in Group 1 were compared with a matching group (Group 2) of 60 full-term normal birth weight (NBW) infants with PUVs managed during the same period. The outcome of both groups was analysed. RESULTS: During follow-up, CKD developed in 17 (32.5%) and 22 patients (36.5%) in Groups 1 and 2, respectively (P = 0.812). Patients with LBW or delivered preterm had significantly higher incidence of oligohydramnios (P = 0.009), increased risk of vesicostomy (P < 0.001), longer hospital stay (P < 0.001), and higher incidence of vesico-ureteric reflux (VUR, P = 0.024). In the LBW patients, initial serum creatinine, nadir serum creatinine, oligohydramnios and Neonatal Intensive Care Unit (NICU) length of stay were significant predictors of final renal outcome (P < 0.001, P = 0.002, P = 0.004 and P = 0.012, respectively). CONCLUSION: In our cohort of LBW and preterm delivery infants with PUVs, outcomes were similar to those of NBW full-term infants with PUVs but with an increased risk of vesicostomy, longer hospital stay, and higher incidence of VUR. LBW was associated with oligohydramnios, longer NICU admission, high initial and nadir serum creatinine, which were associated with a poor prognosis.

Unilateral Renal Agenesis: Necessity of Postnatal Evaluation in a Contemporary Series.

OBJECTIVE: To assess the need for postnatal evaluation and the long-term consequences in patients with prenatal diagnosis of unilateral renal agenesis (URA). MATERIALS AND METHODS: A retrospective review of the records of all patients with prenatal diagnosis of URA between 2004 and 2013 was done. We included all patients who underwent postnatal evaluation with renal bladder ultrasound (RBUS), dimercaptosuccinic acid scan, and a voiding cystourethrogram. Long-term follow-up included physical examination and assessment of contralateral renal growth. RESULTS: A total of 46 children (24 boys and 22 girls) with URA were identified. Ultrasounds showed associated hydronephrosis in 9 patients (19%). Dimercaptosuccinic acid scans confirmed the diagnosis of URA in all patients and showed renal scars in 2 solitary kidneys (4%). Vesicoureteral reflux was diagnosed in 4 patients (12%); all were of low grade and managed conservatively. After a mean follow up of 4 years, RBUS showed compensatory hypertrophy in 31 patients (67%), 3 patients (6%) developed chronic kidney disease, and 2 patients (4%) developed hypertension. CONCLUSION: Prenatally detected cases of URA should be promptly evaluated. RBUS is found to be sufficient for diagnosis. However, additional imaging may be indicated for confirming the diagnosis and detection of other associated urinary anomalies. Serial assessment of the contralateral solitary kidney growth and serum creatinine measurement will be helpful to assess kidney function on the long term.

Uroprotective effect of oleuropein in a rat model of hemorrhagic cystitis.

Hemorrhagic cystitis is one of the devastating complications seen after receiving cyclophosphamide chemotherapy. Oleuropein is the most important phenolic compound of olive leaves that mediates most of its beneficial pharmacological properties. Herein, we investigated the possible uroprotective effect of oleuropein against cyclophosphamide induced hemorrhagic cystitis in a rat model. For this purpose, we measured bladder nitric oxide, reduced glutathione, catalase, tumor necrosis factor-alpha and vascular endothelial growth factor levels in addition to the bladder gene expression of intercellular adhesion molecule-1 after induction of hemorrhagic cystitis in the presence or absence of oleuropein. Histopathological examination of bladder tissues was also performed. After cyclophosphamide injection, we demonstrated a significant decrease in bladder reduced glutathione (39%) and catalase (55.4%) levels and a significant increase of nitric oxide (5.6 folds), tumor necrosis factor-alpha (3.3 folds), vascular endothelial growth factor (2 folds) and intercellular adhesion molecule-1 (8 folds) bladder contents when compared to those in normal control rats. Administration of oleuropein induced a marked elevation in bladder reduced glutathione (37.8%), catalase (100.4%) with a prominent reduction of bladder nitric oxide (40%), tumor necrosis factor-alpha (35.9%) and vascular endothelial growth factor (56.2%) levels along with downregulation of intercellular adhesion molecule-1 bladder expression (73.1%) in comparison to cyclophosphamide treated rats levels. Our data demonstrated that oleuropein counteracts the harmful effects of cyclophosphamide on the bladder through its antioxidant and anti-inflammatory activities. Oleuropein exerts a definite uroprotective effect against cyclophosphamide induced hemorrhagic cystitis in rats.

Cisplatin-induced testicular toxicity in rats: the protective effect of arjunolic acid.

In the present study, the effect of arjunolic acid on testicular damage induced by intraperitoneal injection of rats with 7 mg/kg cisplatin was studied. Cisplatin induced a significant reduction in testicular weights, plasma testosterone, and testicular reduced glutathione levels in addition to a significant elevation of testicular malondialdehyde levels and testicular gene expressions of inducible nitric oxide synthase (iNOS), tumor necrosis factor-α (TNF-α), and p38 mitogen-activated protein kinase (MAPK) when compared with the control group (p < 0.05). Lower tubular diameters and depletion of germ cells and irregular small seminiferous tubules with Sertoli cells only were observed in the cisplatin group. Arjunolic acid administration significantly corrected the changes in both biochemical and histopathological parameters. Arjunolic acid plays a significant protective role against cisplatin-induced testicular injury by attenuating oxidative stress parameters along with downregulation of iNOS, TNF-α, and p38-MAPK testicular expressions.

Percutaneous nephrolithotomy versus open surgery for treatment of staghorn stones in pediatric patients.

INTRODUCTION: We compare percutaneous nephrolithotomy (PCNL) and open surgery in the treatment of staghorn stones in children. METHODS: We retrospectively reviewed the electronic records of children who underwent treatment for staghorn stones between September 2000 and August 2013. They were divided between Group 1 (patients who underwent PCNL) and Group 2 (patients who underwent open surgery). We compared stone-free and complications rates, need for multiple procedures, and hospital stay. RESULTS: The study included 41 patients (35 boys and 6 girls), with mean age 7.4 ± 3.1 years (range: 2-15). Of these 41 patients, 26 had unilateral renal stone and 15 had bilateral renal stones. The total number of treated renal units was 56: 28 underwent PCNL and 28 underwent open surgery. The complication rate was comparable for both groups (32% for open surgery vs. 28.6%, p = 0.771). Multiple procedures were more needed in PCNL group (60.7% vs. 32% in open surgery, p = 0.032). The stone-free rate was 71.4% after PCNL and 78.6% after open surgery (p = 0.537). A significant difference was observed in shorter hospital stay after PCNL (5 vs. 8.8 days, p < 0.001). Our study's limitations include its retrospective design and relatively small sample size. CONCLUSIONS: For the treatment of staghorn stones in children, PCNL was comparable to open surgery in complication and stone-free rates. PCNL had the advantage of a shorter hospital stay and open surgery showed a decreased need for multiple procedures.

Prune belly syndrome with urethral hypoplasia and vesico-cutaneous fistula: A case report and review of literature.

Association between Prune belly syndrome (PBS) and urethral hypoplasia is an unusual condition. It is usually fatal unless there is a communication between the fetal bladder and the amniotic sac. We report a case of PBS with urethral hypoplasia and congenital vesico-cutaneous fistula in a male neonate. Patient underwent cutaneous vesicostomy and was discharged for close follow up of his renal function and for future reconstruction.

Did antenatal diagnosis protect against chronic kidney disease in patients with posterior urethral valves? A multicenter study.

OBJECTIVE: To evaluate the prognostic role of antenatal diagnosis of posterior urethral valves (PUVs) on ultimate renal function. METHODS: Between 1990 and 2010, 315 patients with PUVs were diagnosed and treated at 2 separate tertiary centers. Primary valve ablation was performed in all patients except 18, who underwent initial vesicostomy. Patients were divided into two groups: group 1 included 144 patients who were diagnosed antenatally, and group 2 included 171 patients with a postnatal diagnosis. Long-term functional and radiologic outcomes were assessed. RESULTS: Follow-up was a median 5.5 years (range, 2-15 years). Mean age at ablation was 2.5 years (range, 1 day-15 years). Chronic kidney disease developed at the end of follow-up in 96 patients (30%): 27 (19%) in group 1 and 69 (40%) in group 2 (P <.05). The mean nadir serum creatinine was 0.6 and 0.8 mg/dL in groups 1 and 2, respectively, and the mean final serum creatinine was 0.9 and 1.7 mg/dL, respectively (P <.05). Persistent upper tract dilatation was noted in 43% of group 1 patients and in 69% of group 2 patients (P <.05). CONCLUSION: The potential for recovering renal function is believed to be significant in patients in whom early detection of PUVs and, hence, early intervention was performed. Antenatal screening and detection of these patients might play a significant role in protecting the upper tract and reducing the incidence of chronic kidney disease.

Critical analysis of outcome after open dismembered pyeloplasty in ectopic pelvic kidneys in a pediatric cohort.

OBJECTIVE: To evaluate the functional and morphologic outcome after open pyeloplasty for ureteropelvic junction obstruction (UPJO) in ectopic pelvic kidneys. MATERIALS AND METHODS: A retrospective review of all patients who underwent open pyeloplasty in ectopic pelvic kidneys was conducted. Records were evaluated with respect to age at presentation, preoperative imaging, surgical details, and postoperative course. Patients were followed up regularly for functional and morphologic outcome. Success was defined as symptomatic relief and radiographic improvement of obstruction at the last follow-up. RESULTS: Between 1995 and 2010, 680 patients with primary UPJO underwent open dismembered pyeloplasty at our center. Of these patients, 43 (6.3%) had UPJO in ectopic pelvic kidneys. No perioperative complications were encountered in the study group. Mean follow-up was 42 months (range, 18-90 months), and 5 patients were lost to follow-up. The overall success rate was 82.6%. Postoperative hydronephrosis was improved in 20 (52.6%), stable in 11 (29%), and worsened in 7 (18.4%). Postoperative renal function was improved in 12 (31.6%), stable in 19 (50%), and deteriorated in 7 (18.4%). Redo pyeloplasty was required in 4 patients and secondary nephrectomy in 3. Preoperative differential renal function and surgeon experience were statistically significant predictors of improvement in renal function after pyeloplasty. CONCLUSION: Open pyeloplasty for UPJO in ectopic pelvic kidneys is feasible, but varying degrees of hydronephrosis and radiologic obstruction persist after pyeloplasty that could be attributed to anatomy-related pelvocaliectasis, and so regular follow-up is warranted in this subpopulation.