BackgroundThe literature on nephron-sparing surgery (NSS) in children with bilateral Wilms' tumors (BWT) involving the collection system is mostly comprised of case reports. The present study aimed to summarize the clinical characteristics, treatments, and prognosis of children with BWT involving the collecting system admitted to our pediatric surgery center compared with those whose tumors did not involve the collecting system. A secondary aim was to discuss how to preserve more kidney parenchyma and prevent long-term renal failure under the premise of preventing tumor recurrence.MethodsPatients with BWT admitted to our pediatric surgery center between January 2008 and June 2022 were reviewed. All included patients were grouped according to the relationship between the tumor and collecting system according to the intraoperative findings. Group I included children with tumor infiltrating the collecting system, group II included children with tumor growing into the collecting system, and group III included children whose tumor did not involve the collecting system. The clinical features, treatments and prognosis of the patients were analyzed.ResultsSeventy patients were enrolled, including 20 patients with 25 sides of tumors infiltrating the collecting system in group I,10 patients with 13 sides of tumors growing into the collecting system in group II, and 40 patients in group III. There was no significant difference in patients age and gender between group I and group II. In total, 20 patients in group I and 9 patients in group II had partial response (PR) after neoadjuvant chemotherapy. In group I, 22 of 25 sides of tumors underwent NSS; in group II, 11 of 13 sides of tumors underwent NSS. During an average follow-up of 47 months, in group I, 6/20 patients relapsed and 2/20 patients died; in group II, 3/10 patients relapsed and 1/10 patient died. There was no significant difference in 4-year overall survival (OS) rate among groups I, II and III (86.36% vs. 85.71%vs. 91.40%, P = 0.902).ConclusionsTo preserve renal parenchyma, NSS is feasible for children with BWT involving the collecting system. There was no significant difference in postoperative long-term OS between patients with BWT involving the collecting system and not involving the collecting system.

Background Hypospadias is a common congenital malformation in pediatric urology with surgery being the only curative treatment. Although there are hundreds of surgical methods for hypospadias, no single method can treat all types, and there are still high rates of postoperative complications. We performed this study to investigate surgical procedure selection and perform risk factor analysis of postoperative complications in hypospadias repair. Methods Retrospective analysis was performed of complete clinical and follow-up data of children with hypospadias who were treated and followed up at 15 children’s clinical centers in Mainland China from December 2018 to December 2019. Children were divided into groups according to Barcat classification and surgical methods in order to analyze the surgical choice for different types of hypospadias and the influencing factors of different surgical methods for complications. Results In total, 1011 patients were followed up for 26 months. According to Barcat classification, there were 248 cases of distal type hypospadias, 214 of intermediate, and 549 of proximal type. Transverse preputial island flap urethroplasty (Duckett) and tubularized incised plate urethroplasty (TIP) were performed in 375 (37.1%) and 336 cases (33.2%), respectively. The postoperative complication rate of distal hypospadias was 23.4% (15.8–57.1%), mid shaft 29.0% (22.7–40.0%), and proximal 43.7% (30.2–52.9%). Among the 375 patients in Duckett group, 192 had complications. Multivariate logistic analysis showed that the length of prepuce island flap ( OR = 3.506, 95% CI : 2.258 – 5.442) was an independent risk factor for complications after Duckett operation ( P < 0.001). In TIP group, there were 336 cases with 84 complications. Multivariate logistic analysis showed that the width of urethral plate after longitudinal resection ( OR = 0.836, 95% CI : 0.742 – 0.942) and glans width ( OR = 0.851, 95% CI : 0.749 – 0.965) were independent risk factors for postoperative complications after TIP ( P = 0.003, P = 0.012). Conclusion Several anatomical features play a role during the selection process among the different surgical approaches, including glans size, urethral plate width, and the meatal position. The width of the urethral plate and glans width were risk factors for postoperative complications after TIP. The length of prepuce island flap was a risk factor for complications after Duckett operation.

Background: Urethral plate transection (UPT) is crucial in hypospadias surgery. This study aims to develop a nomogram to predict UPT and assess the severity of hypospadias, to assist in choosing surgical techniques and improving preoperative parental counseling.Methods: We retrospectively reviewed the clinical data of hypospadias patients who underwent urethroplasty from 2018 to 2020 at the National Center for Children's Health (NCCH) and sixteen tertiary institutions in China. Data from NCCH were used to develop the prediction nomogram. The nomogram was internally validated by 10-fold cross-validation and externally validated by the multicenter cohort.Findings: A total of 584 patients in the NCCH cohort and 511 patients in the multicenter cohort were included. The UP width (odds ratio [OR]: 0·49; 95% confidence interval [CI]: 0·42–0·57), preoperative meatus position (OR: 2·65; 95% CI: 2·25–3·13), and preoperative curvature (OR: 1·08; 95% CI: 1·07–1·10) were selected to fit the nomogram (Plate-Meatus-Curvature, PMC model). The nomogram was well-calibrated. The receiver operating characteristic analysis illustrated that the area under the curve was 0·924 (95% CI: 0·845–0·998) in the NCCH cohort and 0·879 (95% CI: 0·845–0·998) in the multicenter cohort. Decision curve analyses revealed great clinical utility. Furthermore, we identified 120·0 as a cut-off value of nomogram points to discriminate patients between severe and non-severe groups. The UPT and postoperative complication rates in the severe group were significantly higher than those in the non-severe group (P < 0·001).Interpretation: The PMC model showed good performance for predicting UPT in hypospadias surgery. Furthermore, we identified a cut-off point for describing the severity of hypospadias, which correlate with postoperative outcomes.Funding: This work was supported by grants from the National Key R&D Program of China (2016YFC 1000807).Declaration of Interest: None to declare. Ethical Approval: The study was approved by the ethics committee of Beijing Children's Hospital, Capital Medical University, National Center for Children's Health (IEC-C-006-A04-V.06, [2022]-E-030-R).

Abstract Purpose To find the cut-off value of urethral defect length (UDL) to guide the choice of single‑stage or two‑stage transverse preputial island flap urethroplasty (TPIFU). Methods We prospectively collected the data of patients with severe hypospadias underwent single-stage and two-stage TPIFU at fifteen tertiary referral institutions from 2019 to 2020. The receiver operating characteristic (ROC) analyzed the cut-off value of UDL to predict postoperative complications with single-stage TPIFU. We extracted patients with long UDL (longer than the cut-off value) in the single-stage and two-stage TPIFU, comparing the postoperative complications. Results A total of 536 patients included 483 underwent single-stage TPIFU and 53 underwent two-stage TPIFU were collected. The ROC analyzed the cut-off value of UDL was 3.5 cm. 169 patients with long UDL (longer than 3.5 cm) were extracted from all 536 patients, with 126 underwent single-stage TPIFU (single-stage group) and 43 underwent two-stage TPIFU (two-stage group). In the single-stage group, complications occurred in 62 (49.2%) patients, including 34 (27.0%) with urethrocutaneous fistula, 21 (16.7%) with urethral stricture, and 25 (19.8%) with diverticulum. In the two-stage group, total complications occurred in four (9.3%), there were both two (4.7%) with urethral stricture and diverticulum, and no urethrocutaneous fistula was recorded. Conclusion Our results show that two-stage TPIFU can significantly reduce the incidence of postoperative complications for hypospadias with UDL longer than 3.5 cm, especially urethrocutaneous fistula and diverticulum.

INTRODUCTION:Laparoscopic pyeloplasty (LP) has been widely used in the treatment of pediatric ureteropelvic junction obstruction (UPJO). However, no prior reports with a large pediatric series have focused on the analysis of complications and impact factors of the outcomes. We hypothesized there were risk factors of higher Clavien grade postoperative complications. OBJECTIVE:To analyze the characteristics of complications and risk factors of high Clavien grade postoperative complications. PATIENTS AND METHODS:All children with UPJO treated with primary transperitoneal LP between July 2016 and July 2018 were retrospectively reviewed. The Clavien complication grades in groups with different weight, intraoperative complication (drainage methods), anteroposterior pelvic diameters (APPD), side, gender, title of surgeon, preoperative presentation and obstruction reason were compared. RESULTS:Of the 279 children, intraoperative complications in which the placement of double-J stents was not accomplished and conversion to open surgery (Satava grade II) occurred in 17 (6.09%) and 2 (0.72%) patients, respectively. A total of 270 patients (277 kidneys) were included in the analysis of postoperative complications. Postoperative complications occurred in 51 (18.89%) patients. The most frequent postoperative incident was febrile UTI in 27 patients. Of the 13 patients who required reoperations, 6 patients had kidney restenosis and were considered as failure of surgery. All complications with an exact onset time occurred within 10 months after surgery. In the univariate and multivariate analysis, weight <10 kg and having intraoperative complication with nephrostomy tube were risk factors of higher Clavien postoperative complication grade (P＜0.05). DISCUSSION:Patients who were <10 kg in weight and having intraoperative complication with nephrostomy tube had a greater risk of a higher Clavien grade postoperative complication. To reduce high Clavien grade postoperative complications, asymptomatic patients under 10 kg in weight and having intraoperative complication with nephrostomy tube need close monitoring. In the 26 patients who had an exact time of the postoperative complications, the longest time we found was 10 months. Thus, we recommend the follow-up time required to observe postoperative complications in patients should be at least 10 months after surgery. CONCLUSIONS:LP has been proven to be safe and effective in children with a low rate of complications. Weight <10 kg and having intraoperative complications with nephrostomy tube were risk factors of higher Clavien grade postoperative complications. Children with low weight and intraoperative complications need more attention in terms of the occurrence of complications.

To evaluate the effect of modified penoplasty in the management of concealed penis.

Objective To compare the treatment efficacy of severe hypospadias between two-stage urethral plate reconstruction procedure and two-stage tubularized transverse preputial island flap procedure. Methods From 2010 December to 2014 December, we retrospectively analyzed 82 cases with severe hypospadias, using two-stage urethral plate reconstruction and two-stage tubularized transverse preputial island flap urethroplasty, respectively.Their mean age in the first operation was 2.4 years, ranged from 2 to 3 years.Hypospadias were penoscrotal type in 32 cases, perineal type in 50 cases.In group A, 42 cases, including penoscrotal type in 15 cases, perineal type in 27 cases, with urethral plate reconstruction procedure underwent two-stage urethroplasty.The transected ventral penile urethral plate and to strip the ventral fibrous tissue were needed in correction of chordee of penis.The patients in group A were underwent the second urethroplasty with urethral plate reconstruction procedure 6-12 months later.In group B, 40 cases,including penoscrotal type in 17 cases, perineal type in 23 cases, who underwent one stage operation in the correction of chordee of penis, only with tubularized preputial island flap forming the part of the urethra.The patients in group B were underwent the second urethroplasty 6 ~12 months later.Results Patients were followed up for 1 to 5 years, mean 3.5 years.No recurrence of penis chordee was noticed.In group A, 6 cases (14.3%) had postoperative fistula.The second fistula repairing was perform 6 to 12 months after the second stage operation.However, still 3 cases were noticed the fistula recurrence, which was cured after the fourth operation.In group B, 3 cases (7.5%) developed fistula, which appeared in the second urethroplasty (P<0.01).They were all treated after the second repairing procedure.In group A, 4 cases were noticed urethral stricture, the urethral stricture rate was 9.5%.The stricture occurred 1 to 3 months after the second operation.After dilation and indwelling catheter, 3 cases resolved the problem. Another case still found the recurrence after 2 months, which the one stage urethral plication and second urethral reconstruction were considered to treat this patient.In group B, 1 cases of urethral stricture, the urethral stricture rate was 2.5%(P<0.01).After dilation and indwelling catheter, this case resolved the problem.In group A, 3 cases had urethral diverticulum, which occurred at a rate of 7.1%, whereas without urethral diverticulum occurred in group B (P<0.01).Those patient with urethral diverticulum accepted the diverticulum removing procedure 3 to 6 months after the second stage procedure.One case was found the urethral fistula, which was treated one year later.The success rate of operation was 69.0%in group A, the success rate of the operation was 90.0%in group B.The difference of the success rate between two groups was statistically significant ( P <0.01 ) . Conclusions With respect to two-stage urethral plate reconstruction procedure in treatment of severe hypospadias, there were the low complication rates of postoperative urinary fistula, urethral stricture and diverticulum in two-stage tubularized transverse preputial island flap procedure.Urethral meatus could be done at the glans of the penis.In addition, the two-stage tubularized transverse preputial island flap procedure provides a good opportunity to practice to master complex hypospadias operation.

Objective To improve the diagnosis and treatment of traumatic pelvi-ureteric junction (PUJ)disruption in children.Methods A retrospective study was conducted for a total of 31 patients with traumatic PUJ disruption from 1993 to 2014.Their clinical records were reviewed for mechanism of injury,diagnostics and treatment.Results There were 23 males and 8 females with a mean age of 6.25 (1.5-10.5) years.The lesions were right (n=18) and left (n=5).And the causes were traffic accident (n =30) and penetrating injury (n =1).For 5 cases of initially presenting at our hospital,the mean interval from trauma to diagnosis was 12.5 (5-20) days.For the remainder transferred to our hospital beyond 24 h,the mean interval from trauma to diagnosis was 42(20-90) days.Pelvi-ureteric reanastomosis and caliceal ureterostomy were performed for 21 and 5 patients respectively.And ileal and appendix replacement for ureteral injuries were finally performed for 2 and 1 patient respectively.Two patients underwent nephrectomy.Hydronephrosis of injured kidney lessened and function improved in 25 patients.Conclusions PUJ disruptions are uncommon and more likely to have a delayed diagnosis because of associated injuries.Haematuria is not indicative of ureteral trauma in children.Computed tomography (CT) with delayed excretory phase imaging may aid its diagnosis.And a definite diagnosis is made by retrograde pyelography under anesthesia.Definitive ureteral reconstruction is preferred with pelvi-ureteric reanastomosis or nephrostomy.Drainage of circumrenal urinoma and nephrectomy should be avoided.

尤因肉瘤／原始神经外胚层肿瘤（ Ewing sarcoma／primitive neuroectodermal tumor ， ES／PNET ）是起源于原始神经外胚层、高度恶性的小圆细胞肿瘤。 ES／PNET 主要发生于胸壁、四肢及脊柱两侧软组织，偶见发生于泌尿生殖系，如肾脏、膀胱、子宫等［1］，罕见发生于输尿管。我院收治1例原发性输尿管ES／PNET，现报告如下。

Objective To investigate the clinical manifestations and treatment of cystic nephroma (CN).Methods A retrospective study was conducted on our 7 patients with CN.The data collected from the medical records of these patients concerning their clinical presentation,imaging findings and treatment were analyzed.Results Three patients (one with bilateral CN) underwent enucleation of the tumors with nephron-sparing surgery and 4 patients underwent nephrectomy.During a follow-up of 6 months to 6 years,the patients were well without recurrence.Conclusions CN is a rare benign disease of the kidney.Imaging features are not specific enough to differentiate CN from cystic partially differentiated nephroblastoma.Nephron sparing resection of tumor is a primary and effective therapeutic method for this disease.

Objective To study the blood vessels distribution of foreskin in children with hypospadias and vesselpedicle, aiming to evaluate and recognize blood supply after application of urethra reconstruction materials. Follow up study was conducted after surgery and early complications were investigated to evaluate the correlation between blood vessel distribution of foreskin and early complications, as well as the therapeutic efficacy. Methods Duckett and Duckett + Duplay procedures were performed for the children with hypospadias. The cold lighting transmission techniques was utilized to record and evaluate the distribution of blood vessels in foreskins, and followed by summarization and analysis. The follow up was conducted for patients. Results Various blood vessels distribution could be found in superficial fascia in foreskin, in which simple main branch blood vessel type was 42.4 percent, double main branches type was 34.7 percent, three main branches types in them was 13.6 percent, and multi-branches type was 2.5 percent. Furthermore, 6.8 percent of them were not found to have branch blood vessel. Among all cases with branches blood vessels, there were no correlations between the number of branch blood vessels and incidences of fistula or stricture. Conclusions Blood vessels distribution could be classified into 5 types according to the number of branch blood vessels in superficial fascia in foreskin, and no correlations between blood vessel distribution type and severity of hypospadias. The distribution of blood vessel in foreskin has less effect on the operative efficacy.

Objective To evaluate the therapeutic efficacy of diagnosis and treatment for renal tumor associated with intravascular tumor embolus in children.Methods From 1996 to 2010,total 15patients suffering from renal tumor associated with intravascular tumor embolus underwent treatment in out center,which had extent into the inferior vena cava (IVC) or atrium.Their clinical information was reviewed.Results The most common clinical signs in patients were abdominal mass and hematuria.Symptom that might have been related to the intravascular extension identified was in one patient was considered to be correlated to associated intravascular tumor embolus.15 patients were diagnosed via CT,in which 14 of them were further confirmed via ultrasound simultaneously.13 patients underwent preoperative chemotherapy with dactinomycin and vincristine for 4 to 8 weeks,and regression of intravascular tumor embolus was noted in 6 of them.The resection of tumor was successfully performed in all patients,as well as intravascular tumor embolus,among them cardiopulmonary bypass was conducted for removal of thrombus in one patient.No intraoperative death and perioperative complications were found in this study.The patients received chemotherapy and irradiation according to NWTS4 staging standard and pathological sub-types.9 patients were followed up and 8 patients survived without tumor embolus at present.Among them 1 patient had survived for 5 years since operation.Conclusions Nephrectomy combining with resection of thrombus is the effective approach to treat Wilmg’tumor patient associated with intravascular tumor embolus.

BACKGROUND:The delayed diagnosis of pelvi-ureteric junction (PUJ) disruption in children following blunt abdominal trauma can result in loss of function of the involved kidney. We examined the potential for kidney preservation and the limits of diagnostic delays.METHODS:A retrospective review of 17 cases of PUJ disruption at Beijing Children's Hospital from 1993 to 2009 was done with respect to diagnosis, treatment and follow-up.RESULTS:The interval from trauma to diagnosis of PUJ disruption was (52 ± 52) days. If one case with nephrectomy was excluded, the interval from trauma to diagnosis was (40 ± 20) days. The average time between injury and first treatment was (49 ± 25) days. Pelvi-ureteric reanastomosis and caliceal ureterostomy were performed separately in 11 and 4 patients, respectively. Ileal replacement for ureter injuries was finally performed in one patient. Hydronephrosis of the injured kidney was reduced and the function improved in 15 out of 17 patients (88%). Only one patient received nephrectomy and the nephrectomy rate was 5.9%.CONCLUSION:Differential renal function at the PUJ disruption side can be saved and the rate of nephrectomy reduced by appropriate surgery if the time to diagnosis and first treatment is limited to within two months.

Objective To investigate the diagnosis,treatment and prognosis of nephroblastomatosis (Nbm) combined with Wilms'tumor (WT). Methods Clinical data of six patients treated for WT combined with Nbm in Beijing Children's Hospital from 2006 to 2010 were reviewed retrospectively.The patients'ages ranged from five to 14 months.Two of the patients were female and four were male.The WTs were left-sided in four cases and right-sided in two cases.The Nbms were ipsilateral with WT in three cases,contralateral in two cases and bilateral in one case. The Nbms were single In three cases and multiple in three cases.WTs were all single and the maximum diameter was 3,4,8,10,11,and 12 cm respectively.Two paitents underwent nephrectomy.Nephron sparing surgery and upper and lower pole nodule biopsy was conducted in two cases,Nephrectomy and contralateral nephron sparing surgery was conducted in an additional two cases.Adjuvant chemotherapy included vincristine,actinomycin and doxorubicin. Results One patient had tumor recurrence 33 months after a 15 month regimen of postoperative chemotherapy. One patient had tumor recurrence and died after nephron sparing surgery 5 months after a 11 month regimen of chemotherapy.Four patients underwent 6 months of chemotherapy,and it was 9,12,and 21 months respectively after stop of chemotherapy.Another patient was still in chemotherapy. Conclusions Nbm is a pre-neoplastic proliferative process with high risk of developing WT.Chemotherapy may reduce the rate of Nbm malignancy.If Nbm is malignant or chemotherapy is invalid,nephron sparing surgery is recommended.

Objective To evaluate the outcomes of modified Duplay urethroplasty for hypospadias reoperation. Methods Records of 27 patients with failed hypospadias repair underwent Modified Duplay urcthroplasty in Beijing Children's Hospital from 2004-2009 were reviewed. The mean age was 10 years and 5 months (3-17 years). All patients had underwent 1-5 operation. The surgical results and postoperative complications were retrospectively analyzed. Results The patients were followed-up for 6-24 months. Sixteen cases were cured in 27 cases repaired by modified Duplay procedure. Eleven cases had postoperative complications, among of them, fistula in 10 and stenosis in 1. Eight cases of fistula were repaired successfully by one additional surgery. Another 2 fistula and 1 stenosis required two additional procedures. Eighteen cases were cured in 29 Duplay repaies. There was no significant difference between two groups (P＞0. 05). Conclusions Modified Duplay urethroplasty is an efficient technique for failed hypospadias repair especially for the patients with urethral plate width of glans ＜8 mm. But local tissue of coronary sulcus should be adequate quality to repair.

Objective To discuss the management of the congenital vesicoureteral junction obstruction (UVJO). Methods A retrospective cohort study was performed of patients who underwent ureteral reimplantation due to UVJO between 2003 and 2008. Of the 73 patients with 83 ureters (male 49 and female 24, age range 8 months to 13 years, median 41 months). Forty-one cases were on the left, 22 were on the right and 10 were bilateral obstruction. The most common presenting symptoms were intermittent abdominal pain and urinary infection. All patients were evaluated preoperatively by ultrasound, voiding cystourethrogram, intravenous pyelogram and diagnosed as UVJO. Reimplantation was done by the Cohen technique in all patients. Results Follow-up of 46 patients ranged 0.5-3.5 years postoperatively, including ultrasound, voiding cystourethrogram, intravenous pyelogram. Hydronephrosis improve in 41 patients, had no change in 5 ureters. Conclusions Symptomatic children or advanced hydroureteronephrosis are definite indication for surgical treatment of UVJO patients. Surgical management could be effective for most of patients.

Objective To evaluate the long-term functional results, complications and patient's satisfaction level in patients performed augmented enterocystoplasty and continent urinary diversion using the appendix. Methods From 1999 to 2005, there were 22 children (12 males and 10 females) underwent augmented enterocystoplasty and continent urinary diversion using the appendix. Surgical results were reviewed retrospectively. There were 11 eases with bladder and urethra dysfunction attributed to neurogenic bladder, 2 cases with complex genitourinary malformation associated with an imperforated anus, 2 cases with exstrophy-epispadias complex, 2 cases with posterior urethral valves, 3 cases with failed urethrovaginal fistula repair and 2 epispadias cases with post failure of Young-Dees- Leadbetter bladder neck reconstruction. Upper urinary tract dilatation and hydroureteronephrosis were found in 17 eases (28 units), including grade Ⅱ-Ⅴ vesicoureteral reflux in 15 children (24 units). Simultaneous procedures included ureteral reimplantation in 15 cases and bladder neck closure in 14 cases. The appendix was used as the catheterizable conduit placed in the right lower abdomen and clean intermittent catheterization was performed in all patients. Outcomes were assessed by urodynamic study, IVU, ultrasound, voiding cystourethrography, BUN, Cr and electrolyte test. Results Mean follow-up was 3.6 years, ranging from 1.5-6 years. Complications included stomal stenosis requiring dilatation in 2 cases and leakage in 2 cases without bladder neck closure and 1 case required surgical revision. All patients achieved excellent stomal continence. No metabolic acidosis and bladder stone was noted. Upper urinary tract impairment had not worsened in all patients. Conclusion Augmented enterocystoplasty and continent urinary diversion using the appendix are associated with high continence, compliance and satisfaction rate and a low complication rate in the treatment of pediatric patients with disorders related to bladder and urethral dysfunction.