Objective:To discuss the diagnosis and treatment 0f WAGR syndrome.Methods:The clinical data of 10 cases of WAGR syndrome children admitted to our hospital from January 2008 to November 2019 were respectively analyzed including the clinical features, diagnosis, and surgical treatments. There were 6 males and 4 females, aged from 13 to 36 months, with an average of 23.6 months. 9 cases were diagnosed as iris absence due to ocular abnormalities in infancy, and 1 case was diagnosed as iris absence due to ocular abnormalities by physical examination because of renal mass. There were 2 boys with cryptorchidism, and 2 boys with hypospadias, 1 of which did not received operation because of mild hypospadias, and another undergoing surgery. There were no abnormality of genitourinary system in the remaining 5 cases. There were 7 cases of unilateral nephroblastoma, with 1 case at the left and 6 cases at the right, and there were 3 cases of bilateral nephroblastoma. Abdominal doppler ultrasound and enhanced abdominal CT were performed for all patients. Abdominal doppler ultrasound indicated solid mass in renal parenchyma or non-uniform echo zone. Abdominal enhanced CT indicated renal tumor with diameter of 1.8 cm-12.7 cm and locally non-uniform enhanced echo. Among the 7 cases of unilateral nephroblastoma, 4 underwent nephrectomy, 1 underwent tumor enucleation, and 2 underwent tumor enucleation for unilateral tumor complicated with nephrogenic rests. There were 3 cases of bilateral nephroblastoma, 2 cases undergoing unilateral tumor enucleation firstly and contralateral tumor enucleation following chemotherapy. One case underwent unilateral tumor nephrectomy followed by contralateral tumor enucleation. One case of unilateral nephrogenic rests did not undergo renal tumor surgery. Preoperative chemotherapy was performed in 7 patients, including 3 bilateral nephroblastoma, 1 unilateral nephroblastoma combined with contralateral nephroblastoma, and 3 unilateral tumors larger enough to pass the midline. The chemotherapy regimen was VCR+ ACTD in 5 cases, VCR+ ACTD+ CTX+ DOX/CDDP+ VP16 and VCR+ CTX+ DOX in another 2 cases respectively.Results:All 10 cases were diagnosed as nephroblastoma. There were 3 patients without preoperative chemotherapy which belongs to COG stageⅠ(1 case) and STAGEⅢ(2 cases); Preoperative chemotherapy was performed in 2 patients with SIOP stage Ⅱ, 2 patients with SIOP stage Ⅲ, and 3 patients with SIOP stageⅤ. Nine children received regular chemotherapy after surgery, among which 1 child in stage Ⅰ received DD4A chemotherapy regimens, 2 children in stage Ⅱ received DD4A and EE4A regimen respectively, and 3 of the 4 children in stage Ⅲ received regular chemotherapy after surgery, including EE4A(1 case)and DD4A(2 cases). EE4A(1 case)and DD4A(2 cases) chemotherapy were performed in 3 patients with stage Ⅴ according to their unilateral tumor stage. Ten cases were followed up, with 9 of the 10 cases having no tumor recurrence or metastasis, and death in 1 case. At present, abdominal doppler ultrasound of 1 child with nephrogenic rests showed no obvious progress. The renal function of 9 children was not significantly abnormal during the regular follow-up. The results of intelligence screening showed that 6 of the 10 patients were significantly behind their peers, and 4 had no obvious abnormality compared with their peers. Gene tests were performed 3 times after surgery, and the results showed the deletion of 11p13 and adjacent distal genes.Conclusions:WAGR syndrome is rare in clinical practice, and renal ultrasound should be monitored after diagnosis to detect renal tumors in early stage. For bilateral cases, renal function should be preserved as long as possible in order to reduce the probability of renal failure. Long-term follow-up of nephroblastoma with this syndrome is particularly important.

Objective To discuss the diagnosis and treatment of unilateral ectopic ureter in children.Methods The clinical data of 41 cases of ureteral ectopic children admitted to our hospital from January 2014 to June 2018 were retrospectively analyzed including the clinical features,diagnosis,surgical treatments.There were 4 males and 37 females patients,aged from 0.4 to 12.7 years,with an average of 3.5 years old.Urinary incontinence was the main manifestation in 35 cases,including 14 cases with urinary tract infection.Preoperative ultrasonography and IVP examination were performed in all the 41 children.The dynamic radionuclide renal imaging was performed in the children who showed no renal inhencement with IVP.There were 27 cases of ipsilateral renal duplication and 9 cases of ipsilateral renal dysplasia.Ectopic fusion of kidney with ipsilateral ectopic ureter has one case on each side,and bilateral renal duplication with ectopic fusion of kidney with left ureteral ectopic in 1 case.The ipsilateral kidneys were normal in 2 cases.There were 27 cases with renal duplication,24 cases with upper renal dysplasia due to upper heminephrectomy,3 cases with well upper renal function,2 cases with lower superior ureteral pelvis anastomosis,and 1 case with ureterocystostomy.Laparoscopic dysplasia nephrectomy was performed on 9 patients with renal dysplasia,and nephrectomy was performed on 3 patients with renal dysplasia with ectopic renal fusion.Ureterocystostomy was performed in 2 cases with normal kidney.Results All 41 patients were followed up for 4-57 months,with an average of 25.3 months.Among the 35 children with urinary incontinence before the operation,33 cases had complete disappearance of urinary incontinence symptoms,and 2 cases had urgent urinary incontinence after the operation,presenting as frequent and small amount of urine discharge,with a strong sense of urination urgency.The micturition interval was shortened,ranging from 30 to 40min in the daytime,and 2 to 3 hours at night.The parents of the children were required to remind them to micturate regularly.Of the 41 cases,3 developed urinary tract infection 6-10 months after operation,and cured by antibiotics without recurrence.Conclusions Ectopic ureter is relatively rare,but urinary incontinence is the most common clinical manifestation.Ultrasound examination could be the preferred examination method.IVP further identified the patients with ectopic ureter who had kidney combined with malformation and renal function.The surgical treatments are mainly based on the corresponding renal function,and the prognosis is good.

Objective To investigate the timing and scheme of surgical treatment for the concomitant ureteropelvic junction obstruction(UPJO) and congenital abnormalities of the kidney.Methods The clinical data of 155 patients with concomitant UPJO and congenital abnormalities of the kidney from January 2006 to January 2016 was retrospectively analyzed.There were 107 males and 48 females,who aged 6 months to 16 years and 6 months.The average time was 5 years and 9 months old when they received operation.There were 8 cases less than 1 year old.There were 93 cases of UPJO on the left side,54 cases on the right side,and 8 cases on both sides.There were 33 cases with duplication of kidney,19 cases with solitary kidney,and 6 cases with renal dysplasia,6 cases with renal ectopia,12 cases with polycystic kidney disease,and 41 cases with dysplasia;2 cases with renal malrotation.There were 100 cases with symptoms such as fever,abdominal pain,vomiting.5 cases had received Anderson-Hynes pyeloplasty in other hospitals,2 cases received nephrectomy with symptoms did not relieve.4 cases were treated with nephrostomy in other hospital.Children with the repeated clinical symptoms,or renal function decreased significantly,or hydronephrosis progressive to the anteroposterior diameter of more than 30 mm received surgical treatment.Results There were 140 cases received Anderson-Hynes pyeloplasty,and 8 cases received nephrectomy with 5 cases were UPJO side and 3 cases were only abnormalities of the kidney without UPJO.All patients received IVP or ultrasonography postoperative 3-6 months,which showed hydronephrosis improved or no obvious change,and 4 cases were improved obviously.The IVP results showed that 5 patients with renal dysplasia together with UPJO had the renal function improved.There were 128 cases followed up for 12 to 106 months,with an average of 64.5 months.All patients had no clinical symptoms.83 cases were reexamined by IVP or ultrasonography,and hydronephrosis was getting better or no change.Conclusions The patients with concomitant UPJO and congenital abnormalities of the kidney don't need surgery in advance.The best choice for those patients is Anderson-Hynes pyeloplasty.The indication of nephrectomy should be considered carefully.

Objective To determine the efficacy and long-term outcome of pyeloureteroplasty in the ureteropelvic junction obstruction (UPJO) patients with poor kidney function(＜ 10％).Methods The data of UPJO patients with poor kidney function treated from January 2006 to September 2016,was retrospectively analyzed.The renal function ＜ 10％ undergoing pyeloureteroplasty were included.Ipsilateral vesicoureteric reflux,ureterovesical junction obstruction and renal dysplasia were excluded.39 patients was enrolled.There were 31 boys and 8 girls.The mean age at surgery was 3.1 years old (range 7 months-14 years).There were 36 primary UPJO and 3 extrinsic vessel cases.Twenty-seven cases of UPJO in left side,9 cases in right side,and 1 case in bilateral side.The preoperative examination included ultrasound,intravenous pyelography (IVU) and 99 mTc DTPA renography.Mean anteroposterior diameter of pelvis was (5.5 ± 2.4) cm before operation (range 3.4-7.4cm);IVU showed non visualized kidney or rim sign.The ill kidneys' mean renal function was (3.25 ± 2.78) ％ (range 0-9％).Results Open pyeloureteroplasty were performed in the 38 patients,additional nephrectomy was required in 1 because of repeated urinary infection,while the other patients had good prognosis,nephrostomy was kept until 2-3 days' successful clipping.Laparoscope pyeloplasty were performed in one patient with double-J.The patient had urinary infection post operation and was cured.The postoperative examination included ultrasound,intravenous pyelography and 99mTc DTPA renography were performed after 3-6 months.Mean anteroposterior diameter of pelvis was (3.2 ± 1.9) cm (range 2.1-4.5 cm);Intravenous urography showed visualized kidney;Mean renal function was (18.16 ± 13.17)％ (9％-27％).There was significant difference between preoperational and postoperational evaluation of renography and pelvis (P ＜ 0.05).There was no correlation between the changed renal function and age (P ＞ 0.05).The mean follow-up time was 10.2 months (range 6-25 months).Conclusions Pyeloureteroplasty could be safe and feasible for the UPJO patients with renal function less than 10％,and could improve the renal function.

Objective To investigate the experience of diagnosis and management of coexisting ureteropelvic junction obstruction (UPJO) and nonreflux megaureter (NRM).Methods The retrospective study of UPJO with NRM was based on 10 years information retrieved from January 2005 to December 2015.The data of 13 patients (8 males and 5 females) were available and recorded.Mean age at surgery was 3.7 years old (range 1.8 to 14 years).The diagnosis and mangement were summarized.Coexisting ureterovesical junction obstruction (UVJO) and vesicoureteral reflux,iatrogenic stricture and vesicoureteral reflux were excluded.Intravenous pyelography,voiding cystourethrography,ultrasound and CT reconstruction were performed before operation.Only six patients had an accurate diagnosis as UPJO with UVJO before surgery.Pyeloplasty was the initial surgical management choice for 10 patients,and ureteroneocystostomy in 3 patients.Results UVJO were diagnosed with pyelography techniques in 3 patients after pyeloplasty,while 4 were diagnosed as nonreflux and nonobstruction megaureter.Of the 10 patients who underwent initial pyeloplasty,additional ureteroneocystostomy was required in 3 and the prognosis was good.Additional pyeloplasty was required in 2 of the 3 patients who initially underwent ureteroneoeystostomy.Mean follow-up time from last operation was 23.3 months (6-53 months),the overall prognosis was good.Conclusions It is often difficult to correctly diagnose coexisting UPJO and NRM.In patients with UPJO,it is highly recommended nephrostomy radiography after pyeloplasty to evaluate the distal ureterovesical junction.Initial pyeloplasty is always recommended as first-line therapy.Additional ureteroneocystostomy was required when hydroureteropelvic was aggravated.

Objective To summarize the management of immature testicular teratomas in children.Methods The clinical data of 19 children (age between 16 days to 13 months) with immature testicular teratomas were retrospectively analyzed.There were 10 cases in left and 9 cases in right side.The main presentation was painless scrotal mass.The size of the tumor was 1.5 cm × 1.2 cm × 0.5 cm-6.0 cm × 5.0 cm × 4.5 cm.Abnormal alpha fetopmtein (AFP)concentrations were detected in 17 patients before surgery.Results Testis-sparing surgeries were done in 11 patients,and the other 8 patients underwent testicle resection because of tumor severely invading testis.Fifteen cases were followed up for 1-10 years.All of them were alive.Conclusions Immature testicular teratomas was rare and almost presenting in children younger than 1 year old.The biological behavior of immature testicular teratomas in children was benign.Testicular-sparing surgery was possible.Close follow-up should be done postoperatively.

Objective To summarize the experience about the diagnosis and treatment of bladder benign neoplasm in children.Methods A retrospective study was conducted for a total of 15 patients with bladder benign neoplasm from October 2006 to May 2016.There were 10 males and 5 females with a mean age of 8.7 years (ranging 1.1-13.8 years).The clinical manifestations of 15 patients included hematuria in 9 patients,frequent micturition with urgent and painful in 3 patients,dysuria in 1 patient,abdominal pain in 2 patients and headache during voiding in 1 patient.Ultrasound showed solid masses in the bladder with iso-echoic or nonhomogeneously hypoechoic.CT scanning showed regular or irregular mass with some enhancement in the bladder.All cases received tumor complete resection by opening operation and bladders were preserved.Among the 15 cases,neoplasms located in the anterior,posterior and lateral wall of bladder in 9 cases,ureteral orifice in 4 cases and trigone of bladder in 2 cases.The size of tumors ranged from 1.2 to 6.0 cm (mean 3.1 cm).The tumors were unifocal and seemed like papillary or cauliflower.The literatures of benign neoplasm of bladder were reviewed,which focused on the clinic characters,pathological classification and therapeutic method.Results Pathologic type included papilloma in 5 patients,inverted papilloma in 1 patient,inflammatory myofibroblastic tumor in 7 patients,hemangioma in 1 patient,pheochromocytoma in 1 patient.Fifteen patients were followed up for 6 to 36 months,mean 26.7 months.All patients recovered well without relapse or metastasis Conclusions Bladder benign neoplasm in children is rare with many kinds of pathological classification.The major clinical manifestation is gross hematuria while dysuria is unusual.Tumors are fewer in trigone of bladder.The best treatment is to resect the tumor completely with bladder preservation if possible.

Objective To investigate the characteristic of extrinsic vessel ureteropelvic junction obstruction (UPJO).Methods From January 2006 to September 2016,37 cases of extrinsic vessel UPJO were retrospective studied.The patients'mean age at surgery was 8.4 years old (range 3-14 years),with 33 boys and 4 girls (left UPJO 27 cases,right 10 cases).There were 3 cases found hydronephrosis during pregnancy and 29 had recurrent abdominal pain.Mean antemposterior diameter of pelvis was (3.1 ±1.2) cm,and renal parenchyma thickness was (0.6-± 0.4) cm.The preoperative ultrasound did not detcet extrinsic vessel,while intravenous pyelography (IVP) showed significant dilation of calyx in extrinsic vessel UPJO.Results All the patients were performed with pyeloureteroplasty,the ureter lumens were found stenosis in 13 cases by pathology,while the remaining cases were not.Mean follow-up time from last operation was 9.3 months (6 to 24 months).Only 1 patient had postoperative complication,which was urinary tract infection and cured by antibiotic therapy.No one need second operation.Three to 6 months after operation,the anteroposterior diameter of pelvis was (1.5-± 1.4) cm which was significantly lower than preoperative (P ＜ 0.05),and renal parenchyma thickness was (0.7 ± 0.33) cm,which was not significantly different with preoperative (P ＞ 0.05).IVP showed the patency of the anastomosis and good function of kidney.Conclusions The preoperative diagnosis of extrinsic vessel UPJO was difficult,the diagnostic rate of extrinsic vessel UPJO could be improved if we found the characteristics of abdominal pain history,ultrasound and IVP.Extrinsic vessel UPJO might be accompanied by the lumens stenosis,so pyeloureteroplasty was recommended.

Surgical treatment of pelvic fracture-associated urethral injury(PFUI) continues to be a difficult problem to be solved in urology.Children with an immature prostate and puboprostatic ligament may suffer from posterior urethral disruptions in a manner different from adults.Considering these distinctions,the management of pediatric PFUI presents a challenge for urologist as no consensus or algorithm has been proposed or accepted.Now,through analyzing the pathogenesis,presentation,diagnosis and treatment of PFUI,give the principle of the management.The choice of immediate repair or delayed repair should be decided according to the particular situation.Immediate Ⅰ stage anastomosis of urethra for PFUI is recommended when the patient's condition is stable,serious complications are treated,and the surgeon is experienced.If the patient's condition is unstable or no experienced urologist is available,a suprapubic catheter should be placed for bladder decompression and try to get a good condition for delayed urethroplasty.

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.