尿道下裂是小儿泌尿生殖系最常见的先天性畸形之一，手术是唯一治疗方法，手术方法操作复杂，难度大，对于初学者掌握有难度。通过总结我院泌尿外科医生学习尿道下裂手术的过程，探讨小儿泌尿外科医师尿道下裂的学习曲线。

Objective To discuss the unique biological,histological and clinical features of pediatric renal cell carcinoma (RCC).Methods A retrospective review and biological analysis of all RCC cases presenting to our hospital from January 1973 to March 2012 was undertaken.Results Twenty-nine RCC pediatric patients (16 boys,13 girls) with mean age of 9.6 (range 2.5-16.0) years were identified.The presentations included hematuria in 17 (58.6％) cases with 3 who developed hematuria after trauma,abdominal mass with hematuria in 3 (10.3％),abdominal mass in 3,abdominal pain in 2,abdominal pain with hematuria in 1,and incidentally finding in 3.The diameter of tumor was from 2.5 cm to 25.0 cm,mean 6.8 cm.According to TNM stage grouping system,16 cases were stage Ⅰ,10 stage Ⅲ,and 3 stage Ⅳ.Xp1 1.2 translocation RCC was identified in 21 patients,clear cell RCC 6,papillary RCC 2.Of the 29 cases,3 patients with the tumor less than 7-cm had nephron-sparing surgery.A 15-cm tumor was incompletely removed in 1 patient and another patient with a 25 cm× 18 cm×15 cm tumor had gross residual.Nephrectomy was performed for the affected kidney in the remaining 24 patients.Twenty-one patients (Xp1 1.2 translocation RCC 13 patients,clear cell RCC 6,papillary RCC only 2) were followed up from 1.5 to 34.0 years,18 were living well (T1N0M0 in 11 cases,T1N1M0 in 2,T2N1M0 in 3,T3N1M0 in 1 and T4N1M1 in 1) and 3 died of recurrence.Conclusions Although RCC is rare in children,pediatric RCC behaves in a distinct fashion compared with adult forms of RCC.Hematuria is the main symptom in pediatric RCC.Xp11.2 translocation RCC is the predominant form,associated with an advanced stage at diagnosis.Nephrectomy is the common treatment for RCC and nephron sparing surgery could be a reasonable option for patients with tumor smaller than 7 cm.For localized RCC (T1-2 N0-1 M0),simple kidney removal surgery is sufficient for treatment without lymph node dissection and postoperative adjuvant treatment.

Objective To review the surgery for hypospadias combined with ventral penile curvature,and to discuss the application of dorsal tunica albuginea plication for residual hypospadias curvature.Methods From Jan 2006 to Dec 2010,there were 746 cases of hypospadias repamy ired by Duckett's procedure.The average age at operation was 3.1 years old.270 cases of residual hypospadias curvature were treated with dorsal tunica albuginea plication technique to straighten the penis.The surgical procedure was as follows:The urethral plate was cut,the dysplastic fascial band around the ventral tunica albuginea was partially excised.Erection test by normal saline injection through a butterfly needle into one corporal body was done.Residual hypospadias curvature was treated with the dorsal tunica albuginea plication after the neurovascular bundle was carefully dissected free.Follow up was done for 6 months after the operation.Results There were 611 cases treated by Duckett's procedure with 207 cases of dorsal tunica albuginea plication.135 cases of Duckett + Duplay's procedure were done with 63 cases of tunica albuginea plication.In the 270 cases of dorsal tunica albuginea plication,there were 56 cases of complications,and in 476 cases of no plication,there were 120 cases of complications.In the cases of tunica albuginea plication,cases of penile type were less than penoscrotal or perineal type (P＜0.001).The cases of Duckett group were less than the group of Duckett +Duplay.Compared with group of tunica albuginea plication and group of no plication,the outcome of surgery showed no significant difference statistically.Conclusions Dorsal plication of the tunica albuginea is a simple and effective method for correcting residual hypospadias curvature.This technique prevents damage to the neurovascular bundles and erectile function.

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.

交叉异位融合肾是指一侧肾脏由原位跨过中线移位到对侧并与对侧肾融合,而输尿管人膀胱位置仍位于原侧[1].尸检发病率1/2000[2].可终生无症状,儿童期多因合并泌尿生殖系、骨骼等其他畸形被发现,发生率约为1/10000[3].复习近10年国内外文献均以个案报道该病[2.4-10].自2009年1月至2011年9月我院收治3例交叉异位融合肾合并复杂泌尿系畸形患儿,报告如下.

Objective To evaluate the management and therapeutic efficacy of bilateral Wilms tumor. Methods From 1997 to 2010, total 315 patients with Wilms tumor were treated in our hospital and 11 cases (3.5％) of them were bilateral, including 8 cases synchronous tumor and metachronous tumor in 3 cases. The mean age in synchronous cases during primary diagnosis was 17 months (ranging 10 months to 2 years) ; the age in metachronous cases was respectively 4 years, 4 years and 3 months, and 20 months. The metachronous tumor was found via ultrasonography in follow up period after unilateral nephrectomy. There were 7 boys and 4 girls in all cases. 4 children had associated congenital abnormalities, including absent of left testis in 1 case, incompletely descended right testis in 1 case, and hypospadias in 1 case, as well as aniridia in 1 case. All 11 cases were favorable histological features. In synchronous cases, one associated with bilateral and one with right sided nephroblastomosis. All the patients underwent pre-operative chemotherapy (vincristine-VCR dactinomycin D-ACTD)for at least 4 weeks. Staged bilateral tumor enucleation was the main procedure. After surgery VCR +ACTD + adriamycin protocol were conducted for 15 months. Follow-up time was more than 5 years. Results 8 children were followed-up for average 4.2 years (ranging 20 months to 14 years) and they were alive and healthy. One metachronous was loss to follow-up. one child received right sided nephrectomy and at the mean time exploration on the left side, died of anuria postoperatively. 1 case (female, 11 months old) died of tumor recurrence. Conclusions The age of patient with bilateral Wilms tumor was more younger. Majority of them has favorable histologic features. Preoperative chemotherapy is an effective way to reduce tumor volume before staged procedure as it can help to preserve functional renal tissue. The associated congenital abnormalities and nephroblastomosis seem not increased complications.

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 investigate the diagnosis and treatment of horseshoe kidney with hydronephrosis in children.Methods The clinical data of 19 children with horseshoe kidney with hydronephrosis were analyzed retrospect.Results From 1997 to 2008,19 children(16 were male and 3 female,ages ranged from 8 months to 16 years,mean 7 years) with horseshoe kidney associated with hydronephrosis under went operation in our hospital.Crossed renal vessels were a main etiology.Intravenous pyelography(IVP) indicated that hydronephrosis was reduced 6~12 months after the operation,abdominal pain disappeared and no abnormalities were found during 6 months to 14 years follow-up periods.Conclusion Horseshoe kidney with hydronephrosis is more common in males,occuring much more commonly in school age children.Pyeloplasty performed by the transposition of crossing vessels and the Anderson-Hynes method showed satisfactory results.

目的 比较先天性前尿道瓣膜及憩室对上尿路的损害,分析二者是否同一疾病不同表现的可能,并了解其对上尿路的损害.方法 回顾性分析本院1990年1月至2009年12月收治的先天性前尿道瓣膜(52例)及憩室(26例)患儿临床资料,比较其临床表现、手术方式及术后恢复情况.两组间率的比较采用x2检验,P＜0.05为差异有统计学意义.结果 20例(38.5%)前尿道瓣膜患儿及38例(30.8%)前尿道憩室患儿存在不同程度膀胱输尿管返流,前者20例(38.5%)及后者12例(46.2%)存在上尿路积水.两组在输尿管反流发生率及程度、上尿路积水方面比较,差异无统计学意义(P＞0.05).前尿道瓣膜患儿存在膀胱憩室和(或)成小梁改变者明显多于前尿道憩室患儿(P＜0.05).11例前尿道瓣膜及6例前尿道憩室患儿接受了2次或2次以上手术,其中5例行膀胱输尿管再植抗反流.存在输尿管反流的11例前尿道瓣膜和4例憩室患儿获随访,随访时反流消失或不超过3级.结论 本研究中,前尿道瓣膜和憩窒患儿上尿路积水和膀胱输尿管反流情况无明显差异,支持二者是同一疾病不同表现形式的判断.对于存在膀胱输尿管反流的患儿,当下尿路梗阻解除后,若反流仍达4级以上,可以考虑采取抗反流手术.

Objective To evaluate the outcomes of tubularized transverse preputial island flap urethroplasty (Duckett)for the repair of hypospadias. Methods We reviewed 558 hypospadias repairs including 457 Duckett and 101 Duckett + Duplay repairs performed between 2006 and 2008. The mean age was 3. 4 years (range: 1-8 years). The surgical results and postoperative complications were retrospectively analyzed. Results We followed-up the patients for 12-36 months. 336 cases were cured in 457 hypospadias cases (73. 5%) repaired by Duckett procedure. 121 patients had postoperative complications, among of them, fistula in 61, stenosis in 10, diverticulum in 42, fistula + diverticulum in 8. 74 cases were cured in 101 Duckett + Duplay repairs (73.3%). 27 patients developed complications, including fistula in 13, stenosis in 6, diverticulum in 5, fistula + diverticulum in 3. There was no significant difference between two groups (P＞0. 05). 108 case of Duckett and 18 cases of Duckett + Duplay complications were repaired successfully by one additional surgery. Another 21 cases required two additional procedures. Conclusions Tubularized transverse preputial island flap urethroplasty provide satisfactory results for hypospadias with chordee.

目的 介绍保留阴蒂背侧血管神经柬的阴蒂短缩、阴蒂头成形术.方法 回顾性分析2000年至2008年本院51例行阴蒂短缩、阴蒂头成形术患儿的临床资料.结果 51例患儿手术年龄1岁至1岁4个月,平均3岁5个月.其中先天性肾上腺皮质增生症40例,真两性畸形8例,混合性腺发育不全3例.手术方法:保留阴蒂背侧血管、神经束,阴蒂缩短、阴蒂头成形,重塑女性外阴外形.术后伤口一期愈合,阴蒂头无坏死,随访0.5～7年,阴蒂外观满意.结论 保留阴蒂背侧血管神经束的阴蒂短缩、阴蒂头成形术符合阴蒂的解剖生理特点,术后女性外阴形态满意.

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 outcomes of surgical repairments for residual rectourethral fistula in patients after anorectoplasty for imperforate anus.Methods From July 2005 to March 2008, 28 patients with residual rectourethral fistula after anorectoplasty underwent surgical repairments in this institution. Of which 22 patients with good anal appearance and function underwent anterior sagittal rectourethral fistula repair and anorectoplasty, 5 patients with severy rectum retraction and 1 patient with antelocation of anus underwent posterior sagittal rectourethral repair and anorectoplasty.Results In patients with residual rectourethral fistula, the urethral end of fistula usually located at the membranous urethra. The average length of fistula was 0.8±0.3cm. Among the 28 patients, successful fistula repair occurred in 27 cases on the first attempt. Twenty five of the 27 patients were follwed up, on which no urethral stricture or diverticulum was found. The anal functions of 21 patients were graded exellent, and 4 were good according to anal function scoring criterion. The post-operative anal functions were significantly improved compared to the pre-opetative anal functions (P＜0.05). The operation time of the anterior sagittal rectourethral fistula repair and anorectoplasty were 72.8±11.2 min, which was significantly shorter than that of the posterior procedure (105.6±14.6min) (P＜0.05).Conclusions Both of the anterior and posterior procedure for rectourethral fistula repairment improve the anal functions successfully. A wider and clearer operative field can be gained via the anterior procedure, which brought convenience and shortened the operation time.

Objective To improve the diagnostic accuracy and therapeutic effect of paratesticu-lar rhabdomyosarcoma (PARA RMS). Methods Fourteen patients diagnosed with PARA RMS from 1977 to June 2008 were retrospectively analyzed. The patients aged from 1 to 15 years, with the mean age of 6 years. Of all the cases, 6 were younger than 5 years. Nine sinistral and 5 dextral PARA RMSs were diagnosed. All patients manifested as scrotum swollen and diagnosed with PARA RMS at stage Ⅰ by roentgenographic findings. Of all the patients, 10 underwent radical inguinal orchiectomy, and 4 patients who had undergone orchiectomy ( 1 case) or tumor enucleation (3 cases) through a trans-scrotal incision in other hospitals underwent inguinal exploration with removal of the testis or the remaining sperrnatic cord. Excision of the scar of scrotal incision were also performed on the 4 above-mentioned patients. Thirteen cases were diagnosed as the spindle cell type of embryonic RMS and one the as the gland alveolar type. Chemotherapy (VAC, vincristine + actinomycin + cyclophosphamide) was performed on all patients. Results Thirteen of 14 patients were followed up for 1 to 24 years (mean, 6 years). Eleven patients were followed up for at least 2 years, including 10 (91%) survivors without tumor recurrence. For the other one patient with the age of 8 years, who underwent only 6 periods of chemotherapy and whose parents gave up the following chemotherapy, had the recurrence of a retroperitoneal metastatic tumor with the diameter more than 8 cm at 2 years postoperatively. A 9 year old patient who was admitted in 1981 had no sperm in his semen at follow-up. Conclusions PARA RMS often shows as a unilateral scrotal swelling or mass, thus it could be diagnosed earlier than other genitourinary tumors. Most cases of early diagnosed with PARA RMS are at stage Ⅰ, with the pathologic diagnosis of spindle cell type which indicated a good prognosis. The curative rate for pa-tients in stage 1 can be up to 90% after radical inguinal orchiectomy combined with VAC chemothera-py. In recent years, only VCR+ ACTD is suggested for type 1 PARA RMS for 1 year, to avoid infer-tility.

目的 探讨小儿肾盂输尿管交界部迷走血管压迫致梗阻性肾积水的临床特点及治疗方法.方法 回顾性分析1997年1月至2008年6月本院收治的34例迷走血管压迫性肾积水患儿的临床资料. 结果34例中.男30例,女4例,左侧28例,右侧6例.平均发病年龄9.5岁.手术方法为离断性肾盂成型术(Anderson-Hynes术式),术中同时将迷走血管移向肾盂后方.病理检查证实12例合并肾盂输尿管交界部狭窄,术后半年行IVP复查,积水缓解,症状消失. 结论小儿肾盂输尿管交界部迷走血管压迫致梗阻性肾积水发病年龄较大,腹痛明显,积水较轻.治疗上在采取离断性肾盂成型术的同时,应将迷走血管转移到肾盂输尿管交接部的后方.马蹄肾合并肾积水时迷走血管压迫是主要的发病原因.

Objective To evaluate the application of preserved urethral plate urethroplasty to treat urethral fistula after hypospadias repair. Methods Two hundred and twenty-four cases with u-rethral fistula after hypospadias repair were treated by preserved urethral plate urethroplasty from 2001 to 2006.Follow-up data were reviewed and analyzed.All of these cases had big(>1 cm)ure-thral fistula.One hundred and two failed hypospadias cases were repaired by tubularized incised plateurethroplasty(Snodgrass).The patients were 2-16 years old and mean age was 3.7 years old.In thesame time period,86 failed hypospadias cases were repaired by Duplay urethroplasty.The children were 2-14 years old and mean age was 3.5 years old.Twenty failed hypospadias cases were repaircd by Onlay island flap urethroplasty and another 1 6 failed hypospadias cases were repaired by Flip-Flap urethroplasty. Results Fistulas were successfully repaired in 86(84.3%)cases out of 102 tubular-ized incised plate urethroplasty procedure.There were 1 3 urethral fistulas and 3 urethral strictures.Seventy-five(87.2%)cases were successful out of 86 Duplay procedure.There were 9 urethral fistulas and 2 urethral strictures.There was no significant difference between these tWO groups of results(P>0.05).Nineteen cases were successful out of 20 Onlay urethroplasty procedures and there was only l urethral fistula.Fourteen cases were successful out of 1 6 Flip-Flap procedures and there was onlv 2 u-rethral fistulas. Conclusions Surgical procedure for urethral fistula repair should be chosen accord-ing to the condition of penis.Tubularized incised plate urethroplasty can get same satisfactory result comparing with Duplay method for failed hypospadias repair.More Snodgrass should be used to assure the cosmetic penis. Key words: Urethra; Hypospadias;  Urethroplasty

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.