Minimally invasive repair of hypospadiac urethral duplication

Urethral duplication is a rare congenital anomaly with various clinical presentations, and multiple techniques have been described for its repair. We report a 1-year-old boy with hypospadiac urethral duplication who presented with double urinary stream. Voiding cystourethrography, retrograde urethrography, and cystourethroscopy showed the normal-caliber ventral urethra was dominant and the distal dorsal (non-dominant) urethra had a good caliber. Urethral reconstruction was performed with an incision of the adjoining walls of the both urethra in a side-to-side urethrourethrostomy fashion.     

小儿Y型重复尿道畸形诊治(附13例报告)

目的 探讨小儿Y型重复尿道的治疗方法,提高Y型重复尿道的诊疗水平.方法 回顾性分析2005年6月到2015年6月收治的13例Y型重复尿道患儿的病历资料.本组均为男孩,就诊年龄1～15岁,平均5.5岁.主要表现为生后自会阴或肛门处排尿,其中4例合并阴茎下弯,其余可伸直,未发现阴茎背曲需要矫正者.术前常规检查包括排尿期膀胱尿道造影(VCUG)、静脉肾盂造影(IVP)及泌尿系超声,明确诊断及有无其他伴发畸形.共有12例进行手术,其中11例腹侧尿道为主尿道,一期行会阴部或阴茎根部尿道造口,二期成型自阴茎根部造口处到阴茎头之间的尿道;1例腹侧会阴部尿道发育纤细而背侧尿道发育正常行腹侧尿道切除术.1例就诊时在当地医院行会阴部瘘管切除术而将腹侧主尿道切除,长期进行尿道扩张治疗,未予处理.结果 13例术后平均随访1～5年.4例经2次尿道成型术后治愈;二期术后发生尿道瘘5例,其中4例再次修瘘,1例待手术;2例二期术后出现尿道狭窄,经尿道扩张及内切开治愈.1例切除腹侧发育不良尿道者排尿正常,术后病理检查证实为移行上皮细胞.当地医院行会阴部瘘管切除术而将腹侧主尿道切除的患儿需每1～2周进行尿道扩张后可正常排尿.结论 Y型重复尿道术前分清主副尿道至关重要,主尿道多位于腹侧,应用带蒂阴囊或包皮岛状皮瓣分期成形尿道可取得较好疗效.     

Blunt pediatric anterior and posterior urethral trauma: 32-year experience and outcomes

ObjectiveTo analyze our experience with delayed repair of pediatric urethral trauma.Materials and methodsFrom 1978 to 2007, 26 boys <18 years old (mean age 15.0) presented for delayed repair of urethral stricture after blunt trauma. Anterior and posterior urethral injuries were separately stratified.ResultsThere were 8 anterior and 18 posterior urethral strictures. All patients presented in a delayed fashion. Mean follow up of the anterior cohort was 2.9 years. All repairs were performed via a ventral onlay buccal graft or anastomotic approach. The mean follow up of the posterior cohort was 1.1 years, and all posterior urethral injuries were repaired via an anastomotic approach.Overall success for anterior stricture disease was 88.9% and for posterior stricture disease was 89.5%. All three urethroplasty failures responded favorably to internal urethrotomy; however, one failed anterior repair and one of the two failed posterior repairs required two internal urethrotomy operations for success. No secondary urethroplasty operations were required and ultimately all patients were voiding per urethra without need for urethral dilation.ConclusionDelayed, definitive repair of pediatric urethral trauma via open urethroplasty has a high success rate.     

Y-duplication of the male urethra: use of anterior anorectal wall for posterior urethral lengthening

We have approached two patients with Y-duplication of the male urethra by a new two-staged technique to provide better results. A strip of anterior anorectal wall in continuity with the posterior urethra was used for posterior urethral lengthening and a tubed pedicled prepucial flap was used to reconstruct the anterior urethra without using the native urethra. This was done under a covering colostomy. After a gap of 6 months to allow for healing of the anorectum and to ensure adequate functioning of the perineal neourethra, second stage reconstruction was done using buried scrotal tube for the mid urethra along with colostomy closure. On follow-up at 8 and 12 months, respectively, both children were well with no stricture or fistula. There was normal anal continence and no stenosis. This technique tackles the problem in Y-duplication of the male urethra of lengthening the posterior urethral channel, which is often difficult to bring to the anterior half of the perineum especially if the opening is high up in the anorectum (case 2).     

Is the Koyanagi Urethroplasty an Acceptable Alternative for Proximal Hypospadias?

PurposeThe 4 most common surgical techniques currently used for severe hypospadias (proximal division of the corpus spongiosum) are the Onlay urethroplasty, the buccal graft urethroplasty, the Bracka procedure, the Koyanagi procedure and its variants. We report our experience of the original Koyanagi in 21 cases.Material and MethodsWe reviewed 21 patients who had a Koyanagi urethroplasty with a mean follow-up of 13 months (6-21 months). All had a preoperative testosterone stimulation. All received the original Koyanagi procedure by the same surgeon. Technical aspects are detailed.ResultsPartial urethral dehiscence was found in 47.6%; fistulae in 19%; urethrocele in 19%; meatal stenosis in 14.3%. Up to now, 57% required further surgical attention i.e. 1.6 operations per patient. Cosmetic results were found good by parents and doctors with a respective score of 13.4/20 and 14/20. Urine stream was found satisfactory in 86% cases and too narrow in 14%.ConclusionsThe redo-operation rate of the Koyanagi procedure is comparable to buccal graft urethroplasty but much greater than the Onlay urethroplasty. Modified Koyanagi (button hole) might provide a lower complication rate. Parents should be warned that most patients who received this type of urethroplasty are likely to receive 2 or more procedures to achieve a satisfactory result. Preoperative hormonal preparation of the penis is likely to be a key issue in the healing capacity of the tissues.     

Correction of penile torsion by mobilization of urethral plate and urethra

ObjectiveTo assess the feasibility of correction of torsion by mobilization of the urethral plate with the corpus spongiosum and the proximal urethra.Patients and methodsOf 27 cases of congenital penile torsion, 18 had hypospadias, seven were chordee without hypospadias, and two were isolated penile torsion. Age of patients varied from 2 to 26 years (mean 6 years, 8 months). Correction of torsion was performed: (1) penile skin de-gloving; (2) mobilization of the urethral plate with the corpus spongiosum up to the corona; (3) mobilization of the proximal urethra up to the perineum; and (4) mobilization of the hypoplastic urethra/urethral plate into the glans. Tubularized incised plate urethroplasty with spongioplasty was done in cases of hypospadias, as compared to spongioplasty alone in cases of chordee without hypospadias.ResultsDegree of torsion varied from 45 to 180 degrees (mean 68.70); 74% of the patients had left and 26% had right penile torsion. Correction of torsion was possible by penile de-gloving (4%), mobilization of urethral plate and spongiosum (26%), mobilization of proximal urethra (22%), and mobilization of urethral plate/hypoplastic urethra with spongiosum into glans (48%).ConclusionsExtended urethral mobilization corrected penile torsion in almost all cases. The technique is simple, safe, reproducible and effective for correction of both torsion and chordee.     

Transpubic Posterior Urethroplasty via Perineal Approach in Children : A New Technique

PurposeIs to evaluate a new the technique of transpubic urethroplasty via perineal approach for managment of pelvic fracture urethral distraction defect (PFUDD) in children.Material and MethodsA prospective study was performed on 52 children undergoing posterior urethroplasty for pelvic fracture urethral distraction defect. After perineal midline approach and excision of all fibrous tissues , 6 out of 52 children had a short urethra that could not bridge the gap (more than 5 cm) to do tension free anastomosis . For the latter group ,transpubic posterior urethroplasty via perineal approach was performed. Firstly, the upper border of the symphysis pubis was palpated and the corporal separation was done distally till this level (peno-scrotal junction). Dissection of one crus started from Medial to lateral edge. After complete separation from the bone, the same was done for the other crus. Two crura was hold laterally with tape and two longitudinal lines were performed over the pubic bone, the distance in between about 4 cm. By cutting diathermy over theses lines, separation of the pubic bone from lateral attachment was performed. The separated bone was hold by nipple forceps and complete separation from the bladder was done by cutting diathermy. Easy bulbo-prostatic anastomosis was performed.ResultsAll six children had a good urinary stream without any post-operative complication related to the technique.ConclusionsTranspubic urethroplasty via perineal approach in children is a feasible easy technique with very minimal morbidity and can be done safely in children when indicated. Easy Bulbo-prostatic anastomosis can be done through the perineal exposure.     

Urethral duplication in males: our experience in ten cases

Urethral duplication is a rare congenital anomaly, affecting mainly boys. Clinical presentation varies because of the different anatomical patterns of this abnormality. We report our experience in ten males affected by urethral duplication. We retrospectively reviewed the records of ten males affected by urethral duplication. Mild cases of distal type I duplications as well as “Y-type” duplication associated to anorectal malformation were excluded. Evaluation included voiding cystourethrography, retrograde urethrography, intravenous urography and urethrocystoscopy. Mean age at diagnosis was 46.7 ± 32.3 months A blind ending duplicated urethra (type I) was present in three patients, two urethras originating from a common bladder neck (type II A2) in three, an “Y-type” duplication in three and a complete bladder with incomplete urethral duplication in one. Surgical management included excision of the duplicated urethra in four patients while a displacement of the ventral urethra (in “Y-type” duplication) in perineal-scrotal or scrotal position was performed in two patients as first stage of urethral reconstruction. Good cosmetical and functional results were achieved in all six treated boys while surgical management was not required in four. Urethral duplication is often associated with genito-urinary and gastro-intestinal abnormalities. Embryology is unclear and a lot of hypotheses have been proposed. We believe that the same embryological explanation cannot be applied to all subtypes of urethral duplication. Management must be evaluated for each case. The overall prognosis is good, in spite of the presence of other severe associate congenital anomalies.     

Outcome of severe hypospadias repair using three different techniques

ObjectiveTo compare the outcomes of three different urethroplasty techniques (onlay, buccal mucosa, Koyanagi type I) used in the reconstruction of severe hypospadias.Patients and methodsOver 10 years (1997–2007), 300 severe hypospadias cases were treated with a mean follow up of 2 years (1–105 months); 203 were operated by the same surgeon of whom 184 completed follow up. Three main techniques were used according to the quality of the urethral plate: onlay urethroplasty (133), buccal graft urethroplasty (25) and Koyanagi type I (26). The mean age at surgery was 36 months (8–298); 76 required preoperative androgen stimulation (onlay 37, buccal 11, Koyanagi 26); 18 required a corporoplasty to straighten the penis (onlay 13, buccal 3, Koyanagi 2).ResultsThirty-eight onlay (28.5%); 14 buccal (56%); 16 Koyanagi (61.5%) urethroplasties had a complication. The fistula rate was 15% for the onlay group; 32% for the buccal mucosa group; 19.2% for the Koyanagi cases. The dehiscence rate was, respectively, 11.3%, 20% and 42.3%. The stricture rate was, respectively, 1.5%, 20% and 34.6%. Urethrocele was found in seven Koyanagi patients. Final functional and cosmetic results were satisfactory in 126/133 (94.7%) onlay, 20/25 (80%) buccal and 14/26 Koyanagi (53.8%) urethroplasties. Primary cases had better results (89%) than redo cases (75.9%). Patients submitted to preoperative androgen therapy developed more complications (onlay: 40.5% vs 23.9%; buccal: 70% vs 43.7%).ConclusionTwo striking results are the low number of severe hypospadias cases requiring an additional corporoplasty, and the increased complication rate found in androgen-stimulated patients. The excellent results of the onlay procedure could be related to the use of dorsal preputial tissue, which in hypospadias is characterized by a well-balanced protein platform compared to the ventral tissues.     

A complex case of megalourethra and its repair

Megalourethra is a rare malformation of the urethra caused by a lack of corpus sponigosum and in some cases corpora cavernosa in the region of the distal urethra. The absence of these structures causes a ballooning of the urethra despite there being no mechanical obstruction. A male child presented with so-called fusiform megalourethra, with absence of the corpora cavernosa and urethral duplication. A voiding cystourethrogram was used to diagnose a fusiform megalourethra with pronounced meatal stenosis and extreme stenosis of pendulous urethra. In addition, there was urethral duplication in the form of an accessory urethra stretching from the urethral colliculus to the perineum. Absence of the corpora cavernosa was also suspected in the distal urethra. The surgical procedure involved pendulous urethroplasty with an onlay technique using urethral duplication and penile reduction. This method of treating megalourethra has not been previously reported. The operative technique for fusiform megalourethra with genital malformation has to be tailored to each individual case, depending on the intraoperative and endoscopic findings.     

Our experience,technique and long-term outcomes in the management of posterior urethral strictures

PurposeTo share our experience, technique and long-term outcomes in posterior urethral stricture management.Materials and methodsThirty-seven boys with post-traumatic posterior urethral stricture underwent resection and end-to-end anastomosis through pre-anal coronal approach or in combination with trans-pubic approach from January 2000 to December 2011. Follow up included symptomatic evaluation by micturating cystourethrogram and retrograde urethrogram in all patients, and urethroscopy in patients with voiding symptoms.ResultsPre-anal coronal approach was used in 29 (78%) cases and in 8 (21%) cases it was combined with trans-pubic approach. In 33 (89.1%) patients it was first attempt, while in 4 (10.9%) it was redo surgery. Two patients required buccal mucosal graft to bridge the deficient urethra. Patient age was 5–17 years (mean 10.8 years). Mean follow up was 48.5 months (range 6–132 months). Thirty-two (86%) patients were symptom free. Failed repairs were successfully managed by urethral dilation in 3 and by redo urethroplasty in the remaining 2. All patients were continent. There was no chordee, penile shortening or urethral diverticula.ConclusionsResection and end-to-end anastomosis of posterior urethral stricture is possible through pre-anal coronal incision; however, if slightest difficulty is envisaged in creating a satisfactory end-to-end anastomosis, extension to trans-pubic approach should be done.     

Severe complications of circumcision: an analysis of 48 cases

ObjectiveCircumcision is still the most commonly performed surgery in Islamic and Jewish societies. We report the findings of 48 cases referred for serious complications after circumcision that needed secondary surgical interventions. The aim of this study is to emphasize the important problem of circumcision complications.Patients and methodsThe 48 cases (mean age 14 years, range 5 months-24 years) with complications of circumcision were reviewed retrospectively. Circumcisions were performed at various medical centers or during religious ceremonies in environments other than health facilities.ResultsThe most commonly observed complication was preputio-glandular fusion, seen in 25 cases (52%). The other complications were: meatal stenosis in 11 (23%), urethral fistula in five (10.4%), partial glandular amputation in four (8%) and opening distal urethra in three (6%). Adhesion freeing and revision were performed in all cases of preputio-glandular fusion, patients with meatal stenosis underwent meatotomy, urethral fistulae were repaired by simple closure, partial glans amputations were patched with buccal mucosa, and patients with complete open distal urethra were repaired by Mathieu (one patient) and tubularized incised plate urethroplasty techniques.ConclusionCircumcision may be associated with many serious complications. To prevent these complications, the operation should be performed by educated and experienced personnel.     

Complete urethral duplication: description of surgical approach mimicking distal epispadias repair

ObjectiveTo present two cases of type IIA urethral duplication and propose a reproducible surgical approach.MethodsTwo cases are presented in this report. The first was a male child with a type IIA1 urethral duplication with two urethral channels arising from the bladder through separate bladder necks coursing to the glans penis. The second infant had a type IIA2 urethral duplication with a single bladder neck. Both children were repaired using a surgical approach that joined both urethral openings into a single orthopic meatus. This was accomplished by incising the common septum and utilizing techniques similar to that of a distal epispadias repair.ResultsAt six months of follow-up both infants are voiding from a single stream without complication. Long-term outcomes remain to be determined.ConclusionsThe technique presented in this report both functionally and cosmetically unites the two urethral meatuses while eliminating the risk of damage to the sphincter.     

Female hypospadias with vaginal stones: A rare congenital anomaly

ObjectiveTo highlight the unusual and late presentation of the rare congenital urethral anomaly of female hypospadias and its management.Patients and methodsWe report on four cases (aged 18–65 years; mean 34 years and 3 months) of female hypospadias; two presented with urinary incontinence, recurrent UTI since birth and dyspareunia, and two were diagnosed during catheterization following retention of urine. One of the younger patients had vaginal stones. We reached the diagnosis by catheterization, voiding cysto-urethrogram and panendoscopy. A periurethral vaginal flap urethroplasty was performed in three layers with excellent results in three cases, and one patient was managed conservatively.ResultsResults were excellent in the three cases treated surgically. Patients were continent after urethroplasty and were happy to pass urine in a stream with complete emptying of the bladder for the first time in their life. Two of them had fertility problems due to urogenital septal defects and are undergoing appropriate treatment.ConclusionsVaginal voiding leads to urinary stagnation in the vagina causing urinary pseudo-incontinence and vaginal stones. Early diagnosis requires a high index of suspicion.     

Post-traumatic urethral strictures in children: What have we learned over the years?

BackgroundUrethral stricture presents an uncommon but difficult urological problem in the pediatric population. Treatment protocols are different from in adults due to anatomical considerations.Material and methodsA thorough manual and Medline search was conducted to review the existing literature on post-traumatic pediatric urethral strictures, with key words: stricture, children, post-traumatic, urethroplasty, pediatric.ResultsOpinion early on was that, due to the confined perineum, high incidence of supramembranous injury resulting in less predictable distraction defects of the posterior urethra and a high incidence of prostatic displacement, transperineal urethroplasty is technically more difficult than in adults and thus the transpubic approach is more feasible. Recent reports revealed that both approaches resulted in almost the same clinical outcomes for children with post-traumatic posterior urethral strictures.ConclusionThe ideal reconstruction for the treatment of post-traumatic posterior urethral strictures in children is bulboprostatic anastomosis. This procedure should be initially attempted through the perineum in every case. A transpubic procedure should be done only when tension-free anastomosis cannot be accomplished through the perineum.     

膀胱粘膜移植术治疗复杂型及重型尿道下裂

目的 对膀胱粘膜移植术Ⅰ期矫治复杂型及重型尿道下裂的适应证及手术技巧进行深入讨论，以期重新认识这一手术方法的应用价值。方法 总结1999年—2001年经多次手术失败及重型尿道下裂15例，平均尿道缺损10．5cm，均采用膀胱粘膜移植术Ⅰ期尿道成形。结果 除1例术后感染外其余14例术后均获得较为满意的疗效，阴茎伸直满意，外形好，尿线粗，开口近似正常位置。手术一次成功率14／15(93．3％)。结论 对多次手术失败、阴茎瘢痕严重、局部取材困难及重型尿道下裂、尿道缺损过长的病例，采用膀胱粘膜移植术I期尿道成形术是目前成功率较高，疗效较理想的手术方法。     

Duplication of the urethra

Accessory urethra or duplication of the urethra is a rare anomaly. The vast majority occur in the sagittal plane; however, collateral or side-by-side duplications in the absence of bladder duplication also exist. We report six cases of urethral duplications. The mode of presentation and the importance of complete examination are discussed. Accepted: 13 July 1998     

Correction of penile torsion and chordee by mobilization of urethra with spongiosum in chordee without hypospadias

ObjectiveTo investigate the feasibility of correcting coexistent penile torsion and chordee without hypospadias by mobilization of the urethra and spongiosum.Materials and MethodsA retrospective study of nine patients with simultaneous penile torsion and chordee without hypospadias was undertaken between January 2006 and December 2012. During this period, a total of 364 cases of hypospadias and 38 of chordee without hypospadias were operated on, making a total of 402 patients with hypospadias-related complexes. The same steps were used for correction of both torque and chordee. After a circumcoronal incision, the penis was fully degloved and the spongiosum with urethra was lifted up off the cavernosa. Next, mobilization of the hypoplastic urethra with spongiosum was extended into the glans. If chordee or torque persisted, urethral mobilization was performed proximally up to the bulbar urethra, as required. Spongioplasty and glansplasty were done and a per-urethral stent was kept in for three to five days.ResultsThe age of the patients ranged from 5 to 16 years (median 6 years). Penile torsion ranged from 30° to 120°, with a median of 75°. Ventral chordee ranged from 45° to 100°, with a median of 50°. A ratio of 1:9.6 was found for chordee without hypospadias compared to the total hypospadias cases, with an incidence of 9.5%. The ratio of chordee without hypospadias with torsion compared to the total hypospadias cases was 1:29. There was an incidence of chordee without hypospadias with penile torsion of 3.5% in all patients with hypospadias. The ratio of chordee without hypospadias with torsion to only chordee without hypospadias was 1:1.71, with an incidence of 37.0%. Every step contributed to the correction of curvature and torsion. Chordee was corrected in two patients by penile degloving and lifting of the spongiosa off the urethral plate; three patients required additional mobilization of the urethra into the glans. Another two patients needed proximal urethral mobilization and one required a dorsal plication. In five patients, torque was corrected by penile degloving, lifting of the spongiosa and mobilization of the urethra into the glans; four patients required further proximal urethral mobilization. All patients had excellent functional and cosmetic results. No residual chordee or torque was observed in any patient on follow-up at 12–24 months.ConclusionThe technique of distally mobilizing a hypoplastic urethra with spongiosum from the corpora into the glans, and proximally up to the bulbar region corrects moderate to severe chordee and torsion with excellent cosmetic results. The incidence of torsion with chordee without hypospadias was 3.5% of all cases of hypospadias.     

分期Duckett术式治疗重度尿道下裂的疗效评价

目的：通过与一期 Duckett 术式手术效果进行比较,探讨分期 Duckett 术式治疗重度尿道下裂的应用及价值。方法2009年1月至2014年10月,我们收治重度尿道下裂72例,均为阴茎阴囊交界型或会阴型尿道下裂,患儿第1次手术时年龄2～3岁,平均2.4岁。其中38例采用管形包皮岛状皮瓣法一期尿道成形术,即一期 Duckett 术式,为 A 组,成形尿道长度4～6 cm。34例采用分期手术,一期手术中矫正阴茎下弯后,采用管形包皮岛状皮瓣法成形部分尿道,即分期 Duckett 术式,为 B 组;第一期成形尿道长度为3～4.5 cm;术后1年行二期尿道成形术,成形尿道长度1.5～2.5 cm,两期手术成形尿道总长度4～6 cm。结果两组均获随访,术后随访6个月至5年,平均3.5年,无一例阴茎下弯复发。A 组有7例（7／38,18.5％）术后发生尿瘘。B 组有3例（3／34,8.8％）术后发生尿瘘,均出现在二期尿道成形术后。A 组有4例出现尿道狭窄（4／38,10.5％）,B 组1例出现尿道狭窄（1／34,2.9％）。A组有4例出现尿道憩室（4／38,10.5％）,B 组无一例尿道憩室发生。A 组手术成功率为60.5％,B 组手术成功率为88.3％,差异有统计学意义（P ＝0．008）。结论与一期 Duckett 术式相比,分期 Duckett术式治疗重度尿道下裂,术后并发尿瘘、尿道狭窄和尿道憩室少。分期 Duckett 术式有效降低了重度尿道下裂的手术难度和并发症的发生率,提高了复杂尿道下裂的疗效,具有较好的临床应用价值。     

Anterior urethral valves: an uncommon cause of obstructive uropathy in children

PurposeAnterior urethral valves (AUV) are rare entities generally described in case reports. They are an uncommon cause of lower urinary tract obstruction in children and can be difficult to diagnose. In the present study, we present our experience in four children with AUV along with a literature review.Materials and methodsWe retrospectively identified four children with AUV presented between 1998 and 2005 at age 4–9 years.ResultsHematuria, urinary tract infection and weak voiding stream were the most common symptoms. Voiding cystourethrography (VCUG) confirmed the diagnosis of AUV. On cystourethroscopy, cusp-like valves in the anterior urethra were seen in all children. Transurethral endoscopic resection of the valves was carried out in three children using a pediatric resectoscope. In one child with a massive anterior urethral diverticulum, open resection of the valve, diverticulectomy and urethroplasty were performed. All patients were cured, none had complications as a result of surgery, and all reported a normal urinary stream at follow-up.ConclusionsChildren with poor stream and recurrent infections should be evaluated carefully and anterior urethral valves should be considered in differential diagnosis of obstructive lesions.