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Introduction: The optimal treatment of proximal hypospadias remains controversial. Several techniques have been described, but the best approach remains unsettled., Objective: To evaluate and compare the complication rates of proximal hypospadias with and without ventral curvature (VC), according to three different surgical techniques: tubularized incised plate (TIP) uretroplasty, dorsal inlay graft TIP (DIG), and staged preputial repair (SR). It was hypothesized that SR performs better than TIP and DIG for proximal hypospadias., Methods: Single-center, retrospective chart review of all patients with primary proximal hypospadias reconstructed between 2003 and 2013. The DIG was selectively employed in cases with narrow urethral plate (UP) and deficient spongiosum. Extensive urethral plate (UP) mobilization (UPM), dorsal plication (DP) and/or deep transverse incisions of tunica albuginea (DTITA) were selectively performed when attempting to spare transecting the UP. Division of UP and SR was favored in cases with severe VC (>50°), which was often concurrently managed with DTITA if intrinsic curvature was present. For SR, tubularization of the graft was performed 6 months later., Results: A total of 140 patients were included. Tubularized incised plate (TIP), DIG, and SR techniques were performed in 57, 23, and 60 patients, respectively. The TIP and DIG techniques achieved similar success rates, although DIG was performed in cases of narrow and spongiosum-deficient plates. Reoperation rates with TIP and DIG techniques was 52.6% and 52.1% (NS). Urethro-cutaneous fistulas were seen in 31.5% and 13% of TIP and DIG techniques, respectively. Staged repair accomplished better results than both TIP and DIG techniques, despite being performed in the most unfavorable cases (reoperation rate 28%). After technical modifications, the DIG technique achieved similar outcomes of SR., Conclusions: Proximal hypospadias remains challenging, regardless of the technique utilized for its repair. Urethro-cutaneous fistulas were more commonly seen after long TIP repairs. Approximately half of the patients undergoing long TIP and DIG procedures needed re-intervention, although the percentage decreased significantly with late modifications in the DIG group. Recurrence of VC after TIP and DIG techniques seemed to be a significant and under-reported complication. Staged repairs, despite being performed for the most severe cases, resulted in overall better outcomes., (Copyright © 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: Low urinary flow rates are common after tubularized incised plate urethroplasty but the etiology remains unclear and may be related to low urethral compliance due to abnormal collagen concentrations and/or fewer elastic fibers in the healed urethral plate. We hypothesized that inserting a preputial mucosal graft over the dorsal raw area after the midline incision may avoid scarring and improve urethral compliance., Materials and Methods: Adult rabbits were submitted to tubularized incised plate urethroplasty with or without inlay preputial graft according to a previously described protocol. Tissular concentrations of collagens I, III, IV, VI, VIII and XIII were measured. Histomorphometric analysis was used to quantify elastic fibers in the urethra. Tubularized incised plate urethroplasty with and without inlay preputial graft was compared to normal rabbit urethras (controls)., Results: mRNA concentrations for collagens I, II and XIII were similar between controls and operated rabbits. The proportions between collagens I and III were 1.05, 0.87 and 1.21, respectively, in controls and animals undergoing tubularized incised plate urethroplasty with and without inlay preputial graft. mRNA concentrations for collagen IV and collagens VI/VIII tended to be higher and lower, respectively, in the operated urethras, despite showing statistical significance only for collagen VIII in animals undergoing tubularized incised plate urethroplasty with inlay preputial graft vs controls (p=0.02). The operated animals did not demonstrate a reduced number of elastic fibers in the urethral tissues compared to controls., Conclusions: Elastic fiber number and distribution were similar between tubularized incised plate urethroplasty cases and controls, suggesting that decreased concentrations of elastic fibers do not explain the reduced urethral compliance after tubularized incised plate urethroplasty. The raw area determined by the dorsal urethral incision regenerated after standard tubularized incised plate urethroplasty, while cicatrization with fibrosis occurred in correspondence to the grafted areas after tubularized incised plate urethroplasty with inlay preputial graft., (Copyright © 2014 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Objective: Abnormal uroflowmetries are common after tubularized incised plate urethroplasties (TIP), perhaps due to low compliance. We hypothesized that (1) abnormal uroflowmetries after TIP might be caused by segmental lower compliance; (2) by adding a graft to the raw area in the incised plate (TIPG), compliance might be improved by preventing secondary intention healing of the dorsal incision., Methods: A standardized penectomy was performed in 27 adult male rabbits: 9 normal non-operated controls (G1), 6 weeks after TIP (G2: n = 9) or TIPG (G3: n = 9). A standardized isolated segment (including the whole urethroplasty in G1 and G2) was progressively distended with air (1, 2 and 3 ml) in the 3 groups. The respective intraluminal pressures were measured with a tensiometer., Results: Pressure measurements were feasible and reproducible for this model. Mean pressures tended to be higher in the experimental groups (G1: 59.7 mmHg vs. G2: 79.6 mmHg vs. G3: 100.1 mmHg for 1 ml injections; G1: 233.1 mmHg vs. G2: 241 mmHg vs. G3: 308.4 mmHg for 2 ml injections and G1: 457.3 mmHg vs. G2: 429 mmHg vs. G3: 520 mmHg for 3 ml injections) without reaching the statistical significance., Conclusion: In this model, the elasticity of the TIP or TIPG neourethras tended to be reduced when compared to controls. The placement of an inlay graft on the dorsal incised area did not increase the compliance. This model allows the measurement of segmental intraluminal urethral pressures generated by controlled air distension and may be a useful tool to evaluate the experimental urethroplasty models.

Purpose: We analyzed the histological and functional characteristics of the tubularized incised plate vs dorsal inlay graft urethroplasty in an experimental rabbit model., Materials and Methods: A total of 24 New Zealand male rabbits were randomly allocated into 4 groups, including sham operation, urethroplasty, tubularized incised plate urethroplasty and dorsal inlay graft urethroplasty. In the urethroplasty group the anterior urethral wall was half excised and the dorsal aspect was tubularized. In the tubularized incised plate group the same steps were followed but tubularization followed a longitudinal midline incision in the dorsal wall. In the dorsal inlay graft group the defect created by the dorsal incision was covered with an inner preputial graft. The animals were sacrificed at 4 and 8 weeks, respectively. The penis was immediately harvested for standardized passive flowmetry and subsequently fixed for histological staining., Results: The grafts took in all animals. The tubularized incised plate defect was bridged by urothelium while in the dorsal inlay graft group the preputial graft kept its original histological characteristics. There was a significant decrease in average flow in the urethroplasty group (1.6 ml per second) compared to that in the sham operated group (3.4 ml per second) and to the other groups (p <0.05). However, no significant difference in average flow was found for the tubularized incised plate and dorsal inlay graft groups (2.4 and 2.2 ml per second, respectively, p = 0.7)., Conclusions: In this short-term rabbit model dorsal inlay graft urethroplasty was feasible with good graft take and integration. Simple tubularization of a reduced urethral plate led to significantly decreased flow. Incision of the reduced plate with or without grafting improved the average flow. Findings in this experimental model do not support the superiority of dorsal inlay graft urethroplasty over tubularized incised plate urethroplasty in terms of urethral flow dynamics., (Copyright © 2011 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Persistent cloaca is a rare urogenital anomaly that can pose significant challenges for adequate diagnosis and management. We describe the case of an infant girl with a cloacal malformation, having a single perineal orifice and an accessory phallic urethra, who presented with ambiguous genitalia at birth. The distal part of the accessory phallic urethra was used to create a mucosa-lined vestibule as a part of the total urogenital sinus mobilization. This technical maneuver allowed a more natural looking, and possibly functioning, vaginal introitus, improving the final cosmetic result., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Objective: Congenital megalourethra is a rare urogenital malformation characterized by dilation and elongation of the penile urethra associated with absence or hypoplasia of the corpora spongiosa and cavernosa. Postnatal complications include voiding and erectile dysfunction as well as renal insufficiency and pulmonary hypoplasia. To date, only a few prenatally diagnosed cases have been reported. We report on 10 cases diagnosed prenatally and their postnatal/autopsy findings., Methods: The study involved retrospective chart review of all cases diagnosed antenatally in three tertiary care centers over 5 years. Antenatal ultrasound images and medical records from obstetrics, genetics, urology and nephrology were reviewed., Results: Ten fetuses with megalourethra were identified at a median gestational age of 19 (range, 13-24) weeks and all were confirmed postnatally or at autopsy. Three pregnancies were terminated and seven continued. All cases presented with a distended bladder and megalourethra and all cases had normal karyotype. Of seven liveborn babies, one died neonatally of pulmonary hypoplasia. All six infants alive at the time of writing had a dysfunctional urethra and three suffered from impaired or end-stage renal disease. Associated anomalies were found in half of the cases., Conclusion: Congenital megalourethra is caused by abnormal development or hypoplasia of the penile erectile tissue, secondary to distal urethral obstruction. When the amniotic fluid volume is normal, survival is possible. However, all liveborn infants have voiding and renal dysfunction and sexual dysfunction is expected. Megalourethra should be considered in all male fetuses presenting prenatally with megacystis and detailed fetal ultrasonography should look for an elongated and/or distended phallic structure as well as any associated anomalies., (Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd.)

Purpose: Although staged buccal mucosa graft urethroplasty is a well accepted technique for salvage urethroplasty, there are few reports on this procedure for redo hypospadias repair in children., Materials and Methods: We reviewed patients who underwent staged buccal mucosa graft urethroplasty for redo hypospadias repair. Age, quality of graft before tubularization, meatal position, presence of balanitis xerotica obliterans and complications were recorded., Results: A total of 30 patients underwent 32 repairs during a 5-year period. Mean age at first stage was 7 years (range 1 to 17) and mean interval between stages was 9.3 months (5 to 13). Mean followup after second stage was 25 months (range 10 to 46). Meatal position before first stage was proximal in 44% of patients, mid shaft in 39% and distal in 16%. Nine patients had biopsy proved balanitis xerotica obliterans. There were no donor site complications. Four patients underwent a redo grafting procedure. Complications after second stage occurred in 11 of 32 repairs (34%), consisting of urethral stenosis in 5, glanular dehiscence in 3 and urethrocutaneous fistula in 3. A third of the patients had some degree of graft fibrosis/induration after the first stage. These patients were prone to more complications at second stage (9 of 11, 82%), compared to patients without these unfavorable findings (4 of 21, 19%; p<0.001). Presence of balanitis xerotica obliterans and meatal position were not significant factors associated with adverse outcomes., Conclusions: Staged buccal mucosa graft urethroplasty is a suitable technique for salvage urethroplasty. Complications after second stage were seen in approximately a third of patients, mainly those with fibrotic/indurated grafts., (Copyright © 2011 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Objective: There is a paucity of literature on a definition or set criteria evaluating urethral plate (UP) quality. We sought to determine whether pediatric urologists are in agreement in their assessment of UP quality., Materials and Methods: A cohort of 21 pediatric urologists attending a national annual meeting were surveyed with a questionnaire inquiring about practice patterns and perceived impressions of UP quality. Nineteen sequentially projected, standardized, digital photographs, depicting plates with variable meatal locations, were presented. Intra- and inter-rater agreement among pediatric urologists was estimated by calculating the Kappa statistic (kappa)., Results: Thirty percent of respondents had more than 15years of practice and one-third repair 10 or more hypospadias per month. Measurement of level of agreement in the impression of UP quality of the 18 projected photographs revealed poor inter-rater agreement (kappa=0.06, P=0.0003), which was not improved by focusing on particular meatal locations or surgeon experience., Conclusions: Despite the inherent shortcomings of digital photography, this study highlights the potential subjectivity and lack of agreement on UP quality. Disagreement seems to be present irrespective of level of the defect or surgeon experience. Setting standards and improving agreement is likely to enhance reporting and interpretation of data in hypospadias studies., (Copyright (c) 2010 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: Despite being the dominant technique for repair of distal hypospadias, application of the tubularized incised plate approach for penoscrotal hypospadias remains controversial. We report our experience with severe hypospadias, comparing tubularized incised plate to transverse island flap onlay urethroplasty., Materials and Methods: We retrospectively reviewed consecutive patients with penoscrotal hypospadias presenting between 1998 and 2006. Based on surgeon preference 35 children underwent tubularized incised plate and 40 underwent onlay urethroplasty. Penoscrotal transposition and degree of ventral curvature, type of ventral curvature repair, complication rate, postoperative uroflowmetry pattern in toilet trained patients and number of reoperations were compared between the 2 groups., Results: Mean patient age at surgery was 17 months (range 9 to 91) for tubularized incised plate urethroplasty and 17.8 months (10 to 58) for the onlay procedure. Urethroplasty was performed over an 8Fr catheter in all patients. With mean followups of 30 months (range 6 to 74) and 38.8 months (16 to 80) the overall complication rates were 60% and 45% for the tubularized incised plate and onlay procedures, respectively. Fistula occurred in 15 patients and repair breakdown in 3 patients (total 51.4%) treated with tubularized incised plate repair, compared to 8 and 2 patients, respectively (25%), treated with onlay repair (p = 0.01). Fistula location also differed significantly between the 2 groups, with proximal fistulas occurring in 11 of 15 tubularized incised plate repairs (73.3%) vs 2 of 8 onlay repairs (25%, p = 0.02). Recurrent ventral curvature was more frequent after onlay urethroplasty (5.7% vs 12.5%, not significant). At a mean age of 5.1 years a plateau uroflow curve (vs normal bell curve) was observed in 16 of 24 children (66.7%) who underwent tubularized incised plate repair and in 7 of 21 (33.3%) who underwent onlay repair (p <0.01)., Conclusions: In this series the overall complication rate was similar for tubularized incised plate and onlay urethroplasty. Despite similar urethroplasty calibers, the uroflow curves and fistula positions in patients undergoing tubularized incised plate repair suggest that the neourethra distal to the fistula may be relatively narrow, creating flow resistance and leading to proximal fistula. Longer followup and close monitoring are needed before embracing one approach over the other.

Purpose: We report the association of complete urethral duplication and bladder exstrophy in 5 males, and describe the main characteristics to achieve diagnosis., Materials and Methods: From 1983 to 2000, 5 males with single bladder exstrophy and complete urethral duplication were seen at 3 institutions. Although bladder exstrophy was obvious at birth, only 1 patient had the preoperative diagnosis of associated urethral duplication. All patients were evaluated with abdominal ultrasound, voiding cystourethrogram after bladder closure and cystoscopy., Results: All patients were totally incontinent, including 4 after primary bladder exstrophy closure. Although it was initially unnoticed by the urologist, most patients had leakage from the tip of the penis during the Valsalva maneuver. In all cases bladder exstrophy was single and deeply situated in the pelvis with excellent elasticity. All patients had a normal sized penis with less severe dorsal chordee and conical shaped glans. The duplicate epispadiac urethra did not have a verumontanum. Only 1 of the 5 patients had the correct diagnosis made before primary closure of bladder exstrophy, while in the other 4 diagnosis of a duplicate ventral urethra was made at the time of epispadias or fistula repair. There were no other associated malformations. In all cases the dorsal epispadiac urethral plate was excised and bladder emptying occurred through the ventral urethra, which at cystoscopy had a normal verumontanum. Postoperatively, all patients became continent and voided with good flow through the ventral urethra and, as opposed to many cases of exstrophy and epispadias, had normal size and excellent cosmesis of the penis., Conclusions: Complete urethral duplication is a rare variant of the exstrophy-epispadias complex. Patients with this anomaly present with a larger and more deeply situated bladder plate than classical exstrophy and a larger penis. A high index of suspicion is necessary to make a preoperative diagnosis. Excision of the dorsal urethral plate and maintenance of the ventral urethra are the treatment of choice, resulting in a continence and normal voiding.