Hypoplastic Urethra Research Articles

Urethral duplication with a large cystic dorsal penile mass in a newborn: a case report

BackgroundUrethral duplication is a rare urogenital anomaly with varying anatomical orientations leading to diverse clinical presentations. We present a case of urethral duplication in a neonate featuring a large dorsal cystic mass on the penis, an unusual presentation.Case presentationA term male neonate with a prenatally diagnosed 10 × 10 cm genitourinary cystic mass was delivered via caesarean section. Examination revealed a large cystic mass extending dorsally from the pubic symphysis over a flattened, elongated penile shaft with a single urethral opening in the glans and undescended testes. A size 6-French feeding tube inserted into the urethra drained the bladder.Urethral communication with the cystic mass was confirmed via voiding cystourethrogram and cyst enlargement noted during voiding. Cyst fluid analysis indicated a urinary origin. Computed tomography and ultrasound were not diagnostic. Initial imaging revealed a dorsal cystic mass projecting from the pubic symphysis without bladder connection.Surgical intervention at 3 weeks revealed a Type IIA-2 urethral duplication, with a dorsal hypoplastic urethra communicating with the posterior urethra. Correction included resection of the dorsal urethra, cyst excision, and reconstruction of the penis with the orthotopic ventral urethra and bilateral orchidopexies.Satisfactory functional and cosmetic outcomes were observed at 2 and 8 months after surgery.ConclusionThis case highlights the significance of identifying unique urethral duplication presentations. The novel occurrence of Type IIA-2 urethral duplication terminating in a dorsal cystic mass underscores diagnostic complexity, surgical intricacies, and aesthetic considerations associated with such cases.

Urethral Mobilisation and Glanuloplasty Technique for Primary and Second-Stage Repair of Hypospadias: A Single Hospital Experience.

Background Over the years, the technique used to correct hypospadias has undergone several modifications to improve outcomes and reduce complication rates. In this study, a modification has been made to the second stage of the two-stage repair of proximal hypospadias. This modification utilises urethral mobilisation and glanuloplasty, traditionally used to repair distal hypospadias, in the second stage of repair of proximal hypospadias. This study aims to assess the implications of this modification on the outcomes in addition to adding to the pre-existing literature on the outcomes of urethral mobilisation and glanuloplasty in the repair of distal hypospadias. Methodology A prospective study was conducted at Salmaniya Medical Complex in Bahrain between January 2016 and December 2021. All patients with either proximal or distal hypospadias who underwent a surgical repair using the urethral mobilisation and glanuloplasty technique were included. The following cases were excluded: patients with proximal hypospadias who did not undergo a first-stage repair, those with a hypoplastic urethra, and those aged 14 years or more. Results The mean operative time for the proximal hypospadias group was 78 minutes, while it was 62 minutes in the distal hypospadias group. Furthermore, the catheter remained in situ for a mean of three days postoperatively in the proximal hypospadias group. Overall, four of 35 patients (11.4%) experienced complications in the proximal hypospadias group. Of these, there were two(5.7%) cases of meatal stenosis, two(5.7%) cases of wound dehiscence, and no cases of diverticula or urethrocutaneous fistulas. Meanwhile, in the distal hypospadias group, one of 117 patients (0.9%) experienced a complication; the complication was meatal stenosis. there was a significant correlation between the age of patients and the complication rate (p = 0.06). The operative time was also found to be a significant factor influencing the occurrence of complications. The follow-up duration ranged between five months and 12 months. All patients had good cosmetic outcomes. Conclusions This study found that urethral mobilisation and glanuloplasty for the second-stage repair of proximal hypospadias resulted in lower complications than the traditional two-stage operation and a short duration of urinary catheterisation. There is a need to conduct studies with longer follow-up durations and objective measures of function to provide a better comparison between the different techniques used.

Imaging characteristics of multiple unusual urogenital abnormalities in an incontinent dog

AbstractA 10‐month‐old, female, neutered Rottweiler was referred for further investigation of lifelong urinary incontinence. On abdominal ultrasonography, the urinary bladder was empty with asymmetrical wall thickening. Cystoscopy revealed a wide and short tubular connection from the vestibule to a poorly distensible bladder, with no distinct identifiable urethral papilla or urethra. The ureters were observed opening into what was thought to be the bladder trigone, but a third opening was additionally noted cranial to the ureterovesical junctions. A hypoplastic urethra and a stenotic vaginal opening were found on a fluoroscopic retrograde vaginourethrocystogram. Histopathology of bladder wall biopsies revealed mild neutrophilic inflammation. The urinary incontinence was attributed to a combination of severe anatomic abnormalities. No treatment was pursued.

Urethroplasty for chordee with a paper-thin hypoplastic urethra: A long-term study.

ObjectivesTo establish a standard surgical procedure for children with chordee with a paper‐thin hypoplastic urethra, which was based on the degree of curvature and urethral plate quality. To guidelines for the management of this disorder.MethodsWe reviewed 58 cases of chordee with a paper‐thin hypoplastic urethra, managed between March 2006 and September 2020. The age of the patients ranged from 23 to 89 months. Operative details and postoperative complications were analyzed.ResultsMild‐moderate chordee with a paper‐thin hypoplastic urethra correction was performed using a tubularized incised plate (13 cases) or onlay island flap (five cases) technique after penile degloving. Severe chordee with a paper‐thin hypoplastic urethra required transection of the urethra and underwent a Duckett (11 cases) or staged (29 cases) urethroplasty. Complications in the tubularized incised plate and onlay island flap groups were 7.7% and 20%, respectively. The overall complication rate was 10.3% in the staged group and 27.3% in the Duckett group.ConclusionsReconstruction of the entire hypoplastic urethra should be recommended in the management of chordee with a paper‐thin hypoplastic urethra. We propose an algorithm based on the degree of curvature and urethral plate quality. The tubularized incised plate or onlay island flap technique can be used for mild to moderate chordee with a paper‐thin hypoplastic urethra, and the outcome of the tubularized incised plate technique tends to be better than that of the onlay island flap technique. Duckett or staged urethroplasty is suitable for severe chordee with a paper‐thin hypoplastic urethra, and the staged technique can improve the overall success rate.

The urethral plate and the underlying tissue; a histological and histochemical study

To examine the urethral plate and the underlying tissues in children with proximal hypospadias associated with severe chordee. The urethral plate and the underlying tissue specimens were excised to correct severe chordee in 17 children with proximal and perineal hypospadias with severe chordee. The median age was 20 months (range 8-36). Sections samples were marked and examined from proximal to distal. Specimens were examined histologically using hematoxylin-eosin (H/E) and Elastic van Gieson (EvG) stain. Histochemical examination was also performed using smooth muscle actin (SMA) and factor 8 antibodies. For control, samples from four patients with hypoplastic urethra proximal to the meatus including the hypoplastic segments until the normal urethra were taken. In addition, the urethra of an adult patient with penile tumor was used as control. The average size of the 17 tissue samples was 0.5cm×0.5cm x 0.3cm in depth. There was a common pattern that was seen in all the 17 specimens with a variable degree of expression. H/E staining showed that the epithelial lining changed from pseudostratified epithelium at the proximal intact urethra to non-keratinized stratified squamous epithelium at the urethral meatus to keratinized stratified squamous epithelium distally at the urethral plate level. EvG staining showed overall very few elastic fibres that increased slightly in the distal urethral plate. SMA staining showed a circular pattern of smooth muscle cells in the proximal intact urethra that changed to a U-shaped pattern at the level of the meatus, to a triangle shaped pattern just distal to the meatus. The distal urethral plate showed an irregular, disorganized rather flat pattern of the smooth muscles. Factor 8 antibodies staining the blood spaces revealed dysplastic unorganized large blood sinusoids underneath the urethral plate that were different from normal capillaries surrounding the proximal urethra. The urethral plate and the underlying tissues in patients with severe chordee have different structure from normal urethra as compared to available literature and the adult control patient. The lack of elastic fibres may help to explain the rigidity of the ventral penis causing chordee. The disorganized irregular distribution of the smooth muscle fibres is suggestive of the hypoplastic corpus spongiosum. The abnormal large blood sinusoids may explain the poor healing quality of the ventral penis in patients with perineal and proximal patients associated with severe chordee. This may explain persistent/recurrent chordee observed later in those patients with severe chordee when dorsal plication is used. The study also supports the recent trend of 2 stage procedure as a plan of management for patients with proximal and perineal hypospadias with severe chordee and excision of all the dysplastic tissues during the first operation.

Urethral mobilization and advancement technique in distal types of hypospadias: short-term local experience

BackgroundA prospective study was conducted on 65 cases with distal hypospadias operated using the urethral mobilization technique between July 2017 and December 2019. Patients with proximal hypospadias and those with distal hypospadias, but with a hypoplastic urethra, were excluded from the study. In this technique, the urethral tube was mobilized proximally in a ratio of 3–4:1 (the ratio of mobilized urethral length to the initial distance between the native meatus and the tip of the glans) then positioned distally after creating wide glans wings. The aim of this study was to assess the outcome of the urethral mobilization technique in distal hypospadias in our center.ResultsThe age of patients was ranged 9 months to 7 years old (mean 37.5 months); 17 (26%) cases were already circumcised. During the postoperative follow-up, 62 (95.4%) patients had a good caliber neomeatus with a good and straight urinary stream; the remaining 3 (4.6%) cases developed meatal stenosis which responded well to urethral dilatation. One (1.5%) patient had a minor retraction of neomeatus but remained within the glans and not requiring further intervention. Four (6%) cases developed minor hematoma which was resolved on conservative measures. Six (9.2%) patients developed minor wound infection which was treated with daily dressing and antibiotic coverage.ConclusionThe urethral mobilization technique is a good choice for repairing distal hypospadias especially for boys who are previously circumcised as the preputial flap is not required in this technique. It provides good cosmetic and functional results, with a fewer complication rate.

The Hypospadias Phenotype With A Distal Meatus In The Presence Of Distal Penile Penoscrotal Angle Fixation.

ObjectiveHypospadias patients may present with the phenotype that features the migration of scrotum to distal penile shaft below a coronal/subcoronal meatus. Patients with this phenotype differ widely in the severity of the hypospadias and the complexity of the surgical repair. We aimed to investigate the operative findings and the outcomes of consecutive patients who presented with this phenotype.MethodsWe retrospectively reviewed the medical charts of 31 consecutive patients who underwent hypospadias repairs from January 2014 to May 2017, and the hypospadias was characterized by i) the external urethral meatus at coronal/subcoronal region, ii) scrotal skin encroaching distally resulting in fixation of penoscrotal angle at distal penile shaft, and iii) urethral plate below the glans on penile shaft invisible or barely visible.ResultsThe median age at the time of surgery was 15.5 months (10–63). The division of corpus spongiosum was noted at distal penile shaft (n=2; 6.5%), mid-shaft (n=5; 16.1%) and proximal location (n=24; 77.4%). The median ventral curvature before degloving was 45 degrees (10–90). Twenty-eight and 3 patients underwent tubularized incised plate and 2-stage preputial flap repairs, respectively. Twenty-nine of 31 patients required cutback of the hypoplastic urethra. At the time of urethroplasty, 2 (6.5%), 7 (22.6%) and 22(70.9%) patients underwent distal, mid-shaft and proximal repairs, respectively. At a median follow-up of 30 months (14–50), 6 (19.4%) patients developed one or more complications (fistula=3; meatal stenosis=5).ConclusionPatients affected by this particular phenotype likely require non-distal hypospadias repair with possibly higher complication rate and should be treated by surgeons with expertise in complex hypospadias repair.

Distal Urethroplasty and Glanuloplasty Procedure Can be Suitable for All Types of Glanular/Subcoronal Hypospadias

ObjectiveTo correct all types of glanular/subcoronal hypospadias, we performed surgery named the distal urethroplasty and glanuloplasty procedure (DUG procedure). We analyzed cases that we have experienced. MethodsA vertical incision with the meatal margin was made in the 12 o'clock direction, with the margin of the external urethral meatus as the center. By this meatoplasty according to Heineke-Mikulicz principle, we changed all type of glanular/subcoronal hypospadias to the hypospadias with wide meatus and wide glans. And then Thiersh-Duplay procedure was performed. ResultsConsecutive 24 underwent modified DUG procedure. The mean age at the time of surgery was 19.0 ± 11.9 months and the mean preoperative glans width was 13 ± 1.5 mm. The external urethral meatus was located glanular in 13 and subcoronal in 11. Hypoplastic urethra (HU) was observed in 7. Skin chordee in 10, penile torsion in 6 and meatal stenosis in 10 were observed. The mean surgical duration was 106 ± 25.4 minutes and the mean postoperative observation period was 40.5 ± 26.2 months. All patients with preoperative skin chordee, penile torsion, and meatal stenosis were improved postoperatively, and in all cases, apart from the patient with meatal regression with longest HU from glanular to distal penile, the slit-like shape of the external urethral meatus was achieved. ConclusionDUG procedure can be used for any type of glanular/subcoronal hypospadias but care should be taken not to indicate too aggressively for glanular/subcoronal hypospadias accompanying long HU to distal penile shaft.

Congenital Anterior Urethrocutaneous Fistula: Report of Three Cases

The Congenital anterior urethrocutaneous fistula is a rare entity with varied anatomic configurations. In this report, we present three different varieties of congenital anterior urethrocutaneous fistula. The anterior urethrocutaneous fistula was associated with no urethra, hypoplastic urethra, and normal urethra, distal to fistula, 1 in each patient. Each required a different treatment strategy. Postoperative outcome was good in all cases.

The Modified Multilayer Coverage of Urethroplasty for Distal Hypospadias.

Purpose:Our purpose is to present the modified multilayer coverage to prevent fistula and to present also the glans dissection respecting the continuity between glans and corpus spongiosum. We think an important factor for glans vascularization that prevents glans disruption for patients with distal hypospadias and report the follow-up.Introduction:Fistula and glans disruption are still the most frequent complications of hypospadias surgery. Neourethral coverage is a mandatory step in this challenging surgery; it reduces the rate of fistula.Materials and Methods:Between September 2013 and December 2015, the modified multilayer coverage using two or three intermediate layers was performed in 110 patients with distal and midshaft hypospadias. Follow-up based on local examination, visual assessment of urine stream, and parent satisfaction was recorded at 1, 3, 6 months, 1 year, and yearly thereafter. Patients lost for the follow-up and the circumcised patients with a bad quality remaining dorsal dartos are excluded from the study.Results:Intraoperatively, the decision whether two or three intermediate layers should be performed depending on the type of the hypospadias and the presence of hypoplastic urethra. Among the 88 patients retained for the study, 86 patients presented satisfactory results, but one patient had a fistula that healed spontaneously and the second patient had a glans disruption.Conclusions:The modified multilayer coverage with a wings glans dissection respecting the continuity of spongiosum and glans is a reliable procedure to reduce the rate of fistula and glans disruption in anterior and midshaft hypospadias.

Management of congenital penile curvature in children

Objective The aim of this study is to assess the surgical approaches for congenital penile curvature retrospectively. Methods From April 2007 to January 2015, 109 patients with congenital penile curvature, age from 13 months to 13 years, mean 64 months, were treated in the Department of Pediatric Urology Surgery, Children′s Hospital of ZheJiang University, China. According to structural defect, the patients were categorized into 4 groups: group Ⅰ, skin tethering (50 cases, 45.9%); group Ⅱ, fascialchordee (29 cases, 26.6%); group Ⅲ, corporal disproportion (24 cases, 22.0%); and group Ⅳ, urethral tethering (6 cases, 5.5%). With the aid of intraoperative artificial erection, the curvanture was corrected by penile degloving in group Ⅰ patients ; group Ⅱ, release of dense fibrous tissue in addition; group Ⅲ, dorsal-midline-plication-based correction; and group Ⅳ, urethral transection and longitudinal-island-flap-urethroplasty-based with dorsal-midline-plication and Tunica-vaginalis-free-graft repair. Results Of the 37 hypoplastic urethra cases, urethral fistula was present in 4 patients, and six months later, urethral repair was successfully performed; and one case undergoing Duckket′s showed urethral diverticulum, and was corrected by diverticulum resection and urethra mobilization; there was no glans dehiscence and urethral stricture. Of the 26 cases with dorsal-midline-plication-based correction, residual curvature occurred in 1 child after one year; and 4 cases recurved during long-term follow-up (ranging from 1 to 9 years, mean 3.2), with a 84.6% cure rate. Conclusions Conclusions Those corresponding methods of penile curvature should be choosed based on different pathological type respectively. The findings are limited because this study was retrospective and lacked long-term follow-up data. Key words: Penis curvature; Hypospadias; Orthoplasty

Correction of penile ventral curvature in patients with minor or no hypospadias: a single surgeon's experience of 43 cases.

To report our experience of correcting penile ventral curvature associated with minor or no hypospadias. We reviewed 43 penile ventral curvature patients treated by a single surgeon from 1997 to 2015. Of these, 23 had minor hypospadias. Curvature was corrected using degloving, chordectomy, dorsal plication (DP), tunica albuginea incision (TAI), or a combination of these. Outcome was confirmed by induced artificial erection and post-operative appearance. Mean age at curvature correction was 3.2±2.6years. 17/43 had degloving and chordectomy (DC), 16/43 had DP after DC, and 10/43 had TAI after DC, because of ventral shortening and severe curvature caused by a short hypoplastic urethra. Other procedures required were primary meatoplasty (n=4) and urethroplasty (UP; n=1) at the time of curvature correction, and UP after correction of curvature (n=11). Complications included recurrence of curvature after DP (n=3/16; 18.8%) and urethral stenosis after UP with tubed peritoneum (n=1/10; 10%). There were no recurrences of curvature in TAI cases. Parents reported penile cosmesis as good (n=38; 88.4%), acceptable (n=4; 9.3%), or poor (n=1; 2.3%). We recommend TAI followed by UP for correcting penile ventral curvature with short hypoplastic urethra. Tubed peritoneum is not recommended for UP.

Urinary reconstruction in vertebral, anorectal, cardiac, trachea‐esophageal, renal abnormalities and limb defects association with chronic renal failure and penile duplication

Various urological complications in VATER association require careful management. A 15-year-old boy with VATER association, including a hypoplastic lower urinary tract and diphallia, presented with chronic kidney disease and incontinence after a right loop ureterostomy. In order to acquire urinary continence without renal function impairment, an ileocecal reservoir with umbilical catheterizable stoma was created as a urinary reconstruction. The ectopic posterior penis was resected for cosmetic reasons, and the stump of the hypoplastic urethra was opened at the perineal skin. Clean intermittent self-catheterization through the umbilicus using disabled bilateral limbs was then achieved. This report describes the management of VATER association in a patient with complicated urological anomalies.

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.

Challenges in the Management of Bilateral Single-system Ectopic Ureters in Male Infants

To describe our experience of managing bilateral single-system ectopic ureters in boys. We discuss difficulties in diagnosis, importance of appropriate preoperative imaging, and the individualized surgical management for this rare congenital malformation. The first patient aged 3months presented with urosepsis-subsequent imaging demonstrated bilateral single-system ectopic ureters, which were reimplanted at age 7months. The second case was an antenatal diagnosis of bilateral hydronephrosis. Postnatal imaging identified bilateral ectopic single-system ureters. A suprapubic catheter was inserted to ensure optimal bladder drainage. He underwent a Cohen reimplantation at age 7months because of recurrent urinary tract infections. The third patient was born with a duodenal atresia (repaired in the neonatal period) and a complex anorectal malformation, including a pouch colon with a colourethral fistula, a dilated, ectopic left ureter, and a hypoplastic urethra. He was initially managed with a colostomy and suprapubic catheter. At age 14months, he underwent a left nephrouretectomy for a nonfunctioning kidney and posterior sagittal anorectoplasty. The right ureter was noted to be ectopic at this time and was reimplanted. Our series highlights the many challenges for diagnosis and management in boys with this condition because of the diverse presentation and need for appropriate preoperative imaging. Treatment is by bilateral ureteric reimplantation at an appropriate age.

How do asymptomatic toilet-trained children void following

Purpose: To evaluate the functional outcome in the form of urinary flow rates in asymptomatic children following uncomplicated tubularized incised plate urethroplasty (TIPU) hypospadias repair.Methods: We reviewed the records of children who underwentTIPU at our institution between April 1997 and September 2007and included only asymptomatic toilet-trained children who hadan uncomplicated postoperative course and had undergone uroflometry not less than 1 year postoperatively. Unfavourable voiding parameters were either a plateau curve, a peak flow below the 5th percentile range in nomogram or a post-void residual (PVR) more than 20% of the total functional capacity of the bladder. Uroflowmetry findings were analyzed against variables, including the surgeon, the severity of hypospadias, the presence of a hypoplastic urethra, the use of double layer closure, the performance of a spongioplasty and the use of a stent. Serial uroflowmetries when available, were compared with respect to the initial flow study.Results: In total, 59 patients were eligible for the study. The mean age at surgery was 2.4 years. Hypospadias was distal penile in 50 (85%) and mid and proximal penile in 9 (15%). Mean follow-up was 3.3±2 (1-9.5) years. The uroflow curve was bell-shaped in 18 (30%), interrupted in 8 (14%), slightly flattened in 27 (46%) and plateau in 6 (10%). Flow rate nomograms revealed that 40 (68%) were above the 20th percentile, 10 (17%) were below the 5th percentile and 9 (15%) were between these ranges. PVR was >20% of the pre-void volume in 9 children (15%). No patient demonstrated all three unfavourable parameters together. The groups of childrenwith unfavourable functional voiding parameters were compared to the children with favourable parameters specifically with respect to the possible predictors of outcome. Follow-up uroflometry in 17 patients showed improvement in the flow curve, flow rate and PVR with significant improvement of maximum urinary flow rate (Qmax) and PVR values.Conclusions: Asymptomatic, urodynamic abnormalities wereobserved in our study following uncomplicated TIPU repair. These abnormalities were not related to the variation of the technique among surgeons. Spontaneous improvement has been noted on serial flow studies.

Chordee without hypospadias: Operative classification and its management

Context:Developing countries.Aims:To propose a operative classification of Chordee without hypospadias (CWH) with its management.Settings and Design:Tertiary referral centre; Retrospective study from January 2000 to January 2011.Materials and Methods:Total 26 patients were classified peroperatively into sixtypes (A: Cutaneous chordee→ Degloving skin and dartos (1/26); B: Fibrous chordee→ chordectomy (4/26);C: Corporocavernosalchordee→ Corporoplasty ± Urethral mobilization (4/26); D: Urethral tethering with Hypoplastic urethra→ Urethral mobilization ± urethral reconstruction because of hypoplastic urethra (14/26); E: Congenital short urethra→ excision of urethra from the meatus and urethroplasty (2/26); and F: Complex chordee→ Degloving ± Corporoplasty ± urethroplasty (1/26 patients). The follow-up over 6 months to 9 years were analyzed.Statistical Analysis:SPSS soft ware version 17.0 for Windows.Results:The mean age of surgery was 5.33 ± 0.11 years. The success rate defined on uroflowmetry and voiding cystourethrography was 65.6%. The coronal urethra-cutaneous fistula developed in 26.9% (7/26) {including 7.7% (3/26) of associated metal stenosis}. The urethral stricture developed in 3.8% (1/26).Conclusions:CWH needs stepwise surgical management. The operative classification may help in better understanding and management of this difficult entity. Meticulous tissue handling and urethroplasty is needed for good and promising results.

How do asymptomatic toilet-trained children void following tubularized incised-plate hypospadias repair?

: To evaluate the functional outcome in the form of urinary flow rates in asymptomatic children following uncomplicated tubularized incised plate urethroplasty (TIPU) hypospadias repair. : We reviewed the records of children who underwent TIPU at our institution between April 1997 and September 2007 and included only asymptomatic toilet-trained children who had an uncomplicated postoperative course and had undergone uroflometry not less than 1 year postoperatively. Unfavourable voiding parameters were either a plateau curve, a peak flow below the 5th percentile range in nomogram or a post-void residual (PVR) more than 20% of the total functional capacity of the bladder. Uroflowmetry findings were analyzed against variables, including the surgeon, the severity of hypospadias, the presence of a hypoplastic urethra, the use of double layer closure, the performance of a spongioplasty and the use of a stent. Serial uroflowmetries, when available, were compared with respect to the initial flow study. : In total, 59 patients were eligible for the study. The mean age at surgery was 2.4 years. Hypospadias was distal penile in 50 (85%) and mid and proximal penile in 9 (15%). Mean follow-up was 3.3±2 (1-9.5) years. The uroflow curve was bell-shaped in 18 (30%), interrupted in 8 (14%), slightly flattened in 27 (46%) and plateau in 6 (10%). Flow rate nomograms revealed that 40 (68%) were above the 20th percentile, 10 (17%) were below the 5th percentile and 9 (15%) were between these ranges. PVR was >20% of the pre-void volume in 9 children (15%). No patient demonstrated all three unfavourable parameters together. The groups of children with unfavourable functional voiding parameters were compared to the children with favourable parameters specifically with respect to the possible predictors of outcome. Follow-up uroflometry in 17 patients showed improvement in the flow curve, flow rate and PVR with significant improvement of maximum urinary flow rate (Qmax) and PVR values. : Asymptomatic, urodynamic abnormalities were observed in our study following uncomplicated TIPU repair. These abnormalities were not related to the variation of the technique among surgeons. Spontaneous improvement has been noted on serial flow studies.

Urethral atresia and anhydramnios at 18 weeks of gestation can result in normal development

IntroductionSecond-trimester anhydramnios is incompatible with survival unless amniotic fluid volume is restored. We describe the long-term outcome of a child with documented anhydramnios at 17 weeks. Case reportA 28-year-old gravida II had anhydramnios at 17 weeks. At 19 weeks, the bladder measured 12 cm, and karyotype was XY. At 21 weeks, a fistula between the bladder and the amniotic space developed. At 36 weeks, a 2800 g boy with prune belly phenotype was delivered. The urachus was patent and drained urine. There was a completely obstructing membrane below the veru montanum and a hypoplastic urethra distal to it. The right kidney had no function and the left had grade V reflux. A P.A.D.U.A. (progressive augmentation by dilatating the urethra anterior) procedure was performed and the urachal fistula closed. At 6 months of age abdominoplasty, bilateral orchidopexy, right nephrectomy and left to right transuretero-ureterostomy were performed. At age 30 months he was toilet trained, with creatinine 0.5 mg/dl and normal growth. At 10 years of age he continues to be continent, with serum creatinine 0.9 mg/dl. ConclusionResolution of anhydramnios at 21 weeks of gestation can result in normal pulmonary function and acceptable renal and bladder function in childhood. Efforts to develop effective methods of fetal bladder decompression should continue.

Surgical treatment of penile duplication

Penile duplication is a rare anomaly with an incidence of 1 in 5,500,000. It is normally associated with other malformations, such as double bladder, presence of the cloaca, imperforate anus, duplication of the rectosigmoid and vertebral deformities. Presented here is the surgical technique used to resolve a rare case of complete penile duplication in a 5-year-old child, without any other malformation. A male Caucasian patient presented with a rare diagnosis of complete penile duplication, without any associated systemic malformation. At 5 years of age, he was successfully submitted to surgical treatment. The procedure included the following steps: penile dissection up to the point of ischial-pubic insertion; dissection of the hypoplastic urethra; amputation of the less developed penis; termino-lateral urethral anastomosis and phalloplasty. Three years after surgery, penile morphology was normal, with a normal urinary flow according to age. Treatment should always be individualized. The malformations that are potentially life-threatening should be solved first. Fortunately, this patient had only penile duplication, successfully corrected after surgical treatment.