The GUDplay technique: A shift of paradigm in glans reconstruction for midshaft and penoscrotal hypospadias with moderate chordee. A proposal of a new approach.

INTRODUCTION: After 5 years experience with the GUD (glandular urethral disassembly) technique for distal hypospadias, we present the GUDplay technique, incorporating Thiersch-Duplay tubularization of the plate till the coronal area, disassembling the glans aggressively and refurbishing the glans. METHODS: We defined the urethral plate and designed an inverted Y incision to open the glans in two wings. The glans was entirely detached from the corpora to gain a great mobility that allowed minor cranial mobilization of the urethra and caudal rotation of the wings. In sequence, there are well-known steps: Duplay urethroplasty, spongioblasts and a Dartos flap to cover the neourethra. The glans was connected to the urethra by 6.0 PDS sutures except in the ventral meatus and the glans wings are joined in the midline. RESULTS: The 5-year-old patient had midshaft hypospadias without previous surgery. The catheter was removed after a week and the healing appears to be good. DISCUSSION: We combined principles of total glans deconstruction in association to Duplay tubularization and then lifted it up to the tip of the glans divided in two wide and mobile wings. We have treated a small series of 6 cases without complications and mean follow-up of 6.2 months.

Macedo ileal catheterizable reservoir adding the scissors maneuver of channel continence: Long term follow-up.

INTRODUCTION: The Macedo ileal catheterizable channel was published in 2000 and consists of an enterocystoplasty with a catheterizable channel that precludes the need of the appendix for the efferent channel. After 25 years of experience with this technique, we decided to review our experience in a select subgroup of cases performed and followed exclusively by the author in a non-teaching hospital facility after the latest modifications of the procedure. MATERIALS AND METHODS: Since 2008, we have refined our technique with the following modifications: small skin transverse incision instead of longitudinal one, with a semicircular flap for further stoma creation in the midline and importantly the scissors maneuver. This maneuver consists of crossing two 2 cm rectus abdominis muscle flaps in the midline to create a neosphincter over the catheterizable channel. Follow-up was defined as the interval between the date of surgery and last visit to the clinic. We defined stoma continence if the dry interval between catheterization was 4hs. Early and late complications were reported as well as the reintervention rate. Additional procedures in association with bladder augmentation were also appointed. RESULTS: We have treated 49 patients. Indications were spinal dysraphism (71.4%), mainly myelomeningocele (65.3%), and others (PUV, exstrophy, anorectal anomalies). Urodynamic evaluation showed bladder pressure of high risk for upper urinary tract involvement (66%), urinary incontinence (24.5%), conversion of incontinent urinary diversion (3.8%), one exstrophy patient not considered (1.9%). Mean age at surgery was 88 months (7.3 years), 21 patients had additional surgeries in addition to Macedo ileal catheterizable channel: Macedo-Malone LACE procedure in 21 patients and bladder neck surgery to improve continence in 5 patients. Urinary stoma continence was 93.9% (46/49) after first surgery, one patient had a serous line valve revised, two patients still leak after 2 h and await revision. Overall enterocystoplasty complications rate was 12.24% and consisted of urinary leakage in 3 cases and channel/stoma stricture in 3 cases. All patients treated for sphincteric deficiency associated with bladder reconstruction are continent per urethra. Urinary stoma revision for stricture was done in 2 cases and consisted on suprafascial approach and reanastomosis, one patient resolved with dilatation of the channel to the stoma. Mean follow up is 100 months (8.3 years). CONCLUSION: We confirmed in a long term follow-up based on face-to-face visits with 8.3 years mean follow-up that the Macedo procedure associated with the scissors maneuver is associated with 93.9% continence of the stoma. Overall complications rate was 12.24%. The cosmetic aspect with a small transverse incision and a midline stoma is another strength of the technique.

Buccal mucosa graft vaginoplasty: A viable option demonstrated step-by-step.

INTRODUCTION: Persistent cloaca, defined as confluence of the rectum, the vagina and the urethra into a single common channel, has an estimated incidence of 1/50,000 live births. We describe the buccal mucosa graft vaginoplasty for a 11 year old female with cloaca, who underwent at the age of 11 months a Peña repair. We performed the vaginoplasty after the onset of uterine pain due to the beginning of menstruation. MATERIAL AND METHODS: We accessed the lower lip for superficial dissection to harvest the graft. The donor site has been kept with as much submucosal fat as possible to avoid damage to the buccinatoria muscles. A second graft was further obtained from the cheek. Both grafts were submitted to multiple small sections to produce a mesh graft and increase its size. A arciform incision anterior to the anal canal and posterior to the urethra was performed followed by consecutive dissection with eletrocautery to gain profundity. The mesh-graft was placed over the neovaginal cavity and sutured with 4.0 PDS monofilament suture used to suture and then quilt the graft in place. The vaginal capacity was confirmed by ease of a two-digit insertion. Haemostasis was confirmed before the insertion of a soft vaginal mold. The patient remained with an indwelling urinary catheter. The mold was equivalent to 24Fr and had 13 cm of profundity and Foley tube were removed after 14 days postoperatively. RESULTS: Patient had an excellent postoperative course and had been instructed to perform vaginal dilatation every 3 h during the day. Current follow up is 10 months. DISCUSSION: Buccal mucosal grafting has advantages over the use of keratinized skin flaps and intestinal flaps. Buccal mucosa is ideal for female genital reconstruction, given its color, texture, lack of hair and mild mucous production. In our particular case, we connected the neovagina after two months of adequate healing to the native 1/3 through laparoscopic approach. CONCLUSIONS: BMG vaginoplasty is a viable alternative to treat females with cloaca at adolescence.

Complete diphallia: How to proceed?

INTRODUCTION: Diphallia is a rare anomaly. It has a range of appearances from a small accessory penile to complete duplication. METHODS: We present a 2 year-old boy with complete penile duplication. The left penile was the largest. NMR (Nuclear Magnetic Resonance) suggested one corporal body for each penile and VCUG (Voiding Cystourethrogram) showed a normal urethra in the right penile and stricture at glandular and mid penile urethra of the left penis. A Y confluence to bulbar urethra was observed confirming only one prostate and bladder. RESULTS: The cystoscopy through the right penile identified the urethral confluence in the bulbar area. We performed a meatotomy in the left penile to insert the cystoscope and confirmed the blind ending urethra. We decided to remove this penile. The penile was degloved entirely and clamped and took out the corpora at the base. DISCUSSION: Diphallia can have three presentations: only glans duplication, bifid diphallia and complete diphallia (two corpora cavernosa and a corpus spongiosum for each penile). In our case, each penile presented only one corpora cavernosa and the decision taken was based on urethral patency. CONCLUSION: The treatment should always be planned individually whereas associated anomalies with the goal of attaining satisfactory functional and cosmetic results.

The Macedo-Malone antegrade colonic enema: A minimal invasive technique that precludes appendix use.

INTRODUCTION: Antegrade continence enema (ACE) is recommended for patients with fecal incontinence/constipation refractory to conservative management. The Malone ACE utilizes the appendix as a catheterizable channel whereas the Macedo-Malone (MM) precludes the use of the appendix. We aimed to illustrate the MM procedure. METHODS: The left colon is brought out through a small transverse incision on the upper left abdomen and a 3-cm transverse flap in a tenia is created. A 12-Fr silicone Foley catheter is placed on the mucosal surface of the flap to allow creating a tubular conduit. After closure of the anterior wall colonic, the continence valve mechanism is produced by embedding the tube over a serous lined tunnel created by interrupted sutures. The distal portion of the tube is anastomosed into a V shape to the skin flap to avoid stoma stenosis. DISCUSSION: The advantage of this technique is the all-the time availability for not requiring the appendix which some authors prefer to use for urinary reconstruction. In a previous study, we have shown that the MM produces a high rate (89%) of fecal continence (Mean follow-up: 75 months). CONCLUSION: We are convinced that this procedure can be incorporated into fecal incontinence/constipation armamentarium.

In utero myelomeningocele repair and incidence of lower urinary tract surgery. Results of a prospective study.

INTRODUCTION: The purpose of this study was to determine the incidence of lower urinary tract surgery in patients with myelomeningocele (MMC) operated in utero as well as in the subgroup of patients with a minimum age of 5 years. METHODS: We use a previously published protocol to categorize bladder patterns and propose the treatment: high risk pattern-anticholinergics and clean intermittent catheterization (CIC); incontinent and normal patterns-clinical surveillance and underactivity pattern- CIC. Non-response to clinical treatment with maintenance of detrusor pressure higher than 40 cmH20 with worsening of hydronephrosis or recurrent urinary infection were imperative indications for surgery and urinary incontinence due to sphincter deficiency and leakage pressure below 40 cmH20 in patients aged 5 years and older were possible indications too. We identified the prevalence of urinary tract surgery and concomitant treatment of constipation and refractory fecal incontinence by convenience, the technique performed and surgery outcomes. RESULTS: A total of 122 patients are part of the prospective protocol. The first UE was performed in 119 patients (median age of 4 months). The categorization of the bladder pattern was high risk in 52.1%, incontinent in 25.2%, hypocontractile 4.2% and normal in 18.5%. Current follow-up was 29.9 months (1-99 months). Surgery was performed on 10 patients (8.4%): 3 vesicostomies, 2 surgeries to treat vesicoureteral reflux, 6 bladder augmentations, 3 ACE and a sling surgery. Considering only patients older than 5 years, we identified 65 patients and seven surgeries performed (10.8%). DISCUSSION: Most bladder reconstruction studies are case series of single institutions or cooperative services, most of which are retrospective. The prospective clinical interpretation and imaging and urodynamic exams over time allowed us prospectively to define the risk of surgery in patients with myelomeningocele operated in utero. It must be considered that the response to the initial urological treatment and even the failure that leads to the need for surgery have to be evaluated in a context of follow-up time. For this reason we have studied separately patients over 5 years-old and also the group considered to be at greatest risk for surgery, the high-risk group of the categorization already described. CONCLUSION: We found an incidence of 10.8% of surgeries in patients over 5 years-old and 12.9% for the high-risk group. This information may be useful to educate parents when stating urological treatment.

A different approach to distal hypospadias repair: The GUD (glandular urethral disassembly) technique.

INTRODUCTION: Distal hypospadias represent the most frequent clinical presentation of hypospadias. In spite of more than 300 techniques available, there is not an ideal approach. We have proposed an alternative procedure based on the combination of minor urethral mobilization and major glans deconstruction and partial disassembly from the corpora, the GUD technique. We want to present our clinical experience with the procedure and describe it in detail. METHODS: The technique consists of disconnecting the spongious tissue and the distal urethra from the corpora and detaching partially the glans as well, from 2 to 10 o'clock. The glans is opened in midline and the procedure combines cranially mobilization of urethra with caudal and medial rotation of glans wings to refurbish the glans correcting the hypospadia without urethroplasty. RESULTS: We have treated 164 patients with distal hypospadia. Median age at the surgery was 22.4 months (1-184 months). The meatal position after penile degloving was coronal at 108 cases, subcoronal at 54 and 2 patients presented megameatus and intact foreskin. Three patients (1.8%) had mild penoscrotal transposition in addition to hypospadia. Twenty-eight patients were treated as a secondary repair (17%). We found complications in 6 patients (3.6%) consisting of five fistulas (3%) and three glans dehiscence (1.8%). Two patients had both complications. Follow up was 21 months (1-42 months) and the median follow-up time was 18 months. DISCUSSION: We acknowledge that this procedure is intended only to distal hypospadias (coronal and subcoronal). We stress that the GUD procedure can be performed irrespectively of any urethral plate "quality" as it does not require a minimum glans width as the TIP repair. Moreover, there is no need for preoperative testosterone treatment. The absence of suture and urethroplasty minimizes the risk of coronal fistulas after surgery. CONCLUSIONS: We believe that this procedure is a viable alternative to distal hypospadias repair.

A salvage procedure for redo penile prosthesis implantation in phalloplasty (De castro technique) for congenital aphallia.

INTRODUCTION: We present a different salvage procedure for penile prosthesis implantation in a De Castro neophallus. METHODS: A patient with congenital aphallia had been operated at the age of 11. Ten years later he had two non successful attempts of prosthesis implantation. Surgery consisted of adapting the implants into the aortic graft and folding it in the middle over the cylinders to reinforce its strength. RESULTS: Patient had a successful postoperative course and has now 6 months of follow-up. DISCUSSION: In a systematic review of penile prosthetic outcomes and complications in gender-affirming surgery, most of the prostheses were inflatable (83.6%) and single-cylinder (61.0%). At a mean follow-up of 3.0 years: 36.2% of prosthesis complications, 60.0% of patients had their original implant present and 83.9% reported achieving penetration. We decided to use vascular grafts as a scaffold because of former non successful attempts. CONCLUSION: The use of vascular prosthesis as a device to implant the penile prosthesis seems to be a safe and effective alternative in an attempt to offer erectile function to the neophallus in case of congenital aphallia.

Robotic-assisted excision of a prostatic utricle cyst in a 12-month boy with proximal hypospadia and 45X0/ 46XY karyotype.

INTRODUCTION: Prostatic utricle cysts result from incomplete regression of Mullerian duct structures and occur most frequently in males with perineal or peno-scrotal hypospadias. Utricular cysts may present with various signs and symptoms including urinary tract infection, pain and post-void incontinence, a palpable abdominal mass or recurrent epididymitis. MATERIAL AND METHODS: We present a 12 months boy we treated due to proximal hypospadias in two stages, in association to a dysplastic undescended testis that was excised. The karyotype of the patient was 45X0/46XY and after successful hypospadias treatment, the mother complained of purulent discharge in the diapers. A sonogram showed a large cyst behind the bladder with heterogeneous content. The diagnosis of utricle cyst was confirmed by a NMR with detailed anatomy and low confluence of the neck of the cyst to the urethra. RESULTS: We performed a robotic excision of the cyst consisting of careful dissection between bladder and rectum and a safe ligation of the communication of it to the urethra. CONCLUSION: Patient outcome was uneventful. To the best of our knowledge this is the youngest patient with utricle cyst treated with robotic approach reported in the literature.

The search for continence in bladder exstrophy: Bladder neck transection and Macedo catheterizable reservoir to augment the nativebladder.

INTRODUCTION: Bladder exstrophy remains one of the most challenging abnormalities in pediatric urology. We propose bladder neck transection and bladder augmentation with a catheterizeable reservoir technique to achieve continence after previous anatomic reconstruction in stages. METHODS: At the age of 5-6 years, we offer the transection of bladder neck and enterocystoplasty to achieve continence. We report on a 6-year-old boy that underwent this procedure. We perform the reservoir from ileum according to Macedo-technique that constructs a catheterizeable channel from the same bowel segment. The continence mechanism of the efferent tube is based on angulation and a serous lined tunnel created with 3.0 prolene sutures. The stoma is placed in the midline. RESULTS: Patient had an uneventful evolution and is continent performing CIC every 4 h with 9 months of follow up. DISCUSSION: In spite of continuous development of bladder exstrophy surgery, the urethral continence and voluntary micturition is still not possible in the majority of patients. We discuss with our patients honestly and offer this method as a viable alternative to achieve continence. CONCLUSION: In our experience, most patients accept urethral transection and suprapubic CIC when educated about results with other alternatives of bladder neck plasty.

The GUD technique: Glandular urethral disassembly for distal hypospadias repair.

INTRODUCTION: We present an alternative procedure for distal hypospadias consisting of urethral mobilization and glandular disassembly, named GUD-technique. MATERIAL AND METHODS: After circumcision and exposure of distal dysplastic urethra, it is entirely mobilized from corpora and glans. Glans is deconstructed and incised in midline producing two wings almost not connected to glandular urethra at all. The urethra is then mobilized cranially and sutured to the tip of glans. Glans wings embrace the distal urethra producing a refurbished conical glans. DISCUSSION: Koff et al. published a technique consisting of extensive urethral mobilization for hypospadias repair. Mitchell & Blagi and Perovic et al. reported on complete penile disassembly for epispadia repair as a way to complete release of the rotation of the penis and treat chordee bringing the urethra to a more functional location. We incorporated these two principles in GUD. The rationale is to avoid suture urethroplasty, simply repositioning the mobilized urethra distally and working aggressively on glans disassembly creating a more conical and cosmetic glans. The aggressive deconstruction of glans makes this technique unique. CONCLUSION: We are convinced that this operation can be regarded as a genuine alternative to distal hypospadias after performing 100 cases in 3 years of experience.

A Comparative Analysis of Bladder Pattern of Patients who Underwent In Utero Versus Postnatal Myelomeningocele Repair.

PURPOSE: In utero myelomeningocele closure is a valid alternative to postnatal repair with unclear benefits to bladder function. We compared bladder status in patients who underwent fetal myelomeningocele surgery versus postnatal repair. MATERIALS AND METHODS: We retrospectively reviewed our database, with group 1 consisting of in utero surgery and group 2 consisting of postnatal repair. Group 3 was a subgroup of group 2, including patients initially presenting at age less than 12 months. We recorded medical history, radiological investigation with renal ultrasonography, voiding cystourethrography, urodynamic evaluation and clinical outcome of the bladder pattern after treatment. RESULTS: We identified 88 patients in group 1, 86 in group 2 and 38 in group 3. The incidence of urinary tract infection was higher in the postnatal period (45% vs 20%). Hydronephrosis occurred in 20.7%, 22.6% and 28.9% of patients in groups 1, 2 and 3, respectively. Vesicoureteral reflux was diagnosed in 15% in all groups. Urodynamic data showed a higher prevalence of detrusor overactivity in group 1 and no difference in other urodynamic parameters. The high risk bladder pattern at initial evaluation occurred in 56%, 50% and 46% of patients in groups 1, 2 and 3, respectively. There was a trend to decrease the percentages of the high risk bladder pattern and to increase the normal pattern after treatment in all groups. CONCLUSIONS: In utero repair did not improve urological parameters compared to repair in the postnatal period.

Urinary and Fecal Continence in 5-Year-Old Patients Who Underwent in utero Myelomeningocele Repair: A Prospective Study.

INTRODUCTION: After the successful results of in utero myelomeningocele (MMC) repair presented by the Management of Myelomeningocele Study, the concept of fetal surgery was introduced in our institution in 2011. Since then, we have been able to follow prospectively a group of patients with attention to urological care. In the present study, we were interested in estimating urinary and fecal continence in this new subset of patients. MATERIAL AND METHODS: We selected from our database patients aged 5 years or older for evaluation of urinary and fecal continence. We reviewed all charts and completed a questionnaire to study aspects of urinary and fecal continence. RESULTS: We identified 14 patients, i.e., 4 (28.6%) males and 10 (71.4%) females. The mean age at MMC surgery was 25.6 gestational weeks. The uro-dynamic class was high-risk in 6 (42.9%), incontinent in 4 (28.6%), hypocontractile in 1 (7.1%), and normal in 3 (21.4%) patients. Three patients had undergone surgery (2 augmentations, i.e., 1 in association with a left colon ACE Macedo-Malone procedure and 1 mini-sling urethroplasty). Twelve patients underwent clean intermittent catheterization (CIC) (85.7%). Only 3 (21.4%) patients had no urinary leakage. Eleven patients (78.6%) used diapers. Eight patients (57.2%) underwent retrograde rectal irrigation and 11 (78.6%) complained of fecal loss. Eleven patients (78.6%) did not report an impact on their self-esteem. CONCLUSION: Despite the use of CIC in 85.7% of the cases, the continence rate in MMC patients operated on in utero was low and 78.6% of the patients used diapers. This data can be used to educate parents about future conditions of their 5-year-old children and may stimulate the debate regarding further attempts (surgical or not) to improve fecal and urinary continence.

In utero myelomeningocoele repair and urological outcomes: the first 100 cases of a prospective analysis. Is there an improvement in bladder function?

OBJECTIVES: To evaluate the first 100 cases of in utero myelomeningocoele (MMC) repair and urological outcomes in a prospective analysis aiming to define possible improvement in bladder function. PATIENTS AND METHODS: We used a protocol consisting of a detailed medical history, urinary tract ultrasonography, voiding cystourethrography, and urodynamic evaluation. Patients were categorised into four groups: normal, high risk (overactive bladder with a detrusor leak-point pressure >40 cm H2 O and high filling pressures also >40 cm H2 O), incontinent, and underactivity (underactive bladder with post-void residual urine), and patients were treated accordingly. RESULTS: We evaluated 100 patients, at a mean age of 5.8 months (median 4 months), classified as high risk in 52.6%, incontinent in 27.4%, with underactive bladder in 4.2%, and only 14.7% had a normal bladder profile. Clean intermittent catheterisation was initiated in 57.3% of the patients and anticholinergics in 52.6%. Antibiotic prophylaxis was initiated in 19.1% of the patients presenting with vesico-ureteric reflux. CONCLUSION: The high incidence of abnormal bladder patterns suggests little benefit of in utero MMC surgery concerning the urinary tract.

Y-type urethral duplication with rectal implantation of the urethra: Which is the best approach?

INTRODUCTION: Y-type urethral duplication describes the condition in which a functional urethra is implanted in the rectum, and there is also a dysplastic topic urethra which produces mostly urinary dribbling. These patients are at risk of urinary tract complications and UTI. We aimed to present the surgical steps of a case treated by ASTRA approach in which we separated the urethra from the rectum and created a perineal urethrostomy. MATERIAL AND METHODS: We treated a 5-month-old boy with Y-type (IIA-2) urethral duplication, in whom the orthotopic urethra was patent just in the penile segment. The patient had urinary flow per anus and minimal dribbling through the orthotopic urethra. We performed a combined cystoscopy with retrograde urethrogram and managed to catheterize the dysplastic urethra with a guide-wire that showed ectopic implantation in the prostatic urethra, below the bladder neck. We performed an ASTRA procedure to separate the urethra from the rectum. The urethral stump was further mobilized to the perineum and anastomosed to a perineal skin flap to create a stoma and minimize the risk of stricture. RESULTS: The patient was followed at 2 month-intervals, and at 6 months follow-up had an excellent outcome. CONCLUSION: The ASTRA approach proved to be an excellent alternative for Y-type urethral duplication with functional urethra implanted in the rectum. We believe that further efforts to reconstruct the urethra should be avoided, with a better and simpler option being to create a definitive perineal urethrostomy.

A 4-Year Prospective Urological Assessment of In Utero Myelomeningocele Repair-Does Gestational Age at Birth Have a Role in Later Neurogenic Bladder Pattern?

PURPOSE: Premature delivery is a major complication of in utero myelomeningocele repair. The prematurity rate in MOMS (Management of Myelomeningocele Study) was 79%, with a mean gestational age at birth of 34 weeks. We speculated that prematurity could also influence urological outcome in patients operated on prenatally for myelomeningocele. MATERIALS AND METHODS: Beginning in November 2011, we prospectively followed a population of patients with myelomeningocele who had undergone in utero repair. RESULTS: We compared patients based on gestational age at birth, ie younger than 34 weeks (group 1, 42 patients) and 34 weeks or older (group 2, 37 patients). Mean gestational age at birth was 28.3 weeks (range 25 to 33) in group 1 and 35.2 weeks (34 to 38) in group 2. Of the cases 47.5% in group 1 were classified as high risk, 35% as incontinent, 10% as hypocontractile and 7.5% as normal. By comparison, 54.5% of cases in group 2 were classified as high risk, 33.3% as incontinent and 12.1% as normal. Differences between the groups were not statistically significant. Mean followup was 27.9 months in group 1 and 24.3 months in group 2. CONCLUSIONS: Our results show that gestational age at birth has little impact on bladder pattern. These data reinforce the need to follow this population closely.