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.

High grade persistent ventral curvature after multiple hypospadias surgery: how to correct?

ABSTRACT Introduction: A challenging situation in proximal hypospadias is the presentation of patients with successful urethroplasty but with persistent or recurrent ventral curvature (VC) after multiple hypospadias repair. Materials and Methods: We present a 13 year-old boy with 7 previous surgeries (long TIP, Duplay, meatoplasty) to treat hypospadias presenting with 60 degrees of VC, in spite of a well-accepted coronally neomeatus. We degloved the penis and artificial erection clearly appointed corporal disproportion causing curvature. We disconnected urethra from corpora. After excision of remnant fibrotic tissue, there was a residual curvature so a lenghtening corporoplasty with dermal graft from groin was performed. We have adjusted the urethral meatus position into a proximal penile shaft. We used a buccal mucosa graft placed in an inverted U-shape position planning a second stage urethroplasty (1). An indwelling silicone Foley tube was left for one week. The patient was discharged the day after surgery. Results: The aspect after corporoplasty proved satisfactory curvature correction. Patient had an excellent outcome and is scheduled for a second-stage after 6 months. Discussion: Snodgrass and Bush (2) reported that on 73 patients with an average of 2.7 operations for proximal shaft to perineal hypospadias; of which, 83% had VC at re-operation averaging 50°. We do believe that some good results with minimal dorsal plicature may recur in adolescence and therefore when these procedures may be considered, they should be performed by classic Nesbit technique (3). Otherwise, the choice for primary ventral lengthening should be taken. Conclusion: Severe curvature associated with hypospadias should undergo a major procedure at early stage to avoid decompensation after dorsal plicature in adolescence. We had a very satisfactory result, the patient awaits the second stage procedure (Figure-1). Figure 1 End aspect after complete healing of buccal mucosa. Notice that penis is straight and second stage will be performed after 6 months of interval.

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.

High grade persistent ventral curvature after multiple hypospadias surgery: how to correct?

INTRODUCTION: A challenging situation in proximal hypospadias is the presentation of patients with successful urethroplasty but with persistent or recurrent ventral curvature (VC) after multiple hypospadias repair. MATERIALS AND METHODS: We present a 13 year-old boy with 7 previous surgeries (long TIP, Duplay, meatoplasty) to treat hypospadias presenting with 60 degrees of VC, in spite of a well-accepted coronally neomeatus. We degloved the penis and artificial erection clearly appointed corporal disproportion causing curvature. We disconnected urethra from corpora. After excision of remnant fibrotic tissue, there was a residual curvature so a lenghtening corporoplasty with dermal graft from groin was performed. We have adjusted the urethral meatus position into a proximal penile shaft. We used a buccal mucosa graft placed in an inverted U-shape position planning a second stage urethroplasty (1). An indwelling silicone Foley tube was left for one week. The patient was discharged the day after surgery. RESULTS: The aspect after corporoplasty proved satisfactory curvature correction. Patient had an excellent outcome and is scheduled for a second-stage after 6 months. DISCUSSION: Snodgrass and Bush (2) reported that on 73 patients with an average of 2.7 operations for proximal shaft to perineal hypospadias; of which, 83% had VC at re-operation averaging 50°. We do believe that some good results with minimal dorsal plicature may recur in adolescence and therefore when these procedures may be considered, they should be performed by classic Nesbit technique (3). Otherwise, the choice for primary ventral lengthening should be taken. CONCLUSION: Severe curvature associated with hypospadias should undergo a major procedure at early stage to avoid decompensation after dorsal plicature in adolescence. We had a very satisfactory result, the patient awaits the second stage procedure (Figure-1).

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.

Conversion of vesicostomy into continent catheterizable reservoirs in myelomeningocele

ABSTRACT Introduction: Vesicostomy should be considered in children with neuropathic bladder in case first-line therapies fail. This simple and reversible procedure can reduce febrile urinary tract infections and protect the upper urinary tract (1) until more definitive alternatives can be proposed. We describe in this video how we approach patients that underwent vesicostomy and want it to be converted into a continent catheterizable reservoir. Material and methods: We perform an infra-umbilical longitudinal incision with a semicircular flap where the stoma will be placed (outside vesicostomy). After releasing the bladder, we proceed with usual steps of the Macedo-Pouch technique (2). We perform the reservoir from 35cm of ileum that constructs a catheterizable channel from the same bowel segment from a 3cm width flap from anterior and posterior wall of ileum in the mid part of it. The continence mechanism of the efferent tube is based on angulation and a serous lined tunnel created with 3-4 3.0 prolene sutures. The stoma is placed in the midline (3). Results: Patient had an uneventful evolution and is continent performing CIC every 4 hours with 9 months of follow-up. We have in the last 3 years a consecutive series of 12 patients operated according to this principle. Discussion: Vesicostomy should be regarded as an alternative for patients with neurogenic bladder refractory to clinical treatment at a younger age in order to postpone definitive treatment such as any an enterocystoplasty. This option must be considered as transient, since definitive reconstructive surgery can provide preservation of renal function and continence achievement. In this context, our video demonstrates that performing a bladder augmentation on a patient with a vesicostomy is safe and feasible. We reinforce that our method precludes the need of appendix or creation of a Monti tube as the outlet channel and the whole procedure is performed from a single piece of bowel.

The GUD technique: glandar urethral disassembly. An alternative for distal hypospadias repair

ABSTRACT Introduction We present an alternative procedure for distal hypospadias consisting of urethral mobilization and partial glandar disassembly, namely GUD (glandar urethral disassembly) technique. Materials and Methods A subcoronal circumcision exposes distal dysplastic urethra. We incise the Buck´s fascia on both sides of urethra releasing it partially from the corpora. We keep a thin bridge of urethral plate to the glans and disassembly almost completely the glans from the corpora, except for the bridge. The glans is incised creating two wide wings that are extremely mobile. The urethra is mobilized, advanced and sutured to the tip of the glans. The glans wings embrace the distal urethra producing a conical glans. Discussion The concept of urethral mobilization has been reported and popularized by Koff in the literature to correct distal hypospadias. One of the limitations of this procedure is the risk of urethral retraction due to extensive proximal dissection. We got inspiration from Mitchell and Bagli' s work of penile disassembly in epispadias to develop the GUD concept. We adopt minimal urethral mobilization mainly in glandar/proximal penile shaft and complete deconstruction of the glans, detaching the corpora from the glans and rotating the wide glans wings to embrace the urethra. Therefore we avoid suture urethroplasty and refurbish the glans to a better conical shape. Conclusion We are convinced that this operation can be regarded as a genuine alternative to distal hypospadias (coronal and subcoronal) but should not be addressed to midshaft forms.

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: glandar urethral disassembly. An alternative for distal hypospadias repair.

INTRODUCTION: We present an alternative procedure for distal hypospadias consisting of urethral mobilization and partial glandar disassembly, namely GUD (glandar urethral disassembly) technique. MATERIALS AND METHODS: A subcoronal circumcision exposes distal dysplastic urethra. We incise the Buck´s fascia on both sides of urethra releasing it partially from the corpora. We keep a thin bridge of urethral plate to the glans and disassembly almost completely the glans from the corpora, except for the bridge. The glans is incised creating two wide wings that are extremely mobile. The urethra is mobilized, advanced and sutured to the tip of the glans. The glans wings embrace the distal urethra producing a conical glans. DISCUSSION: Koff et al. published a modification of the Barcat technique known as extensive urethral mobilization and confirmed excellent cosmetic and functional results on 168 patients with only 3.5% of reoperation. This procedure has several modifications but none has reported an aggressive disconnection of corpora to the glans, but simply incising two glans wings. 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 dorsal chordee. We joined these two procedures to propose the GUD technique. The rationale for this procedure is to avoid suture urethroplasty and create a more conical and cosmetic glans. CONCLUSION: We are convinced that this operation can be regarded as a genuine alternative to distal hypospadias (coronal and subcoronal) but should not be addressed to midshaft forms.

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.

An unusual presentation of urethral duplication presenting with chronic bladder retention, left scrotal transposition and left renal agenesis

ABSTRACT Introduction and objective Urethral duplication is a rare congenital anomaly, with roughly 200 cases reported in the literature (1). It is more frequent in males, with few cases reported in females. The clinical presentation differs according to the anatomical variant present. The duplication most commonly occurs in the sagittal plane with one urethra located ventrally and the other dorsally (2). Usually the ventral urethra is the more functional of both. Duplications occurring in the coronal plane are quite rare and they are usually associated with bladder duplication (3). The purpose of this paper was to present a video of a boy with an unusual urethral duplication form. Materials and Methods Patient was born premature due to oligohydramnios at 7 months-gestational age and he has initial diagnosis of hypospadia. Since then, he presented at least 7 febrile UTI and mother complained of difficult micturition and a presence of a mass at lower abdomen. Patient was referred to our institution and we identified urethral duplication with a glandar and scrotal meatus, palpable bladder and left penile-hemiscrotum transposition. US and CT-scan showed left kidney agenesis and overdistended bladder. VCUG and retrograde urethrography showed duplication, presence of contrast in the seminal vesicles and complete catheterizing of both urethras was not possible. Results The topic urethra was dysplastic and not patent to a 4Fr plastic tube so we were unable to access it endoscopically. We performed initially a Mitrofanoff procedure to allow CIC and treat chronic retention. Six months later, we assessed both urethras surgically and concluded that dorsal urethra was dysplastic after 3cm still in the penile area and scrotal urethra was not possible to be catheterized. We excised the ventral urethra because of dribbling complaints up to bulbar area and reconstructed the scrotal transposition, keeping the topic urethra for cosmetic issues. Patient had excellent outcome, performs CIC every 4 hours and has not presented further UTI episodes. Discussion and conclusion The urethral duplication is an anomaly that has multiple anatomical presentations. There are several theories about the etiology, but none can explain all types of presentations. There is also more than one rating available, and the Effmann classification is the most detailed. The case exemplifies this varied spectrum of anatomic urethral duplication. It resembles the urethral duplication type IIa-Y, however, ventral urethra meatus was located in penoscrotal area and both urethras were at least partially hypoplastic/dysplastic associated with obstruction and bladder retention. In determining how to best manage a patient with Y-type urethral duplication, the caliber and quality of the orthotopic urethra must first be assessed. Published reports suggest that best outcomes are those using the ventral duplicated urethra for the reconstruction (4). In this case, none of urethras were functional and a supravesical outlet channel had to be provided. The treatment of this condition requires an individualized planning and a vast technical knowledge of reconstructive surgery.

An unusual presentation of urethral duplication presenting with chronic bladder retention, left scrotal transposition and left renal agenesis.

INTRODUCTION AND OBJECTIVE: Urethral duplication is a rare congenital anomaly, with roughly 200 cases reported in the literature (1). It is more frequent in males, with few cases reported in females. The clinical presentation differs according to the anatomical variant present. The duplication most commonly occurs in the sagittal plane with one urethra located ventrally and the other dorsally (2). Usually the ventral urethra is the more functional of both. Duplications occurring in the coronal plane are quite rare and they are usually associated with bladder duplication (3). The purpose of this paper was to present a video of a boy with an unusual urethral duplication form. MATERIALS AND METHODS: Patient was born premature due to oligohydramnios at 7 months-gestational age and he has initial diagnosis of hypospadia. Since then, he presented at least 7 febrile UTI and mother complained of difficult micturition and a presence of a mass at lower abdomen. Patient was referred to our institution and we identified urethral duplication with a glandar and scrotal meatus, palpable bladder and left penile-hemiscrotum transposition. US and CT-scan showed left kidney agenesis and overdistended bladder. VCUG and retrograde urethrography showed duplication, presence of contrast in the seminal vesicles and complete catheterizing of both urethras was not possible. RESULTS: The topic urethra was dysplastic and not patent to a 4Fr plastic tube so we were unable to access it endoscopically. We performed initially a Mitrofanoff procedure to allow CIC and treat chronic retention. Six months later, we assessed both urethras surgically and concluded that dorsal urethra was dysplastic after 3cm still in the penile area and scrotal urethra was not possible to be catheterized. We excised the ventral urethra because of dribbling complaints up to bulbar area and reconstructed the scrotal transposition, keeping the topic urethra for cosmetic issues. Patient had excellent outcome, performs CIC every 4 hours and has not presented further UTI episodes. DISCUSSION AND CONCLUSION: The urethral duplication is an anomaly that has multiple anatomical presentations. There are several theories about the etiology, but none can explain all types of presentations. There is also more than one rating available, and the Effmann classification is the most detailed. The case exemplifies this varied spectrum of anatomic urethral duplication. It resembles the urethral duplication type IIa-Y, however, ventral urethra meatus was located in penoscrotal area and both urethras were at least partially hypoplastic/dysplastic associated with obstruction and bladder retention. In determining how to best manage a patient with Y-type urethral duplication, the caliber and quality of the orthotopic urethra must first be assessed. Published reports suggest that best outcomes are those using the ventral duplicated urethra for the reconstruction (4). In this case, none of urethras were functional and a supravesical outlet channel had to be provided. The treatment of this condition requires an individualized planning and a vast technical knowledge of reconstructive surgery.