In utero myelomeningocele repair and high-risk bladder pattern. a prospective study.

OBJECTIVES: High-risk bladder pattern can be defined by Urodynamic Evaluation (UE) as overactive bladder with detrusor leak point pressure higher than 40 cmH2O and/or higher filling pressures also above 40 cmH2O. We wanted to evaluate response to treatment in myelomeningocele patients operated in utero in this subgroup. PATIENTS AND METHODS: From our prospective cohort of in utero MMC we have identified patients in the high-risk group. Treatment consisted of anticholinergics (Oxybutynin 0.2 mg/Kg) 2 or 3 times daily in association with CIC. At every UE, patients were reclassified in high-risk or low-risk patterns. Patients not responding were proposed bladder reconstruction or diversion according to age. RESULTS: Between 2011 to 2020, we have been following 121 patients and 60 (49.6%) of them were initially categorized as high-risk. The initial UE was performed at a mean age of 7.9 months and detrusor overactivity was found in 83.3% (mean maximum pressure of 76.5cmH20). When evaluating patients with 2 or more UE, we identified 44 patients (follow-up: 36.8months).  It was observed in the group of patients who underwent 2 to 5 UE, that response to treatment was validated by the finding of 40% of low-risk bladder patterns in the second UE and between 62% to 64% in the third to the fifth UE. The incidence of surgery was 13.3%. CONCLUSIONS: Early urological treatment of high-risk bladder pattern was effective in approximately 60%. We reinforce the need to correctly treat every patient with myelomeningocele, in accordance with UE, whether undergoing in utero or postnatal treatment.

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).

In utero myelomeningocele repair: The natural history of patients with incontinent pattern (sphincteric deficiency: leakage below 40 CMH20).

INTRODUCTION: Since 2011 we have been following prospectively myelomeningocele patients treated in utero with particular interest to patients with sphincter weakness/deficiency. We investigated the changes of bladder pattern and upper urinary tract with time in children who underwent in utero repair and had low-pressure incontinence based on urodynamic evaluation (UE). MATERIALS AND METHODS: From the 120 patients in our database, 117 had at least one UE. Of these, 30 were classified as incontinent when leaking at low pressure (<40 cmH20). We reviewed clinical evaluation, urinary tract ultrasound, voiding cystourethrography (VCUG), and other UE parameters at first and last evaluation. RESULTS: We found 30 cases (25.64%). Mean age at initial evaluation was 4.97 months followed by UE done initially at mean age of 5.73 months. Follow-up was 28.4 months. Febrile urinary tract infection has been found in four patients (13.3%), hydronephrosis in four patients, and bladder neck thickening in three (10%). The VCUG showed vesicoureteral reflux in three cases (3/27, 11.1%). A total of 90% of patients had detrusor overactivity with mean maximum detrusor pressure (33.37 cmH20). Only 16.67% of patients showed normal bladder capacity. From the 30 patients, 23 had at least two UE. We noticed a change of bladder pattern as follows: six patients became of high-risk pattern, five normal, and two with underactive bladder pattern. The average interval between the first and last UE was 25.5 months (median: 15 months). CONCLUSION: We concluded that 43.47% of patients with low DLPP have kept the incontinent pattern. If the initial LPP was below 30 cmH20, 70% remained with the incontinet pattern.

Study of serum and urinary markers of the renin-angiotensin-aldosterone system in myelomeningocele patients with renal injury detected by DMSA.

INTRODUCTION: The Renin-Angiotensin-Aldosterone System (RAAS) has been suggested as a possible marker of renal injury in chronic diseases. This study proposes to analyze the serum and urinary markers of the RAAS in myelomeningocele patients with renal function abnormalities detected on DMSA. MATERIAL AND METHODS: Seventeen patients followed in our institution that presented with renal injury on DMSA. We review nephrologic and urologic clinical aspects and evaluated ultrassonagraphy, voiding urethrocystography and urodynamics. Urinary and serum samples were collected to evaluate possible correlations of renal lesions with RAAS. Control group urine and serum samples were also sent for analysis. RESULTS: Serum ACE 2 activity means in relation to urodynamic findings were the only values that had a statistically significant difference (p = 0.040). Patients with normal bladder pattern presented higher ACE 2 levels than the high risk group. Statistical analysis showed that the study group (SG) had a significantly higher mean serum ACE than the CG. The means of ACE 2 and urinary ACE of the SG and CG were not statistically different. The ROC curve for serum ACE values had a statistically significant area for case and non-case differentiation, with 100% sensitivity and 53% specificity for values above 60.2 mg/dL. No statistically significant areas were observed in relation to ACE 2 and urinary ACE values between SG and CG. CONCLUSION: The analysis of serum ACE, ACE 2 and urinary ACE were not significant in patients with myelomeningocele and neurogenic bladder with renal injury previously detected by renal DMSA.

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.

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.

Left-colon antegrade enema (LACE): Long-term experience with the Macedo-Malone approach.

AIMS: We evaluated the long-term results with a left antegrade continence enema (LACE) approach: "Macedo-Malone (MM) procedure" to define parameters such as clinical durability of the technique and patients' compliance with the method. METHODS: We reviewed the medical records of all patients that underwent the MM procedure in our institution since 2001 and conducted a telephonic interview to investigate the use of the enema and satisfaction with the procedure. RESULTS: Thirty-five MM procedures were performed, but eight patients lost to follow-up were excluded. Fifteen patients (55%) were female. Myelomeningocele was the clinical diagnoses in 25 (93%). Mean age at surgery was 9 years (3-27 years) and mean follow-up was 75 months (median: 56 months). The indication for LACE was clinically intractable constipation with fecal leakage. Most patients still used the stoma regularly to do the enema (74%). Mean washout time was 27 min (2-90 min). The revision rate due to stenosis was 22.2% (6/27) and all underwent suprafascial revision. We performed one classical MACE by infrafascial approach as a salvage procedure. Overall reoperation rate was 25.8% and fecal continence was 89%. There were no reports of leakage through the stoma. Among patients who still used the stoma, 74% were satisfied with surgery and would strongly recommend the procedure to another patient. CONCLUSIONS: The MM procedure is a straightforward procedure, which can be performed in 15-20 min, avoids additional entero-entero anastomosis, precludes the use of the appendix, and has shown comparable results to either "classical" MACE or other LACE variants. Neurourol. Urodynam. 36:111-115, 2017. © 2015 Wiley Periodicals, Inc.

Neophalloplasty in boys with aphallia: A systematic review.

OBJECTIVE: Aphallia is a rare penile congenital abnormality. The aim of this systematic review was to assess all available literature on neophalloplasty in infancy with an interest in technical alternatives and clinical outcome. MATERIALS AND METHODS: We performed a Pubmed search using the terms: neophalloplasty, neophallus, and phalloplasty, and selected articles that presented technical alternatives for penile construction in cases of penile dysgenesis, especially aphallia in children and/or adolescents. RESULTS: A total of 319 articles were identified in the initial search. Among the different techniques presented in 19 papers collected, we categorized reconstructive procedures into two groups: microsurgical and non-microsurgical techniques. Among the microsurgical techniques, procedures such as the osteocutaneous fibular flap (n = 18), the myocutaneous latissimus dorsi flap (n = 24), and the radial forearm free flap (n = 293) were reported. Among the non-microsurgical techniques, we found a wider range of technical options (6 in total). Most were based on groin, abdominal, and scrotal flaps. Long-term reports on the functionability of the neophallus and psychological aspects of the patients are lacking. CONCLUSION: In assessing recent literature, it seems rational that neophalloplasty should be regarded as the preferred strategy for boys born with aphallia, in opposition to female gender assignment.

Penile prosthesis implantation in a patient with congenital aphallia treated using the De Castro technique 10 years previously. Is it feasible?

OBJECTIVE: Aphallia is a rare congenital abnormality with an incidence of 1 in 30 million births. In this video, we demonstrate implantation of a penile prosthesis in a neophallus performed 10 years previously in a patient aged 21. METHODS: Through a midline perineal incision aiming to reach the inferior surface of the pubic arch, we created a 16-cm tunnel for prosthesis insertion into the neophallus. We dressed the prosthesis with a polypropylene mesh to give stability to the component and avoid its extrusion. We anchored the lateral mesh to the inferior aspect of the pubic arch with 2.0 vicryl sutures in both sides. RESULTS: The patient had an excellent initial outcome, without any complaints of pain or other inflammatory findings. CONCLUSION: We acknowledge limited clinical experience with this technique. Further psychological evaluation will confirm if patients can have pleasant sexual experiences.

Neophalloplasty to congenital aphallia using two transverse skin-flaps: an alternative approach.

OBJECTIVE: Penile agenesis is a rare congenital abnormality with an incidence of 1 in 30 million births. We want to demonstrate in this video a new technique of neophalloplasty based on two transverse skin flaps from lower abdominal wall. METHODS: The patient was placed into a prone position; the urethral meatus was found inside the rectum, 2 cm from the anal border. An Anterior Sagittal Transrectal Approach (ASTRA) approach was performed; the urethra was isolated and repositioned as a perineal urethrostomy. We created the neophallus based on two flaps, each measuring 7 × 3 cm. After incision, flaps were rotated 90° and sutured to each other creating the neophallus. We also created a Mitrofanoff channel to secure bladder emptying in cases of urethrostomy stricture. RESULTS: The patient had an uneventful immediate postoperative evolution, but later showed some degree of penile retraction. He voids preferably through the urethrostomy and his mother uses the Mitrofanoff once a day. The current follow-up is 18 months. CONCLUSION: We acknowledge limited clinical experience with this technique. However, the same principle has been used to create catheterizable urinary channels, with favorable results and longer follow-up. The use of transverse skin flaps might be an interesting alternative for neophalloplasty in aphallia.