Riberto Liguori

Long-Term Results with A One-Stage Complex Primary Hypospadias Repair Strategy (The Three-in-One Technique)

PURPOSE Complex primary hypospadias repair that warrants urethral plate division is treated mostly in two steps, not necessarily in two surgeries. Our aim was to review long-term results with a one-stage strategy based on reconstruction of the urethral plate with buccal mucosa graft and onlay transverse preputial flap anastomosis protected by a tunica vaginalis flap (the three-in-one concept). MATERIAL AND METHODS We were able to report on 35 patients operated for primary scrotal, penoscrotal and perineal hypospadias between March 2002 and June 2008. We reviewed all charts and had phone interviews with patients not seen for the last 24 months. We investigated parameters such as UTI occurrence, fistula, residual curvature, meatal stenosis, urethral diverticula, dehiscence, orchitis and parental perception. RESULTS Surgical complications occurred in 13 patients (37%): 4 meatal stenosis, 4 diverticula, 5 fistulae and 2 residual penile curvatures (total 42%). Meatal dilatation was successful in 2 cases, reflected in fistula resolution. The reoperation rate was 31.5% consisting mostly of simple procedures like fistula closure, meatotomy and penile curvature release, and complex diverticula repair in 4 cases. Parental perception was excellent for 57% (20 patients) and good or acceptable for the remaining. Mean follow-up was 4.6 years. CONCLUSIONS The one-step strategy is associated with 68.5% success in a single operation, whereas 31.5% will need a second repair. We recognize that meatal problems are mostly associated with fistulae and diverticula; therefore, we recommend a final acceptable proximal glandar opening that will not compromise the neourethra.

A new extra-abdominal channel alternative to the mitrofanoff principle: experimental and preliminary clinical experience

INTRODUCTION The appendix is the gold-standard channel for the Mitrofanoff principle in pediatric urology, but the search for alternatives is justified considering it may not be available or preferably used for colonic stomas (Malone antegrade continence enema). The aim of this study is to report on technical feasibility of a new approach for creating catheterizable channels in a rabbit model and to present our preliminary clinical experience. MATERIAL AND METHODS We configured a tube from two rectangular skin flaps 1x4 cm opposite each other in the middle line of the lower inferior abdomen. The channel was anastomosed to the bladder dome with embedding sutures to create a valvular mechanism. The experimental study consisted of 12 rabbits, divided in 4 groups according to the sacrifice schedule at 2, 4, 8 and 12 weeks. At 30th postoperative day, an urodynamic evaluation was performed to record continence of the stoma. A histological analysis of the specimens stained with hematoxylin-eosin, Masson trichrome and Picrosirius red was also done in group 2 (sacrifice at 4 weeks postoperatively). We used this method in 3 patients with congenital non-neurogenic bladder disease presenting with massive residual volumes without compliance deficits. RESULT The technique proved feasible in all animals, 9 of 12 could be easily catheterized and underwent urodynamic study. No stoma leakage was observed in 7 animals at high bladder pressures (> 50 cm H20) and only 2 animals had some leakage at 40 cm H20. Urodynamics performed through the stoma showed urethral leakage at 20 cm H20, therefore demonstrating the efficacy of the valvular mechanism. Histological analysis confirmed good integration between the tube and the bladder. Mean follow-up of the clinical series (3 patients) was 7.2 months. Two patients remained continent up to 4 hours, whereas 1 patient had some leakage after 2 hours. CONCLUSION We were able to confirm feasibility of a new extra-abdominal channel based on the Mitrofanoff principle and successfully reproduced the method in a clinical setting. Follow-up was short and long term results are required before any conclusive judgment can be made.

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.

Is continent urinary diversion feasible in children under five years of age

PURPOSE To review our clinical experience with urinary continent catheterizable reservoir in children under five years of age. MATERIALS AND METHODS A total of 23 patients (16 males, 7 females) with a median age of 3.64 years were evaluated. Among these, 6 (26.08%) had a posterior urethral valve, 9 (39.13%) myelomeningocele, 4 (17.39%) bladder exstrophy, 2 (8.69%) genitourinary rabdomyosarcoma, 1 (4.34%) had spinal tumor and 1 (4.34%) an ano-rectal anomaly. RESULTS Perioperative complications were observed in four patients consisting of one febrile urinary tract infection, one partial operative wound dehiscence, one partial stomal dehiscence and one vesico-cutaneous fistula after a secondary exstrophy repair. The overall long-term complications rate was 40.90% and consisted of two stomal stenoses (9.09%), one neobladder mucosal extrusion (4.54%), three neobladder calculi (13.63%) and persistence of urinary incontinence in three patients (13.63%). The overall surgical revision was 36.36% and final continence rate was 95.45% with mean follow-up of 39.95 months CONCLUSION Continent urinary diversion is technically feasible even in small children, with acceptable rates of complications.

A neosphincter for continent urinary catheterizable channels made from rectus abdominal muscle (Yachia principle): Preliminary clinical experience in children

PURPOSE We investigated continence outcomes for patients undergoing primary or redo reconstruction of a urinary catheterizable reservoir involving the Yachia technique of intersecting two rectus abdominis strips over the outlet channel. MATERIALS AND METHODS A retrospective evaluation of 22 consecutive patients operated from March 2009 to August 2010 was performed, consisting of 16 primary reconstructions (Macedo catheterizable ileal reservoirs) and 6 rescue cases for leaking stomas. Our data comprised 18 spina bifida patients, 1 sacral agenesis, 1 posterior urethral valves and 1 genitourinary tuberculosis. Mean age at surgery was 8.5 years (3-21 years). We evaluated continence at 3, 6, 12 months, and at the last follow-up based on data from urinary charts. RESULTS Mean follow-up was 21.1 months (12-29 months). Overall continence was 100% for the primary cases and 66% for the redos (2/6 failed). Three patients had initial difficulty in performing clean intermittent catheterization but this resolved with time and experience. CONCLUSION Using Yachias technique has improved the continence rate of our catheterizable reservoirs and was partially successful for suprafascial revision of incontinent conduits.

Urethral duplication II-A Y type with rectal urethra: ASTRA approach and tunica vaginalis flap for first stage repair.

INTRODUCTION Urethral duplication is a rare congenital anomaly affecting mainly boys. Generally, the duplication develops on the sagittal plane; the accessory urethra may run dorsally or ventrally to the orthotopic one. We present a patient with urethral duplication in which the orthotopic urethra was patent in the penile segment but atresic in the bulbar and prostatic segment. The patient had urinary flow from the rectum and the ectopic urethra could be well identified by anal examination. MATERIALS AND METHODS Age at surgery was 13 months. The procedure consisted of an ASTRA (anterior sagittal trans-ano-rectal) approach for dividing the urethra and rectum and was successful to move the urethra up to the perineal area. The rectum was reconstructed and the patient placed into a lithotomy position. A urethral catheter inserted in the penile urethra oriented us were the atresic urethra in bulbar area started. The scrotum was opened in the middle and the distance between the two urethral stumps proximal and distal defined the extension of no urethral tissue that consisted of 5 cm. We opened the right scrotal space and a tunica vaginalis flap was obtained and attached to the bulbar tissue for a two-stage urethroplasty strategy. RESULTS Patient had a nice healing and the tunica vaginalis was nicely incorporated to the adjacent tissue, having the two urethral stumps well delineated. CONCLUSIONS ASTRA approach in combination with a two-stage urethroplasty with tunica vaginalis dorsal flap proved to be an excellent combination for a rare case of urethral Y duplication having the main urethra into the rectum.

Elective appendicovesicostomy in association with monfort abdominoplasty in the treatment of prune belly syndrome

OBJECTIVE To evaluate the role of elective appendicovesicostomy in association with Monfort abdominoplasty to avoid urinary tract infection (UTI) and renal damage in the post-operative follow-up of patients with prune belly syndrome. MATERIALS AND METHODS We followed 4 patients operated in our institution (UNIFESP) (Monfort, orchidopexy and Mitrofanoff) and compared them to 2 patients treated similarly, but without an appendicovesicostomy, in a second institution (UFBA). We evaluated postoperative clinical complications, UTI and preservation of renal parenchyma. Patients were followed as outpatients with urinalysis, ultrasonography (US) and occasionally with renal scintigraphy. RESULTS Mean follow-up was 23.5 months. Immediate post-operative course was uneventful. We observed that only one patient with the Mitrofanoff channel persisted with UTI, while the 2 patients used as controls persisted with recurrent pyelonephritis (> 2 UTI year). CONCLUSION Our data suggest that no morbidity was added by the appendicovesicostomy to immediate postoperative surgical recovery and that this procedure may have a beneficial effect in reducing postoperative UTI events and their consequences by reducing the postvoid residuals in the early abdominoplasty follow-up. However, we recognize that the series is small and only a longer follow-up with a larger number of patients will allow us to confirm our suppositions. We could not make any statistically significant assumptions regarding differences in renal preservation due to the same limitations.

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.

Two-stage complex hypospadias repair when urethral plate has to be divided: ventral corporoplasty using a tunica vaginalis flap.

OBJECTIVE To demonstrate ventral corporoplasty, with tunica vaginalis flap to reconstruct the corpora cavernosa, in a two-stage strategy for proximal hypospadias surgery. METHODS Assessment of residual curvature after complete urethral plate division and transverse superficial cuts in albuginea. Ventral incision of tunica albuginea to elongate the ventral surface of the penis and use of a tunica vaginalis flap to reconstruct the defect in corpora cavernosa. Dorsal preputial island flap was used to cover the penile ventral surface, to be tubularized in a second stage, together with the original urethral plate. RESULTS Patient had an uneventful follow-up. Penile aspect was very satisfactory with no residual curvature. CONCLUSION Tunica vaginalis is an attractive alternative for ventral corporoplasty in hypospadia repair and has the advantages of prompt disposability, autologous use, and does not represent an extra cost for treatment.

Cloacal Exstrophy: a complex disease

INTRODUCTION Cloacal exstrophy is a rare occurrence with an incidence of 1:200,000 to 1:400,000 live births. It represents one of the most challenging reconstructive endeavors faced by pediatric surgeons and urologists. Aside from the genitourinary defects, there are other associated anomalies of the gastrointestinal, musculoskeletal and neurological systems that require a multidisciplinary approach when counseling anxious parents. MATERIAL AND METHODS We present a video of a patient with cloacal exstrophy treated with 21 days of life. Surgery consisted of separation and tubularization of the cecal plate from the exstrophied bladder halves and colostomy construction. The bladder was closed primarily and umbilical scar reconstructed and used for ureteral and cistostomy drainage. A urethral catheter was used to guide bladder neck tubularization. A final epispadic penis was obtained and planned for further repair in a second step. RESULTS The patient had an initial uneventful postoperative course and immediate outcome was excellent. The bladder healed nicely but patient presented with abdominal distension in the 5th day of postoperative setting requiring parenteral nutrition. The distal colon persisted with lower diameter although non obstructive, but causing difficulty for fecal progression. Continuous colostomy dilatation and irrigation were required. CONCLUSIONS Approximating the bladder halves in the midline at birth and primary bladder closure is a viable option, intestinal transit may be a issue of concern in the early postoperative follow-up.