Atila Rondon

Urological evaluation of patients that had undergone in utero myelomeningocele closure: A prospective assessment at first presentation and early follow-up. Do their bladder benefit from it?

To report our data on initial urological presentation after in utero myelomeningocele (MMC) closure.

Prenatal diagnosis of bladder exstrophy by fetal MRI

PURPOSE To review our experience with prenatal diagnosis of bladder exstrophy by fetal magnetic resonance imaging (MRI). Bladder exstrophy can be diagnosed by ultrasonography (US) evaluation of the fetus based on absence of bladder filling, low-set umbilicus, small genitalia and lower abdominal mass, although in some instances more accurate anatomical information is desired. MATERIAL AND METHODS We studied three patients at mean gestational age of 27.3 weeks. The fetal MRI exam was performed on axial, sagittal, coronal planes and echo gradient in the best plan for acquisition of fetus. Images were analyzed by a group of three radiologists with experience in fetal MRI. RESULTS The MRI defined a lower abdominal mass prolapsing below the umbilical vessels, having the ureters ending on it in an anterior position. A cloacal malformation or a cloacal exstrophy could be excluded, as well as other accompanying spinal abnormalities. The renal system and oligohydramnios could be well documented. CONCLUSIONS The MRI showed a detailed scenario of the abnormality with advantages over the US evaluation in regard to excluding cloacal anomalies. MRI allowed accurate sexual differentiation and may be indicated after suspected bladder exstrophy on US evaluation.

Phalloplasty and urethroplasty in a boy with penile agenesis

OBJECTIVE New concepts in ambiguous genitalia have questioned the strategy of female gender reassignment in cases of penile agenesis. Hence, we present a video of the construction of a phallus according to the De Castro technique as an alternative approach for this devastating condition. METHODS A 12-year-old boy with congenital penile agenesis and posterior urethra emerging in the distal rectum was selected. An ASTRA approach was used and a phallus was constructed with a skin flap from the abdominal wall, tubularized to produce a phallic appearance. A buccal mucosa graft was tubularized to create the new urethra. RESULTS Immediate outcome was excellent. Partial dehiscence of the dorsal urethral sutures occurred and the patient started voiding through a scrotal urethrostomy at 9 months postoperatively. Phalloplasty provided an adequate male appearance with a good cosmetic aspect. CONCLUSION The technique is a feasible alternative. A procedure in 2 stages by first creating the neourethra at a later date might be an interesting option to try to avoid the urethral complications observed. Long-term follow-up is however necessary to confirm the initial results.

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.

The buccal mucosa fenestrated graft for Bracka first stage urethroplasty: experimental study in rabbits

OBJECTIVE To histologically evaluate, in an experimental study in rabbits, the integration process of the buccal mucosa fenestrated graft applied in the corpora cavernosa for Bracka first stage urethroplasty. MATERIALS AND METHODS A urethral defect was surgically created in 16 male rabbits of the New Zealand breed through the excision of the penile urethra. The urethral defect was corrected by applying buccal mucosa fenestrated graft through two cruciform incisions in the distal portions of its longitudinal axis. The animals were sacrificed at 2, 4, 8 and 12 weeks post surgery and their genitals were subjected to clinical and histological assessment. RESULTS The buccal mucosa fenestrated graft showed complete uptake in all groups, with keratinization squamous metaplasia and mucosal proliferation of the fenestrated areas. The fenestrated graft area represented an increase in length of 25 % in length in relation to the original standard graft. CONCLUSIONS The fenestrated buccal mucosa graft presented total integration to the adjacent epithelia with re-epithelization of the incision areas of the graft (fenestrations) and no significant inflammatory or scarring reactions when compared to other mucosa transplanted areas; therefore its application is viable in cases of extensive urethral defect whenever the donating area might be insufficient.

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.

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.

Anthropometric measurements as an indicator of nutritional status in spina bifida patients undergoing enterocystoplasty

ABSTRACT Objective: To use anthropometric measurements to compare nutritional status in children with neurogenic bladder dysfunction secondary to meningomyelocele who underwent enterocystoplasty and those who did not undergo surgery. Methods: A case-control study was conducted in 20 children, divided into two groups: those who had enterocystoplasty (Group A) and those who did not undergo surgery (Group B), matched for genre and age. Weight, height, arm circumference, and triceps skinfold thickness were the parameters used. Nutritional assessment was determined by calculating the indexes, based on age and genre. Classification was based on the percentile and the results were compared with the reference values. Results: The mean age was 6.41 years in Group A and 6.35 years in Group B. The interval between surgery and evaluation was 11 months. The following measures were found for Group A: 80% of children were eutrophic, a percentage 30% greater than that in Group B; arm muscle circumference was adequate in 40% of patients, a percentage 20% greater than that in Group B; arm muscle area was adequate in 90%, a percentage 30% greater than that in Group B. Values in Group B were as follows: for triceps skinfold thickness, 60% of patients had values above the mean, a percentage 20% greater than that in Group A; for arm fat index, 60% of patients were above the mean value, 40% greater than in Group A. Conclusion: Patients who had undergone enterocystoplasty showed better nutritional status, while the control group presented higher fat indexes in anthropometric measures. However, the differences between groups were not statistically significant.

Comparative analysis of the pressure profilometry of vesicocutaneous continent catheterizable conduits between patients with and without rectus abdominis neosphincter (Yachia principle)

To assess whether crossing rectus abdominis muscle strips, as proposed by Yachia, would change urinary catheterizable conduits pressure profilometry, in static and dynamic conditions.