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.

The Ochoa urofacial syndrome: recognize the peculiar smile and avoid severe urological and renal complications / Síndrome urofacial de Ochoa: reconheça o sorriso peculiar e evite complicações urológicas e renais graves

Ochoa syndrome is rare and its major clinical problems frequently unrecognized. We describe facial characteristics of six patients to help health professional recognize the inverted smile that these patients present and refer them to proper treatment. Patients’ medical records were reviewed and patients’ urological status clinically reassessed. At last evaluation patients’ mean age was 15.5 years, and age ranged from 12 to 32 years. Mean follow-up was 35 months (12 to 60). Initial symptoms were urinary tract infections in four patients (67%) associated with enuresis and incontinence in three of them (50%). One patient had only urinary tract infection and two lower urinary tract symptoms without infections. Initial treatment consisted of clean intermittent catheterization with anticholinergics for all patients. Four patients (67%) were submitted to bladder augmentation. Two patients had end-stage renal disease during follow-up, one received kidney transplantation and one patient remained on the waiting list for a renal transplantation. Familial consanguinity was present in only one case. This significant condition is rare, but it must be recognized by pediatricians, nephrologists and urologists in order to institute early aggressive urological treatment.

A síndrome de Ochoa é rara, e seus principais problemas clínicos são frequentemente não reconhecidos. Descrevem-se aqui características faciais de seis pacientes para auxiliar profissionais de saúde a reconhecer o sorriso invertido que eles apresentam e encaminhá-los para o tratamento adequado. Os prontuários médicos foram revisados e a condição urológica dos pacientes foi reavaliada clinicamente. A média de idade na última avaliação foi de 15,5 anos, variando de 12 a 32 anos. O seguimento médio foi de 35 meses (12 a 60). Os sintomas iniciais foram infecções do trato urinário em quatro pacientes (67%) associadas com enurese e incontinência urinária em três deles (50%). Um paciente apresentou infecções do trato urinário isoladamente e dois apresentaram sintomas do trato urinário inferior, porém sem infecções. O tratamento inicial consistiu em cateterismo intermitente limpo, com anticolinérgicos em todos os pacientes. Reconstrução urinária foi realizada em quatro pacientes (67%) por meio de ampliação vesical. Dois pacientes apresentaram doença renal em estágio terminal no seguimento, um recebeu transplante renal e outro manteve-se em lista de espera para transplante renal. Consanguinidade familiar esteve presente em apenas um caso. Essa condição significativa é rara, porém deve ser reconhecida por pediatras, nefrologistas e urologistas, a fim de instituir tratamento urológico agressivo precoce.

The Ochoa urofacial syndrome: recognize the peculiar smile and avoid severe urological and renal complications.

Ochoa syndrome is rare and its major clinical problems frequently unrecognized. We describe facial characteristics of six patients to help health professional recognize the inverted smile that these patients present and refer them to proper treatment. Patients' medical records were reviewed and patients' urological status clinically reassessed. At last evaluation patients' mean age was 15.5 years, and age ranged from 12 to 32 years. Mean follow-up was 35 months (12 to 60). Initial symptoms were urinary tract infections in four patients (67%) associated with enuresis and incontinence in three of them (50%). One patient had only urinary tract infection and two lower urinary tract symptoms without infections. Initial treatment consisted of clean intermittent catheterization with anticholinergics for all patients. Four patients (67%) were submitted to bladder augmentation. Two patients had end-stage renal disease during follow-up, one received kidney transplantation and one patient remained on the waiting list for a renal transplantation. Familial consanguinity was present in only one case. This significant condition is rare, but it must be recognized by pediatricians, nephrologists and urologists in order to institute early aggressive urological treatment.

Total urogenital mobilization by CAH: a step-by-step illustration of the technique.

INTRODUCTION: Congenital adrenal hyperplasia (CAH) resulting from deficient 21-hydroxylase activity is an autosomal recessive disorder with an incidence of 1:5000-25,000 in Caucasian populations. Despite various techniques to treat CAH, total urogenital mobilization (TUM) has gained popularity. This technique has low morbidity, and can be performed by the perineal route with the patient in the dorsal lithotomy position without the need to separate the urethra from the vagina. We aim to demonstrate in this video the TUM technique step by step. METHODS: A 9-month-old child, born with ambiguous genitalia (Prader 3) with a 46XX karyotype and CAH diagnosis presented to our facility. Endoscopy showed a 2-cm common channel. RESULTS AND DISCUSSION: The patient was treated as described in the video and she had an uneventful clinical outcome with complete healing. TUM is an excellent alternative for treating CAH.

Complete female epispadia: the case for perineal approach.

INTRODUCTION: Complete female epispadia (CFE) is a rare congenital anomaly occurring in 1 of 500,000 live births. The goals of CFE management include achieving continence, while protecting the kidneys, and creating functional, cosmetic external genitalia. PATIENT AND METHODS: We demonstrate in this video the steps of the surgery and present midterm follow-up of a 6-year-old child with CFE. RESULTS AND DISCUSSION: The patient had an excellent cosmetic result, and reported continence of 3 h period with mild leakage. The perineal infrapubic approach offers the possibility of restoring cosmesis and providing resistance in one surgery precluding the need for abdominal bladder neck surgery.

Categorization of bladder dynamics and treatment after fetal myelomeningocele repair: first 50 cases prospectively assessed.

PURPOSE: We categorized bladder patterns and principles of treatment applied to patients who underwent myelomeningocele repair during gestation in a prospective urological assessment. MATERIALS AND METHODS: We performed urinary tract ultrasound, voiding cystourethrogram and urodynamic evaluation. We then categorized patients into 4 patterns, including normal, high risk (overactive bladder with detrusor leak point pressure greater than 40 cm H2O and high filling pressure also greater than 40 cm H2O), incontinent and underactive bladder. RESULTS: A total of 51 patients were enrolled in study at the first medical appointment. Urodynamic evaluation was done in 48 of these patients as the initial investigation, enabling attribution of a bladder pattern. The high risk pattern was found in 27 patients (56.2%), 18 were incontinent and 1 had an underactive bladder. Only 2 patients (4.2%) in this series had a normal bladder. CONCLUSIONS: Of patients who underwent myelomeningocele closure during gestation 93.7% had significant lower urinary tract dysfunction consisting of high bladder pressure or incontinence. These data reinforce the absolute need to follow these patients closely. The potential benefits of fetal surgery in the urinary tract remain to be proved.

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

AIMS: To assess whether crossing rectus abdominis muscle strips, as proposed by Yachia, would change urinary catheterizable conduit's pressure profilometry, in static and dynamic conditions. METHODS: Non-randomized selection of 20 continent patients that underwent Macedo's ileum-based reservoir, 10 including Yachia's technique (Study Group) and 10 without this mechanism of continence (Control Group). Demographics and cystometric data were assessed. Conduit's pressure profilometry was obtained by infusing saline through a multichannel catheter, at rest and during Valsalva maneuver. We assessed the pressure: (a) in the bladder; (b) in conduit's proximal segment; and (c) in conduit's distal segment, which is presumably the abdominal wall and crossed muscle strips site. RESULTS: Mean age at surgery was 6.1 years in the Control Group and 7.7 years in the Study Group. There was no statistically significant difference between groups regarding maximum cystometric bladder capacity and leakage point pressure. At rest, the pressure profilometry showed similar results between groups in all segments analyzed. During Valsalva maneuver, pressure profilometry showed similar results between groups in bladder and conduit's proximal segment pressure. In this condition, conduit's distal segment pressure in the Study Group (Mean = 72.9 and Peak = 128.7 cmH2 O) was significantly greater (P < 0.05) than conduit's distal segment pressure in the Control Group (Mean = 48.3 and Peak = 65.1 cmH2 O). CONCLUSIONS: Crossing muscle strips over the conduit significantly increases the pressure in its distal segment during contraction of the rectus abdominis muscle, which can be important in moments of sudden increase in abdominal pressure in order to keep continence.

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?

AIMS: To report our data on initial urological presentation after in utero myelomeningocele (MMC) closure. METHODS: A prospective urological assessment at first presentation was designed for patients that had undergone in utero MMC closure and referred to our urological facility. The protocol consisted of detailed medical history, renal sonography, voiding cystourethrogram, and urodynamic evaluation. RESULTS: In utero MMC closure was performed in 19 patients at gestational age of 25.6 weeks 25-27. Birth occurred at a mean gestational age of 31.8 weeks 26-36. Hyperactive bladder was observed in 89.5% 17/19. Bladder compliance was normal in two cases (10.5%), was markedly reduced in 10 patients (52.6%) and not possible to be determined due to urinary leakage in 7 patients (36.8%). We observed normal bladder capacity in 8 patients (42.1%), reduced in 11 (57.9%), and detrusor-sphincter dyssynergia in 9 patients (47.4%). Underactive bladder was diagnosed in one case. Clean Intermittent Catheterization was initiated by 11 patients (57.9%) mostly in association with anticholinergics 10/11. Vesicoureteral reflux was found in 5 patients (26.3%) and 9 had pyelonephritis at a mean follow-up of 5.4 months 2-17. CONCLUSIONS: Our data suggested that despite in utero MMC surgery, patients are at risk for bladder abnormal function and renal deterioration and should be aggressively treated, not differently from those operated in the post-natal term. This study has the merit of being a prospectively set evaluation performed by one investigator, including the urodynamic study. We acknowledge the need of long-term follow up.

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. .

Keratinized versus non-keratinized preputial flap onlay urethroplasty: does it make any difference in the histological analysis? An experimental study in rabbits

Purpose To compare the histological characteristics of keratinized versus non-keratinized onlay island flaps in an experimental rabbit model. Materials and Methods Sixteen male rabbits were randomly allocated into two experimental groups: keratinized and non-keratinized onlay island flaps. A defect was created in the ventral aspect of the penile urethra. In the keratinized group, a longitudinal island flap was harvested from the external prepuce and rotated to cover the urethral defect. In the non-keratinized group a transverse island flap was harvested from the inner prepuce. The animals were sacrificed after 2, 4, 8 and 12 weeks. Results The flaps were viable in all animals, and no deaths were associated with the procedure. Two urethrocutaneous fistulas were identified, one in each experimental group. A similar pattern of fibrosis was identified in both groups. The keratinized epithelium of the external prepuce kept its histological aspect and keratin production. Both keratinized and non-keratinized groups presented a slight decrease on the epithelial thickness, however without a statistically significant difference between groups. Conclusions In this short-term rabbit model, we observed that the stratified squamous keratinized epithelium from the external prepuce kept its keratin production. There was no statistical influence of the flap type on the mean epithelial thickness. .

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

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.

Testosterone supplementation's effects on age-related bladder remodeling - experimental study in rats.

OBJECTIVE: This study was designed to evaluate the effect of testosterone replacement on the fibrotic process of the detrusor bladder muscle during the normal aging process. METHODS: 15 Wistar senile rats, aged between 18 and 20 months were divided into two groups: testosterone group - 11 animals submitted to the administration of testosterone undecanoate (50 mg/kg intramuscular), once per month; and, Control group - four animals underwent a sham procedure. At the end of eight weeks, animals from both groups were sacrificed; bladders were removed and subsequently stereologically evaluated to determine the volumetric density of collagen fibers. The success of testosterone administration was confirmed by the measurement of serum testosterone at the beginning and end of the experiment. RESULTS: In the replacement group, testosterone average was 3.2 ng/ml, whereas in the control group, the mean testosterone at the end of the experiment was 0.64 ng/ml (p < 0.05). Analysis of stereological collagenous fiber showed higher density in the control group compared to the testosterone group I (56% versus 37.02%, respectively). The difference of volume concentration of collagen between both groups was statistically significant (p < 0.000). CONCLUSION: Bladder wall fibrosis was reduced in senile rats subjected to testosterone replacement.

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.

Medidas antropométricas como indicadores do estado nutricional em pacientes com espinha bífida submetidos a enterocistoplastia / Anthropometric measurements as an indicator of nutritional status in spina bifida patients undergoing enterocystoplasty

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.

OBJETIVO: Utilizar as medidas antropométricas para comparar o estado nutricional em crianças com bexiga neurogênica secundária à mielomeningocele submetidas a enterocistoplastia àquelas não submetidas à cirurgia. MÉTODOS: Estudo tipo caso-controle conduzido em 20 crianças, divididas em dois grupos, aquelas que realizaram enterocistoplastia (Grupo A) versus crianças não operadas (Grupo B), pareados por gênero e idade. Os parâmetros utilizados foram peso, altura, circunferência do braço e prega cutânea triciptal. A avaliação nutricional foi determinada calculando-se índices baseados na idade e no gênero. A classificação foi baseada em porcentagens, e os resultados foram comparados com valores de referência. RESULTADOS: A média da idade foi 6,41 anos no Grupo A e de 6,35 no Grupo B. O intervalo entre a cirurgia e a avaliação foi de 11 meses. As seguintes medidas foram encontradas para o Grupo A: 80% das crianças eram eutróficas, 30% maior do que no Grupo B; circunferência braquial foi adequada em 40% dos pacientes, 20% maior do que no Grupo B; a área do músculo do braço foi adequada em 90%, 30% maior do que no Grupo B. Os valores no Grupo B foram os seguintes: 60% apresentaram prega cutânea triciptal acima do valor médio, uma porcentagem 20% acima do que no Grupo A; para índice de gordura braquial, 60% dos pacientes estavam acima da média, 40% maior do que no Grupo A. CONCLUSÃO: Pacientes submetidos à enterocistoplastia demonstraram melhor estado nutricional enquanto o grupo controle apresentou índices de gordura mais elevados nas medidas antropométricas. Entretanto, a diferença entre os grupos não foi estatisticamente significativa.