Fetal intervention for severe lower urinary tract obstruction: a multicenter case-control study comparing fetal cystoscopy with vesicoamniotic shunting.

OBJECTIVE: To evaluate the efficacy of fetal intervention using fetal cystoscopy or vesicoamniotic shunting in the treatment of severe lower urinary obstruction (LUTO). METHODS: A cohort of 111 fetuses with severe LUTO attending two centers between January 1990 and August 2013 were included retrospectively. Fetuses were categorized into three groups based on the method of intervention: (1) fetal cystoscopy, (2) vesicoamniotic shunting or (3) no intervention. Multivariate analyses were performed to determine the probability of survival and normal renal function until 6 months of age by comparing fetal cystoscopy and vesicoamniotic shunting to no fetal intervention. RESULTS: Of the 111 fetuses with severe LUTO that were included in the analysis, fetal cystoscopy was performed in 34, vesicoamniotic shunting was performed in 16 and there was no fetal intervention in 61. Gestational age at diagnosis, method of fetal intervention and cause of bladder obstruction were associated with prognosis. In multivariate analysis and after adjustment for potential confounders (considering all causes of LUTO) the overall probability of survival was significantly higher with fetal cystoscopy and vesicoamniotic shunting when compared to no intervention (adjusted relative risk (ARR), 1.86 (95% CI, 1.01-3.42; P = 0.048) and ARR, 1.73 (95% CI, 1.01-3.08; P = 0.04) respectively). A clear trend for normal renal function was present in the fetal cystoscopy group (ARR, 1.73 (95% CI, 0.97-3.08; P = 0.06)) but was not observed in the vesicoamniotic shunt group (ARR, 1.16 (95% CI, 0.86-1.55; P = 0.33)). In cases in which there was a postnatal diagnosis of posterior urethral valves, fetal cystoscopy was effective in improving both the 6-month survival rate and renal function (ARR, 4.10 (95% CI, 1.75-9.62; P < 0.01) and 2.66 (95% CI, 1.25-5.70; P = 0.01) respectively) while vesicoamniotic shunting was associated only with an improvement in the 6-month survival rate (ARR, 3.76 (95% CI, 1.42-9.97; P < 0.01)) with no effect on renal function (ARR, 1.03 (95% CI, 0.49-2.17, P = 0.93)). CONCLUSION: Fetal cystoscopy and vesicoamniotic shunting improve the 6-month survival rate in cases of severe LUTO. However, only fetal cystoscopy may prevent impairment of renal function in fetuses with posterior urethral valves. Our data support the idea of performing a subsequent randomized controlled trial to compare the effectiveness of fetal cystoscopy vs vesicoamniotic shunting for severe fetal LUTO.

Cystoscopic placement of transurethral stent in a fetus with urethral stenosis.

We report the case of a fetus with severe megabladder, displaying the 'keyhole' sign on ultrasound imaging, that underwent cystoscopy at 22 weeks' gestation. There was a familial history of mild urethral atresia. Fetal cystoscopy revealed congenital urethral atresia. A guide wire was advanced through the fetal urethra and a transurethral vesicoamniotic stent was placed successfully. The fetus was delivered at 36 weeks' gestation and postnatal cystoscopy confirmed the absence of posterior urethral valves or urethral atresia. The infant was 5 years old with normal renal function at the time of writing. We conclude that fetal cystoscopic placement of a transurethral stent for congenital urethral stenosis is feasible.

One-stage external iliac fixation device and bilateral fascial and groin flaps facilitate abdominal wall closure after posterior sagittal iliac osteotomy in cloacal exstrophy.

PURPOSE: Repairing abdominal wall defects after cloacal exstrophy reconstruction always poses a challenge. Our proposal for repair consists of bilateral posterior iliac osteotomy and external iliac fixation with Schanz pins, together with abdominoplasty through bilateral groin flaps of skin and muscular aponeuroses, in a single staged procedure. METHODS: 7 patients (5 male and 2 female; mean age 3.1 years) with cloacal exstrophy underwent reconstructive surgery at our institution. Cloacal exstrophy reconstruction was performed in 3 stages, whenever possible. In Stage 1, the intestinal tract is separated from the hemi-bladders; the small colon is tubularized (colorrhaphy) with an opening on the left flank. The hemi-bladders are joined and the pathology is converted into classic bladder exstrophy, followed by primary repair (cystorraphy). At this stage, closure of the abdominal wall is made by groin flap plasty, following bilateral posterior iliac osteotomy with an external iliac fixator. Stage 2 consists of bladder augmentation and the management of urinary continence. Stage 3 is genitoplasty. The aim of this study was to demonstrate our results for the first stage. RESULTS: After a mean follow-up of 7 years, closure of abdominal wall was found to be excellent and successful in all 7 patients. Their abdominal walls are strong and solid, with no retraction, fistula or eventration. CONCLUSION: The association, in a single stage, of a posterior osteotomy with an external iliac bone fixator and bilateral groin flaps for the closure of soft tissue defects of the abdominal wall in cloacal exstrophy appears to be a safe and cosmetically acceptable alternative technique.

Early fetal cystoscopy for first-trimester severe megacystis.

OBJECTIVES: To report the feasibility of early fetal cystoscopy for the prenatal diagnosis and therapy of severe first-trimester megacystis. METHODS: Between January 2008 and February 2010, early fetal cystoscopy at 16 weeks of gestation was offered to 15 patients whose fetuses presented with severe first-trimester megacystis. All infants were followed up for 6-12 months after birth. Autopsy was always performed whenever fetal or neonatal deaths occurred. RESULTS: Seven patients decided to undergo fetal therapy, and eight elected to continue with expectant observation. One fetus died before early fetal cystoscopy was performed. Therefore, six fetuses underwent early fetal cystoscopy. Urethral atresia was diagnosed in three fetuses during fetal cystoscopy and confirmed at autopsy following termination of pregnancy at 19-20 weeks in all cases. Posterior urethral valves were diagnosed and successfully fulgurated by laser during early cystoscopy in three fetuses, two of which survived with normal renal and bladder function after birth; the remaining fetus had a postnatal diagnosis of megacystis-microcolon intestinal hypoperistalsis syndrome and died neonatally. In the expectantly managed group, no survivals were observed, even among cases with 'isolated' posterior urethral valves. CONCLUSIONS: Percutaneous early fetal cystoscopy is feasible for prenatal diagnosis and therapy of severe megacystis.

Genital trauma due to animal bites.

PURPOSE: Animal bites to the external genitalia are rare. We retrospectively evaluated our experience with treating genital trauma caused by animal attacks. MATERIALS AND METHODS: We studied the medical records of 10 patients treated in the surgical emergency department at our hospital who presented with genital injury caused by an animal bite from 1983 to 1999. Special attention was given to the severity of injury, surgical treatment, antibiotic prophylaxis and outcome. RESULTS: Of the 2 men and 8 boys 8 were attacked by dogs, 1 by a horse and 1 by a donkey, respectively. In all cases initial local treatment involved débridement and copious wound irrigation with saline and povidone-iodine solution. Five patients who presented with minimal or no skin loss underwent primary skin closure, including 2 in whom urethral lacerations were surgically repaired. There was moderate to extensive tissue loss in 5 patients, including degloving penile injury in 2, traumatic spermatic cord amputation in 1, complete penile and scrotal avulsion in a 5-month-old infant, and partial penectomy in 1. Reconstructive procedures provided satisfactory cosmetic and functional results in 8 cases. Antibiotic prophylaxis was administered in all patients and no infectious complications developed. CONCLUSIONS: Animal bite is a rare but potentially severe cause of genital trauma and children are the most common victims. Morbidity is directly associated with the severity of the initial wound. Because patients tend to seek medical care promptly, infectious complications are unusual. Management involves irrigation, débridement, antibiotic prophylaxis, and tetanus and rabies immunization as appropriate as well as primary wound closure or surgical reconstruction. Good functional and cosmetic results are possible in the majority of cases.

Incontinent epispadias: surgical treatment of 38 cases.

We review 38 cases of surgically corrected incontinent epispadias with a followup of 5 months to 18 years. In 20 cases the Leadbetter, in 8 the Tanagho and in 8 the Young-Dees techniques of bladder neck reconstruction were used. Of 3 patients with minimal (15 to 25 ml.) bladder capacity the Arap procedure was performed in 1, while small constriction of the bladder neck to improve the bladder capacity and compliance was done in 2. In 1 of the latter patients a 60 ml. capacity was achieved and a secondary Leadbetter operation provided an excellent result. Continence was attained after the initial operation in 18 patients, followup is too short to determine the result in 3 and 15 did not acquire urinary control. Revision of the bladder neck plasty was performed in 11 patients, which resulted in continence in 4 and partial continence in 2. Among 34 patients with an adequate followup 22 (73.3 per cent) are continent and 8 (26.4 per cent) are incontinent. The results were similar with the 3 techniques.

Duplicated exstrophy: report of three cases.

Three cases of duplicate exstrophy are described and the theoretical mechanisms of embryogenesis discussed. In all 3 cases there was classic pelvic skeletal deformity. Urinary control was normal in 2 of the 3 patients. The surgical approach consisted of plastic repair of the abdominal wall defect in all patients and correction of urinary incontinence in 1 patient.

[Vesicostomy: a temporary urinary diversion in childhood]. / La vésicostomie: dérivation urinaire temporaire dans l'enfance.

The authors report on 21 pediatric cases of tubeless cystostomies (19 males and 2 females). 15 cases of posterior urethral valves (septic condition and very narrow urethra). 3 primitive vesico-renal refluxes (small bladder, severe impairment of renal function and extensive dilatation of the upper urinary tract). 2 prune-belly syndromes. 1 neurogenic bladder. (Impracticable intermittent catheterization). 19 of the 21 cases showed dramatic improvement (narrowing of the upper urinary tract, disappearance of infection and resumption of staturo-ponderal thrive). Only 1 child with valves had to be put on cutaneous ureterostomy. The improvement in the upper urinary tract, as seen on the IVP, is very rapid in valve cases without reflux. The improvement is visible earlier on isotopic renal scans than on IVP. 11 children suffered acute pyelonephritis during vesicostomy. There were no cases of cutaneous peristomal modification. Simultaneous closure of the derivation and surgery of the primitive disease were performed in 10 cases after a 1-3 year period of vesicostomy. Vesicostomy will succeed in those babies who have shown clinical and biological improvement after a short period of trans-urethral or suprapubic catheterization. The suprapubic endoscopis treatment of the valves becomes possible through the vesicostomy hole excluding urethral trauma.

Experiencia com osteotomia pelvica na reconstrucao cirurgica da extrofia vesical. / Experience with pelvic osteotomy in surgical reconstruction of blader exstrophy

Os autores estudaram retrospectivamente uma serie de 16 pacientes submetidos a osteotomia pelvica, como recurso auxiliar na reconstrucao cirurgica da extrofia vesical. Onze pacientes eram do sexo masculino e cinco do feminino, com media de idade de 3,1 anos; em oito casos processou-se a osteotomia iliaca posterior, em sete a osteotomia inominada e em um caso a osteotomia pubiana. A analise dos resultados mostrou as vantagens, os inconvenientes e as complicacoes da osteotomia. Concluem que: a osteotomia e eficiente no fechamento da parede abdominal como artificio cirurgico, diminuindo a tensao dos tecidos; a imobilidade no leito foi em media de 30 dias; a hospitalizacao de 75 dias utilizando-se a osteotomia posterior e 45 dias nas demais tecnicas; a rotacao medial e inferior da pelvis provocou sepultamento adicional do penis, assim como a reconstrucao do monte de venus foi prejudicada, permitindo cicatriz glabra mediana; um caso com osteomielite dos iliacos com sequelas graves sobre a marcha e postura; presenca de fistulas urinarias, supuracoes nos casos em que se usou o gesso

Nonrefluxing colonic conduit: efficiency and complications of ureterocolic anastomosis.

The efficiency of and complications in 65 cases of colonic conduit have been studied (64 cases of bladder exstrophy and 1 case of cloacal exstrophy). For the ureterocolic reimplantation, we employed the Leadbetter technique and a modified kelalis technique. The results after 1-12 years in 128 renoureteral units showed 28 complications (21 obstruction and 7 refluxing units). Other types of complications have been observed and reported in this work. The high incidence of obstruction was probably due to the long submucous tunnel in the ureterocolic reimplantation. We believe that the sigmoidal conduit is an adequate method for temporary or permanent diversion.

Estenose da juncao pieloureteral na infancia: diagnostico, tratamento e evolucao de 49 casos. / Pyeloureteral junction stenosis in children: diagnosis, treatment and course of 49 cases

Os autores apresentam uma experiencia de 10 anos no manuseio de 49 criancas portadoras de estenose da juncao pieloureteral, internadas na Clinica Urologica do Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo. Descrevem a casuistica, os procedimentos adotados no pre-operatorio, os tipos de cirurgias realizadas, os resultados obtidos e as complicacoes observadas. Tecem consideracoes especificas sobre os cuidados cirurgicos que devem ser tomados para evitar as complicacoes