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Occult spinal dysraphism: clinical and urodynamic outcome after division of the filum terminale. Similar findings were reported in healthy, asymptomatic infant siblings of children with vesicoureteral reflux (Bachelard et al, 1999). A retrospective review of onlay versus tubularized preputial flap repairs by Wiener and colleagues (1997) found that 12% of those tubularized developed diverticulum during follow-up to a mean of 20 months, which might suggest that tubed flaps have greater risk than onlays for this complication. It should also be recognized that "obstruction" does not mean surgical therapy is required. Resident pericytes and infiltrating hematopoietic stem cells can also differentiate into fibroblasts. Sacral sensation is relatively spared, even in the presence of extensive sacral motor deficits (Boemers et al, 1994a). Further descriptive subdivision of ureterocele types has been published, particularly by Stephens (Stephens, 1971; Stephens et al, 1996). Diagnosis General Issues the challenge in diagnosing "obstruction" has been made more difficult because of disagreement regarding the definition of obstruction. Compilation of the cases reported in the literature between 1950 and 1970 by Waldbaum and Marshall (1970) showed that 86% of the 56 accurately traceable patients had died, with or without surgical intervention. Data from Reiner and colleagues (2008) from a large series of male patients revealed that 14% experienced suicidal ideation. The case of Seitzman and Patton involved an ectopic ureter that emptied, along with the ipsilateral vas deferens, via a persistent common mesonephric duct into the proximal posterior urethra (Seitzman and Patton, 1960). Ureteral access sheath for the management of pediatric renal and ureteral stones: a single center experience. Therefore, it is doubtful that growth of this abnormally configured prostate can have much impact on urinary continence after failed bladder neck repair, and improved continence at puberty may be a result of changes in the pelvic floor with maturity. What is known with certainty is that enuresis is a common medical condition in children (Shreeram et al, 2009). Depending on the degree of proteinuria, consideration should be given to obtaining a serum creatinine level to estimate the glomerular filtration rate. The major steps are mobilization of any structures extending distal to the internal ring, including epididymis and vas and gubernacular remnant, transection of the peritoneum lateral to the vessels and distal to the vas, and proximal mobilization of the vessels while maintaining collateral blood supply between the vas and spermatic vessels. OtherBladderAnomalies Nephrogenic Adenoma Nephrogenic adenoma of the urinary bladder is a rare benign tumor mostly found in adults. The main difference is that the urethra is separated from its attachments to the underlying corporeal bodies and pelvic diaphragm during the first stage of the procedure. This also moves the pelvic floor muscles into a more anterior position, thus providing more support for the anterior pelvic organs. A low total motile count was found Surgical Repair of Varicocele Several approaches exist to correct the adolescent varicocele: inguinal or subinguinal, laparoscopic or retroperitoneal, or venographic. Division is seldom performed as a primary procedure but may be considered if the previously mentioned repairs fail (Khoury et al, 1999; Bergman et al, 2006; Landau et al, 2009; De Troyer et al, 2011; Baradaran et al, 2012; Kavanagh et al, 2012). Expanded polytetrafluoroethylene patch in hernia repair: a review of clinical experience. Is oral vitamin B12 therapy effective for vitamin B12 deficiency in patients with prior ileocystoplasty. Anorectal malformation and associated end-stage renal disease: management from newborn to adult life. Interleukin 8 receptor deficiency confers susceptibility to acute experimental pyelonephritis and may have a human counterpart. There is evidence that a deletion of the seventh chromosome (7q36) leading to the absence of a transcription factor may be responsible for this anomaly (Papapetrou et al, 1999). The congenital form of buried penis is believed to result from the inelasticity of the dartos fascia, which normally allows the penile skin to slide freely on the deep layers of the shaft, with restricted extension of the penis because the penile skin is not anchored to the deep fascia. Gait is usually not affected, although it may be delayed, and the children tend to roll to their sides and use their arms to sit from a supine position. Medical management of mild and moderate vesicoureteral reflux: followup studies of infants and young children.

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DisimpactionandLargeBowel/RectumWashout Decreasing fecal load in the large intestine and rectal vault is the first step toward establishing an optimal medical regimen. Reconstruction of the urinary tract by cecal and ileocecal cystoplasty: review of a 15-year experience. Expected Bladder Capacity and Optimal Bladder Capacity the most relevant parameters for interpretation of uroflowmetry are voided volume, Qmax, and uroflow patterns. In addition, other patient factors known to modify surgical outcomes such as preexistence or subsequent onset of elimination dysfunction must be considered. Fetal obstructive uropathy without renal dysplasia: a study of the renal findings in 13 cases presenting with megacystis. Other diagnoses, especially when augmentation cystoplasty is not necessary, may allow for spontaneous voiding. Robot assisted laparoscopic partial nephrectomy: a viable and safe option in children. Recent studies support the idea that degeneration of the Chwalle membrane (Chwalle, 1927) is important for generating a patent connection between the ureter orifice and the bladder (Mendelsohn, 2009). Excretory urography often fails to visualize the portion of the ureter beyond the J hook. In contrast, a concave (left) or flat papilla refluxes because its collecting ducts open at right angles onto a flat papilla. In reflux nephropathy using color Doppler ultrasonography, renal resistive index measurements derived from blood flow in interlobar and arcuate arteries are significantly increased in higher grades of reflux and correlate positively with scintigraphic findings from the same renal unit (Radmayr et al, 1999). An incision along the superior scrotal border is made as described by Bianchi and Squire for any palpable testicles. It is prudent for the surgeon who may see only a few patients with this condition to consider referral of these complex management situations to a center where special expertise and experience exist. The most common malformation was a duplicated collecting system (6); others included hypoplastic or absent kidney (3), pelvic kidney (2), ureteropelvic junction obstruction (1), and multicystic dysplastic kidney. A second scoring system, from the Toronto group, was published in 2000 (Farhat et al, 2000). If this trend continues without rigorous prospective analysis, it could erode equipoise, to potentially position this technique as another first-line therapy after reflux diagnosis. With the recognition that infection and progressive renal insufficiency are the factors that most often pose the greatest threat to quality of life and survival, surgical reconstruction to normalize the anatomy and function of the genitourinary tract was advocated. The use of expanded polytetrafluoroethylene (Gore-Tex) in rhinoplasty: a 6-year experience. Minimizing any type of catheter balloon filling is important to prevent inducing bladder spasms in the small, hypertrophic bladder and potentially occluding the ureteric orifices (Jordan and Hoover, 1985). Genome-wide expression profiling of urinary bladder implicates desmosomal and cytoskeletal dysregulation in the bladder exstrophy-epispadias complex. This segment should be kept as short and straight as possible to facilitate easy intermittent catheterization. Functional ectopic ureter in case of ureteric duplication in children: initial experience with laparoscopic low transperitoneal ureteroureterostomy. Testicular growth from birth to two years of age, and the effect of orchidopexy at age nine months: a randomized, controlled study. Cassar and colleagues (2008) evaluated Doppler waveforms in cases of torsion with decreased or preserved testicular flow and observed subtle waveform abnormalities, including increase or decrease in amplitude relative to the normal testis and reversal of diastolic flow. Those with an elevated leak point pressure and good initial compliance seem to have the most long-lasting results from the procedure. Contralateral inguinal hernia after negative laparoscopic evaluation: a rare but real phenomenon. Use of a technique that staged the closure over a 2- to 3-week period after performance of the osteotomy by gradually cranking the fixator medially was also shown to be of benefit in providing a successful primary or secondary closure (Mathews et al, 2006). Prevalence of urinary tract infection and vesicoureteral reflux in children with lower urinary tract dysfunction.

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Success is enhanced by elevation of the epithelium of the bladder neck, which may be compromised by scarring from previous operative procedures. Evaluation of renal function, renal drainage, or both, is required in those with renal insufficiency. Bilateral undescended testes classified according to preoperative and postoperative status of gonadotropins and Sathyanarayana S, Beard L, Zhou C, et al. Anderson and Rickwood (1991) have reported perforations occurring in bladders with significant uninhibited contractions after augmentation, as have others (Pope et al, 1998). This space is lined by the obliterated umbilical arteries, with its base on the anterior dome of the bladder and the tip directed toward the umbilicus. All operative techniques that create an increase in outlet resistance do so at the expense of detrusor contractibility. Rather than providing strict guidelines for treatment, these principles provide ample room to individualize treatment on the basis of the particular preferences and tolerances of physicians, families, and patients for the various burdens each of the principles entails. We often remind parents that at the initiation of therapy, the child may fail to awaken and that parents should wake the child when the alarm sounds. Suture size depends on the prevailing anatomy, but most often a 6-0 or 5-0 resorbable monofilament suture on a round needle is used. Ten-year review of groin laparoscopy in 1001 pediatric patients with clinical unilateral inguinal hernia: an improved technique with transhernia multiple-channel scope. The original Young procedure has evolved and remains of primary consideration when reconstructing the exstrophic bladder neck (Ferrer et al, 2001). They refuted the existence of the pop-off mechanism accounting for new contralateral reflux in their series because the risk for new contralateral reflux did not correlate with the preoperative reflux grade (grades 4 and 5). Impaired reproductive development in sons of women occupationally exposed to pesticides during pregnancy. The authors concluded that earlier orchidopexy may be more technically demanding and supported the concept that expertise of the surgeon is relevant for more challenging cases. The long-term outcome of the endoscopic subureteric implantation of polydimethylsiloxane for treating vesico-ureteric reflux in children: a retrospective analysis of the first 195 consecutive patients in two European centres. Downregulation of aquaporin-2 parallels changes in renal water excretion in unilateral ureteral obstruction. The value of intermittent self catheterization in the early management of traumatic paraplegia and tetraplegia. Migration and granulomatous reaction after periurethral injection of polytef (Teflon). Glucomannan is not effective for the treatment of functional constipation in children: a double-blind, placebo-controlled, randomized trial. Therefore, appropriate treatment of these genitourinary malformations can significantly affect and improve QoL. An ultrasound examination of the bladder will usually confirm the diagnosis, and kidney images will further support this. The presence of a cremasteric reflex correlates with intact testicular blood flow but does not unequivocally indicate normal testicular perfusion, especially if the clinical presentation is otherwise suggestive of torsion. Smallercalibernephroscopes(15to18Fr)withoffset lenses have greatly facilitated standard percutaneous treatment techniquesinpediatricpatients. Consideration must eventually be given to removal of the original segment and replacement with another after repeated perforation. Cloacal exstrophy and cloacal exstrophy variants: a proposed systemofclassification. Use of the dysfunctional voiding symptom score to predict resolution of vesicoureteral reflux in children with voiding dysfunction. A voiding diary kept by the parents should help assess the times at which a child voids; the relationship between voiding and common events such as meals, breaks at school, and play activities; the occurrence of urgency or incontinence; and voided volume. Artificial urinary sphincter in the treatment of urinary incontinence: preoperative urodynamics do not predict the need for future bladder augmentation. Onlay flap repair for proximal hypospadias was reported in 126 patients with mean follow-up to 22 months.

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Although it is possible to achieve some penile lengthening and release of chordee at the time of initial closure, it is always necessary to perform formal penile elongation with release of chordee at the time of urethroplasty in exstrophy patients. True continence, as defined by a 4-hour dry period between voidings or catheterizations, has been reported to be at most 78% and has ranged as low as 5% (Leonard et al, 1990a; Capozza et al, 1995; Bomalaski et al, 1996; Perez et al, 1996b; Sundaram et al, 1997; Silveri et al, 1998; Guys et al, 2001; Godbole et al, 2003; Halachmi et al, 2004; Dean et al, 2007; Kitchens et al, 2007; De Vocht et al, 2010; Kaye et al, 2010; Alova et al, 2012a; DaJusta et al, 2013). This results in a variety of effects, including growth alterations, fibrosis, as well as altered blood flow. Fifteen patients older than 16 years had engaged in satisfactory intercourse, and all reported orgasms and ejaculation with a straight penis on erection. The dorsal prepuce is split down its midline and the two parts are transferred ventrally with their dartos vascular pedicles and sutured into the defect from the meatus to the glans. Trans-scrotal approach for surgical correction of cryptorchidism and congenital anomalies of the processus vaginalis. Cystoscopy can provide useful information immediately before open surgery, such as confirmation of orifice position and duplication and the proximity of diverticula to the orifice, and clarify urethral patency if indicated. The measurement of amniotic fluid as an indicator of fetal urine production is a critical portion of every antenatal ultrasound. Every effort should be made by the examiner to determine the lowest position the testis may attain. The epithelial edges of the trough are then secured to the lateral aspect of the tube. A single-blinded, randomized comparison of laparoscopic versus open hernia repair in children. Dilations of the fetal bladder caused by anatomic obstructions are mostly due to urethral anomalies or external obstruction. These investigators found a higher rate of behavior problems in children with voiding postponement compared to those with urge incontinence as their major complaint (Lettgen et al, 2002; von Gontard et al, 2011a). It may be that detrusor filling pressures need to be looked at in a more critical way to determine whether they are an important factor in upper urinary tract deterioration. These children can present at any age, although the condition is rarely recognized in early childhood. They may also be downstream effects reflecting specific alterations mediated by other cytokines, which in themselves are not directly relevant but are indicative of the level and pattern of the obstructive effect. Autosomal recessive polycystic kidney disease: genetic disease online reviews at GeneTests-Geneclinics. Despite reports to the contrary in select patients (Husmann and Cain, 1999), concern about fecal incontinence secondary to use of the ileocecal valve persists for patients with neurogenic dysfunction. They no longer, however, recommend such extensive reconstructive surgery before the age of 3 to 6 months. We learned that this objective patient selection resulted in double the number of boys receiving stimulation compared to our previous subjective use for a "small-appearing" glans. After 6 months of follow-up, however, they reported that there was no significant difference between the two treatment groups regarding the efficacy and number of children who relapsed. Parental counseling regarding the signs and symptoms of incarceration should occur. The incidence of positive contralateral inguinal exploration among preschool children: a retrospective and prospective study. The use of estrogen and progestin contraceptives may increase the risk of thromboembolic events in those with decreased mobility, and the use of intrauterine devices for contraception is unsafe owing to pelvic infections (Jackson and Mott, 2007). If one is incompetent, the corresponding bladder neck can be closed and the connected urethra excised. The timed voiding regimen is often linked to a positive reinforcement program using a diary in which the child tracks the number of times voided during the day. Typically, all patients have been maintained on nasogastric decompression until bowel function recovers, although two studies (Gundeti et al, 2006; Erickson et al, 2007) have suggested that nasogastric suction may not be necessary after ileocystoplasty. Fortunately, if the correct diagnosis is missed in such a case, there are unlikely to be significant consequences, because a totally nonfunctioning hydronephrotic kidney is rarely salvageable and probably will cause no problems other than a predisposition to infection or hypertension. Micropenis in hypogonadotropic hypogonadism: response of the penile androgen receptor to testosterone treatment. The desired graft is outlined, injected with 1: 100,000 epinephrine, and harvested. Questionnaire, hormone, semen analysis, and paternity data were analyzed for a large cohort of men who underwent orchidopexy between 1955 and 1975 and a control group of similar age who were matched for timing of unrelated surgery. In an extensive review of the literature by Gordon and colleagues, additional inguinal incisions were needed in 4.

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Peak retrograde flow: a novel predictor of persistent, progressive and new onset asymmetry in adolescent varicocele. Isolated cryptorchidism: no evidence for involvement of genes underlying isolated hypogonadotropic hypogonadism. Anorectal Defects the perineum is short and broad and the anus is situated directly behind the urogenital diaphragm; it is displaced anteriorly and corresponds to the posterior limit of the triangular fascial defect. Treatment of distal ureteral stones in children: similarities to the American Urological Association guidelines in adults. As a nonionizing, noninvasive imaging platform, coupled with its ability to assess renal vasculature, it is ideally suited to serial follow-up of renal growth and development. DiagnosisandClassification the vast majority of varicoceles in children and adolescents are identified incidentally by a primary care practitioner. With perhaps the most compelling evidence to date, Kaefer and colleagues (1999a) found that only 17% of patients with hostile neurogenic bladder dysfunction treated immediately on diagnosis required augmentation cystoplasty as compared with 41% of similar patients treated expectantly. Robotic-assisted pyeloplasty has a shorter and more efficient learning curve compared to that of laparoscopy because of advanced visualization through threedimensional vision and the dexterity of the instruments provides for easier manipulation, precise dissection, and suturing. Does the type of bladder augmentation influence the resolution of pre-existing vesicoureteral reflux Effects of the artificial urinary sphincter on prostatic development and sexual function in pubertal boys with meningomyelocele. In acquired bladder diverticula, the infravesical obstruction has to be eliminated first. Genital malformations and coexistent urinary tract or spinal anomalies in patients with imperforate anus. Renal preservation in children with neurogenic bladder-sphincter dysfunction followed in a national program. The response is more gradual and sustained than for desmopressin, with approximately two thirds of children becoming dry during active treatment and nearly half remaining dry after treatment completion (Glazener et al, 2005). Notwithstanding the previous discussion, modern digital fluoroscopic equipment has further reduced the radiation exposure of conventional fluoroscopy, thereby narrowing the difference in radiation exposure between the two modalities. PregnancyandReflux the morphology of the urinary tract is altered with the onset of pregnancy and increases throughout gestation (Beydoun, 1985). In modern exstrophy reconstructive techniques, most surgeons try to preserve the urethral plate at the time of exstrophy closure. Efforts to reduce the inflammation include the use of ice packs, nonsteroidal anti-inflammatory agents, scrotal elevation, and rest to avoid traumatic exacerbation. Total continence reconstruction using the artificial urinary sphincter and the Malone antegrade continence enema. In addition, ambulatory status did not correlate with urodynamic findings (Tanaka et al, 2006). In most series of autoaugmentation, no matter what the technique, occasional patients have been noted or concern has existed at the time of the initial procedure that adequate expansion was not achieved. The duplication most commonly occurs in the sagittal plane with one urethra located ventrally and the other dorsally. If an abnormality is found, the evaluation should then be directed toward determining the underlying cause and distinguishing whether the dysfunction stems from an anatomic or functional issue. Hassan and colleagues (2003) found that the presence of urinary reflux did not correlate with renal outcomes, underscoring that the presence of reflux alone should not be seen as an indication for intervention. UrinaryReconstruction Modern Staged Reconstruction the staged management of the urinary tract follows that used for the management of bladder exstrophy (Gearhart and Jeffs, 1991b). A combination of medications and intermittent catheterization have a positive impact, particularly in children with neurogenic dysfunction (Rink and Mitchell, 1984; Aslan and Kogan, 2002; Verpoorten and Buyse, 2008). Thus the entire reflux literature, which historically reports results in terms of five separate grades, must be considered with some circumspection because the veracity of grade at the time of the contrast study may be, to some extent, arbitrary. The adventitia of the ureter immediately proximal to the cuff is then approximated to the full thickness of the ileal wall at the hiatus so that the cuff protrudes into the lumen. These may not be readily identified preoperatively (Smith and Parrott, 1994), and their complexity may create surgical challenges, particularly with endoscopic incision (see later). After the testis has been delivered, the distal sac and overlying cremaster are mobilized proximally as far cranially as possible, "high above the inguinal canal" (Iyer et al, 1995). In a review by Baker and colleagues (1999), posterior urethral obstruction was found in 41 patients.

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Bladder neck sling for treatment of neurogenic incontinence in children with augmentation cystoplasty: long-term followup. Rumplessness of chicken embryos produced by the injection of insulin and other chemicals. Therapy with the alarm can be reinitiated for relapse (more than two wet nights in 2 weeks). Towards a rational terminology in the study of the gubernaculum testis: arguments in support of the notion that the cremasteric sac should be considered the gubernaculum in postnatal rats and other mammals. Since the first report, modifications have been made to improve flap viability, minimize fistula formation, and extend the indications for the procedure beyond that of the neurogenic bladder (Salle et al, 1997). Resolution of vesicoureteral reflux in completely duplicated systems: fact or fiction Vesicoscopic ureteral reimplantation: a minimally invasive technique for the definitive repair of vesicoureteral reflux. These structures can be very difficult to differentiate from an umbilical-urachus sinus because no connection to the bladder or bowel can be seen on sinugram. A 6-Fr stent is passed into the bladder and tubularization is done in two subepithelial layers, the first using an interrupted 7-0 polyglactin stitch and the second a continuous 7-0 polydioxanone stitch. Other Testicular Anomalies Associated with Cryptorchidism Several rare anomalies of testicular development associated with cryptorchidism, each with 100 to 150 reported cases in the literature, include polyorchidism, splenogonadal fusion, and transverse testicular ectopia. Briefly, a 7-day bowel diary is advisable and includes the Bristol Stool Form Scale. It is critical that the patient be well hydrated at the time of surgery even if it necessitates the use of intravenous fluids during the preoperative day. Cyst puncture and aspiration with or without contrast medium injection has been popular in the past, but with current imaging modalities the need for cyst puncture is very rare. Increased activity of the hypothalamic-pituitary-testicular axis in infancy results in increased androgen action in premature boys. Impaired transformation of gonocytes to spermatogonia is reported in cryptorchid testes and may help define fertility potential. Preoperative stone attenuation value predicts success after shock wave lithotripsy in children. Optimal surgical treatment and urological outcomes in boys with pelvic and urogenital rhabdomyosarcomas and soft tissue sarcomas. The understanding of the normal anatomy and embryologic development of the male external genitalia is essential to recognizing and treating penile and scrotal anomalies. The embryology of anterior urethral valves is not clear, but abortive corpus spongiosum over the affected portion of the anterior urethra indicates a place on the hypospadias spectrum or a faulty union between urethral mucosa and the epithelium of the fossa navicularis. During the third trimester of pregnancy, the fetus is voiding at the rate of approximately 30 times every 24 hours (Goellner et al, 1981). Vesicoureteral reflux diagnosed in adulthood: incidence of urinary tract infections, hypertension, proteinuria, back pain and renal calculi. UrethralDuplication Urethral duplication is another rare anomaly of the urethra with several known anatomic variants. Several limitations reiterate the significant challenges in conducting such studies. C,Perimeatalshaft skin to be excised before the inner preputial "collar" is made ventrally. Epidemiology of bladder exstrophy: a communication from the International Clearinghouse for Birth Defects monitoring systems. A history of cryptorchidism is associated with a twofold to fivefold increased risk of testicular cancer, lower than historical estimates. A comparison of calcium hydroxyapatite and dextranomer/hyaluronic acid for the endoscopic treatment of vesicoureteral reflux. Diagnosis and management of eosinophilic cystitis: a pooled analysis of 135 cases. Standardized tools have been used to reflect improvements in quality of life (Shankar et al, 1998), self-esteem, psychosocial function (Aksnes et al, 2002), and fecal incontinence-related and constipation-related quality of life (including anxiety and family worry) (Ok and Kurzrock, 2011).

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Detrusor myectomy (autoaugmentation) in the treatment of hyper-reflexive low compliance bladder. These include bladder exstrophy, epididymal anomalies, connective tissue disorders, cystic fibrosis, and posterior urethral valves. In another series, extrascrotal testes were less likely to descend by 1 year of age (50%) than high scrotal testes defined as cryptorchid at birth (87. In reducing the capacity of this system to support function by the administration of captopril, a decrease is detected in the postcaptopril study. Work has shown that one dilated ureter typically is enough for cystoplasty (Zubieta et al, 1999; Kajbafzadeh et al, 2010). Controversy exists as to whether skeletal cremaster muscle fibers originate from abdominal wall musculature or from within the gubernaculum itself (van der Schoot, 1996; Barteczko and Jacob, 2000; Niikura et al, 2008), but their unique innervation and recent studies of transgenic mice (see later) suggest the latter. Papillary Anatomy Another factor governing renal susceptibility to scarring is the configuration of the papillae as their ducts open to the calyces. Family and twin studies suggest locus heterogeneity and poor phenotype-genotype correlation (von Gontard et al, 2011b). In 53 sessions requiring a mean number of 3489 shock waves per session, stone-free and complication rates were 83. This also will afford the opportunity for a new cohort of older patients to provide longer term resolution rates on a systematic basis. Using blunt dissection, a plane between the posterior bladder neck and vagina in girls or rectal wall in boys is developed. Unilateral ureteral obstruction in early development alters renal growth: dependence on the duration of obstruction. The ultimate determination of the need for intervention may come as a single diagnostic test, or it might be the determination based on a pattern of change, or the lack thereof, throughout time. This procedure has been performed clinically with early reports of good results in most patients (Gonzalez et al, 1994). Most studies note a 10-year minimum lag time between the augmentation and presentation of disease. Studies by Stec and colleagues (2003) using sections from the bony pelvis in fetal exstrophy specimens and normal aborted fetuses found that the ultrastructure, bone development, microscopic growth patterns, and endochondral ossification were absolutely the same. Injection can be done directly through the working channel of the endoscope, often one with an offset lens system. The psoas hitch should be carried out with nonabsorbable suture before creation of the submucosal tunnel. This draws the extravesical and intravesical points of the intramural ureter closer together, shortening and widening the tunnel, and allows passage of the urine bolus into the bladder. Acquired cystic disease of the kidney in patients with end stage chronic renal failure: a study of prevalence and etiology. Various flaps have been described as a method for avoiding stomal stenosis (Keating et al, 1993; Kajbafzadeh, et al, 1995; Kaefer and Retik, 1997; Frane-Guimond et al, 2006; Berrettini et al, 2008; Landau et al, 2008). The precalyceal canalicular ectasia may involve one or more renal papillae in one or both kidneys. Two retrospective series obtained intravenous pyelography or renal sonography in 41% and 72% of patients, both reporting 18% to be abnormal. The rectal defect may be closed primarily, but if redundant intestinal tissue is available, then the fistulous portion of intestine is excised. Urinary Tract Infection Bacteriuria is common after intestinal cystoplasty, particularly in patients requiring intermittent catheterization (Gearhart et al, 1986; Hendren and Hendren, 1990; King, 1991). Because we choose not to perform flap urethroplasties, we lengthen the shortened ventral tunica albuginea of the corpora using three corporotomies without grafting. Currently, however, most authors recommend assigning gender that is consistent with karyotypic makeup of the individual if at all possible. These results were mirrored in the long-term follow-up study of Mollard and associates (1998). An innovative trial by Chrzan and colleagues (2013) was undertaken in 18 patients, using laparoscopic techniques that allowed for "vesicoscopic" exposure of the bladder neck. To qualify for a staccato label, the fluctuations should be larger than the square root of the maximum flow rate. Indeed, this common finding should argue against performing such studies too early after surgery. Prospective study on the prevalence and associated risk factors of cryptorchidism in 6246 newborn boys from Nice area, France.

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Eventually, the enlarged diverticulum can become responsible for ureteral obstruction. A traction suture is passed percutaneously at the level of the bladder dome under cystoscopic vision to anchor the bladder wall to the abdomen and prevent it from pulling away when the camera port incision is made at the dome. Both muscle types were also observed by other (Wyndham, 1943; Lemeh, 1960) but not all (Heyns, 1987; Costa et al, 2002; Niikura et al, 2008) researchers. In our experience, this osteotomy is superior to the pubic mobilization seen with simple bilateral transverse anterior innominate osteotomy or even pubic ramotomy. Repeated botulinum-A toxin injections in treatment of children with neurogenic detrusor overactivity. We make this decision with the patient under anesthesia at the time of primary closure with our pediatric orthopedic colleagues. If the appendix is used in situ as a continence mechanism in a continent urinary reservoir, the reservoir by necessity will include the right colon. Further maneuvers to provide spermatic cord length include transection of lateral fascial bands along the cord, cranial retroperitoneal dissection, medial transposition of the testis beneath the epigastric vessels (Prentiss maneuver), and, if required, cranial extension of the incision. However, in most of these failures the bladder capacity is small and augmentation is required. Infants void with significantly higher maximum detrusor pressures than in normal adults, and male infants void with significantly higher pressures than females. The conclusion was that male patients with genital reconstruction and closure of the urethra demonstrated high risk of infertility. Skin coverage can also be accomplished by penoscrotal Z-plasty, lateral penile shaft Z-plasty, island pedicle of ventral preputial skin, or a skin graft. Transgenic deletion of either of two homeobox genes, Hoxa10 and Hoxa11, is associated with isolated cryptorchidism in mice (Satokata et al, 1995; Potter and Branford,1998) with evidence for delayed muscle development in the Hoxa11 gubernaculum (Harisis et al, 2013). An alternative but equally efficacious technique involves meatal tailoring using clamps without the need for suture (Cubillos et al, 2012). Worsening of bladder function is noted in 4% to 30% (Simeoni et al, 1996; Castera et al, 2001; Catti et al, 2008). Normally the bladder is a highly compliant vesicle in that it will accommodate an increasing volume of urine without a corresponding increase in intravesical pressure. Shukla and colleagues (2004) reported results in only 10 patients with an average 43 months of follow-up, noting urethroplasty complications in 70%, including seven fistulas, three meatal stenoses, and one diverticulum. Considerations the amount of bowel used in continent urinary diversion may vary depending on the patient. During sleep the bladder is normally quiescent and stable with lack of facilitation of detrusor contractions, whereas during wakefulness marked detrusor overactivity is observed. Nocturnal bladder emptying: a simple technique for reversing urinary tract deterioration in children with neurogenic bladder. Mobilizing lateral mons veneris flaps brings the clitoral halves closer to the midline and makes them easier to reconstruct into a single entity. As previously noted, there is poor correlation between the extent of abdominal wall deficit and the degree of hydronephrosis or renal dysplasia, or both. However, if a 6-year-old with normal bladder function presents with grade 3 reflux, it is much less likely to resolve. Nevertheless, with increasing experience modest success rates appear to improve (Curry et al, 1999; Kokoska et al, 2001). Success with this approach has been reported in numerous series, and it is now a procedure commonly practiced around the world (Squire et al, 1993; Griffiths and Malone, 1995; Koyle et al, 1995; Malone, 1995; Dick et al, 1996; Gerharz et al, 1997a; Schell et al, 1997; Peeraully et al, 2014). The outcome of this situation is guarded but good, because most cases resolve completely as edema of the cord in response to the injury subsides, leaving no permanent damage (Iwatsubo et al, 1985; Fanciullacci et al, 1988). Decisions regarding the suitability of bladder closure or the need for waiting should be made only by surgeons with a great deal of experience in the exstrophy condition (Gearhart and Jeffs, 1998). A genetic study of the human T gene and its exclusion as a major candidate gene for sacral agenesis with anorectal atresia. Type 1 constitutes three entirely separate ureters with unique attachment to the bladder or distally and accounts for 35% of triplications. Patients with microscopic hematuria (more than 5 red blood cells per high-power field), both dialysis patients and transplant recipients, should be radiographically evaluated because of the increased risk of tumor formation in these groups (Kim, 2010). The exclusion of these patients from the diagnosis of "obstruction" has tended to force people to create convoluted descriptions of hydronephrotic kidneys.

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The normal urethra is replaced by a broad, mucosal strip lining the dorsum of the penis extending toward the bladder, with potential incompetence of the sphincter mechanism. EpidemiologyandNaturalHistory Enuresis is a common problem, with an estimated 7 million children in the United States alone with this condition. Laparoscopy is the procedure of choice to confirm or exclude the presence of a viable or remnant abdominal testis, unless a prominent scrotal nubbin is palpable with other clinical signs of monorchism. Although uncommon, and depending on the solution used, water intoxication and/or electrolyte abnormalities may occur (Hunter et al, 1993; Schreiber and Stone, 1999). In both types of paraureteral diverticula, the Waldeyer sheet eventually becomes damaged as the diverticulum expands in size. Congenital megacystis is defined currently as a dilated, thinwalled bladder with a wide and poorly developed trigone. Infertility in cryptorchidism is linked to the stage of germ cell development at orchidopexy. In overlearning, additional fluids are given at bedtime while alarm training is continued after dryness has been achieved (Young and Morgan, 1972). In particular, phytotherapy (favored by parents who equate "natural" with safety) can elicit severe adverse reactions because of contamination, inability to quantify active compounds, unknown adulteration, or direct toxic effects. Although many methods of epispadias repair exist, meticulous follow-up of the urethra, appropriate selection of patients, and surgical experience remain the milestones of success. This has been noted clinically as well; most patients do not require any routine bladder irrigations for mucus after gastrocystoplasty. This led to focus on biodegradable, collagen-rich tissues serving as scaffold with and without cell "seeding" for bladder regeneration (Kelami, 1971; Fishman et al, 1987; Atala et al, 1992; Kambic et al, 1992; Kropp et al, 1995a; Zhang et al, 2004; Harrington et al, 2008). ClinicalCorrelates the previous discussion clearly suggests that multiple opportunities exist for modifying the course of reflux if secondary causes are appreciated, identified, and treated. In a large review of an exstrophy database of over 815 patients, Lowentritt and colleagues (2005) reported 25 exstrophy complex variants, of which six were cloacal exstrophy variants. A short-term, multicenter, randomized double-blind dose titration study of the efficacy and anticholinergic side effects of transdermal compared to immediate release oral oxybutynin in the treatment of patients with urge urinary incontinence. Hafez and Helmy (2011) reported on three patients with isolated penopubic epispadias who underwent postpubertal reconstruction using penile disassembly, indicating good cosmesis with a meatus at the tip. Bilateral vanished testes diagnosed with a single blood sample showing very high gonadotropins (folliclestimulating hormone and luteinizing hormone) and very low inhibin B. Cysts of the ejaculatory system-a treatable cause of recurrent epididymo-orchitis in children. Interestingly, this improvement was most pronounced in children with severe wetting (>5 nights per week), family problems, or behavioral problems. The confusion arises in part from the variability of the histologic picture: the appearance of the primitive stroma; the maturity of tubular and even on occasion of muscle elements; and the degree of epithelial atypia that differs not only from patient to patient but also within the same lesion. Subepithelial urethroplasty was superior in a retrospective analysis of Mathieu operations by Ulman and associates (1997). The YoungDees-Leadbetter bladder neck plasty, Marshall-Marchetti-Krantz suspension, and ureteral reimplantation are performed when the bladder capacity reaches approximately 100 mL, which usually occurs between 4 and 5 years of age when the child is ready to be dry. Video-urodynamics should be obtained for transplant candidates to determine the safe storage pressures and contractile function of the future reservoir. Those with proximal hypospadias had more spraying (46 of 106 [43%]) than did patients with distal repairs (245 of 818 [30%]). When a caudal block cannot be performed, penile and scrotal blocks are used, with a wider area of infiltration at the scrotal base and an additional region of injection superolateral to the scrotum on the side where tunica vaginalis will be harvested. Pyelonephritic scarring in 213 patients with upper and lower urinary tract infections: long-term followup. Occasionally a child with neurogenic dysfunction may be a candidate for orthotopic bladder substitution (Stein et al, 2000, 2005), but it is not clear how many of those patients with neurogenic dysfunction can be expected to void adequately. B,Patient after having undergone anterior innominate and vertical iliac osteotomy and placement of intrafragmentary pins and external fixator. Implantation of a testicular prosthesis should occur at least 6 months after any scrotal procedure or after puberty and is best performed through an inguinal approach. Localization of mutation producing autosomal dominant polycystic kidney disease without renal failure. Urothelial cell carcinoma accounts for close to 60% of the tumors, followed by squamous cell carcinoma in 21%, adenocarcinoma in 16%, and signet cell tumor of the gastric augment in 5% (Austin et al, 2007). This allows the bladder to fill with urine from the upper tract at low pressures (compliance) (Mattiasson, 1994).

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However, failure after injection therapy does not preclude a successful outcome when it is salvaged by more invasive measures (Alova et al, 2012b). Immunolocalization of androgen receptor and estrogen receptors alpha and beta in human fetal testis and epididymis. Green and colleagues (1993), however, pointed out that because many of the deformities are unilateral, oligohydramnios is the most likely etiology. A consensus view on the embryologic origins of posterior urethral valves remains elusive, though various mechanisms have been proposed. It may be beneficial to mobilize only one edge over to the other side to avoid overlapping suture lines. Their work agreed with that of Berglund and colleagues (1987) and Studer and Zingg (1997) in suggesting that ileal reservoirs have lower basal pressures and less motor activity. A single-centre long-term outcome analysis of artificial urinary sphincter placement in children. Formerly, collagen was the bulking agent of choice, but often multiple injections were required (Ben-Chaim et al, 1995a). It should be the renal response primarily that is used to make the determination of obstructive effect. Cases of congenital cryptorchidism may go undetected or untreated in infancy owing to prematurity or other morbidity, may undergo longer than necessary observation for spontaneous descent, or may represent cases of undetected reascent after spontaneous descent in infancy. Histologically, there is a characteristic triad present that includes (1) irregular thickening and disintegration of the tubular basement membrane, (2) marked tubular atrophy with cyst development, and (3) interstitial cell infiltration with fibrosis. However, a dramatic increase in pediatric urolithiasis has been observed in more recent years (Srivastava and Alon, 2005), especially among adolescents without known metabolic disturbances. In an updated series, Gargollo and colleagues (2011) found that 80% of patients with long-term follow-up required bladder neck repair. Echo-enhanced voiding urosonography for detection of vesicoureteric reflux in children. The FowlerStephens procedure is now typically performed laparoscopically with spermatic vessel clipping (Bloom, 1991) followed by laparoscopic or open testicular mobilization in the same setting, or in a staged approach 6 months later. Several series have suggested increased risk with maternal use of acetaminophen or more than one analgesic in midpregnancy or earlier (Jensen et al, 2010a; Kristensen et al, 2011; Snijder et al, 2012). The chance for fertility in adolescent boys after corrective surgery for varicocele. Once identified after birth, the distended bladder requires drainage by intermittent catheterization or vesicostomy placement. For a ureterocele in a duplex system, the availability of endoscopic incision creates more options (Table 134-1 and. Neither group had any difference in number of surgical procedures, development of continence with or without the need for bladder neck reconstruction, or eventual need for continent urinary diversion. Polyorchidism is the presence of a supernumerary testis that is more commonly unilateral and on the left side, with rare cases of bilateral duplication or triplication reported in a comprehensive meta-analysis of the pediatric and adult literature (Bergholz and Wenke, 2009). The timing of the rupture of this defective cloacal membrane determines the variant of the exstrophy-epispadias complex that results. Predominant characteristics include an elongated, low-set umbilicus and divergent rectus muscles that attach to the separated pubic bones. They usually appear after the age of 2 years, gradually become more prominent with time, and persist throughout life. Although glomerular filtration and renal plasma flow are the most commonly measured elements of renal function, renal tubular function is equally important, yet is often ignored in obstructive uropathy. Metabolic consequences and long-term complications of enterocystoplasty in children: a review. The type 3 valve is similarly contentious as a diagnosis, but is most commonly described as an annular ring similar to that seen with a congenital urethral stricture. Other treatments include application of an iced glove for 5 minutes, application of granulated sugar for 1 to 2 hours, and placement of multiple punctures in the edematous skin (Mackway-Jones and Teece, 2004). Equally successful outcomes have been reported by Baird and colleagues (2005a) in adolescents who had undergone a mean of eight prior procedures before their definitive repair. The next 20 had two-layer subepithelial urethroplasty using interrupted 7-0 polyglactin and continuous 7-0 polydioxanone.