Jeffrey W. Taub MD

Unfortunately cholesterol medication zetia ezetimibe 10 mg on-line, these two groups were small and not clinically matched prospectively to limit other potential confounding factors such as testicular position cholesterol lowering foods and fruits purchase ezetimibe on line, and sperm counts in the surgery-only group were lower than typical for unilateral cryptorchidism cholesterol test ranges effective 10 mg ezetimibe. Overall cholesterol causes best buy for ezetimibe, these studies provide preliminary definition de colesterol total generic ezetimibe 10 mg fast delivery, suggestive evidence that buserelin may have both short- and long-term effects on testicular histology and/or fertility potential. However, the suboptimal design of the studies on which this evidence is based mandates that future well-designed prospective studies be conducted before buserelin treatment can be used routinely in cryptorchidism. In summary, little if any high-quality evidence exists showing benefit of hormonal therapy for cryptorchidism or for stimulation of germ cells. In follow-up studies of 391 boys with 464 acquired cryptorchid testes, 90% of high scrotal and 64. In contrast, a study from the same institution showed that testicular volumes were significantly smaller for age in 155 postpubertal patients who had undergone orchidopexy at diagnosis (the majority before puberty) after a mean of 6. Because these individuals were selected for orchidopexy and the majority (92%) had suprascrotal testes, they are likely not comparable to the group of boys treated after puberty at the same institution (Hack et al, 2010). Furthermore, these studies were not randomized and provided no long-term data regarding functional outcome. Consequently, they fail to provide strong evidence in support of observation as the recommended approach for cases of acquired cryptorchidism. MedicalTherapy Hormonal therapy has been used for a variety of indications in patients with cryptorchidism, including differentiation of retractile from true undescended testes, stimulation of testicular descent or germ cell maturation, and as an adjunct to abdominal orchidopexy. Hormone therapy is not currently recommended, given the lack of rigorous data supporting its efficacy (Thorsson et al, 2007; Kolon et al, 2014). Several published reports address the usefulness of hormonal therapy in distinguishing retractile from true undescended testes. Although the efficacy of either hormonal treatment is about 20% and superior to placebo in randomized trials, this effect is not clearly clinically significant. In view of a lack of large prospective studies, it is unclear if hormone therapy for cryptorchidism is beneficial or harmful to germ cells in the short or long term. Despite this, the average age at which orchidopexy is performed remains approximately 4 years in many series (Barthold and Gonzalez, 2003; Kokorowski et al, 2010; Bayne et al, 2011; Snodgrass et al, 2011; Barthold et al, 2012; Bradshaw et al, 2014; Nah et al, 2014). The reason for delayed intervention likely reflects a combination of factors, primarily including delayed referral of congenital cases and the occurrence of acquired cryptorchidism. 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. Delay may also be exacerbated by difficulty in distinguishing undescended from retractile testes. The traditional approach to surgical treatment of palpable testes is inguinal orchidopexy with repair of an associated hernia if present (Hutcheson et al, 2000a), although a primary scrotal approach as originally described and advocated by Bianchi and colleagues (Bianchi and Squire, 1989; Iyer et al, 1995) is an alternative approach. E, the tunica vaginalis is opened over the testis, and F, the incision is extended proximally alongthelengthofthecord. After identification of the external ring, the external oblique fascia is incised to expose the canal, with care taken to avoid injury to the ilioinguinal nerve. The spermatic cord is isolated and the testis is dissected distally to its attachment to the gubernacular remnant. Transection of the gubernaculum distal to the sac will avoid potential injury to a long-looping vas. Longitudinal incision of the internal spermatic fascia allows free mobilization of an intact hernia sac, if present, and minimizes skeletonization of the vas and spermatic vessels. Alternatively, the sac may be opened overlying the testis and the incision extended proximally along the length of the cord. Once isolated, the sac is mobilized to the level of the internal inguinal ring and suture-ligated. Incision of the internal spermatic and transversalis fascia at the level of the ring facilitates additional retroperitoneal mobilization of the vas and vessels, if needed. 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. Very rarely, the testis cannot be brought to dependent scrotal position after these maneuvers and a two-stage procedure may be considered as an alternative to orchiectomy, which is preferentially reserved for visibly abnormal or atrophic testes, postpubertal patients, or cases associated with insufficient vasal length. After mobilization of the spermatic cord, a transverse scrotal incision is made and a subdartos pouch created. A large clamp or a finger can be used to create a tunnel just anterior to the pubis. The testis is passed through an opening in the dartos without twisting of the spermatic cord. Existing appendages should be excised and the epididymis inspected and any anomalies recorded. Recording of testicular volume by direct caliper measurement in three dimensions and similar (estimated) measurements of contralateral testicular volume can establish a baseline for postoperative assessment. Secure fixation of the testis within the pouch can be achieved by tension-free closure of the opening in the dartos around the cord, incorporating the cut edge of the tunica vaginalis. If needed, additional absorbable fixation sutures can be placed between the visceral tunica vaginalis and the dartos. Alternatively, suture fixation through the tunica albuginea to the scrotal wall may be performed. There is a theoretic risk of injury to the testis via inflammatory or vascular insult with suture placement through the tunica albuginea. Supplemental local or regional techniques for perioperative pain control are advisable and may include local anesthetic infiltration, ilioinguinal nerve block, or caudal anesthesia; the latter is particularly useful in younger patients undergoing bilateral inguinal or concomitant penile surgery. Testicular biopsy has been performed routinely in some centers and has been advocated by Hadziselimovic and colleagues as a method to determine prognosis for fertility (Hadziselimovic and Zivkovic, 2007). This approach is controversial and not recommended outside of research protocols because it does not change the current approach to treatment (Ritzen et al, 2007; Beckers and van der Horst, 2008). Biopsy is indicated in cases of sexual ambiguity or if clinical evidence of testicular dysgenesis is present. Complications of inguinal orchidopexy are uncommon; those of greatest significance include testicular retraction and atrophy. Docimo reported a comprehensive review of the orchidopexy literature in 1995, before the routine use of laparoscopic orchidopexy, and including both palpable and nonpalpable testes (Docimo, 1995). He concluded that the overall risk of atrophy or nonscrotal position was approximately 15% overall in published reports, significantly higher in abdominal or peeping testes (24%) compared with those distal to the internal ring (10%) and higher in boys undergoing surgery after 6 years of age. More recently, analysis of boys with postoperative testis retraction over an 18-year period at a single institution consisted of less than 2% of 1886 primary open orchidopexies performed during that period, with a slightly higher risk of failure in older boys (McIntosh et al, 2013). However, these boys were not actively followed by the authors, so detailed clinical data were not available and the frequency of testicular atrophy could not be defined. In another series of 418 orchidopexies in 356 boys from a single institution with a median of 1-year follow-up, the risk of atrophy was 1. However, in this series the risk of complications was higher when surgery was performed at a younger age, and all boys with acquired cryptorchidism had a successful result. 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. Long-term follow-up should be considered for counseling of the patient regarding fertility issues, risk of testicular malignancy, and self-examination. Torsion of a scrotal testis after orchidopexy has been reported but is very rare, and the risk may be minimized by routine extravaginal testicular fixation in a subdartos pouch. If complete intrascrotal testicular atrophy occurs postoperatively, further intervention is not needed, but the option of testicular prosthesis placement should be offered to the patient and family (Bodiwala et al, 2007). 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. Fixation of the prosthesis to the dartos and closure of the scrotal fascia above the implant using purse-string nonabsorbable suture are required. Complications including displacement, pain, or infection occur in less than 5% of cases. Clinical experience suggests that cryptorchid boys may request prosthesis implantation less frequently than males with acute testicular loss after puberty (Bodiwala et al, 2007). If the testis is prescrotal, a primary scrotal approach can be considered and may allow adequate mobilization of the testis. If inguinal exploration is needed to provide sufficient cord length, several approaches are available. Redman described a primary or secondary orchidopexy that involves a lateral approach to the cord after mobilization of the external oblique and cremaster fasciae (Redman, 2000). This approach avoids traversal of the previously scarred layers anterior to the cord and affords a clearer view of the anatomy. Cartwright and colleagues described mobilization of the intracanicular cord with an overlying patch of external spermatic fascia (Cartwright et al, 1993). The importance of correcting a persistently patent processus vaginalis and/or of adequate retroperitoneal mobilization of the cord in cases of high recurrent cryptorchidism has been stressed (Redman, 2000; Pesce et al, 2001; Ziylan et al, 2004). The results of secondary orchidopexy appear to be similar to the primary procedure, although the risk of vascular and vasal injury is theoretically higher (Pesce et al, 2001). Various scrotal incisions thathavebeenreported;A,Bianchiincision(Bianchi);B,transverse low scrotal approach (Misra); C, midline scrotal approach. Modifiedscrotal[Bianchi]mid raphe single incision orchiopexy for low palpable undescended testis:earlyoutcomes. After induction of anesthesia, the patient is re-examined to confirm the position of the testis. An incision along the superior scrotal border is made as described by Bianchi and Squire for any palpable testicles. Alternatively, a transverse low scrotal approach (Misra et al, 1997) and midline scrotal approach (Cloutier et al, 2011) have been described for those testes that can be drawn into the scrotum. 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). Some cases require conversion to an inguinal approach for ligation of the sac or to gain further length on the spermatic cord (Parsons et al, 2003; Dayanc et al, 2007). Rajimwale and colleagues confirmed in several cases that the hernia sac had been effectively ligated above the internal ring via the scrotal incision when a secondary inguinal incision was required for further mobilization of the testis (Rajimwale et al, 2004). Fixation sutures through the tunica albuginea have been used in many series of scrotal orchidopexy (Jawad, 1997; Russinko et al, 2003; Bassel et al, 2007; Dayanc et al, 2007; Takahashi et al, 2009), followed by placement of the testis in a subdartos pouch. In an extensive review of the literature by Gordon and colleagues, additional inguinal incisions were needed in 4. The single institution longterm results reported by these authors included a reoperative rate of 4. In a literature review of 1558 cases in 20 series reporting 3 months to 5 years of follow-up, a hernia was present in 30% and 3. Scrotal incision orchidopexy is used selectively in many series, but the available evidence suggests that efficacy and complication rates are similar to those of standard inguinal orchidopexy. Orchiectomy is appropriate for patients with testes that are poorly viable and/or at higher risk for tumor, which may include testes in postpubertal patients or very small or dysgenetic testes in postpubertal patients, and is in our opinion best performed laparoscopically. Open Transabdominal Orchidopexy Extensive dissection of the vas and vessels is facilitated by a longitudinal opening of the internal oblique and peritoneum through an extended inguinal incision (Kirsch et al, 1998) or via a higher incision medial to the pubic tubercle and a preperitoneal approach (Jones and Bagley, 1979; Gheiler et al, 1997). In the procedure described by Jones and Bagley, the internal ring is approached via a muscle-splitting incision, the peritoneum is opened, the testis delivered, and the vas and vessels freed from their peritoneal attachments. A tunnel is created to the scrotum and the testis is secured in place as for an inguinal orchidopexy. The reported success rate for this procedure for abdominal testes was 95% (Gheiler et al, 1997). Laparoscopic Orchidopexy and Fowler-Stephens Orchidopexy Operative laparoscopy emerged over 15 years ago as the procedure of choice for abdominal orchidopexy (Caldamone and Amaral, 1994; Jordan and Winslow, 1994), and the basic surgical approach and high success rates have stood the test of time (Table 148-1). The feasibility of primary versus Fowler-Stephens orchidopexy depends on the length of the vas and vessels, presence or absence of looping ductal structures, and age of the patient. Although laparoscopy allows the surgeon to assess some of these features before choosing a specific surgical procedure, the choice may be difficult (Yucel et al, 2007). Observed testicular position alone may correlate poorly with the ultimate length of the cord after mobilization. After induction of anesthesia, a further attempt to palpate the testis is made, although a laparoscopic approach may be considered for mobilization of high canalicular testes as well. After decompression of the bladder and stomach, an infraumbilical 5-mm trocar is placed for passage of a 30-degree lens, and both internal rings are visualized. An open Hasson or Bailez technique is preferable for umbilical trocar placement in the pediatric age group to minimize risk of injury (Franc-Guimond et al, 2003). The size and position of the testis within the abdomen are determined before further decision making. For single-stage laparoscopic orchidopexy, additional 2- or 3-mm trocars are placed in the right and left lower quadrants to triangulate with the umbilicus and ipsilateral internal ring, or in the midclavicular line at the level of the umbilicus bilaterally for bilateral abdominal testes. 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. Samadi and colleagues advocate initial mobilization of the gubernaculum to be used as a handle for further mobilization of the testis, and minimal use of cautery during this maneuver (Samadi et al, 2003). Ability to mobilize the testis to the opposite internal ring has been used as a measure of adequate length for placement in the scrotum but was not predictable in some series. Once mobilized, the testis is brought through a new hiatus medial to the epigastrics and lateral to the medial umbilical ligament or through the existing internal inguinal ring. This maneuver can be completed using a trans-scrotal clamp or an additional port passed up from the scrotum. With tension on the extra-abdominal testis, peritoneal attachments overlying the cord can be more easily transected, thus providing addi- tional length. In some cases the testis can only be brought into the upper scrotum; the long-term adequacy of this approach is not clear. Excessive tension on the vessels during placement of the testis should be avoided, because injury or avulsion of the spermatic vessels may occur (Esposito et al, 2002). A key strategy should be preservation of the blood supply between the vas and spermatic artery during dissection so that the Fowler-Stephens procedure can be performed if necessary. Formal closure of the dissected internal ring is not necessary (Handa et al, 2005; Riquelme et al, 2007); indeed, previous experience with open hernia repair suggests that ligation is not needed if the internal ring is dissected (Mohta et al, 2003).

The prenatal diagnosis of lower urinary tract obstruction using B scan ultrasound: a case report cholesterol chart for cheese ezetimibe 10 mg order on-line. Testicular histology in fetuses with the prune belly syndrome and posterior urethral valves cholesterol and high blood pressure ezetimibe 10 mg buy line. Congenital absence of abdominal muscles cholesterol medication being recalled buy cheap ezetimibe 10 mg line, with distended and hypertrophied urinary bladder cholesterol test price philippines ezetimibe 10 mg amex. Urethral obstruction malformation complex: a cause of abdominal muscle deficiency and the "prune belly cholesterol lowering diet in spanish cheap ezetimibe 10 mg visa. A possible relationship between Beckwith-Wiedemann syndrome and prune belly syndrome. Congenital absence of the abdominal muscle associated with malformation of the genitourinary and alimentary tracts: report of cases and review of the literature. Giant omphalocele and "prune belly" sequence as components of the Beckwith-Wiedemann syndrome. Voiding function in patients with prune belly syndrome after Monfort abdominoplasty. Ultrasonic detection of fetal ascites and bladder dilation with resulting prune belly. Über die symmetrischen krongenitalen Bauchmuskeldefekte und über die Kombination derselben mit anderen Bildungsanomalien des Rumpfes. Patterns of congenital lower urinary tract obstructive uropathy: relation to abnormal prostate and Chapter140 Prune-BellySyndrome 3251. Prune-belly syndrome: ongoing controversies regarding pathogenesis and management. Two stage orchiopexy with laparoscopic clip ligation of the spermatic vessels in prune belly syndrome. As the most common cause of bladder outlet obstruction in infants, the presentation of posterior urethral valves is often antenatal, and pathognomonic findings on postnatal imaging confirm the diagnosis. Although an endoscopic ablation or urinary diversion will address the immediate uropathy, the embryologic insults to the bladder and kidneys manifest to varying extents, requiring that these boys remain under the extended care of urologists and nephrologists. It was Hugh Hampton Young, however, who described the first endoscopic diagnosis of a urethral obstruction that was termed posterior urethral valves (Young et al, 1919). Despite the rudimentary nature of the early endoscopic instruments, Randall carried out the first endoscopic resection of valves in 1920, providing a description that remarkably encapsulates the visual findings of the posterior urethra even today: the prostatic urethra is markedly dilated. Deep pittings penetrate down into either lateral wall of the prostatic urethra and at the extremity the verumontanum is seen a fine frenulum which extends distally for about 1 cm, and in dividing, forms what is apparently a definite valve on either side of the urethra, rising from the floor to each side wall. The type 1 lesion, pertinent to 95% of cases, is theorized as a hypertrophied variant of the inferior urethral crest formed by the insertion of the distal ends of the wolffian ducts into the anterolateral walls of the cloaca (Stephens, 1983). The urethral valves are actually leaflets that arise from the verumontanum, take an anterior course, and then fuse in the midline just proximal to the external striated urethral sphincter. Some argue that the cleft seen in the midline-the "two forklike processes" as Young and coworkers described, or the leaflets fanning out from the verumontanum-are actually iatrogenic and created by retrograde instrumentation in the perinatal period (Dewan et al, 1994). The type 2 valve of Young and colleagues, described as arising from the verumontanum and extending posteriorly and superiorly to the bladder neck, is not obstructing and has not been reported definitively since early reports. Stephens (1983) reported seeing no type 2 valves in 210 boys with posterior urethral valves examined with cystoscopy and suggested that the description was that of the secondary effects at the bladder neck of more distal obstruction. 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. Young and colleagues (1919) described a complete obstruction "attached to the entire circumference of the urethra, there being a small opening in the center. A consensus view on the embryologic origins of posterior urethral valves remains elusive, though various mechanisms have been proposed. The earliest theory held that hypertrophy of the urethral mucosal folds was the cause of obstruction, which was furthered later to suggest cloacal remnants caused the appearance of valves after division by the urogenital membrane (Krishnan et al, 2006). Lowsley placed the origin of posterior urethral valves in an abnormal development of the wolffian and müllerian ducts based on an autopsy study in 1914 (Lowsley, 1914), but a more recent autopsy study corroborates the presence of a congenital obstructing posterior urethral membrane, consistent with a persistent oblique urogenital membrane that is punctured at the time of catheter or feeding tube placement by the medical practitioner in the immediate postpartum period (Dewan et al, 1994; Krishnan et al, 2006). Since birth incidence requires the often inaccurate count of early spontaneous pregnancy loss, a later study following a similar cohort based in the West Midlands between 1995 and 2007 calculated a prevalence ratio of posterior urethral valves-number of affected births divided by the total number of live and stillbirths-of 2. This prevalence was also significantly higher in black and minority ethnic groups when compared with white Europeans. Considering that the United States alone may expect 300 to 500 new cases of infants with posterior urethral valves born annually- of which one third will go on to end-stage renal failure (Heikkilä et al, 2011)-the economic repercussions in terms of dialysis and renal transplantation arising from this congenital anomaly are enormous. Over time, however, polyuria caused by renal dysplasia and continuing glomerular and tubular damage leaves the bladder without adequate periods of bladder emptying, leading to decompensation. This phase leads to increased bladder residuals that are causally linked to exacerbation of hydronephrosis and further renal damage. LowerUrinaryTract It cannot be overstated that the potential comorbidities arising from posterior urethral valves-renal dysfunction, urine reflux, worsening hydronephrosis-are due to bladder dysfunction. Mitchell (1982) coined the term valve bladder syndrome when he described 11 patients in whom bladder filling and emptying were noted to be intricately related to extent of renal pelvocaliectasis and overall renal function and dysfunction. This concept was subsequently illustrated as a "vicious cycle" leading to the valve bladder syndrome. Bladder hypertrophy secondary to the fetal obstruction results in higher voiding pressures that maintain complete bladder emptying in the compensated phase. The increased voiding pressures lead to gradual remodeling of the bladder wall, further increasing voiding pressures, and ultimately to higher postvoid residuals as emptying begins to fail. The long-term sequelae of valvular disease can be correlated to bladder dysfunction, renal dysplasia, polyuria, and multiple other anatomic and physiologic factors. As this bolus of urine fills the bladder even as the bladder empties partially, a lack of extended periods of an empty, relaxed bladder pushes the compensated bladder toward decompensation. This, of course, leads to even greater postvoid residuals, overflow incontinence, and further renal damage (Close et al, 1997). The storage and voiding of urine at high pressures has been simulated in fetal sheep and rabbit models in vitro to simulate and then investigate the cascade of events effectuating bladder smooth muscle alterations. Increased bladder dilation is seen following partial bladder outlet obstruction (Kirsch et al, 2003), and the dilated, poorly contracting bladders (simulating the decompensated bladder) lead to more upper tract dilation. This model and the work of other researchers confirmed that extracellular matrix elements line the detrusor smooth muscle cells of the bladder after obstruction-as noted in valve bladders (Workman and Kogan, 1990). Importantly, the pathologically significant elevated storage pressures must be distinguished from the neonatal and infantile elevated voiding pressures that are a normal feature of bladder development (Sillén et al, 1992). There remains some controversy as to whether the changes in bladder morphology in the valve-affected bladder are reversible, as opposed to the phenotypic changes in neurogenic bladders that are more permanent (Keating, 1994; Hutcheson et al, 2004). The deposition in the extracellular matrix causes altered contraction and passive relaxation of the bladder, which may cause multiple intracellular changes ranging from a reduction in detrusor blood flow causing ischemia to free radical toxicity. These changes alter the phenotype of detrusor muscle myosin bundles and actin-associated filaments (Ghafar et al, 2002; Shukla et al, 2004; Levin et al, 2005). The valvular obstruction will also lead to marked dilation of the posterior urethra, hypertrophy of the bladder neck, and a flattening of the verumontanum with dilation of the ejaculatory ducts-the pathognomonic findings of this diagnosis on voiding cystourethrogram. These changes seem to return to a more normal appearance following valve ablation as the offending distal obstruction is removed. This bladder dysfunction manifests in varied ways during infancy and later, and is discussed in a later section. What is apparent is that a sustained increase in intravesical storage pressures over prolonged time intervals transmits that pressure to the ureter, the renal pelvis, and ultimately the glomerular units-causing architectural and functional changes at each ascending structure (Koff et al, 2002). These architectural changes can be inferred clinically by the severe hydroureteronephrosis that is often seen in the setting of posterior urethral valves. Increased echogenicity, parenchymal thinning with cortical cysts, and lack of corticomedullary differentiation similarly imply significant renal dysplasia. Ureteral dilation occurs as a direct transmission of pressure from the dysfunctional bladder, as well as the vesicoureteral reflux that is seen in up to 70% of patients with posterior urethral valves (Puri and Kumar, 1996; Sarhan et al, 2011). Polyuria caused by progressive renal damage and congenital renal dysplasia compound ureteral dilation as well (Smyth et al, 1991) the chronicity of ureteral dilation is classically believed to cause ureteral wall thickening, loss of peristalsis, and loss of mucosal coaptation, increasing the risk of urine stasis, infection, and increased pressures in the renal units. The elevated renal pelvic pressures secondary to the bladder and ureteral pressure increases lead to significant alterations in renal morphology and function. The renal dysfunction seen in posterior urethral valves has two specific etiologies: (1) obstructive uropathy and (2) renal dysplasia. Obstructive uropathy causing renal damage is a well-known phenomenon in various models. Fetal sheep ureteral obstruction models clearly demonstrate that, although hydronephrosis occurs rapidly after obstruction, irreversible dysplastic changes in the renal architecture are seen by term, and these changes were confirmed after outlet obstruction in the same model (Peters et al, 1992; Chevalier, 2004). Apoptosis and increased oxidative stress in mouse kidneys with ureteral obstruction are also seen in the face of bladder outlet obstruction (Kawada et al, 1999; Chevalier, 2004). The increased pressure from obstruction damages luminal cells in the renal tubules and will also lead to poorly concentrated urine production (Li et al, 2004; Nguyen et al, 2005a). Obstruction may also affect urinary concentration by reducing blood flow to the medulla, causing a loss in the medullary concentration gradient that results in significant polyuria and even in the postobstructive diuresis seen after catheter placement in an infant with posterior urethral valves (Dinneen et al, 1995). Others have demonstrated known primary dysplastic malformations of fetal cartilage tissue or dysplastic glomeruli and tubules in posterior urethral valves that affected renal tissue at the time of nephrectomy (Haecker et al, 2002). A correlation between severe renal hypodysplasia and decreased activity of the renin-angiotensin system that modulates renal development has been noted in posterior urethral valves, as well as a decrease in the angiotensin receptor type 1 genetic polymorphisms (Peruzzi et al, 2005). Bajpai and associates (2005) found that plasma renin activity increases precede common clinical findings of renal damage such as rising serum creatinine, renal scars, and lowering glomerular filtration rate. These cytokines are considered potential biomarkers that seem to decrease with improvement in renal function after valve ablation, whereas elevations imply worsening outcomes. This relationship, in that original series, was found in 13% of patients with posterior urethral valves, and the theory that these children would have better long-term renal function as a result of the pop-off phenomenon was widely accepted. The glomerular filtration rate, importantly, was already abnormal in 75% of these same patients even in the first 2 years of life. Massive, dilating vesicoureteral reflux is seen on the left side on this voiding cystourethrogram and, in this case, is typically associated with a poorly functioning kidney on the ipsilateral side, referred to as the vesicoureteral reflux with dysplasia syndrome. Posterior urethral valves are detected in approximately 1 in 1250 ultrasound screenings, accounting for 10% of significant antenatally detected genitourinary disease and afflicting one third of surviving infants with bilateral renal disease (Thomas and Gordon, 1989; Gunn et al, 1995). Renal echogenicity will be increased in posterior urethral valves, and is a reliable indicator to infer renal damage as well. This study should be completed in the early postnatal period after renal and bladder sonography, and as soon as an infant with suspected prenatal findings of valves is hemodynamically stabilized and able to undergo the contrast study. Fetal magnetic resonance imaging demonstrating lower urinary tract obstruction that was postnatally diagnosed as posterior urethral valves. A dilated bladder funneling to a visible posteriorurethra(arrow)isseenonthisT2image. The bladder often appears thickened and trabeculated with multiple diverticuli, mimicking the appearance of a neuropathic bladder. High-grade vesicoureteral reflux may be seen in approximately 50% of patients with valves at the time of diagnosis (Hassan et al, 2003). Images obtained during the voiding phase will show contrast traveling across a hypertrophied, elevated bladder neck and grossly dilated posterior urethra. The urethra funnels abruptly at a transverse membrane, or cusp, representing the obstructing valve leaflets seen at cystoscopy. The study commences with the insertion of a 6- or 8-Fr feeding tube into the urethra. This tube may curl within the capacious posterior urethra or hypertrophied bladder neck, requiring the use of a coudé catheter to advance into the bladder. Often a catheter may already be in place at the time of the study, and it is important that the catheter be withdrawn gradually distal to the posterior urethra during the voiding phase of the study to offer unobstructed views of that segment. A, Voiding cystourethrogram image demonstrates a dysmorphic, elongated bladder with dilated posterior urethra and classic appearance of posterior urethral valves. RadionuclideRenalScan the radionuclide renal scan offers quantification of differential renal function, and cortical deficits seen on the study may imply renal dysplasia when completed in the neonatal period. Mercaptoacetyltriglycine is a useful agent to evaluate renal functional contribution, though delayed emptying of nuclear tracer from the often dilated collecting systems should not be necessarily interpreted as ureterovesical junction obstruction requiring intervention. Placement of a urinary catheter is essential in a patient with vesicoureteral reflux to minimize error in the calculation of renal function. However, the serum creatinine plateau even in unaffected children may not be seen until days 65 to 220 of life (DeFoor et al, 2008, Boer et al, 2010). The infant may be noted to have difficulty with voiding, and the urinary stream may be weak or intermittent. A 5- or 7-Fr feeding tube, or similar caliber urinary catheter, should be inserted per urethra in an infant presenting to the LaboratoryEvaluation Laboratory evaluation of a newborn with a diagnosis of posterior urethral valves will, as with any newborn, reflect maternal values and must be interpreted with caution. Catheter placement into the bladder may be impeded by the hypertrophied and often elevated bladder neck and curling of the catheter within the dilated posterior urethra. In such cases, a coudé-tipped catheter or, alternatively, using a stylet to curl the tip of the feeding tube dorsally will facilitate bladder drainage. 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). Placement of the catheter within the bladder may be confirmed by bladder sonography or, in some instances, a one-shot cystogram. Urinomas A urinoma is associated with posterior urethral valves in 3% to nearly 10% of cases. The introduction of renal ultrasonography in 1979 increased the detection of urinomas to 15% in one study (Heikkilä et al, 2011). Forniceal rupture will appear on renal ultrasonography as distorted renal parenchyma resulting from fluid trapped within the renal capsule, whereas transperitoneal transudation of fluid or bladder rupture will present as neonatal ascites (Greenfield et al, 1982). It is only in cases in which the ascites is causing respiratory distress, severe abdominal distention, or other clinical symptoms that percutaneous drainage or tapping of ascites becomes necessary; these interventions are uncommon. There is some debate as to whether a urinoma heralds better or worse renal function for the affected side. Numerous studies have postulated that the urinoma serves as a pop-off mechanism, thereby reducing renal dysplasia on a given side, and some studies demonstrate globally preserved renal function, including an index of long-term renal severity (Rittenberg et al, 1988; Wells et al, 2010). Other studies hold that the urinoma, especially one retained within the renal capsule and compressing the kidney, impairs ipsilateral renal function and is a harbinger for worsened renal prognosis or has no bearing at all on long-term renal function (Patil et al, 2003; Kleppe et al, 2006; Heikkilä et al, 2011). The frequently cyanotic baby requires complex ventilatory support, and this is often the factor delaying definitive surgical intervention to address the valves. Pulmonary hypoplasia seen in the infant with a history of antenatally detected oligohydramnios may be the contributing factor to perinatal mortality in these children, requiring intensive and rapid supportive treatment (Pinar, 2004).

Other genomic rearrangements and trisomies cholesterol test normal 10 mg ezetimibe mastercard, including Down syndrome (trisomy 21) cholesterol check up bangalore discount ezetimibe 10 mg visa, are associated with cryptorchidism (Hadziselimovic cholesterol levels how to lower purchase ezetimibe with visa, 1983) cholesterol test cvs buy 10 mg ezetimibe otc. These include all cases of classic prune-belly (triad or Eagle-Barrett) syndrome cholesterol efflux definition generic 10 mg ezetimibe with visa, 80% of spigelian hernia (Durham and Ricketts, 2006; Bilici et al, 2012; Balsara et al, 2014), 41% to 54% of cerebral palsy (Rundle et al, 1982; Cortada and Kousseff, 1984), 38% of arthrogryposis (Fallat et al, 1991), 15% of myelomeningocele (Ferrara et al, 1998), 16% to 33% of omphalocele, 5% to 15% of gastroschisis (Kaplan et al, 1986; Koivusalo et al, 1998; Yardley et al, 2012), 19% of imperforate anus (Cortes et al, 1995b), 12% to 16% of posterior urethral valve (Krueger et al, 1980; Heikkila et al, 2008), and 6% of umbilical hernia (Kaplan et al, 1986) patients. Multisystem anomalies are often associated with omphalocele (80%) and prunebelly syndrome (45%), suggesting a syndromic cause (Loder et al, 1992; Koivusalo et al, 1998). Cortes and colleagues reported associations between renal and T10 to S5 spinal anomalies and cryptorchidism, with the affected testis on the same side as the renal anomaly in 90% of cases (Cortes et al, 1998). The occurrence of cryptorchidism with other nongenital anomalies may complicate and/or alter the timing of treatment. For example, spigelian hernia may be difficult to diagnose, and associated absence of the gubernaculum and inguinal canal may complicate orchidopexy (Bilici et al, 2012; Balsara et al, 2014). Spontaneous testicular descent is reported in 50% to 55% of gastroschisis patients (Hill and Durham, 2011; Yardley et al, 2012) suggesting that observation is warranted. Abduction of the thighs contributes to inhibition of the cremasteric reflex, which is elevation of the testis that is elicited by scratching the inner thigh. The examination should include documentation of testicular palpability, position, mobility, size, and possible associated findings such as hernia, hydrocele, penile size, and urethral meatus position. Sustained gentle traction on the cord can help to inhibit the cremaster reflex and allow a retractile testis to remain at least temporarily in stable scrotal position. Scrotal asymmetry can be a useful clinical sign because it is commonly present in boys with unilateral cryptorchidism. In large clinical series, the majority (75% to 80%) of undescended testes are palpable and 60% to 70% are unilateral; involvement of the right side is more common overall but less frequent in series of nonpalpable testes (Hadziselimovic, 1983; Cendron et al, 1993; Cortes et al, 2001; Giannopoulos et al, 2001). Position of undescended testes varies markedly with the population studied, which may in part be the result of different classification techniques. In a meta-analysis of surgical patients, testes were abdominal in 34%, near the internal ring ("peeping") in 12%, canalicular in 27%, and beyond the external ring in 27% (Docimo, 1995). Moul and Belman classified all subinguinal testes with a lateral gubernacular attachment as ectopic (66% of their total cases). Associated genital findings may warrant additional diagnostic studies that are best completed in the neonatal period. If neither testis is palpable, particularly if penile development is abnormal, karyotype and hormonal analyses are performed urgently to rule out congenital adrenal hyperplasia and obviate the potential adverse effects of undiagnosed salt wasting. Routine circumcision should be delayed until evaluation confirms a genetically normal male. Hypospadias is associated with cryptorchidism in 12% to 24% of cases (Cendron et al, 1993; Moreno-Garcia and Miranda, 2002; Cox et al, 2008). If proximal hypospadias is present, chromosomal analysis is warranted because the frequency of abnormalities is high (32% to 47%) (Cox et al, 2008; Sekaran et al, 2013). Micropenis was reported in 46% of boys with anorchia caused by bilateral vanishing testes (Zenaty et al, 2006) (also called testicular regression syndrome), and small penile size in association with cryptorchidism is also observed in cases of hypogonadotropic hypogonadism. Every effort should be made by the examiner to determine the lowest position the testis may attain. Manual downward pressure with one hand along the ipsilateral inguinal canal from the anterior iliac spine to the scrotum and palpation with the opposite hand helps to identify the lowest position of a palpable testis. Difficulty in the clinical classification of cryptorchidism when the testis is palpable is related to both documentation of testicular Peripenile Superficial inguinal Transverse scrotal Contralateral scrotum Perirenal position and differentiation of truly undescended from retractile testes, complicated by the fact that these entities may coexist. The gold standard for diagnosis remains careful examination of a child in several positions and confirmation of incomplete descent of the testis to a dependent scrotal position after induction of anesthesia. Prospective studies of intraobserver and interobserver variation show major differences in documentation of testicular position among examiners (Wit et al, 1987; Olsen, 1989). Olsen noted complete agreement between two examiners on scaled measures of testicular position and mobility in only 5 (13. Cendron and colleagues reported that preoperative testicular position correlates poorly with intraoperative findings (Cendron et al, 1993). Variation in observed testicular position preoperatively and postoperatively may influence assessment of prognosis and outcome in boys with cryptorchidism. NonpalpableTestes When a testis is nonpalpable, possible clinical findings at surgery include (1) abdominal or transinguinal "peeping" location (25% to 50%). If both testes are nonpalpable and not distal to the internal inguinal ring in a genetic male, at least 95% are abdominal, with cases of bilateral vanishing testis occurring rarely (Cendron et al, 1993; Moore et al, 1994). If neither vas nor spermatic artery is found at the time of laparoscopy, laparoscopic or surgical dissection of the perivesical area and retroperitoneum up to the level of the kidney is required for exclusion of the presence of a testis, because true agenesis is extremely rare. In most cases, laparoscopic or surgical abdominal exploration is performed, although hormone testing can also be useful and may be sufficient for the diagnosis of anorchia. Overall, the sensitivity and specificity of ultrasound in localizing the nonpalpable testis are 45% and 78%, respectively (Tasian and Copp, 2011). Some authors advocate very selective use of imaging if recommended by the managing surgical specialist after referral; in this situation the sensitivity of ultrasonography in identifying inguinal testes is reported to be as high as 95% to 97%, and abdominal testes are also seen in some cases (Cain et al, 1996; Nijs et al, 2007), but examination under anesthesia is likely to provide the same information (Tasian et al, 2011). Imaging is not indicated for diagnosis of the nonpalpable testis, because it has limited accuracy and does not obviate the need for definitive surgical intervention. Diagnostic laparoscopy, followed by laparoscopic orchidopexy if an abdominal testis is present, has become the preferred approach to the nonpalpable testis for many clinicians. Laparoscopy is preceded by an examination under anesthesia, which may be a useful adjunct that helps to define the appropriate course of action. Important laparoscopic observations include the size and position of the spermatic vessels and vas; testicular size, quality, and position if visible; and patency of the internal inguinal ring. The combination of a closed internal ring and a blind-ending spermatic artery and vas confirms an abdominal vanishing testis. An atretic spermatic cord coursing through a closed inguinal ring is suggestive of a distal vanishing testis, but this finding may be subjective and, conversely, normalappearing vessels may be associated with both viable and vanishing testes (Zaccara et al, 2004). Moreover, the laparoscopic view may suggest abdominal blind-ending vessels despite a testis being present distally or in an ectopic abdominal position (Zaccara et al, 2004; Kim et al, 2005; Ellsworth and Cheuck, 2009). Therefore if laparoscopy does not unequivocally localize the testis or blindending spermatic artery, additional surgical exploration is needed for definitive diagnosis. This may be performed laparoscopically after the placement of additional working ports. The need for excision and contralateral scrotal orchidopexy in vanishing testis cases remains controversial. The cause of vanishing testis is not known but is thought to represent spermatic cord torsion or vascular accident occurring after completion of genital masculinization but before fixation of the testis in the scrotum. Evidence supporting this includes the presence of hemosiderin in remnant testicular nubbins excised at surgery (Turek et al, 1994) and reported cases of contralateral postnatal torsion (Gong et al, 1996). An enlarged contralateral testis (Huff et al, 1992) and absence of palpable intrascrotal appendage tissue (processus vaginalis, wolffian structures,or gubernaculum) are highly predictive of a vanishing testis (Mesrobian et al, 2002). Diagnosis of a vanishing testis requires documentation of blindending spermatic vessels in the abdomen, inguinal canal, or scrotum. Excision is appropriate when the spermatic vessels traverse the internal inguinal ring, to provide confirmation that no viable (or atrophic) testis is present, because the laparoscopic appearance of the spermatic vessels and processus vaginalis may be deceiving and may not reliably exclude the presence of an inguinal testis (Ellsworth and Cheuck, 2009). Vanishing testes are often in or near the scrotum; therefore, initial scrotal exploration should be considered when a palpable scrotal nubbin and contralateral testicular hypertrophy (testicular length 1. However, a trans-scrotal search will be timeconsuming and unproductive when a vanishing testis is intraabdominal. Moreover, in cases of testicular-epididymal dissociation, a scrotal nubbin may actually be the epididymis instead of a vanishing testis; a laparoscopic approach facilitates more accurate diagnosis and subsequent management of such cases (Wolffenbuttel et al, 2000; De Luna et al, 2003). 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. Contralateral fixation of a solitary testis in cases of monorchism is advocated by some but not universally supported. The possibility that prenatal torsion is the cause of vanishing testis (Gong et al, 1996) does not imply that the contralateral testis is likely to undergo a similar fate after the postnatal period. However, some surgeons empirically recommend contralateral fixation to eliminate the risk of such a devastating complication (Rozanski et al, 1996) and/or because a contralateral bell-clapper deformity (incomplete testicular fixation to the tunica vaginalis) may be present (Bellinger, 1985; Al-Zahem and Shun, 2006). However, review of the anatomy of the tunica vaginalis contralateral to vanishing testes suggests that the bell-clapper anomaly is rare and the risk of torsion of the solitary testis in these cases is minimal (Martin and Rushton, 2014). AssociatedPathology Testicular Maldevelopment Many observational studies of the histologic development of prepubertal cryptorchid testes have been published. Over 40 years ago, Mancini and colleagues systematically reported germ cell counts and related arrested development of spermatogonia with progressive loss in cryptorchid testes (Mancini et al, 1965). Subsequently, several large series, some with additional normal autopsy (Hedinger, 1982) or affected control (hernia, hydrocele) (Hadziselimovic et al, 1986) data and others that refer to these established age-dependent norms (Schindler et al, 1987; Gracia et al, 1995; McAleer et al, 1995; Cortes et al, 2001; Huff et al, 2001), have provided mainly consistent findings in cryptorchid boys. These data show that the number of spermatogonia (germ cells) per tubule is reduced after infancy and fails to increase normally with age in cryptorchid and to a lesser degree in contralateral scrotal testes. The frequency of abnormal histology in the contralateral testis varies among studies, ranging from 22% to 95%, and is likely reflective of differences in patient populations, use of control data, and methodology. Moreover, variability within and among biopsies from single testes has been reported (Hedinger, 1982; Schindler et al, 1987). However, these data provide strong evidence that abnormal germ cell development is often present after early infancy in cryptorchid testes. Findings are similar in boys with secondary cryptorchidism after hernia repair, suggesting that these may in fact be cases of primary cryptorchidism (Fenig et al, 2001). In other studies, higher germ cell counts were correlated with reduced age-dependent interstitial fibrosis (Suskind et al, 2008), lower age at surgery, and increased likelihood of testicular palpability (Tasian et al, 2009; Kraft et al, 2011). In a series of 723 boys with cryptorchidism (14% bilateral), testicular volume did not predict germ cell count (Noh et al, 2000), but in a subsequent series of 1326 boys with unilateral cryptorchidism from the same institution, testis volume was positively correlated with germ cell counts in both undescended and contralateral descended testes (Kraft et al, 2011). Detailed studies of peritubular myoid and Sertoli cells in cryptorchidism are limited, but their abnormal development or function may contribute to the observed germ cell abnormalities. The available data suggest disruption of prepubertal Sertoli cell morphology, failure of maturation at puberty, and evidence for reduced number after 4 months of age in cryptorchid testes (Lackgren and Ploen, 1984; Rune et al, 1992; Regadera et al, 2001; Zivkovic and Hadziselimovic, 2009). Impaired transformation of gonocytes to spermatogonia is reported in cryptorchid testes and may help define fertility potential. Although the ratio of gonocytes to spermatogonia appears to be normal in cryptorchid testes at about 1. As noted for germ cell counts, the reported percentage of cryptorchid testes lacking Ad spermatogonia varies widely from 17% to 85% (Zivkovic et al, 2009; Thorup et al, 2013). Ad spermatogonial number may also be reduced in the contralateral testis of boys with unilateral cryptorchidism (Kraft et al, 2011). In addition, the appearance of primary spermatocytes at age 4 to 5 years is delayed in cryptorchid testes (Huff et al, 1989). Kollin and colleagues studied testicular growth in undescended and scrotal testes and measured the effect in orchidopexy on testicular size using serial ultrasonography in prospective randomized studies of congenital cryptorchidism (Kollin et al, 2006, 2007, 2012, 2013). They showed that the undescended testis is smaller at birth and grows less well than the scrotal testis, even if spontaneous descent occurs. Postoperative testicular growth was superior in boys who underwent orchidopexy at age 9 months as compared with those randomized to surgery at age 3 years. Biopsies at the time of orchidopexy in these patients showed marked reductions in germ cell number and a less prominent reduction in Sertoli cells when orchidopexy was delayed until age 3. In these studies, inhibin B levels correlated with testicular volume, as reported previously for neonates with normal testes, where inhibin B levels were interpreted to primarily reflect differences in Sertoli cell number (Main et al, 2006b; Sharpe, 2006). These well-conducted prospective studies provide strong support for the concept that cryptorchidism is associated with both primary and secondary effects on testicular development, and that the extrascrotal position of the testis may have adverse effects even in infancy. Anomalies of the Epididymis, Processus Vaginalis, and Gubernaculum Attachment of the epididymis to the testis may be abnormal in cryptorchid boys (Marshall and Shermeta, 1979), but the reported frequency varies widely from 16% to 75% (Heath et al, 1984; Merksz and Toth, 1987; Gill et al, 1989; Mollaeian et al, 1994; Kraft et al, 2011), most likely because characterization of this anomaly may be subjective. Anatomic findings in decreasing order of frequency include partial or complete nonfusion between the caput and/or cauda epididymis and the testis, epididymal elongation and/or looping, and atresia. Epididymal anomalies were more common in congenital (37% to 60%) than acquired (11% to 31%) cryptorchidism (Barthold et al, 2012; van Brakel et al, 2012). Complete epididymal detachment or nonunion between the testis and epididymis and vas is rare and more likely to be associated with abdominal testes (Foley et al, 2005; Wakeman and Warner, 2010; Karaman et al, 2011; Sharma and Sen, 2013) but did not correlate with testicular histology (Kraft et al, 2011). However, the degree to which these anomalies alter sperm transport, if at all, and potentially contribute to subfertility in males with a history of cryptorchidism remains undefined. Failure of closure of the processus vaginalis (inguinal hernia) and abnormal attachment of the gubernacular remnant are common in association with cryptorchidism. In their study of 759 patients, Cendron and colleagues identified a persistently patent processus vaginalis ipsilateral to 87% of unilateral and 71% of bilateral undescended testes. Specific notation of the gubernacular position available from this and another large study indicates aberrant attachment lateral to the scrotum in 66% to 75% of cases (Moul and Belman, 1988; Cendron et al, 1993). The processus is patent in 45% to 50% of boys with ascending testes, possibly related to older patient age and/or reduced severity of cryptorchidism in this group (Barthold and Gonzalez, 2003; Barthold et al, 2012; van Brakel et al, 2012). Inguinal hernia is also more common in family members of boys with cryptorchidism (Barthold et al, 2012). Anomalies of the tunica and processus vaginalis in cryptorchidism predispose to development of testicular torsion or clinical hernia, respectively, in rare cases. Torsion of an undescended testis can occur at any age (reviewed by Zilberman et al, 2006) and may be confused with an incarcerated inguinal hernia. The risk of torsion is higher in undescended than in scrotal testes and may be particularly high in children with neuromuscular diseases such as cerebral palsy. Delay in diagnosis is common, and a high index of suspicion is needed to reduce the high risk of testicular loss. Of particular concern is the potential risk for delayed diagnosis with resultant testicular necrosis during the postnatal period when infants are observed for spontaneous testicular descent (Singal et al, 2013). 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. Because abdominal cryptorchidism commonly occurs in these cases, laparoscopy is useful in both diagnosis and treatment.

Multilocular cysts of the kidney: a study of 29 patients and review of the literature cholesterol score of 3 buy discount ezetimibe on-line. Proceedings of the Tenth Annual Conference of the National Computer Graphics Association level of cholesterol in shrimp ezetimibe 10 mg buy on line. Autosomal dominant polycystic kidney disease: presentation kresser cholesterol ratio buy 10 mg ezetimibe visa, complications and progression cholesterol test by mail cheap ezetimibe american express. Renal cell carcinoma of native kidneys: prospective study of 129 renal transplant patients cholesterol lowering foods in kerala order ezetimibe 10 mg on line. Acquired cystic disease of the kidneys: a hazard of long-term intermittent maintenance hemodialysis. Molecular studies in Finnish patients with familial juvenile nephronophthisis exclude a founder effect and support a common mutation causing mechanism. Multicystic dysplastic kidney: natural history from in utero diagnosis and postnatal followup. 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Obstructive nephrouropathies constitute the single largest entity leading to renal insufficiency in male children younger than 1 year of age, and renal insufficiency is the largest single cause of renal failure needing transplantation, occurring in about 23% of children undergoing transplant (Benfield et al, 2003; Seikaly et al, 2003). A large number of children are affected by lesser degrees of obstruction and may undergo years of clinical monitoring and imaging studies. The wide spectrum of obstructive changes constitutes one of the major challenges in the clinical management of these conditions in that there is no definitive dividing line between obstruction that warrants intervention and obstruction that does not (Peters, 1995). The presence of an obstructive lesion is readily determined with current imaging, but the criteria for intervention remain controversial. This is largely due to the absence of effective markers of the patterns and progression of obstruction as well as limited information regarding the natural history of differing degrees of obstruction (Chevalier, 2004). We are left with few guideposts along the spectrum of obstruction by which clinical decisions may be made. This chapter will review the current state of knowledge regarding the pathophysiology of congenital urinary obstruction, how it may be correlated with clinical scenarios, and its distinction from postnatal obstruction. It is anticipated that with increasing understanding of the mechanisms of congenital urinary obstruction, we will become better able to discriminate between children requiring therapeutic interventions from those in whom this would be unnecessary. Obstructive hypertension appears to be renin mediated (Riehle and Vaughan, 1981; Urata et al, 1985; Mizuiri et al, 1992) and may be reversible with surgical repair (de Waard et al, 2008). The pathologic correlates of these functional alterations have been described in the congenitally obstructed kidney to varying degrees (Elder et al, 1995; Stock et al, 1995; Han et al, 1998; Poucell-Hatton et al, 2000; Zhang et al, 2000; Huang et al, 2006).

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