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Persistent anhydramnios after placement of a vesicoamniotic shunt is a rare but potentially serious concern in a patient in utero. We present the case a male fetus who at 32 weeks gestation had bilateral hydroureteronephrosis, a distended bladder, and anhydramnios. A vesicoamniotic shunt was placed, but the anhydramnios persisted. Postnatally, the child was found to have a migrated shunt, left grade V vesicoureteral reflux (VUR) and posterior urethral valves (PUV). He underwent a PUV ablation and removal of the migrated shunt. The patient is now two years old and is doing well with no urinary tract infections or renal deterioration.

A 3-month-old boy was seen for routine follow-up at the pediatric nephrology outpatient clinic. He had been diagnosed as having Sotos syndrome manifesting with craniofacial dysmorphism, feeding difficulties, pulmonary artery stenosis, and atrial septal defect, as well as complex urological abnormalities. He had bilateral hydronephrosis with megaureter and grade V vesicoureteral reflux to the left and grade I to the right kidney. At the age of 6 weeks, static renal scintigraphy using DMSA (99mTc-dimercaptosuccinic acid) to assess renal morphology, structure, and function had demonstrated almost symmetrical kidney function (split kidney function left 44% vs right 56%) without cortical scarring. While his baseline serum creatinine had been 40 μmol/L (0.45 mg/dL), a sudden rise to 69 μmol/L (0.79 mg/dL) was noted. Urinary tract infection was ruled out, as was dehydration. On renal ultrasound, dilation of the right collecting system and ureter had increased significantly and a novel fluid collection at the upper pole was noted, which prompted an MRI study (Fig. 1). In addition to serum creatinine, cystatin C measurement was ordered and was within the reference interval for age (1.13 mg/L). Fig. 1. Abdominal MRI. Bilateral hydronephrosis and fluid collection at right upper pole (asterisk). Creatinine, the most commonly used endogenous marker of glomerular filtration rate (GFR),4 has in recent years been supplemented by cystatin C. In fact, today's most reliable equations for estimating GFR both in adults (1) and in children (2) make use of a combination of the 2. Still, in certain conditions the 2 markers can give very discordant findings, which may be diagnostic, as in the case presented here. Discordant findings may occur …

To describe a laparoscopic surgical technique for ureterocystoplasty in pediatric patients with the Mitrofanoff procedure.The procedure was performed in 4 patients (2 females and 2 males), 8-11 years old (average, 9.5 years), with a history of myelomeningocele and secondary neurogenic bladder. The patients were evaluated before the surgery with renal ultrasound, voiding cystourethrography, and renal scintigraphy. All subjects reported left hydronephrosis with severe dilatation of ureter and the collector system, left megaureter with grade V vesicoureteral reflux, and left functional exclusion, with right renal normal function. The urodynamic investigations revealed low bladder size and bladder leak point pressure (BLPP) above 40 cm H2O. The laparoscopic ureterocystoplasty augmentation procedure and the Mitrofanoff procedure with the proximal ureter were performed in these patients. Complications and outcomes were recorded and compared with those of the postoperative urodynamic test.The 2-4 years of follow-up of the patients and its urodynamic postoperative evaluation reported at least 75% of the capacity according to their age, compliance that varied between 15 to 20 mL/cm H2O, and a BLPP of less than 40 cm H2O. This last parameter is considered of low risk to damage the upper urinary tract. There was no leaking of urine by the stoma over the 4 hours of catheterization.Even though enterocystoplasty is the gold standard to increase the capacity of the neurogenic bladder, it has an elevated morbidity. So the use of a dilative ureter to increase bladder size and create a Mitrofanoff stoma in patients with neurogenic bladder, pop-off phenomenon, and renal ipsilateral atrophy could be considered by the laparoscopic approach.

The reasons for referral and treatment strategies in patients who underwent complete primary repair of bladder exstrophy elsewhere in the newborn period were evaluated.An institutionally approved database identified patients who underwent complete primary repair of exstrophy and were subsequently referred for continued care.A total of 10 females and 55 males were referred for treatment between 1996 and 2010. Six females and 23 males were referred for complications following initial complete primary repair of exstrophy. Female complications included dehiscence (3 patients), prolapse (2) and stricture (1). Male complications included dehiscence (10 patients), prolapse (9), pubic separation (1) and stricture (3). Nine males had posterior urethral loss and 13 had major penile soft tissue injuries. Grade V vesicoureteral reflux and severe hydronephrosis were seen in the 4 patients with urethral strictures. Patients with dehiscence or prolapse underwent successful reclosure with osteotomy. Cases with posterior urethral loss or strictures were repaired with grafts. Four females and 32 males were referred for incontinence. Nine of these patients had poor bladder capacity, of whom 5 underwent bladder augmentation with continent stoma and 4 are awaiting further bladder growth. A total of 27 patients underwent bladder neck repair, with 15 (56%) subsequently continent, 5 (19%) daytime continent with nocturnal incontinence and 7 (26%) continuously incontinent. At initial closure osteotomy had been performed in all patients who were continent following bladder neck reconstruction and in 4 of 5 who were daytime continent.Complications of complete primary repair of bladder exstrophy can result in undesired upper urinary tract changes and penile soft tissue loss. Surgical reconstruction of such complications and acceptable continence are attainable in select cases.

Left tibial agenesis, polysyndactyly with talipes equinovarus deformity and Grade IV vesicoureteral reflux of the right kidney are described in a 40-day-old male and an unrelated 1-month-old male, is also reported with right tibial agenesis, polysyndactyly with talipes equinovarus deformity and right kidney agenesis and left Grade V vesicoureteral reflux. No other pathology was recorded. Follow up at 1 year and 3 years, respectively, revealed normal motor and mental development. As this combination has been unpublished before, we believe that this a new syndrome.

Since DX/HA was approved by the Food and Drug Administration in 2001 as an acceptable tissue-augmenting substance for subureteral injection, endoscopic treatment has become increasingly popular for treating vesicoureteral reflux (VUR). However, most paediatric urologists still continue to recommend ureteral reimplantation as the treatment of choice in the management of grade V VUR. The purpose of this study was to prospectively evaluate the effectiveness of endoscopic subureteral injection of DX/HA in the treatment of grade V reflux.During 2001-2009, 56 children (35 males, 21 females) with primary grade V VUR underwent endoscopic treatment using DX/HA. Their ages ranged from 4 months to 11 years. Forty-nine patients had unilateral grade V VUR, and seven had bilateral grade V (63 refluxing ureters including 17 duplex systems). Renal scarring on dimercaptosuccinic acid was observed in 39 kidneys (mild scarring 13, moderate scarring 14 and severe scarring 12). In all patients, endoscopic injection was made after inserting the needle within the wide ureteral orifice. Renal ultrasound and voiding cystourethrogram was performed 3 months after the endoscopic treatment. If VUR had not resolved, the patient was considered for further endoscopic treatment. After the VUR was resolved, children were followed with renal ultrasound at 1 year and every 2 years thereafter.The VUR completely resolved after first injection of DX/HA in 33 (52.4%) ureters and downgraded to grade I VUR in 7 (11.1%) ureters. 19 (30.2%) ureters required a second injection, and 4 (6.3%) ureters required a third injection to resolve VUR. No children in this series needed reimplantation of ureters or presented with ureteral obstruction during follow-up.Endoscopic treatment with DX/HA is effective in eradicating grade V primary reflux. This simple and minimally invasive treatment should be offered to all parents of children with grade V VUR as an alternative to ureteral reimplantation.

We encountered a patient with megaureter-megacystis syndrome showing a giant bladder and dilated ureters with marked reflux, which is very rare; to our knowledge, only 2 patients have been reported in Japan. The patient was a 4-year-old boy, who showed inborn polyposia and polyuria, and proteinuria at the age of 1 year. He visited the pediatric department in our hospital complaining of cold-like symptoms, stomachache and diarrhea. Urinary infection and kidney dysfunction were observed, and the patient was hospitalized for close examination. Bilateral pyelocaliceal hydronephrosis was detected by ultrasonography, and the patient was referred to our department. CT revealed bilateral hydronephrosis (right atrophic kidney), hydroureters and megacystis. Bilateral grade V vesicoureteral reflux, an increase in the bladder volume (> 300 ml), and urination without residual urine were noted by voiding cystourethrography. Uroflowmetry revealed that maximum flow rate was 21.6 ml/sec, voided volume was 110 ml, and residual volume was 24 ml. From these examinations, the patient was diagnosed as having megaureter-megacystis syndrome, and underwent antireflux operation of the bilateral ureters using Cohen's procedures.

OBJECTIVES The aim of this study was to determine circulating levels of ELAM-1 in sera collected from patients with vesicoureteral reflux, and to investigate the relationship between circulating concentrations of ELAM-1 and reflux nephropathy. METHODS The serum levels of ELAM-1 were measured in 117 patients aged between 3 months and 13 years, with grade III to grade V vesicoureteral reflux, and 22 controls, by ELISA technique. Renal scarring was demonstrated in 46 of the 117 children on radionuclide scans. RESULTS The mean serum ELAM-1 levels were 54.23 +/- 20.11 ng/ml in controls and 77.79 +/- 34.50 ng/ml in children with vesicoureteral reflux (p < 0.05). The mean serum ELAM-1 levels in children with vesicoureteral reflux and renal scarring (104.48 +/- 34.53 ng/ml) were significantly higher than in children with VUR without renal scarring (60.51 +/- 20.85 ng/ml; p < 0.001). CONCLUSION These findings suggest that circulating ELAM-1 levels reflect increased tissue damage in children with vesicoureteral reflux, probably as a result of progressive localised inflammatory responses occurring in these patients.

Multicystic renal dysplasia is a common cause of childhood end-stage renal failure and is categorized under the entity known as congenital anomalies of kidney and urinary tract. With the increasing knowledge about their natural history, it is now known that most of the cases of unilateral multicystic dysplastic kidney undergo spontaneous involution; thus, most of the pediatric urologists now prefer conservative management with long-term nephrological and urological observation. However, the presence of recurrent urinary tract infection as in the present case report due to associated ipsilateral Grade V vesicoureteral reflux, a rare associated finding mandates nephrectomy.

Objectives Although endoscopic treatment provides a high rate of success in children with grades II–IV vesicoureteral reflux (VUR), its role in the management of grade V reflux has been questioned. In this study we reviewed our 21-yr experience of endoscopic treatment in children with grade V primary VUR. Methods We retrospectively reviewed the medical records of 132 children who underwent endoscopic treatment for primary grade V reflux from 1984 to 2004. VUR was unilateral in 39 patients and bilateral in 34, and 59 patients had ipsilateral grade V reflux with a lower grade of VUR on the contralateral side. Endoscopic treatment was performed in a total of 166 grade V ureters; polytetrafluoroethylene was used from 1984 to 2000 and dextranomer/hyaluronic acid from 2001 to 2004. Median follow-up was 12.2 yr and mean follow-up was 13.4 yr. Results VUR was completely resolved after first injection in 88 (53%) ureters and downgraded to grade I or II in 26 (15.7%). VUR resolved after a second and third injection in 36 (21.7%) and 10 (6%) of ureters, respectively. Endoscopic treatment failed to correct VUR in 6 (3.6%) ureters, requiring ureteral reimplantation in 5 and nephrectomy in 1. Thirteen patients developed urinary tract infections during the follow-up period, and on investigation 9 ureters (5.4%) had recurrence of VUR. No injection or morbidity related to tissue-augmenting substances was noted in any patient. Conclusion Endoscopic treatment should be the first-line of treatment in management of grade V vesicoureteral reflux.

Objective. To determine if urinary N-acetyl-beta-glucosaminidase (NAG) assays could be applied as a screening test for early detection of vesicoureteral reflux. Methods. Two hundred eighty-eight urine samples from children undergoing voiding cystourethrography (VCU) for a variety of urologic problems were assayed for N-acetylbeta-glucosaminidase using spectrophotofluorimeter techniques. Sample creatinine levels were also determined using an auto analyzer. The urinary NAG to creatinine ratio for each patient was then compared to their VCU results, which were interpreted independently of the assay values and graded according to the International Reflux Classification system. The NAG to creatinine ratios were then classified according to their grade of reflux, and the mean NAG levels, plus one standard deviation, were calculated for each as well as for the nonrefluxing controls. Statistical analysis was done with P values obtained by Student's t-test. Results. A total of two hundred thirty-two specimens were evaluable with ninety (38.8%) samples collected from refluxing patients and one hundred forty-tw o (61.2%) samples from nonrefluxers. The NAG levels standardized to urine creatinine revealed a mean value among refluxers of 15.514 μmol/g creatinine. This compared with a mean value of 14.611 μmol/g creatinine in the nonrefluxing group, with the difference being insignificant. When the mean NAG levels were compared for each grade of reflux to the nonrefluxing controls, only grade V had a significant elevation (44.561 μmol/g creatinine) above the nonrefluxers (P = 0.0001). Conclusions. With the exception of grade V vesicoureteral reflux, urinary NAG levels do not reliably detect the presence of reflux and therefore cannot be accurately applied as a screening test for detection of this common urologic problem.

Herniation of the ureter through the sciatic foramen is extremely uncommon. In 1994 Epner and Lautin reported on 11 patients with this condition.' Several other cases have been reported since then, including some in which the hernia was intermittent.' CASE REPORT A 3-month-old female infant diagnosed with Down's syndrome just after birth had a urinary tract infection and was admitted to our department. Abdominal ultrasound revealed bilateral hydronephrosis and voiding cystourethrography showed bilateral grade V vesicoureteral reflux. Fluoroscopy demonstrated the left ureter herniating into the sciatic foramen, as evidenced by ureteral curling when the infant cried (fig. 1). Otherwise both ureters were normal. DISCUSSION Sciatic hernia is the protrusion of the pelvic peritoneal sac and its contents through the greater or lesser sciatic foramen (fig. 21.3 The ureter may be involved in this peritoneal sac. It is important to recognize ureteral-sciatic hernia, since it may cause urinary disturbances ranging from asymptomatic pyuria' to hydronephr~sis~ and even pyonephro~is.~ The physician should bear in mind the possibility of ureteral-sciatic herniation when hydronephrosis is associated with cachexia: neuromuscular or hip disease leading to atrophy of the piriform muscle,5 or urinary disturbances are associated with a sciatic nerve syndrome and intestinal ob~truction.~ The differential diagnosis of lesions in the gluteal space must also include this di~ease.~ Symptomatic ureteral-sciatic hernia

CASE REPORT We present a patient with serious contracted bladder and bilateral grade V vesicoureteral reflux which developed after intravesical Mitomycin­C treatment. The 78­years­old male patient had a history of transurethral bladder tumor and prostate resection at the same session in another center, because of a solitary, papillary tumour with a diameter of 20 mm near the left ureteral orifice and an enlarged prostate. Before the operation intravenous urography was done, showed normal bladder capacity (figure 1). The histology revealed transitional cell carcinoma (T1, G1) and benign prostatic hyperplasia. Intravesically 20 mg Mitomycin­C was given weekly for the first six weeks, then monthly for 10 months. During the treatment he developed increasing frequency and urge incontinence, but the treatment was not interrupted. Two years after the end of instillation the patient presented to our department with right flank pain, frequency and urge incontinence. Serum levels of creatinine and urea were 228 μmol/L and 11.3 mmol/L, respectively. Urine culture, including culture for tuberculosis, was negative. His renal ultrasound showed bilateral hydronephrosis, and thin renal cortex. Cystogram revealed contracted bladder and bilateral grade V vesicoureteral reflux (figure 2). Cystoscopy showed golf­hole ureteral orifices and low capacity (50 ml) of the bladder. There were no sign of tumour recurrence. Due to co­morbidity of the patient (hypertension, ischemic heart disease) reconstructive surgery could not be performed; therefore percutaneous suprapubic cystostomy was placed. One year after suprapubic cystostomy placement, the patient was very well, he had no symptoms and his serum level of creatinine stabilized at about 150 μmol/L. DISCUSSION Mitomycin­C, doxorubicin and epirubicin are the most frequently used intravesical chemotherapy to prevent recurrence of superficial bladder cancer after transurethral resection. These drugs can be used for single, immediate, postoperative instillation or for adjuvant repeat instillations. The efficacy of these drugs are similar [1, 2]. Side effects of intravesical chemotherapy (cystitis, bladder irritation) are usually moderate and localized to the bladder. However, rarely it can cause severe, even fatal, complications, especially after immediate post­operative instillation when there is a bladder wall perforation [3]. Mitomycin­C is considered to be a safe intravesical chemotherapeutic drug, but rarely it can cause contracted bladder, which can require urinary diversion [4]. The cause of contracted bladder in the present case was most likely chemocystitis caused by intravesical Mitomycin­C therapy; we were able to exclude other common cause of contracted bladder (tuberculosis, schistosomiasis, interstitial cystitis, irradiation cystitis). Perhaps, long­term (1­year­long) treatment and the absence of treatment interruption, even though the patient’s symptoms facilitated this serious adverse effect. CLINICAL IMAGES OPEN ACCESS

Augmentation cystoplasty is a commonly used method to treat patients with inadequate bladder capacity. Calculus formation within the altered urinary tract has been a frequent complication. We report the unique finding of calculi within a retained ureteral stump in a patient who had undergone enterocystoplasty. CASE REPORT A 29-year-old female presented with recurrent urinary tract infections that were refractory to treatment. The patient had been born with sacral agenesis and imperforate anus. Neurogenic bladder and vesicoureteral reflux had subsequently developed, and the patient underwent ileal conduit formation for management of progressive bilateral hydroureteronephrosis as a young child. At age 15 years she underwent urinary undiversion with ileocecoplasty to the native bladder. Videourodynamics were repeated at age 28 years and revealed a volume capacity of 700 ml., and bilateral grade V vesicoureteral reflux and reflux into the retained ureteral stumps. The patient performed clean intermittent catheterization every 2 to 4 hours to minimize the post-void residual urine volume. Recurrent urinary tract infections were managed with outpatient oral antibiotic therapy. In addition, in 1999 a bladder calculus had developed in the bowel segment of the bladder. The stone was managed with cystolithotripsy, with complete stone removal. In 2001 urinary tract infections recurred despite appropriate antibiotic coverage for each recurrent infection and antibiotic prophylaxis. Symptoms consisted of painful urethral catheterizations, small urethral catheterized volumes and pyuria. Plain x-ray of the kidneys, ureters and bladder demonstrated multiple calcified densities in the pelvis (see figure). In preparation for cystolithotripsy, cystoscopy of the native and augmented bladder was performed, which showed an absence of bladder calculi. However, a dilated and patulous right ureteral orifice was identified. Retrograde ureterogram revealed several filling defects in the right ureteral stump that correlated with the calcifications seen on x-ray. The ureteral stump measured approximately 8 cm. The calculi were fragmented via semirigid ureteroscopy with holmium laser lithotripsy and basket stone extraction. The bladder was copiously irrigated. The patient was discharged home on the same day with postoperative instructions to perform bladder irrigation twice daily in addition to clean intermittent catheterization. Stone analysis demonstrated calcium phosphate. DISCUSSION