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PURPOSE: Nephrolithiasis in preterm infants rarely requires surgical management. When it persists despite conservative therapy, treatment options are not clearly defined. We report a single institutional experience with extracorporeal shock wave lithotripsy (ESWL)* for the treatment of these small infants. MATERIALS AND METHODS: We treated 8 infants (mean age 13 months) with a history of prematurity and 9 persistent stones with a Dornier HM3 lithotriptor between 1996 and 1999. Mean weight was 7,700 gm. Of the infants 7 had been treated with furosemide for bronchopulmonary dysplasia and 1 presented with multiple anatomical abnormalities. Gantry modification with a wooden platform and polystyrene foam positioning was used for lung and visceral protection. Ureteral stents were placed in 5 patients before ESWL. Renal ultrasonography was performed before, and 2 and a mean of 8 weeks after ESWL. Stone risk factors in our population were investigated through a multispecialty approach. RESULTS: Average stone burden was 47.9 mm.2. A total of 9 sessions of ESWL were required for complete fragmentation of the 9 renal stones. A mean total of 2,100 shocks at a mean 16.1 kV. were administered. One patient with bilateral stones was treated in 2 separate sessions after a 4-week interval. No repeat ESWL sessions or other surgical interventions were required in any patient. Renal ultrasonography demonstrated no post-ESWL morphological changes. Practices leading to a higher incidence of neonatal nephrolithiasis at our institution were also identified. CONCLUSIONS: ESWL is effective treatment for nephrolithiasis in small infants. Short-term safety has been established but continued long-term functional followup is essential. Multifactorial etiologies of nephrolithiasis must be identified and modified promptly in the care of preterm infants.

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PURPOSE: We report the association of complete urethral duplication and bladder exstrophy in 5 males, and describe the main characteristics to achieve diagnosis. MATERIALS AND METHODS: From 1983 to 2000, 5 males with single bladder exstrophy and complete urethral duplication were seen at 3 institutions. Although bladder exstrophy was obvious at birth, only 1 patient had the preoperative diagnosis of associated urethral duplication. All patients were evaluated with abdominal ultrasound, voiding cystourethrogram after bladder closure and cystoscopy. RESULTS: All patients were totally incontinent, including 4 after primary bladder exstrophy closure. Although it was initially unnoticed by the urologist, most patients had leakage from the tip of the penis during the Valsalva maneuver. In all cases bladder exstrophy was single and deeply situated in the pelvis with excellent elasticity. All patients had a normal sized penis with less severe dorsal chordee and conical shaped glans. The duplicate epispadiac urethra did not have a verumontanum. Only 1 of the 5 patients had the correct diagnosis made before primary closure of bladder exstrophy, while in the other 4 diagnosis of a duplicate ventral urethra was made at the time of epispadias or fistula repair. There were no other associated malformations. In all cases the dorsal epispadiac urethral plate was excised and bladder emptying occurred through the ventral urethra, which at cystoscopy had a normal verumontanum. Postoperatively, all patients became continent and voided with good flow through the ventral urethra and, as opposed to many cases of exstrophy and epispadias, had normal size and excellent cosmesis of the penis. CONCLUSIONS: Complete urethral duplication is a rare variant of the exstrophy-epispadias complex. Patients with this anomaly present with a larger and more deeply situated bladder plate than classical exstrophy and a larger penis. A high index of suspicion is necessary to make a preoperative diagnosis. Excision of the dorsal urethral plate and maintenance of the ventral urethra are the treatment of choice, resulting in a continence and normal voiding.

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Fetal lower urinary tract obstructive uropathy, when associated with oligohydramnios, is usually associated with a poor outcome. We present a case of successful in utero endoscopic ablation of posterior urethral valves in which the infant survived the neonatal period without evidence of renal dysplasia. The role, indications, and potential benefits of this novel technique are discussed.

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Hydronephrosis due to congenital ureteropelvic junction obstruction (UPJO) is commonly diagnosed by antenatal sonography. We report the case of an infant who developed new-onset hydronephrosis in the right kidney following uneventful left-sided pyeloplasty for a congenital UPJO. The furosemide minus 15-minute diuretic renogram (F-15 DR) was used to confirm obstruction in the affected renal unit when standard diuretic renography was equivocal. The indications for surgery or observation as well as the role of the F-15 DR are discussed.

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PURPOSE: Inadequate bladder emptying is a common urinary dysfunction in children. The role of alpha-blockers for managing bladder outlet obstruction remains relatively unexplored in children. Because of the well established impact of alpha-blocker therapy in men, we investigated its use for treating inadequate bladder emptying in the pediatric population. MATERIALS AND METHODS: We treated 17 children 3 to 15 years old with documented poor bladder emptying of various etiologies, including dysfunctional voiding, the Hinman syndrome, the lazy bladder syndrome, posterior urethral valves, myelomeningocele and the prune-belly syndrome, using the alpha-1 adrenergic receptor antagonist, doxazosin. The initial dose of 0.5 to 1.0 mg. nightly was increased according to patient response and as tolerated. Patients were followed weekly to monthly by symptomatic history, and urine flow and/or post-void residual urine volume measurement. Two patients with neurogenic bladder were also followed with cystometrography and leak point pressure determination. RESULTS: Bladder symptomatology and/or emptying improved in 14 patients (82%). Ten patients had decreased post-void residual urine during treatment and in 3 uroflowmetry showed increased maximum flow. Two patients with neuropathic bladder secondary to myelomeningocele had decreased leak point pressure on alpha-blocker therapy and in 2 with a history of posterior urethral valves new onset bilateral hydronephrosis completely resolved. Only 1 patient had mild postural hypotension, which resolved with dose reduction. CONCLUSIONS: Selective alpha-blocker therapy seems to be well tolerated in children and appears effective for improving bladder emptying in various pediatric voiding disorders at short-term followup. Long-term followup and further investigation are warranted to validate the potential role of alpha-blocker therapy in pediatric urinary dysfunction.

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PURPOSE: We evaluated the difference in response patterns of captopril versus standard renography for assessing hydronephrosis due to suspected ureteropelvic and ureterovesical junction obstruction. This technique may identify hydronephrotic kidneys in which normal function is maintained by vasoactive compensatory mechanisms. Sustained obstruction may cause these mechanisms to fail, and expose the kidneys to permanent functional deterioration in the long term. MATERIAL AND METHODS: We prospectively studied 15 boys and 8 girls with a mean age of 3.5 years with grades III to IV/IV hydronephrosis. Evaluations included renal sonography, standard diuretic and captopril renography, glomerular filtration rate, voiding cystography, serum creatinine, blood pressure, and urinalysis with culture and sensitivity. Obstruction was suspected at the ureteropelvic junction in 19 kidneys and at the ureterovesical junction in 9. We compared differential function values obtained by standard diuretic and captopril renography. RESULTS: We observed certain patterns in response to captopril renography, including pattern 1--unilateral decrease in hydronephrotic kidney relative function in 5 of 23 cases, 2--bilateral decreased function in 2, 3--bilateral increased function in 4 and 4--no change in function in 12 on standard renography. When half-time was more than 20 minutes on standard diuretic renography in 8 cases, captopril renography showed an ipsilateral decrease and bilateral increase in glomerular filtration rate in 4 and 1, respectively, and no change in 3. In 12 of the 23 patients (52%) there was no difference in the results of captopril and diuretic renography as well as no change in differential function on standard diuretic renography during 1 1/2 years of observation. Surgical correction was performed in 4 patients in whom half-time was greater than 20 minutes and differential function was decreased on captopril renography. CONCLUSIONS: Our preliminary study reveals that there may be a role for captopril renography for detecting renin-angiotensin system mediated compensatory mechanisms in obstructive uropathy. When such compensatory mechanisms are activated, they may be unmasked by captopril, producing 1 of 4 patterns on renography and glomerular filtration rate. Patterns may indicate different degrees of impending renal function impairment and, thus, they may become useful for determining the progression of injury, when present, and the appropriate timing of surgical intervention.

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PURPOSE: We assess and clarify diagnostic features for making the prenatal diagnosis of cloacal exstrophy. MATERIALS AND METHODS: We evaluated 9 patients born with cloacal exstrophy at our institutions (2 prospectively and 7 retrospectively) for diagnostic features on prenatal ultrasound studies. We also thoroughly reviewed the literature on 13 previous prenatally diagnosed cloacal exstrophy cases. Diagnostic criteria were assessed by combining the findings in our patients and those in previous reports. RESULTS: Of the 22 patients with prenatal ultrasound studies and cloacal exstrophy whom we analyzed 1 of our 9 and 2 in the literature had a cloacal membrane that persisted at 22 weeks of gestation. Major ultrasound criteria for diagnosing cloacal exstrophy prenatally are nonvisualization of the bladder, a large midline infraumbilical anterior wall defect or cystic anterior wall structure (persistent cloacal membrane), omphalocele and lumbosacral anomalies. Seven less frequent or minor criteria include lower extremity defects, renal anomalies, ascites, widened pubic arches, a narrow thorax, hydrocephalus and 1 umbilical artery. CONCLUSIONS: We propose major and minor criteria to assist in the prenatal diagnosis of cloacal exstrophy. Despite these major and minor criteria the certainty of establishing a prenatal diagnosis remains challenging. Persistence of the cloacal membrane beyond the first trimester in 1 patient was an exception to the classic concept of cloacal exstrophy embryogenesis. An accurate prenatal diagnosis requires validation of these criteria by further correlation of prenatal and postnatal observations.

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OBJECTIVE: The objective of this study was to identify a cost-effective method of predicting a therapeutic response to desmopressin (DDAVP) by correlating daytime functional bladder capacity, age and urine osmolalities in patients with monosymptomatic nocturnal enuresis. MATERIALS AND METHODS: Thirty-five children out of 51 who initially presented to our institutions were included in the study. The remainder was excluded for lack of complete data. Constipation was actively addressed and was managed by administration of evening enemas for 3 consecutive days and dietary adjustment prior to initiating the study. Each micturition as measured throughout the day and the maximal daytime functional bladder capacity was determined as the largest void over a 2-day period. Urine samples were collected at home at 08:00, 16:00 and 22:00 (times that would best reflect the fluctuations in plasma vasopressin levels). Intranasal DDAVP was then administered, titrating the dose over a 2-week period. The initial dose was 10 micrograms and the dose was increased 10 micrograms every 3 days. RESULTS: The response to DDAVP was then evaluated and of the 35 children, 27 demonstrated a complete response to DDAVP (all at doses between 10 and 30 micrograms). These were then related to the possible predictive factors. There was a significant correlation between a high maximum daytime functional bladder capacity and response to DDAVP (p = 0.006). Similarly, age was also predictive of a good response to DDAVP treatment (p = 0.008). However, spot urine osmolalities were not predictive of a response to DDAVP (p > 0.1). CONCLUSIONS: Functional bladder capacity is a reliable predictor of response to desmopressin; children with larger capacities are more likely to exhibit a successful response. Older children have a better response rate than younger ones. Spot urine osmolality measured on specimens collected in the home setting is not predictive of response to desmopressin.

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PURPOSE: There is ongoing controversy concerning the management of multicystic dysplastic kidney disease, particularly with regard to the potential for malignant transformation. Our report fuels the debate by adding the 2 youngest patients in whom malignancy was present from birth or developed subsequently. MATERIALS AND METHODS: Two well documented cases of malignancy associated with multicystic dysplastic kidney disease are presented in 2 female infants (5 and 3 months old). The 5-month-old female infant was followed for multicystic dysplastic kidney disease and had no evidence of tumor either antenatally or at birth. The 3-month-old presented with hypertension and interventricular septal defect. A renal tumor was present on initial ultrasound. RESULTS: Even though malignant degeneration is rare in multicystic dysplastic kidney disease, 9 cases have been reported in the literature so far. Of these cases 3 were Wilms tumor, 5 were renal cell carcinomas and 1 mesothelioma. CONCLUSIONS: Our 2 cases lend support to the surgical management of multicystic dysplastic kidney disease, particularly as nephrectomy can now be performed in a day surgery setting with minimal morbidity. Only the risks of coexisting malignancy and possible malignant degeneration transformation are specifically addressed in this article. Other complications of multicystic dysplastic kidney disease such as hypertension, infection, abdominal pain, hematuria and persistent dysplastic renal tissue despite ultrasonographic resolution of multicystic dysplastic kidney disease are additional risk factors to be considered. A recommendation for nephrectomy in all cases of multicystic dysplastic kidney disease cannot be based only on these 2 cases. Several other factors must be weighed before making that decision.

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PURPOSE: We investigated the long-term metabolic impact of gastrointestinal composite reservoirs. MATERIALS AND METHODS: Nine patients underwent construction of a gastroileal (7) or gastrocolonic (2) reservoir for continent urinary diversion. Four cases of metabolic acidosis were converted from a preexisting conduit and the other 5 patients had diversion for either preexisting metabolic acidosis or the short bowel syndrome. All were reconstructed using a medium sized gastric segment (8 x 4 cm.) from the greater curvature of the stomach. The anti-incontinence segment was constructed from a tapered and reimplanted ileal segment. All patients underwent preoperative and postoperative measurements of serum pH, serum electrolytes, and urinalysis. Serum gastrin was measured in all patients postoperatively. Followup from surgery ranged from 47 to 61 months (mean 54.4). RESULTS: All 9 patients demonstrated electrolyte neutrality in serum on long-term followup. Postoperative serum pH (mean 7.40) was significantly different (p < 0.01) from preoperative serum pH (mean 7.36) and serum bicarbonate was also significantly different (p < 0.01) preoperatively versus postoperatively (mean 22.3 versus 25.14). Urine pH values were not significantly different throughout the study. One patient with mildly acidic urinary pH (6.0 to 6.5) had ulcerative skin changes at the stoma site. Three patients had elevated serum gastrin levels on short-term followup but all patients had normal serum gastrin levels on long-term followup. One patient, with persistent alkaline urine, had urolithiasis and symptomatic urinary tract infections. CONCLUSIONS: Our results demonstrate that a composite urinary reservoir constructed using gastric and intestinal segments achieved serum electrolyte neutrality on long-term followup. These results indicate a long-term metabolic advantage over other intestinal reservoirs associated with hyperchloremic metabolic acidosis and may be beneficial in patients compromised by either preexisting metabolic acidosis or the short bowel syndrome.

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PURPOSE: We reviewed our experience with the safety and long-term efficacy of percutaneous endopyelotomy in children with secondary ureteropelvic junction obstruction who previously underwent open pyeloplasty. MATERIALS AND METHODS: Nine patients a median of 7 years old underwent endopyelotomy between June 1985 and July 1995. Anderson-Hynes pyeloplasty had previously been performed in all children. Antegrade endopyelotomy was done with a cold knife in conjunction with balloon dilation in some cases. Postoperative stenting lasted 6 weeks. RESULTS: The median interval between pyeloplasty and endopyelotomy was 7.5 months. Mean operative time was 240 minutes. Percutaneous endopyelotomy was successful in 8 of the 9 patients. One patient who required repeat endopyelotomy after 4 years is presently well at 5 years of followup. Average followup was 5.6 years (range 2 to 10). There was a low morbidity rate, including 1 case of urinary tract infection, 1 of pneumonia and 1 that required blood transfusion. CONCLUSIONS: Percutaneous antegrade endopyelotomy is a safe, effective and durable alternative for children who previously underwent unsuccessful pyeloplasty.

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The objective of the current study was to find a cost-effective way of correlating spot urine osmolalities, bladder capacity and age in patients with monosymptomatic nocturnal enuresis with response to treatment with desmopressin (Minirin, DDAVP). A total of 35 children fulfilled the entry criteria and were included in the study. Constipation was eliminated in these children by appropriate enema treatment and diet adjustment prior to enrollment. Urine samples were collected at home at times that would best reflect fluctuations in plasma vasopressin levels (08:00, 16:00 and 22:00) over three consecutive 24-hour periods. Maximal functional bladder capacity was determined from the largest voided volume. A 2-week dose-titration treatment period with intranasal desmopressin was then conducted. With doses of desmopressin being increased by 10 micrograms every 3 days. Response to desmopressin treatment was then assessed and factors that were observed to be markers of a favourable response were noted. Of the 35 children, 27 demonstrated a complete response to desmopressin treatment, at doses of 10-30 micrograms. Spot urine osmolalities were not predictive of the response to desmopressin (P > 0.1). In contrast, there was a significant correlation between a high maximum functional bladder capacity and response to desmopressin (P = 0.006). Age was also predictive of a good response to desmopressin treatment (P = 0.008).

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Neonatal hydronephrosis is being detected with increasing frequency. The majority of these cases have a tendency to resolve during infancy. Hydronephrosis is an anatomical entity that is not synonymous with obstruction. Review of the pathoembryology, the pathophysiology, the diagnostic techniques currently used and the natural history of hydronephrosis is given. The management and the controversies involved are discussed.

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To our knowledge we report the first case of prenatally diagnosed bilateral neonatal torsion of the spermatic cord. Prenatal ultrasound revealed bilateral hydroceles surrounded by a second fluid layer bilaterally and bilateral inhomogeneous testicles.

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Laparoscopy is useful in both the diagnosis and the management of impalpable testes. Intra-abdominal testicles can be removed laparoscopically if atrophic or can be partly devascularized by spermatic vessel clipping if apparently normal. Assessment of testicular revascularization would be desirable prior to subsequent orchidopexy. A second-stage vasal-based orchidopexy can then be performed once adequate testicular reperfusion via the deferential pedicle is believed to have occurred. We have used both diagnostic and therapeutic laparoscopy in the management of 103 non-palpable testes over a period of 6 years. Open procedures following laparoscopy included 57 orchidopexies, 11 orchiectomies and one microvascular testicular autotransplant. Thirteen laparoscopic interventions were performed: 5 orchiectomies for atrophic testes and 8 testicular vessel clippings followed by 6 second stage open inguinal orchidopexies. Color Doppler duplex ultrasonography was not found to be reliable for assessment of testicular revascularization following spermatic vessel clipping. There were 3 complications which were all related to puncture with the Veress needle.

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Micturition disorders simulating neurogenic bladder disease have been loosely termed "dysfunctional voiding". No underlying neuropathy can be found. A variety of voiding disturbances have been identified since the early 1970s, each with its own characteristics and clinical relevance. We have classified voiding dysfunctions into mild, moderate and severe, according to their potential impact on the upper tracts. Bladder instability, the Hinman syndrome and the Ochoa syndrome are the only dysfunctional voiding syndromes that are associated with reflux or ureterovesical obstruction. Each syndrome is briefly described.

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Laparoscopy is useful in both diagnosis and management of impalpable testes. Intra-abdominal testicles can be removed laparoscopically if atrophic or can be partly devascularized by spermatic vessel clipping if apparently normal. Assessment of testicular revascularization would be desirable prior to subsequent orchiopexy. A second-stage vasal-based orchidopexy than can be performed once adequate testicular reperfusion via the deferential pedicle is believed to have occurred. We have used both diagnostic and therapeutic laparoscopy in the management of 103 non-palpable testes over a period of six years. Open procedures following laparoscopy included 57 orchidopexies, 11 orchiectomies, and 1 microvascular testicular autotransplant. Thirteen laparoscopic interventions were performed: 5 orchietomies for atrophic testes and 8 testicular vessel clippings followed by 6 second-stage open inguinal orchidopexies. Color Doppler duplex ultrasonography was not found to be reliable for assessment of testicular revascularization following spermatic vessel clipping. There were 3 complications which were all related to puncture with the Veress needle.

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We present the manifestations, their recognition, and treatment of urachal disorders, as well as report on 3 cases of urachal abscess in children. A review of the English literature is included.

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