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Background: Reports on increasing hypospadias trends are based on birth defect registries, which are prone to inaccuracy. We assessed the prevalence of hypospadias precisely, by prospective examination of all newborns in Rotterdam over a 2-year period.Methods: A total of 7292 consecutive male births were examined for the presence of hypospadias, classified by severity.Results: The frequency of hypospadias in newborn boys was 0.73% (53/7292). The rate among live births was 38 per 10 000, which is 6 times the previously reported rate for the Southwestern Netherlands (6.2) (P < 0.0001). This registry excludes glandular hypospadias. Without glandular cases, our rate is 26 per 10 000, which is still 4-fold higher (P < 0.0001). The ratio of minor to major hypospadias was 0.3. In 79% of cases, surgery was indicated.Conclusions: We found a 4-fold higher than expected hypospadias rate, which may be explained by case ascertainment differences. The proportion of major cases was higher than generally assumed. This study provides evidence for substantial geographical differences. Explanations for temporal and geographical differences need to be explored. To monitor hypospadias rates and trends accurately, complete case ascertainment, including standardized classification of severity, is warranted. [ABSTRACT FROM AUTHOR]

Objectives: Varicocele in adolescent men is one of the most challenging topics in pediatric urology. What should be considered as a good indication for treatment and at what age? Does treatment improve fertility? Can we wait? What is the best surgical and minimal invasive treatment? To answer these questions we provide a guideline to use in daily practice., Methods: We performed a search of the English literature of the last ten years through the Pub Med database using the following key words "varicocele", "diagnosis", "treatment", "fertility" and "adolescent". We then summated and compared the results of these studies., Conclusion: Adolescents with varicocele need regular follow-up including clinical examination and ultrasound measurements of the testis. Those with a persistent discrepancy between left and right testis size of more than 20% over a period of 12 months and those with pain need varicocelectomy independent of patient age, Tanner stage or varicocele size. The peak retrograde flow (PRF) seems to be a good diagnostic non-invasive tool in the follow-up and can be of help to select patients for surgery. PRF=38 cm/s in combination with testicular asymmetry =20% is a reason for surgery independent of age, Tanner stage or varicocele size. PRF<30 cm/s in combination with testicular asymmetry <20% should be followed annually. In case of worsening PRF or asymmetry surgery must be performed. Patients suitable for conservative management should be followed until semen analysis is possible. We present a flow-chart to use in the management of adolescent varicocele. The best results of varicocele treatment are obtained with microscopic subinguinal and laparoscopic lymphatic spearing techniques as shown in a meta-analysis performed in this review.

A 91-year-old woman with type 2 diabetes presented at the emergency ward subconscious with lower abdominal swelling. Evaluation revealed dehydration and hyperglycaemia, and abdominal x-ray showed an air space surrounding a severely swollen bladder. After excluding enterovesical fistulae, the patient was diagnosed with emphysematous cystitis. Treatment for urinary retention, antibiotic treatment and control of the diabetes mellitus resulted in a rapid recovery. A second patient, a 65-year-old woman with a history of recurrent urinary tract infections and urolithiasis, presented with irritative urinary symptoms and pain in the lower abdomen. Explicit inquiry revealed that she also had intermittent pneumaturia. Urethrocystoscopy revealed submucosal bullae, which are a hallmark of emphysematous cystitis. The patient was given intravenous antibiotic therapy. Diabetes mellitus is a risk factor for emphysematous cystitis. The disorder is treated by draining the bladder with an indwelling catheter and intravenous antibiotic therapy, selected according to the urine culture results. In general, orally administered antibiotics are insufficient.

The cases of spermatic cord torsion reported here show that this condition may occur even some time after fixation. Three men, aged 22, 30 and 35 years, visited the urologist with complaints of an acute scrotum following earlier orchiopexy for spermatic cord torsion. Different techniques are employed to fixate the testicles after spermatic cord torsion. These cases confirm that Jaboulay's bottleneck procedure is the method of choice for bilateral fixation in patients presenting with this condition.

During the last 40 years more and more myelomeningocele (MMC) patients have survived to adulthood and since the 1980s there is growing interest in sexual functioning of these patients. However, most of what is known about the impact of MMC on sexual functioning pertains to males. We have reviewed the literature pertaining to female sexuality and MMC with regard to sexual development, activity, dysfunction, sexual knowledge and education, pregnancy and delivery, as well as future trends.

The importance of the secretion and action of androgens during the critical period of male sexual development is exemplified in patients with androgen insensitivity syndrome. Their karyotype is always 46XY. In 2 sisters, aged 11 and 13 years, the androgen insensitivity syndrome was diagnosed based on an androgen receptor gene mutation. Ambiguous genital development of a new-born was shown to be due to a lack of testosterone production, based on a luteinizing hormone receptor gene mutation. Finally, in a phenotypically female new-born a gene mutation of 17-beta hydroxysteroid dehydrogenase type 3 was found to be responsible for insufficient testosterone synthesis during embryonic development. The presentation of a patient, and specifically a neonate, with abnormal genital development represents a difficult diagnostic and therapeutic challenge. Referral to a centre with experience in the diagnosis and management of disorders of sexual development is advised where the emphasis should be on psychological and genetic counselling.

It is unknown whether changes in bladder function due to urethral obstruction follow a specific sequence. To answer this, we adapted a small animal model to allow repeated complete pressure-flow studies, enabling individual follow-up of changes in bladder function on urethral obstruction. Obstruction was induced in guinea pigs by placing a silver ring around the urethra. Urodynamic studies were repeated under anesthesia with ketamine/xylazine. Bladders were filled and bladder pressure measured through a single suprapubic catheter. Urine flow rate was measured using an ultrasound probe around the penis. Accurate measurements of bladder pressure and urine flow rates were obtained at 1-week intervals for 11 weeks in individual guinea pigs. In the control animals, the urodynamic parameters did not show significant changes. In the obstructed group, urethral resistance (P(low,ave)) increased from 20 to 35 cm H(2)O after 4 weeks and remained at that level. The maximum flow rate (Q(max)) increased from 0.17 to 0.24 mL/s after 2 to 3 weeks. After this peak, it gradually decreased to lower than the starting value after 10 to 11 weeks. The pressure at maximum flow rate (p(Qmax)) increased from 24 to 47 cm H(2)O after 6 to 7 weeks and thereafter declined. During weeks 1 through 4 of obstruction, unstable contractions were seen. All animals followed a similar sequence of patterns but at variable rates. Our animal model allows complete urodynamic follow-up of individual animals with urethral obstruction. We observed a specific sequence of changes in urodynamic patterns and parameters of bladder function., (Copyright 2001 Wiley-Liss, Inc.)

Prenatal ultrasound studies have significantly contributed to the understanding of congenital abnormalities of the urinary tract and the development of pediatric urology over the past decade. Besides providing more insight into pathophysiology of the developing urinary tract, it has promoted the possibilities of early postnatal intervention and of studying the natural history of developmental abnormalities such as pelviureteral junction obstruction and vesico-ureteric reflux. The downside of this development has been and probably still is a considerable amount of diagnostic and therapeutic interventions for so-called abnormalities which, left alone, will disappear with time and without significant sequelae. On the other hand, many children with severe obstructive uropathy are alive today because of early intervention. Longterm follow-up studies are needed to prove the efficacy of prenatal screening and to ascertain the contribution of these technical possibilities to quality of life.

Purpose: Detrusor instability and hyperreflexia are characterized by involuntary detrusor contractions in the filling phase of the voiding cycle. The diagnosis is made when urodynamic evaluation reveals such contractions. To compare patients and evaluate treatment a method is needed to quantify the degree of instability. We developed an instability parameter based on the area under the curve of involuntary detrusor contractions on conventional filling cystometry., Materials and Methods: We developed an automatic method to calculate the area under the curve of involuntary detrusor contractions in conventional filling cystometry. Logistic regression was used to construct decision rules to differentiate stable from unstable bladders. These rules, derived from a group of 100 children, were applied to a second group of 77 who were independently assessed by 3 urodynamics experts., Results: Typically 88% of the second group were correctly classified as stable or unstable by the automatic procedure. In the unstable subgroup there was poor correlation between the calculated instability parameter and the instability score assigned by the experts. Most likely this difference occurred because the experts based their opinion mainly on the amplitude of the highest unstable contraction and the percentage of filling time that instability was found., Conclusions: The proposed method of automatically grading detrusor instability based on the area under detrusor contractions differs from the intuitive method used by experts. Since no standard is available, it cannot be concluded which method is better. Our proposed method is objective and it results in a single physical value.

Distribution and regulation of androgen receptor expression during fetal and neonatal virilization of the rat fetus was assessed by immunohistochemistry. In mesonephric duct derivatives the androgen receptor expression became evident first in the efferent ductules and epididymis (on fetal day 14), subsequently in the vas deferens and finally in the seminal vesicle. Mesenchymal cells of the urogenital tubercle were positive for androgen receptors from fetal day 14 onwards. In the mesenchymal cells of the prostate anlagen, androgen receptor positive cells were found first on fetal day 16. Administration of 5alpha-dihydrotestosterone to pregnant rats from day 11 to day 20 of gestation caused a stabilization of the wolffian duct in female fetuses. The androgen receptor expression pattern became similar as found in mail fetuses, and showed an increase in density and in frequency of androgen receptor positive cells. Administration of the androgen antagonist flutamide during the same interval caused a reduction in density and frequency of androgen receptor positive cells in male fetuses. These findings indicate that androgens enhance the expression of androgen receptors in the developing rat genital tract by induction of androgen receptor positive cells, and by increasing the frequency. The developmental pattern of androgen receptor expression in the rat mesonephric duct system reflects the androgen-responsiveness of the ducts, and is consistent with induction of the androgen receptor along the ducts by testosterone reaching these structures in an exocrine fashion.

Objective: To elucidate whether diminished levels of androgen receptor (AR) are the underlying cause for the development of hypospadias by determining AR levels in the foreskins of boys with hypospadias., Patients and Methods: The content of AR was determined by a sensitive immunoblotting technique in extracts from preputial tissue obtained from 15 patients (mean age 2.5, SD +/- 1.5 years) with hypospadias and from seven controls (mean age 2.5, SD +/- 1.5 years). Immunoreactivity of the protein was measured by densitometry., Results: No significant difference in mean AR content was found between those boys with hypospadias (2.1 +/- 0.9 fmol/mg protein, +/- SD) and the age-matched control group (2.2 +/- 0.3 fmol/mg protein). Stratification by the severity of hypospadias (distal and proximal on the shaft, and penoscrotal hypospadias) showed that the severity was not related to tissue AR levels., Conclusion: It is unlikely that hypospadias is caused by a decreased expression of AR.

From January 1980 to January 1992, urodynamic examinations were performed in 339 enuretic children without constipation or anatomical or neurogenic causes of incontinence. Nocturnal enuresis was seen in 178 patients, 130 children suffered from nocturnal and diurnal enuresis, and 31 had only diurnal enuresis. M. detrusor instability was found in 44% of the children and 73% showed more than 15% reduction in functional bladder capacity. In 5% an infravesical obstruction was suspected on pressure-flowmetry and in 12% dysfunctional voiding was seen. Routine urodynamic studies are not indicated in children with enuresis.

Prenatal diagnosis, management and outcome were studied retrospectively in 24 cases of unilateral and bilateral uretero-pelvic junction obstruction (UPJO). Moderate-to-marked pelvic dilatation was documented in 19 out of 24 cases. The low morbidity and mortality observed in this study are probably determined by the late (third trimester) detection of UPJO, resulting in an underestimation of the prevalence of more severe renal pathology. Amniotic fluid was increased in 4 out of 14 cases of unilateral UPJO and in 5 out of 10 cases of bilateral UPJO, the underlying mechanism of which is still unclear. There was no oligohydramnios. The incidence of extrarenal structural pathology was low, i.e., 3 out of 24 cases. A close agreement could be demonstrated between pre- and postnatal sonographic grading of pelvic dilatation. However, postnatal ultrasonic grading of pelvic dilatation correlated poorly with the degree of functional obstruction as determined by IVP and lasix renography. Despite the severity of pelvic dilatation in the majority of cases, enlarged kidneys were revealed by postnatal clinical examination in only three instances, underlining the importance of prenatal sonographic detection of UPJO.

Urethral advancement is a less well known technique for the repair of distal hypospadias. We treated 37 patients with a median age of 3 years for coronal and subcoronal hypospadias, with excellent results.

Between March 1985 and January 1987 2547 ESWL treatments were performed on 2064 patients, including 33 boys and 28 girls younger than 18 years. Almost identical results were obtained in children as in adults. All stones desintegrated and success was achieved in 97%; 79% was considered stone-free after 6 months and 18% had passable asymptomatic residual fragments (biggest fragments less than 3 mm) which did not grow larger during a mean follow-up of 17.8 months. One patient had a recurrent stone, after having been stone-free for 13 months. Post-ESWL complications and morbidity were minimal. Auxiliary measures were necessary in 5 patients before ESWL and in 6 patients after ESWL. Failure to render the kidney free of stones occurred in 2 patients with complex urolithiasis. Because of these results ESWL should be considered the treatment of choice in the paediatric age group for urolithiasis.