[Pilot study on drinking and voiding management in child day-care facilities in the district of Garmisch-Partenkirchen]. / Pilotstudie zum Trink- und Entleerungsmanagement in Kindertagesbetreuungseinrichtungen im Landkreis Garmisch-Partenkirchen.

BACKGROUND AND OBJECTIVES: The development of child day care is currently the focus of a public discussion. More and more very young children are cared for in a day-care facility rather than by the family. Therefore, key aspects of education are shifted into these institutions. The aim of this survey was to inquire about the current situation of drinking and voiding management in day-care facilities in the district of Garmisch-Partenkirchen. MATERIALS AND METHODS: In all, 322 questionnaires were sent to employees of 40 child day-care facilities. Organization of drinking and voiding behavior advices and personal assessments of continence education were queried. The evaluation was carried out anonymously. RESULTS: A total of 29 facilities (73%) replied. The average fluid intake was 260 ml (50-750 ml) at 12 o'clock in all day-care facilities included. The possibility to decide when to have breakfast and when to drink according to the individual thirst is practised in 43% (62/143) of the facilities. A fixed amount of fluid intake was offered by 11% (16/150) of the institutions. Of those responding, 92% (139/151) specifically send the children to the toilet, while 85% of the participants consider a continence education concept would be useful. CONCLUSION: According to a general trend, children attend day-care facilities earlier and spend more time there. This means that educational staff are taking care of children who are not yet continent. A verifiable continence educational concept does not exist in any of the institutions surveyed; however, most participants would favor such a concept. It would be desirable to establish an interprofessional health education program in which drinking and voiding training is integrated according to national recommendations.

[The German research network CURE-Net : A benefit for patients with rare diseases]. / Das deutsche Forschungsnetzwerk CURE-Net : Ein Gewinn für Patienten mit seltenen Erkrankungen.

With the help of the media, there is growing public awareness for the problems associated with rare diseases and their impact on the lives of those affected and their families. Bladder exstrophy-epispadias complex (BEEC) is also a part of the group of rare diseases within the urological field. The German network CURE-Net was founded in 2009 to systematically collect data regarding the epidemiological and molecular causes, and clinical and psychosocial effects of congenital urorectal malformations. With the help of self-help groups a national registry could be established for systematic data retrieval. This research can help to improve existing medical care and follow-up for affected individuals with BEEC.

[Epispadias and urethral duplication]. / Epispadie und Harnröhrenduplikatur.

Epispadias is the mildest form of the exstrophy-epispadias complex and rarely occurs in an isolated form. The characteristic clinical sign is a mucosal strip on the penile dorsum. Clinically, epispadias presents with urinary incontinence. Primary surgical correction is recommended at the age of 12 months and should only be performed at a specialized center. During follow-up, further bladder neck surgery may be necessary. Despite a functional and cosmetically appealing penis, psychosocial and psychosexual development of the affected individuals can severely be affected. The incidence of urethral duplication remains unclear. Several classifications reflect the anatomical variation. The clinical presentation depends on the continuity of the duplicated urethra and its relationship to the sphincteric mechanism. In addition to clinical examination, a voiding cysturethrography is part of the basic diagnostic workup. After endoscopic examination an individual operative approach can be defined.

[Urinary bladder function disorders in childhood]. / Harnblasenfunktionsstörungen im Kindesalter.

Bladder dysfunction in children is a very common and heterogeneous problem. There can be disturbances either in bladder storage or during voiding phases. First of all it is important to distinguish between non-neurogenic and neuropathic bladder dysfuction. In this article the most common causes, the basic diagnostic evaluation and therapeutic interventions are summarized.

[Transition of adolescents with the exstrophy-epispadias complex to adult medicine: influence of long-term outcome results on management]. / Transition von Jugendlichen mit Blasenekstrophie-Epispadie Komplex in die Erwachsenenmedizin: Implikationen des Langzeitverlaufes für das Management.

Today, young individuals with rare congenital anomalies as the Exstrophy-Epispadias-Complex (EEC) are mostly monitored interdisciplinary with a high standard of care and enthusiasm during childhood. However, when growing up through adolescence to adulthood adequate care-givers are not available at the moment in adult medicine in Germany and a concrete transition process has yet not been established. Over the past years, we put much effort in systematic evaluation of long-term outcome after reconstruction of the EEC in the newborn period to further improve outcome results. Beside predictive parameters for continence and long-term bladder function, genital function and fertility, as well as postoperative pelvic floor morphology and gynecological outcome, orthopedic results and psychosexual and psychosocial development in EEC were of major interest. As a consequence we currently develop a German-wide follow-up concept in EEC patients regarding age- and gender specific outcome issues. Long-term observations of the EEC outcome however, underline the unrestricted importance of careful long-term follow-up of all EEC patients, as well as the necessity of close cooperation of pediatric urologist, pediatric surgeons, urologists, orthopedic surgeons, gynecologists, andrologists, psychologists and urotherapists from early childhood and the need of knowledge transfer and hopefully a successful transition of the EEC individuals to general medicine.

[Tumour registry and reality: differential diagnosis of testicular tumours in childhood: survey and personal experience]. / Tumorregister und Realität - Differenzialdiagnose von Hodentumoren im Kindesalter - Übersicht mit eigenem Patientengut.

Painless scrotal swelling must always be considered as a potential malignant testicular tumour until proven otherwise. Prepubertal testicular tumours are seldom entities, for their differential diagnosis testicular sonography is gaining increasing importance. Although, according to national and international tumour registries, most patients tend to have malignant tumours, some clinical series from paediatric centres confirm that prepubertal testicular tumours are mainly benign lesions, especially up to the age of 12 years. Testis-sparing procedures are favoured when AFP is in a normal range and testicular parenchyma is detected sonographically. Oncological principles should be applied to paediatric patients consequently, staging examinations should be requested when a definite histology is proven. Cases should be reported to national tumour registries and, in addition to close surveillance, in some cases adjuvant therapy is necessary. In addition to the clinical guidelines from the tumour registries, differential diagnosis, testis-sparing surgery and the presence of TIN in the paediatric population should be discussed.

[Analysis of laparoscopic orchidopexy in intra-abdominal testis]. / Ergebnisse der laparoskopischen Orchidopexie beim nicht palpablen Hoden.

Laparoscopy is an established diagnostic procedure but is less frequently used as a therapeutic method for impalpable testes. To compare the results of the classic two-stage orchidopexy described by Fowler-Stephens with a testicular vessel-sparing single-stage approach in the management of intra-abdominal testes we retrospectively analyzed our data.From January 2005 to September 2010, 105 patients (mean age 32 months) underwent laparoscopy for impalpable testes. In cases of intra-abdominal testes, laparoscopic orchidopexy was performed either in a two-stage procedure including initial ligation of the testicular vessels or as a direct single-stage procedure without ligation of the vessels. The results of both approaches were evaluated postoperatively clinically and by ultrasonography. Among the 122 impalpable testes 63 were located intra-abdominally; single-stage orchidopexy was performed in 29 testes whereas a two-stage approach was conducted in 14 testes. Fourteen gonads had to be removed due to atrophy and in six cases no testis was found at all. In the other 59 cases inguinal exploration followed resulting in 22 orchidopexies, 34 removals and 3 blind-ending vessels. During a mean follow-up of 17 months none of the 29 testes treated by single-stage orchidopexy atrophied or reascended. By contrast, two cases of atrophy, one reascent and one subumbilical wound infection occurred after the two-stage procedure. Considering our excellent experiences with single-stage management, we conclude that the single-stage approach is a reliable, safe and efficacious treatment modality of intra-abdominal testes. However, the two-stage procedure including testicular vessel ligation should be restricted to high abdominal testes with very short vessels.

[Perioperative pain management in major reconstructive surgery in pediatric urology: a plea for continuous epidural anesthesia]. / Perioperatives Schmerzmanagement bei großen plastisch-rekonstruktiven Eingriffen in der Kinderurologie: Ein Plädoyer für die Periduralanästhesie.

Regional analgesia is firmly established in modern pediatric anesthetic practice and its popularity continues to grow. In our department continuous epidural anesthesia (CEA) is a frequently used technique of pain management following major reconstructive procedures of the lower urinary tract. The aim of this study was to investigate the efficacy, safety, and potential benefits of CEA over standard analgesics.We retrospectively reviewed the records of 21 infants who underwent single-stage bladder exstrophy repair in our department. In 15 children an epidural catheter was placed preoperatively for CEA; 6 patients treated without CEA served as controls. Total doses of narcotics and analgesics, length of intensive care unit (ICU) stay and ventilatory assistance, time to first bowel activity, anticholinergic requirements, and CEA-related side effects were documented and compared for both groups.Children given epidural anesthesia required six- to tenfold lower doses of morphine intra- and postoperatively compared to those without CEA; ventilatory support upon completion of surgery was remarkably shorter (59 versus 210 min) in the CEA group as well as ICU stay (1.1 versus 1.8 days). The total consumption of anticholinergics was twice as high as in patients without CEA. There were no relevant CEA-related complications.Being a retrospective audit of practice in our institution with a small number of patients, our results are in line with previously published data on CEA in pediatric patients. CEA has been shown to significantly reduce the need for anesthetics and morphine and allows early extubation with all subsequent advantages for a speedy recovery post surgery. Thus, the technique is to be recommended as a safe and efficacious method for pain management following major reconstructive surgery in pediatric urology. Importantly, this type of anesthesia should be performed only by experienced anesthesiologists in institutions where appropriate equipment, staff, and monitoring are available.

[Pathological findings in the scrotum and testis and initial diagnostic management]. / Pathologische Befunde des Skrotums und des Hodens und deren initialdiagnostisches Management.

This is a summary of typical clinical pathology of the scrotum and testis in childhood. Starting with the most common clinical pictures we describe their obvious appearance as the red, the blue, the painless distended, the empty and maldeveloped scrotum. Most important for differential diagnosis seem to be the history and clinical examination, whereas colour duplex sonography can help you with decision-making and interpretation of initial and follow-up findings. Associated anomalies should be screened and the parents should be informed about the relevance and required procedure.

Biometry of the pubovisceral muscle and levator hiatus assessed by three-dimensional ultrasound in females with bladder exstrophy-epispadias complex after functional reconstruction.

OBJECTIVE: The congenital bony and musculoskeletal defect of the pelvis in bladder exstrophy-epispadias complex (BEEC) highly predisposes females to uterine prolapse. There is a paucity of knowledge on the anatomy of the pelvic soft tissue structures following surgery. The aim of this study was to investigate with transperineal three-dimensional (3D) ultrasound the pubovisceral muscle in females with BEEC who had undergone surgical reconstruction in childhood. METHODS: In a cross-sectional observational study we examined 12 Caucasian female BEEC patients, mean age 19.9 (range, 15.5-27.4) years, from a single center after a single-stage functional reconstruction with closure of the anterior pelvic ring. As a control group we used 13 Caucasian nulligravidae. 3D transperineal ultrasound volumes were acquired with the patient at rest in the supine position and with an empty bladder, and established pelvic floor parameters were measured. Analysis was conducted offline by two independent investigators. RESULTS: No statistical difference between the BEEC patients and the control group was observed in the anteroposterior diameter or the area of the levator hiatus, or in the maximal thickness of the levator muscle. However, significantly greater values were observed in BEEC patients in the transverse diameter of the levator hiatus (mean, 4.31 vs. 3.81 cm, P = 0.046) and in the levator angle (mean, 80.1 vs. 70.0 degrees, P = 0.040). The measurements obtained in the control group were consistent with those previously reported in the literature. CONCLUSIONS: This is the first study showing that transperineal 3D ultrasound can be used for the assessment of BEEC patients after functional reconstruction. Biometric pelvic floor parameters may be useful in the long-term follow-up of BEEC patients.

[Combined flexible and semirigid ureteroscopy with laser lithotripsy. Alternative to percutaneous nephrolithotomy of complex EWSL refractory nephrolithiasis]. / Kombinierte flexible und semirigide Ureterorenoskopie mit Laserlithotripsie. Alternative zur perkutanen Litholapaxie komplexer ESWL-refraktärer Nephrolithiasis.

BACKGROUND: Flexible ureteroscopic laser lithotripsy of intrarenal calculi is technically demanding and challenging due to potential dislocation from the calyx and damage to the tissue or the fiber optic during in situ laser lithotripsy. MATERIAL AND METHODS: In 12 consecutive patients ESWL resistant renal stones were collected with the flexible ureteroscope using the nitinol basket and repositioned into the renal pelvis. After removing the flexible ureteroscope from the ureter the stones were treated through an additionally inserted semirigid ureteroscope with frequency-doubled double-pulse Neodym:YAG (FREDDY) laser under direct vision. RESULTS: Operating time was on average 110 min. During the complication-free 3 month follow-up, 8 patients had complete success, 3 partial success, 1 patient had residual fragments>3 mm and 88% of lower pole calyces were stone-free. CONCLUSIONS: As an alternative to percutaneous nephrolithotomy, repositioning of renal stones to the renal pelvis with the flexible instrument allows in selected cases safe laser fragmentation through a semirigid instrument. Access or exposition-related problems can be solved and repair costs of the instruments will be minimized.

Epidemiological survey of 214 families with bladder exstrophy-epispadias complex.

PURPOSE: We sought to identify causative nongenetic and genetic risk factors for the bladder exstrophy-epispadias complex. MATERIALS AND METHODS: A total of 237 families with the bladder exstrophy-epispadias complex were invited to participate in the study, and information was obtained from 214 families, mainly from European countries. RESULTS: Two families showed familial occurrence. Male predominance was found among all subgroups comprising epispadias, classic bladder exstrophy and cloacal exstrophy, with male-to-female ratios of 1.4:1, 2.8:1 and 2.0:1, respectively (p = 0.001). No association with parental age, maternal reproductive history or periconceptional maternal exposure to alcohol, drugs, chemical noxae, radiation or infections was found. However, periconceptional maternal exposure to smoking was significantly more common in patients with cloacal exstrophy than in the combined group of patients with epispadias/classic bladder exstrophy (p = 0.009). Only 16.8% of mothers followed the current recommendations of periconceptional folic acid supplementation, and 17.6% had started supplementation before 10 weeks of gestation. Interestingly, in the latter group mothers of patients with cloacal exstrophy were more compliant with folic acid supplementation than were mothers of the combined group of patients with epispadias/classic bladder exstrophy (p = 0.037). Furthermore, mothers of children with cloacal exstrophy knew significantly more often prenatally that their child would have a congenital malformation than did mothers of children with epispadias/classic bladder exstrophy (p <0.0001). CONCLUSIONS: Our study corroborates the hypothesis that epispadias, classic bladder exstrophy and cloacal exstrophy are causally related, representing a spectrum of the same developmental defect, with a small risk of recurrence within families. Embryonic exposure to maternal smoking appears to enforce the severity, whereas periconceptional folic acid supplementation does not seem to alleviate it. There is a disproportional prenatal ultrasound detection rate between severe and mild phenotypes, possibly due to the neglect of imaging of full bladders with a focus on neural tube defects.

[Urological manifestations of Down syndrome: significance and long-term complications -- our own patient cohort with an overview]. / Urologische Manifestationen des Down-Syndroms : Bedeutung und Langzeitkomplikationen -- eigenes Patientengut mit Ubersicht.

BACKGROUND: In the literature urogenital anomalies are associated with Down syndrome. Anomalies of the external genitals, posterior urethral valves, and renal dys- or hypoplasia are reported. However, the incidence, etiology, and clinical course of neurogenic and functional bladder dysfunction in Down syndrome are unclear. MATERIAL AND METHODS: We report on 23 boys and 1 girl with Down syndrome with a mean age of 79.4 months. RESULTS: Thirteen boys had uni- or bilateral cryptorchidism. Two had posterior urethral valves, two bladder exstrophy, and two hypospadias. Three children had neurogenic and eight non-neurogenic functional bladder dysfunction. Due to loss of renal function nephrectomy was necessary in three patients. CONCLUSIONS: Children with Down syndrome seem to often have associated neurogenic or functional bladder dysfunction, which can lead to upper urinary tract deterioration. Therefore, early and regular sonographic and urodynamic evaluation of the bladder function is mandatory.

[Topical therapy of balanitis xerotica obliterans in childhood. Long-term clinical results and an overview]. / Die topische Therapie der Balanitis xerotica obliterans im Kindesalter. Klinische Langzeitergebnisse und Ubersicht.

Balanitis xerotica obliterans (BXO) is a chronic and progressive dermatitis of unknown aetiology and incidence. Its management in childhood is controversial. Although in most cases only the prepuce is affected, meatal and urethral involvement may lead to major surgical reconstruction. Therefore complete surgical excision of the affected skin is considered to be mandatory. In case of involvement, incidental histological evidence or a relapse, or when complete removal of the affected skin is not possible, a topical therapy should be implemented. In a retrospective analysis of our study population (13 children) with BXO, relapse rate was lower after topical therapy with tacrolimus (Protopic), a highly selective immune modulator, than after the standard anti-inflammatory therapy with betamethasone. The use of tacrolimus ointment is a safe therapy with no severe side effects. Due to the fact that there are no predictive factors for progression or relapse of BXO, we consider a topical anti-inflammatory therapy is always indicated after any type of surgery for BXO. Follow-up monitoring should be close, so that any relapse can be detected and treated as early as possible.

[Development of treatment for extrophy-epispadias in Germany]. / Geschichte der Blasenekstrophiebehandlung in Deutschland.

While repair of exstrophy has a long tradition in Germany, a comprehensive review of the medical history of exstrophy-epispadias repair and its frequent inconsistencies and setbacks in Germany has not previously been published. Numerous resources were used to collect the information described in this review. Older textbooks from the library of German Society of Urology (DGU) in Düsseldorf and from the School of Medicine Library at the Friedrich-Alexander University in Erlangen were used to collect information on the important steps in the treatment of bladder exstrophy and epispadias from the first report of it onward. A Medline search was done to collect appropriate information from after 1969. Schenck von Grafenberg was the first to describe exstrophy of the bladder, in his report published in 1597, while the term 'exstrophy' was first used by Chaussier in 1780. Isolated epispadias remained unknown and untreated until it was described by Morgagni in 1761. The initial attempts to treat this anomaly were restricted to controlling the incontinence. In 1869 Karl Thiersch described the etiology and anatomy of epispadias and reported on a case of epispadias reconstruction with a long-term follow-up of 11 years. As long ago as in 1897, Mickulicz enlarged a small exstrophic bladder by adding an ilial segment to make it possible to close the bladder. After the first really successful reconstruction of bladder exstrophy in 1942 by Young in the USA, various multiple-stage and single-stage reconstruction approaches emerged in Germany. From the start, urinary diversion was seen as an alternative to reconstruction in the treatment of bladder exstrophy. The first ureterosigmoidostomy was done in an exstrophy patient by Simon in Heidelberg in 1852, after which Maydl described a new technique based on transtrigonal sigmoidostomy for urinary diversion in 1894. There are now three approaches in current use in Germany for the management of bladder exstrophy-epispadias: reconstruction in various staged approaches, complete reconstruction in a single session, and the primary rectal reservoir (Mainz pouch II).