[Trampolining-induced gross hematuria : Unmasking the nutcracker syndrome]. / Trampolinspringen-induzierte Makrohämaturie : Das demaskierte Nussknackersyndrom.

The case of a 12-year-old boy with sports-induced recurrent macrohematuria and left-sided flank pain is reported. After extensive laboratory and imaging diagnostics, the diagnosis of nutcracker syndrome is made based on the characteristic clinical manifestation. Under a conservative approach and abstention from the triggering sport, a clinical as well as image-morphologically confirmed maturation occurred.

[Rare diseases with clinical relevance-the Silver-Russell syndrome]. / Seltene Erkrankungen mit klinischer Relevanz ­ das Silver-Russell-Syndrom.

The Silver-Russell syndrome (SRS) is a rare imprinting disease associated with pre- and postnatal growth retardation, craniofacial features, and asymmetry. Genitourinary abnormalities are seen in up to 20% of affected individuals. Apart from structural renal anomalies, cryptorchidism and hypospadias occur frequently in boys, while girls often have anomalies similar to those in Mayer-Rokitansky-Küster-Hauser syndrome with congenital hypoplasia or aplasia of the uterus and upper part of the vagina. Frequently hypospadias repair and orchiopexy are difficult because of lack of buccal mucosa due to facial dysmorphism and intraabdominal position of the testicles, respectively. Anesthetic problems with SRS children can be profound and mostly concern a difficult airway due to facial dysmorphism. Especially the young, very thin SRS patients are prone to hypoglycemia and hypothermia and require close perioperative monitoring. Children with SRS and their families face challenges from birth to adulthood. In case of urogenital abnormalities, they should receive multidisciplinary care by pediatric urologists/surgeons and pediatric anesthesiologists in a center of expertise in rare diseases.

Acquired cryptorchidism: More harm than thought?

INTRODUCTION: Acquired cryptorchidism (AC) has been recognized as a subgroup of undescended testes (UDT). There is growing evidence that the compromising effect equals that of congenital UDT (cUDT). This prospective study included an extensive histological examination of biopsies taken from AC patients. PATIENTS AND METHODS: From August 2013 to December 2014, 21 boys (3-12 years of age) underwent testicular biopsy during orchiopexy for AC. Patient and family histories were taken. The amount of germ cells (GC) per tubule (T) and the amount of adult dark spermatogonia (Ad-S) per T were determined by resin semi-thin sections examination. The samples were also scanned for signs of malformation. Immunohistochemical stains were performed as markers for atypical germ cells. RESULTS: Four (19%) boys were born prematurely, two (9.5%) were small for gestational age (SGA), and nine (43%) had a positive family history of UDT. The median of GC/T was 1.06 in boys <9 years, and 0.60 in boys ≥9 years. The median of Ad-S/T was 0.02 in boys <9 years and 0.01 in boys ≥9 years. There were no signs for malformation and no atypical cells. The immunohistochemical stains were negative in all specimens. CONCLUSIONS: Prematurity, SGA, and a positive family history appeared to be predictors for AC. Extensive histopathological examination of AC revealed a significant reduction of germ cell count and fertility markers, comparable with that in cUDT. The alterations were more severe in boys aged ≥9 years. It is unclear as to whether or not this was possibly caused by a longer duration of inguinal position, but this finding suggests that routine checks of testicular position throughout childhood are needed, and that there is a cause for continued efforts in educating parents and primary care physicians regarding AC. Current data support the notion of surgical correction once the diagnosis is made.

[Scrotal ultrasound examinations in infants and toddlers]. / Sonographie des Skrotalinhalts bei Säuglingen und Kleinkindern.

BACKGROUND: Besides physical examination, ultrasonography is the most valuable diagnostic tool to assess the scrotum and testes in the case of an acute scrotum or scrotal pathology. PROBLEMS: In infants and toddlers the examination can be challenging. Due to the limited patient compliance, the small testicular size (< 0.5 ml), and low blood flow velocity (< 3 cm/s), it can be difficult to achieve a proper flow curve when assessing blood flow. CONCLUSION: The examiner's skills are as important as adequate equipment (i. e., linear ultrasound probe, 12-14 MHz) and optimal program settings (Doppler scale < 3 cm/s, gate 1 mm). However, if there is doubt, surgical exploration is unavoidable.

[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.

[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.

[Late diagnosis of cryptorchidism]. / Der spät diagnostizierte Hodenhochstand

Cryptorchidism is a risk factor for testicular cancer and reduced fertility. Failure of critical maturation steps, with the persistence of gonocytes beyond 6 months, results in a decreased number of adult dark (A-dark) spermatogonia, which causes a deficiency of primary spermatocytes at 3 years of age. We evaluated the histological findings in boys older than 4 years who were diagnosed with a testis located in the inguinal channel. From September 2007 to September 2008, 15 boys (mean age 8 years) underwent orchidopexy with a biopsy taken from the undescended testis of one side. Sonograms were performed in all 15 boys. The incidences of spermatogonia, primary spermatocytes, and Leydig cells (semiquantitative) were estimated as well as the exclusion of intratubular carcinoma in situ cells (Department of Pathology, Central EM Lab, University Medical Center Regensburg, Germany). Orchidopexy was performed on the right side in eight boys, the left side in one, and both sides in six. Sonographically, no parenchymal echotexture abnormalities were found. A-dark spermatogonia could be detected in nine biopsies. The Leydig cell score was reduced in 11 boys. No carcinoma in situ cells were detected. Late diagnosis of undescended testis will have a poor prognosis for future fertility.