[Renal cell carcinomas in childhood]. / Nierenzellkarzinome im Kindesalter.

BACKGROUND: Renal cell carcinoma is a rare childhood disease. However, it should be considered in the differential diagnosis in the detection of a renal mass. OBJECTIVES: Incidence and mortality of renal cell carcinomas in childhood. Presentation of clinical symptoms, diagnostics, histology, therapy and course of the disease. PATIENTS AND METHODS: Evaluation of our own prospective data of a small patient population of 8 patients, as well as discussion of the current literature on this tumor entity. RESULTS: The average follow-up period was 27 months. Preoperative staging studies showed no metastases. The histologically primary predominant subtype was papillary renal cell carcinoma. Tumor stage and lymph node status were the most important prognostic factors in our study. CONCLUSIONS: Renal cell carcinoma in childhood is a rare disease whose treatment is challenging and should be performed in an interdisciplinary team. Typical clinical symptoms, as in adults, are absent. The partial nephrectomy should be, if practicable, the surgical treatment of choice. The most important risk factors for survival are tumor stage and lymph node status. Data on adjuvant target therapy for metastatic disease in childhood are lacking. Further prospective, multicenter studies are necessary to generate more information on the biology and course of this disease and to obtain adjuvant treatment options in locally advanced disease.

[Early bilateral nephrectomy in neonatal autosomal recessive polycystic kidney disease : Improved prognosis or unnecessary effort?] / Frühe beidseitige Nephrektomie bei Säuglingen mit pränataler ARPKD : Prognoseverbesserung oder unnötiger Aufwand?

BACKGROUND: Neonatal autosomal recessive polycystic kidney disease (ARPKD) is associated with giant kidneys, lung hypoplasia, pulmonal hypertension, and end-stage renal failure. Depending on the study, mortality is reported to range between 20 and 80%. OBJECTIVES: Does bilateral nephrectomy improve survival? PATIENTS AND METHODS: Between 2010 and 2016, we treated 7 children with prenatally diagnosed ARPKD. All had a planned delivery by cesarean section. After birth, oscillated ventilation with nitrogen enrichment was initiated to achieve maximum oxygenation and to decrease pumonary hypertension. All children had bilateral massive kidney hyperplasia (length 13-16 cm). RESULTS: Nephrectomy on one side was performed within 72 h together with placement of a peritoneal dialysis catheter in the intensive care unit. Contralateral nephrectomy was performed after 1-2 weeks when the child was stabilized by dialysis. In 2 children, kidney transplantation has already been performed and they are doing fine. One child died after 10 months due to infection. The other children are stable on home peritoneal dialysis awaiting transplantation. CONCLUSIONS: Early bilateral nephrectomy in neonatal ARPKD is feasible, but requires distinctive care at a pediatric intensive care unit and a high amount of organizational efforts to treat these children adequately in the first few days. In our experience, the procedure is a promising approach to improve ventilation and enable dialysis. However, kidney transplantation, best from a living donor, is required within the first years of life.

[Acute Kidney Failure Due to Urachal Cyst?] / Akutes Nierenversagen bei Urachuszyste?

A 34-year-old para V woman was referred to our centre at 35+1 weeks of gestation for an assumed fetal malformation with prenatal renal impairment and anhydramnios. Prenatal ultrasound demonstrated unilateral renal agenesis; the bladder was not detectable. The baby was born by caesarian section at 36+2 weeks of gestation because of placental insufficiency. Postnatal adaptation was uneventful, but the newborn presented external stigmas of trisomy 21 and progressive renal impairment with anuria. Nevertheless, the postnatal ultrasound showed two enlarged kidneys in loco typico with impaired perfusion but without signs of malformations. In the lower abdomen, a rosette-shaped structure of unknown origin was noted. Its origin could not be cleared by imaging including voiding cystourethrography and colon contrast radiography. Explorative laparotomy identified the structure as a persistent urachal cyst with secondary obstruction of the upper urinary tract. After removal of the urachus with reconstruction of the bladder dome, renal function recovered completely while urine was drained continuously via suprapubic catheter. A voiding cystourethrogram 3 weeks later showed a posterior urethral valve as an additional unexpected diagnosis. The valve was slit at the age of 6 months without complications, the renal function remained stable in the further course. In retrospect, the main cause for the renal failure remains unclear. It appears to be the obstruction due to the space-consuming character of the urachal cyst, especially because the megacystis typically associated with urethral valve was not viewable. Alternatively, the additional proximal stenosis may have only masked the typical findings of PUV.

[Late consequences of urethral injuries. Reconstruction options]. / Spätfolgen von Urethraverletzungen. Rekonstruktive Möglichkeiten.

BACKGROUND: Stricture excision and posterior urethroplasty is the most common procedure after posttraumatic urethral strictures. RESULTS: Re-strictures and fistulas are treated by repeat urethroplasty. Tension-free anastomosis is prerequisite for surgical success. Urinary incontinence after posttraumatic injuries is treated by an artificial urinary sphincter.

The T-plasty: a modified YV-plasty for highly recurrent bladder neck contracture after transurethral surgery for benign hyperplasia of the prostate: clinical outcome and patient satisfaction.

OBJECTIVE: To describe a modified surgical technique for treatment of highly recurrent bladder neck contracture (BNC) after transurethral surgery for benign hyperplasia and to evaluate success rate and patient satisfaction of this novel technique. METHODS: Ten patients with highly recurrent BNC and multiple prior attempts of endoscopic treatment underwent the T-plasty. Perioperative complications were recorded and classified according to the Clavien classification. Patient reported functional outcomes were retrospectively analysed using a standardized questionnaire assessing recurrence of stenosis, incontinence, satisfaction and changes in quality of life (QoL). The questionnaires included validated IPSS and SF-8-health survey items. RESULTS: Mean age at the time of surgery was 69.2 years (range 61-79), and the mean follow-up was 26 months (range 3-46). No complications grade 3 or higher according to the Clavien classification occurred. Success rate was 100 %. No de novo stress incontinence occurred. Urinary stream was described as very strong to moderate by 80 % of the patients, mean post-operative IPSS-score was 11.3 (range 4-29), and mean post-operative IPSS-QoL was 2.4 (range 1-5). Patients satisfaction was very high or high in 90 %, and QoL improved in 90 %. The SF-8-health survey showed values comparable to the reference population. CONCLUSION: The T-plasty represents a safe and valuable option in treating highly recurrent BNC after surgery for benign hyperplasia. It offers multiple advantages compared to other techniques such as a single-staged approach and the opportunity for reconstruction of a reliable wide bladder neck by usage of two well-vascularized flaps. Success rate, low rate of complications and preservation of continence are highly encouraging.

Evidence-based (S3) Guideline on (anogenital) Lichen sclerosus.

Lichen sclerosus (LS) is an inflammatory skin disease that usually involves the anogenital area. All patients with symptoms or signs suspicious of lichen sclerosus should be seen at least once initially by a physician with a special interest in the disease in order to avoid delay in diagnosis, as early treatment may cure the disease in some and reduce or prevent scarring. The diagnosis is made clinically in most cases. Biopsies should only be performed under certain circumstances. The gold standard for treatment remains potent to very potent topical steroids; however, mild and moderate disease in boys and men may be cured by circumcision. Certain triggers should be avoided. http://www.euroderm.org/images/stories/guidelines/2014/S3-Guideline-on-Lichen-sclerosus.pdf http://www.awmf.org/fachgesellschaften/mitgliedsgesellschaften/visitenkarte/fg/deutsche-gesellschaft-fuer-gynaekologie-und-geburtshilfe-dggg.html.

[Complications of hypospadias repairs]. / Komplikationen bei Hypospadiekorrekturen.

Hypospadias is the most common congenital abnormality of the lower urinary tract affecting one of 300 male newborns. More than 300 different surgical hypospadias repair techniques have been described. Currently, tubularized incised plate and meatal advancement and glansplasty integrated repair are the preferred techniques for distal hypospadias, whereas two-staged procedures are most frequently used in proximal forms. Success rates are high in the hands of dedicated surgeons, although studies on long-term results are sparse. The most frequent complications of hypospadias repairs include urethrocutaneous fistulas, meatal stenosis, and urethral strictures. Urological follow-up into puberty is warranted, as well as further studies with standardized reporting of long-term results and complications.

Panurethral and complex urethral strictures. Reconstruction in several steps: current techniques and indications.

Patients with panurethral and complex urethral strictures after failed urethral reconstruction due to strictures and hypospadias repair is a rare but challenging condition. Contemporary surgical techniques include one and two staged urethroplasties using different graft substitutes (i.e., buccal mucosa) or full thickness skin grafts (i.e., from the inner thigh(, thereby providing satisfactory results with reducing the re-stricture rate in these patients. However, all current techniques do so at the expense of higher revision rates and thus requiring multiple procedures. Studies investigating the outcomes of reconstruction in panurethral and complex urethral strictures often have heterogeneous patient cohorts including children and adults, different underlying causes, and different techniques, thus allowing only limited interpretation of the published data. In the field of urethral reconstruction, where personal experience and expertise presents an accepted necessity, however, leading to rather small single center studies,only well-designed randomized clinical trials can truly answer the question of which technique will be advantageous in these patients.

[Two-stage urethral reconstruction with buccal mucosa for hypospadias cripples : Long-term results]. / Zweizeitige Harnröhrenrekonstruktion mit Mundschleimhaut bei Hypospadiekrüppeln : Langzeitergebnisse.

So-called hypospadias cripples are a challenging group of patients who present with multiple previous repair operations with unsatisfactory functional and cosmetic results. Attempts at renewed correction can be carried out with various techniques and different materials and staged repair with buccal mucosa according to Bracka has shown good results.

[The buried penis : Indications, a new technique and the results]. / Der vergrabene Penis : Indikationen, Vorstellung einer neuen Technik und Ergebnisse.

The buried penis describes a bunch etiologies and clinical presentations of which the congenital form is rare. In the past different techniques had been described, using inner prepuce after mobilization to cover the penile shaft. This can lead to persistent edema of the skin. We developed a new technique to reduce the rate of postoperative edema.