EuroGuiderm guideline on lichen sclerosus-introduction into lichen sclerosus.

INTRODUCTION: Lichen sclerosus (LS) is an inflammatory skin disease affecting all ages. LS typically involves the anogenital site where it causes itching and soreness. It may lead to sexual and urinary dysfunction in females and males; however, it may be asymptomatic. First signs of LS are redness and oedema, typically followed by whitening of the genital skin; sometimes fissuring, scarring, shrinkage and fusion of structures may follow in its course. LS is associated with an increased risk of genital cancer. LS has a huge impact on the quality of life of affected patients, and it is important to raise more awareness of this not uncommon disease in order to diagnose and treat it early. OBJECTIVES: The guideline intends to provide guidance on the diagnostic of LS, highlight important aspects in the care of LS patients (part 1), generate recommendations and treatment algorithms (part 2) on topical, interventional and surgical therapy, based on the latest evidence, provide guidance in the management of LS patients during pregnancy, provide guidance for the follow-up of patients with LS and inform about new developments and potential research aspects. MATERIALS AND METHODS: The guideline was developed in accordance with the EuroGuiDerm Methods Manual v1.3 https://www.edf.one/de/home/Guidelines/EDF-EuroGuiDerm.html. The wording of the recommendations was standardized (as suggested by the GRADE Working Group). The guideline development group is comprised of 34 experts from 16 countries, including 5 patient representatives. RESULTS: Ultrapotent or potent topical corticosteroids in females and males, adults and children remain gold standard of care for genital LS; co-treatment with emollients is recommended. If standard treatment fails in males, a surgical intervention is recommended, complete circumcision may cure LS in males. UV light treatment is recommended for extragenital LS; however, there is limited scientific evidence. Topical calcineurin inhibitors are second line treatment. Laser treatment, using various wave lengths, is under investigation, and it can currently not be recommended for the treatment of LS. Treatment with biologics is only reported in single cases. CONCLUSIONS: LS has to be diagnosed and treated as early as possible in order to minimize sequelae like scarring and cancer development. Topical potent and ultrapotent corticosteroids are the gold standard of care; genital LS is often a lifelong disease and needs to be treated long-term.

EuroGuiderm guideline on lichen sclerosus-Treatment of lichen sclerosus.

INTRODUCTION: Lichen sclerosus (LS) is an inflammatory skin disease affecting all ages. LS typically involves the anogenital site where it causes itching and soreness; it may lead to sexual and urinary dysfunction in females and males; however, it may be asymptomatic. First signs of LS are usually a whitening of the genital skin, sometimes preceded by redness and oedema; fissuring, scarring, shrinkage and fusion of structures may follow in its course. LS is associated with an increased risk of genital cancer. LS has a huge impact on the quality of life of affected patients, and it is important to raise more awareness of this not uncommon disease in order to diagnose and treat it early. OBJECTIVES: The guideline intends to provide guidance on the diagnostic of LS (part 1), highlight important aspects in the care of LS patients, generate recommendations and treatment algorithms (part 2) on topical, interventional and surgical therapy, based on the latest evidence, provide guidance in the management of LS patients during pregnancy, provide guidance for the follow-up of patients with LS and inform about new developments and potential research aspects. MATERIALS AND METHODS: The guideline was developed in accordance with the EuroGuiDerm Methods Manual v1.3 https://www.edf.one/de/home/Guidelines/EDF-EuroGuiDerm.html. The wording of the recommendations was standardized (as suggested by the GRADE Working Group). The guideline development group is comprised of 34 experts from 16 countries, including 5 patient representatives. RESULTS: Ultrapotent or potent topical corticosteroids in females and males, adults and children remain gold standard of care for genital LS; co-treatment with emollients is recommended. If standard treatment fails in males, a surgical intervention is recommended, complete circumcision may cure LS in males. UV light treatment is recommended for extragenital LS; however, there is limited scientific evidence. Topical calcineurin inhibitors are second line treatment. Laser treatment, using various wave lengths, is under investigation, and it can currently not be recommended for the treatment of LS. Treatment with biologics is only reported in single cases. CONCLUSIONS: LS has to be diagnosed and treated as early as possible in order to minimize sequelae like scarring and cancer development. Topical potent and ultrapotent corticosteroids are the gold standard of care; genital LS is often a lifelong disease and needs to be treated long-term.

[Lichen sclerosus in children]. / Lichen sclerosus im Kindesalter.

Lichen sclerosus (LS) is a chronic inflammatory disease of the skin. It mainly affects the anogenital area. More knowledge of the disease is needed to avoid delay in diagnosis as early treatment may cure the disease in some and reduce or prevent scarring. Initial treatment for girls and boys comprises the daily application of a potent cortisone ointment for 3 months. After remission the treatment should be continued long-term intermittently. If there is no complete remission in boys complete circumcision is recommended. LS usually cannot be healed but only well suppressed, and also after circumcision recurrences may occur years later. Therefore, patients have to be well informed and should be followed up long-term.

[Bladder exstrophy: Quality of primary care and long-term prognosis]. / Blasenekstrophie : Qualität der Primärversorgung und Langzeitprognose.

BACKGROUND: It is universally agreed that successful and gentle initial bladder closure is decisive for favorable long-term outcome. Due to a number of reasons, including a lack of comparable multicenter studies, there are numerous concepts for initial exstrophy closure. DISCUSSION: Therefore, we describe our concept of delayed, staged reconstruction without osteotomy in classical bladder exstrophy, while taking into considerion the available literature on long-term follow-up as well as on own clinical and research data. CONCLUSION: Most notably there are multiple medical but also psychological advantages of a delayed procedure. Primary closure without osteotomy is feasible and has no disadvantages in the long-term follow-up when compared to the invasive procedure of osteotomy. Due to high intravesical pressure, initial bladder neck surgery might have negative effects on bladder development and on the upper urinary tract.

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.

[Circumcision and orchiopexy: management of complications]. / Zirkumzision und Orchidopexie: Komplikationsmanagement.

Circumcision and orchidopexy are among the most frequently performed pediatric urological procedures. Although they may be classified as surgical interventions suitable for junior surgeons, either procedure can lead to serious consequences including the loss of the respective organ. In this paper, the general aspects of pediatric urological surgery (e.g., distinctive features of informing underage patients, the handling of children in the operating room, and relevant pediatric anesthesiological aspects) are described first. Then, the most important sources of error will be highlighted and potential complications and their management during all stages of the procedure will be illustrated. Apart from the so-called freehand circumcision, this paper also deals with potential complications when using the Plastibell® device. In regard to orchidopexy, laparoscopic procedure shall be discussed in addition to the standard procedure.

[Intrascrotal lipoblastoma in childhood: diagnostic strategies and therapy planning]. / Intraskrotales Lipoblastom im Kindesalter : Diagnostische Strategien und Therapieplanung.

Based on a case report of an intrascrotal lipoblastoma in childhood, we present the preoperative diagnostic algorithm and the main differential diagnoses in testicular and paratesticular tumors as well as their surgical management. An 8-week-old infant was admitted with a scrotal tumor known since birth. MRI showed a pinnate vessel supply, originating in the left internal iliac artery. The tumor was exposed operatively and could be completely removed. Intrascrotal lipoblastoma are rare. Like most testicular and paratesticular tumors in childhood lipoblastomas are benign. Today ultrasound and Doppler sonography are basic tools for diagnosis and surgical planning in testicular and scrotal tumors in childhood. In some cases MRI can provide additional important information for surgical planning. In contrast to adults testis sparing surgery is favoured in children.