Endocrine outcome and seminal parameters in young adult men born with hypospadias: A cross-sectional cohort study.

BACKGROUND: Hypospadias affects around 1/200 newborn males. Intrauterine testicular dysfunction may underlie a subset of cases. The long-term endocrine and reproductive outcomes in these men remain largely unknown. METHODS: Cross-sectional study in Ghent and Vienna University Hospitals to assess the endocrine and seminal parameters of young adult men (16-21 years) born with non-syndromic hypospadias (NSH) (n = 193) compared to healthy typical males (n = 50). Assessments included physical exam, semen analysis, hormone assays and exome-based gene panel analysis (474 genes). FINDINGS: All participants had experienced a spontaneous puberty, in spite of higher LH and INSL3 levels than typical males. Oligo- or azoospermia was observed in 32/172 (18·6%; 99%-CI: 12·2-27·4%) of NSH men; but in 5/16 (31·3%; 99%-CI: 11·1;62·4%) of complex NSH men and in 13/22 (59·1%; 99%-CI: 33·2-80·7%) of those born small for gestational age (SGA). No (likely) pathogenic coding variants were found in the investigated genes. Suboptimal statural growth affected 8/23 (34·8%; 99%-CI: 15·4-61·0%) of men born SGA with NSH. INTERPRETATION: Spermatogenesis is significantly compromised in NSH men, especially in those born SGA or those with complex NSH. Long-term andrological follow-up is recommended, including end-pubertal semen analysis. No clear monogenic causes could be demonstrated in our cohort even in proximal or complex NSH. Being born SGA with NSH is frequently associated with poor catch-up growth, requiring growth hormone therapy in some. FUNDING: Research grants from the European Society of Paediatric Endocrinology, the Belgian Society of Pediatrics, the Belgian Society of Pediatric Endocrinology and Diabetology and the Research Foundation Flanders (FWO).

Ultrasound-guided epidural anesthesia and sedation for open transvesical Cohen ureteric reimplantation surgery in 20 consecutive children: a prospective case series and proof-of-concept study.

BACKGROUND: Epidural anesthesia is usually combined with general anesthesia (GA) for children undergoing sub-umbilical surgery and GA in children is associated with a potential for respiratory events. Aiming to reduce airway manipulation and the use of GA drugs, we designed a study of transvesical Cohen ureteteric reimplantion under epidural anesthesia in sedated, spontaneously breathing children. METHODS: We enrolled 20 children (3-83 months, 6.3-25.0 kg) scheduled for open transvesical abdominal surgery with Pfannenstiel incision. Sedation was followed by ultrasound-guided epidural anesthesia. Increases in heart rate by >15% and or patient movements upon skin incision were rated as block deficiencies. Intubation equipment for advanced airway management was kept on standby. The primary study endpoint was successful blockade, meaning that no sequential airway management was required for the spontaneous breathing patients during surgery. Secondary endpoints included any use of fentanyl/propofol intraoperatively and of postoperative analgesics in the recovery room. RESULTS: All 20 blocks were successful, with no block deficiencies upon skin incision, no need for sequential airway management, and stable SpO2 levels (97-100%). Surgery took a median of 120.5 minutes (IQR: 89.3-136.5) and included one bolus of fentanyl in one patient 120 minutes into a protracted operation. No more systemic analgesia had to be provided in the recovery room. CONCLUSIONS: Sedation and epidural anesthesia emerged as a useful alternative to GA from our consecutive case series.

Ischemic Priapism in a 12 Year Old Patient Associated With Coronavirus Disease 2019 (COVID-19): A Case Report.

Infection with Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) has been associated with changes in blood coagulation resulting in increased incidence of venous thromboembolic events and coagulopathy. Moreover, single cases of ischemic priapism have been reported in adult patients with SARS-CoV-2 infection. In this report, we describe the case of ischemic priapism in a 12-year-old child with recent SARS- CoV-2 infection.

Rules and regulations for a pregnant endourologist: the European perspective.

INTRODUCTION: Working in surgery while pregnant is challenging. Navigating this period safely is of paramount importance. Anecdotal observation suggests that there exists great variation among European nations in regard to maternity leave and radiation safety. The aim of this article was to gain insight into policy patterns and variations across Europe regarding these issues. METHODS: A series of core question items was distributed to representatives across 12 nations Austria, Belgium, Germany, Greece, Iceland, Italy, Netherlands, Norway, Poland, Republic of Ireland, Spain and the United Kingdom). RESULTS: The total number of weeks with full pay ranged from as little as 4 weeks in Belgium to 32 and Iceland. All countries included in this study give the option of additional weeks beyond the initial period, however at reduced pay. Some offer unpaid leave beyond this. Only 5/12 countries had a specific policy on when the pregnant surgeon should come off the on-call rota. Only Austria, Italy and Poland stipulate a requirement for the pregnant clinician to be replaced or be completely exempt in cases involving radiation. Only Germany, Iceland, Norway and Poland highlight the need to limit radiation dose in the first trimester. Beyond this, Germany alone provides written guidance for reduction in gown weight and along with Poland, display arguably the most forward-thinking approach to resting. CONCLUSION: There is a marked range in maternal leave policies across Europe. There also exists a lack of universal guidance on radiation safety for the pregnant urologist. There is urgent need for this void to be addressed.

Adolescent and Young Adult Urogenital Outcome following Childhood Hypospadias Repair: Perfection Revisited.

PURPOSE: We assessed the long-term surgical, functional urinary and sexual outcomes of adolescent and young adult men who underwent childhood hypospadias repair. MATERIALS AND METHODS: Men born with nonsyndromic hypospadias and healthy male controls aged 16-21 years old were recruited, and their surgical, urinary, sexual functional and aesthetic outcomes assessed. Good outcome was defined as a patent and orthotopic meatus without fistulas, and straight erections (<30 degree curvature) without erectile or ejaculatory problems. Statistics included regression analyses, chi-square/Fisher exact tests and Student's t/Mann-Whitney U and Kruskal-Wallis tests. RESULTS: A total of 193 patients and 50 controls participated 16.4 years (range 8.2-21.2) after initial repair. At least 1 reintervention was performed in 39.2%. The highest reintervention rate was found in those younger than 12 months at initial repair, even when excluding proximal hypospadias cases. A disturbed urinary and/or suboptimal sexual functional outcome was seen in 52.9% of cases. Suboptimal voiding was found in 22.1%, although few had relevant residual urine. More reinterventions and proximal hypospadias cases were associated with suboptimal urinary outcome, and the latter also with impaired sexual function. Poor inter-observer agreements were found between physician and patient genital appraisal. CONCLUSIONS: In 52.9% of cases, at least 1 concern was identified that required long-term followup. Hypospadias repair below 12 months was associated with more reinterventions. Adopting a restrictive attitude toward aesthetic refinement, unless on the patient's own request, could improve urinary outcomes.

Psychosexual Outcome, Sexual Function, and Long-Term Satisfaction of Adolescent and Young Adult Men After Childhood Hypospadias Repair.

BACKGROUND: The psychosexual outcome in adolescents and young adults (AYA) men born with hypospadias is precarious. However, the factors responsible for impaired outcome in some AYA men have been understudied. AIM: To explore the outcome after hypospadias repair in childhood of AYA men aged 16-21 years and examine their opinion and their parents' opinion about this type of surgery. METHODS: Cross-sectional assessment of 193 AYA men born with hypospadias and 50 male controls was performed. Questionnaires such as the Decision Regret Scale, Pediatric Penile Perception Score, Sexual Quality of Life-Male, International Index of Erectile Function, and a custom-made questionnaire were used. The Decision Regret Scale and a custom-made questionnaire were also completed by the participants' parents. Physical examination including Hypospadias Objective Penile Evaluation and measuring stretched penile length was performed. OUTCOMES: This study reports the psychosexual functioning (ie, social, relational, and sexual), erectile and sexual function after childhood hypospadias repair, using ad hoc measures. In addition, the opinion about hypospadias repair of patients and their parents is represented. RESULTS: The number of surgeries and satisfaction regarding penile appearance were the most important factors associated with the opinion on hypospadias repair and the psychosexual outcome. Most AYA men were more satisfied with their penile appearance than the physician. 80% of men were satisfied with having had a childhood hypospadias repair, even though they had not been able to consent to surgery themselves. Erectile and ejaculation problems were mild and seen in approximately 10% of the population. CLINICAL IMPLICATIONS: Based on our data, deferring hypospadias repair until the patient can decide himself is not warranted. However, physicians who accept a suboptimal esthetic outcome and withdraw from repeated surgery may contribute importantly to the patient's well-being, especially in proximal forms of hypospadias. STRENGTHS & LIMITATIONS: This is one of the rare studies addressing the AYA's psychosexual outcome after childhood hypospadias repair. Strengths include the combination of clinical and psychosexual data from a very large cohort of men and their parents to provide a more holistic view. By entering this study, participants might have a different comfort level regarding their sexuality or have a different body image than the overall population of young men. CONCLUSION: Uncomplicated hypospadias surgery results in equal psychosexual outcome as controls and in high satisfaction rates; multiple surgeries are a risk factor for poorer outcomes. 80% of men are satisfied with childhood hypospadias repair. Tack LJW, Springer A, Riedl S, et al. Psychosexual Outcome, Sexual Function, and Long-Term Satisfaction of Adolescent and Young Adult Men After Childhood Hypospadias Repair. J Sex Med 2020;17:1665-1675.

The External Genitalia Score (EGS): A European Multicenter Validation Study.

CONTEXT: Standardized description of external genitalia is needed in the assessment of children with atypical genitalia. OBJECTIVES: To validate the External Genitalia Score (EGS), to present reference values for preterm and term babies up to 24 months and correlate obtained scores with anogenital distances (AGDs). DESIGN, SETTING: A European multicenter (n = 8) validation study was conducted from July 2016 to July 2018. PATIENTS AND METHODS: EGS is based on the external masculinization score but uses a gradual scale from female to male (range, 0-12) and terminology appropriate for both sexes. The reliability of EGS and AGDs was determined by the interclass correlation coefficient (ICC). Cross-sectional data were obtained in 686 term babies (0-24 months) and 181 preterm babies, and 111 babies with atypical genitalia. RESULTS: The ICC of EGS in typical and atypical genitalia is excellent and good, respectively. Median EGS (10th to 90th centile) in males < 28 weeks gestation is 10 (8.6-11.5); in males 28-32 weeks 11.5 (9.2-12); in males 33-36 weeks 11.5 (10.5-12) and in full-term males 12 (10.5-12). In all female babies, EGS is 0 (0-0). The mean (SD) lower/upper AGD ratio (AGDl/u) is 0.45 (0.1), with significant difference between AGDl/u in males 0.49 (0.1) and females 0.39 (0.1) and intermediate values in differences of sex development (DSDs) 0.43 (0.1). The AGDl/u correlates with EGS in males with typical genitalia and in atypical genitalia. CONCLUSIONS: EGS is a reliable and valid tool to describe external genitalia in premature and term babies up to 24 months. EGS correlates with AGDl/u in males. It facilitates standardized assessment, clinical decision-making and multicenter research.

Midline Incision of a Graft in Staged Hypospadias Repair-Feasible and Durable?

Purpose: In severe hypospadias staged repair is commonly used and it is regarded as feasible, safe, and durable. In this article we want to describe the results of a modification of the staged repair: a midline incision of the graft during the second stage. Materials and Methods: This is a consecutive single team (2 surgeons) retrospective series. Between 2014 and 2017, 250 patients underwent hypospadias repair, among them 35 patients that had primary staged hypospadias surgery with completed first and second stage repair. 24 (68.6%) cases received a preputial skin graft and 11 (31.4%) buccal mucosa graft. Median age at first stage was 1.5 (0.5-22.1) years, mean time between first and second stage operation was 0.72 (0.4-1.76) years. Follow up rate was 100%, mean follow up period was 1.50 (0.4-3.8) years. Results: The total complication rate was 22.9%. In buccal mucosa repair the complication rate was 36.4% and in preputial graft repair the complication rate was 16.7%, respectively. In 23 patients (65.7%) during second stage urethroplasty a midline incision was performed (8 glandular graft, 15 penile graft, 6 at level of urethral opening). Complication rate in non-incised urethroplasty was 8.3%, in incision at glandular level 37.5%, in incision at penile level 13.3% and in incision at urethral opening 16.7%, respectively. Conclusions: Two stage repair is the method of choice in the correction of severe hypospadias. In selected cases a midline incision of the graft is feasible and can be applied if needed. Randomized studies will be needed to evaluate the true benefit of incising the graft.

Fetal magnetic resonance imaging of lymphangiomas.

OBJECTIVES: To evaluate the fetal magnetic resonance imaging findings of lymphangiomas. METHODS: The magnetic resonance scans of eight fetuses with lymphangiomas were evaluated. Magnetic resonance evaluation included: number; size; signal intensities of the lesions; thickness of the septae; configuration of the margins; presence of blood breakdown products; change in size or signal intensity (in four patients with multiple examinations); exact expansion of the lesions to the adjacent anatomical structures; and concomitant pathological findings. Results were compared with postpartum clinical assessment and imaging in seven patients and with autopsy in one patient. RESULTS: Two retroperitoneal, three thoracic, and three cervical lymphangiomas (diameters between 3.3 and 15.6 cm) were included. All lesions consisted of macrocysts, and additional microcystic parts were found in three lymphangiomas. Blood breakdown products were found in one lesion. Agreement with postpartum imaging was excellent. One patient received intrauterine drainage for chylothorax, and one pregnancy was terminated. CONCLUSIONS: Fetal lymphangiomas display the same magnetic resonance imaging features as postnatal lymphangiomas. Intrauterine magnetic resonance characterization of lymphangiomas provides the exact delineation, detection of associated and/or concomitant pathologies, and differential diagnosis among other cystic pathologies. Patient management may be altered with respect to the type and/or time of treatment, and with regard to the continuation or termination of pregnancy.

Neuroendocrine tumor of the common hepatic duct, mimicking a choledochal cyst in a 6-year-old child.

We report a rare case of a neuroendocrine tumor of the extrahepatic biliary tree in a child. A 6-year-old girl who presented with jaundice and pruritus was found to have elevated liver enzymes along with a cystic dilatation of the common hepatic duct. After further diagnostic testing, a working diagnosis of a type I choledochal cyst was established. Laparotomy revealed hydrops of the gall bladder, an elongated and dilated cystic duct and a cystic dilatation of the common hepatic duct. An unusually thickened common bile duct was also noted at the time. Follow-up histology revealed an invasive neuroendocrine tumor which lead to a second look operation in which extended resection and lymphadenectomy was performed. No histological or gross pathological evidence of lymph node metastasis was found. Postoperatively the patient continues doing well 2 years after the original diagnosis was established. Neuroendocrine tumors of the extrahepatic bile duct are extremely rare with only 4 pediatric cases of a total of 51 cases published in the literature. To our knowledge, this is the youngest patient reported so far. The rarity of this entity made it challenging to diagnose a case of a neuroendocrine tumor in an atypical location that radiologically mimicked a choledochal cyst.