Apart from listening to the cry of a healthy newborn, it is the declaration by the attending paediatrician in the labour room that the child is normal which brings utmost joy to parents. The global incidence of children born with congenital anomalies has been reported to be 3%-6% with more than 90% of these occurring in low- and middle-income group countries. The exact percentages/total numbers of children requiring surgical treatment cannot be estimated for several reasons. These children are operated under several surgical disciplines, viz, paediatric-, plastic reconstructive, neuro-, cardiothoracic-, orthopaedic surgery etc. These conditions may be life-threatening, e.g., trachea-oesophageal fistula, critical pulmonary stenosis, etc. and require immediate surgical intervention. Some, e.g., hydrocephalus, may need intervention as soon as the patient is fit for surgery. Some, e.g., patent ductus arteriosus need 'wait and watch' policy up to a certain age in the hope of spontaneous recovery. Another extremely important category is that of patients where the operative intervention is done based on their age. Almost all the congenital anomalies coming under care of a plastic surgeon are operated as elective surgery (many as multiple stages of correction) at appropriate ages. There are advantages and disadvantages of intervention at different ages. In this article, we present a review of optimal timings, along with reasoning, for surgery of many of the common congenital anomalies which are treated by plastic surgeons. Obstetricians, paediatricians and general practitioners/family physicians, who most often are the first ones to come across such children, must know to guide the parents appropriately and convincingly impress upon the them as to why their child should not be operated immediately and also the consequences of too soon or too late.

To assess the effect of age at hypospadias surgery on emotional and behavioural problems, somatic symptoms, irritability, and penile perception.

We retrospectively identified the patients who underwent single distal hypospadias surgery and age-matched healthy controls were included. There were two further subgroups according to the age at the time of hypospadias repair (<2 vs. >2 years). The Strengths and Difficulties Questionnaire (SDQ), Revised Children's Anxiety and Depression Scale (RCADS), Affective Reactivity Index (ARI), Level 2 Somatic Symptom Scale, and Penile Perception Score (PPS) scale were used. The groups were compared using multivariate variance analysis (MANOVA).

Both groups consisted of 70 patients (mean age 14.0 ± 0.2 years, for both), while there were 34 patients in the hypospadias groups who underwent surgery at <2 years of age. Depressive, panic, separation anxiety, social phobia, and somatic complaint symptom scores of the hypospadias group were lower than those of the control group. Obsessive-compulsive symptom levels were significantly higher in patients who underwent hypospadias surgery at >2 vs. <2 years of age. Additionally, PPSs rated by the surgeon were significantly higher in the former. A multivariate linear regression model indicated that panic disorder symptom scores predicted child PPS in the hypospadias group. Limitations include retrospective design.

Single hypospadias surgery seems not to have a negative impact on emotional and behavioural status. Children who underwent distal hypospadias surgery after 2 years of age had higher levels of obsessive-compulsive symptoms. Following emotional status may help the early diagnosis of future psychopathologies.

Retrospective comparative study.

III.

Specialist facilities for children are still unavailable in some Sub-Saharan African contexts. It is the case of pediatric urology, whose recent advances are still largely unshared. Prenatal diagnosis of urinary abnormalities (CAKUT) is largely unknown. Early recognition and referral of Undescended testis (UDT), Hypospadia, bladder exstrophy epispadias complex, ambiguous genitalia, stone disease, and tumours are uncommon in rural areas. Missed diagnosis is not uncommon and delayed management is associated with poor outcomes. We present a cross-sectional, descriptive study about the epidemiology of Pediatric urological admissions to three sub-Saharan East African Hospitals. All the urological cases between 0-18 years referred to three distinct East African Hospitals over 124 weeks were considered. Prevalence of different groups of diseases, age, and mode of presentation were reported. We found 351 cases (M/F 127/24) out of 2543 surgical referrals (13%). Seventy percent of cases were Hypospadias and UDT. Fifty percent of UDT were beyond 6, and most Hypospadias were between 4 and 7 yrs. CAKUT had a very low prevalence (4.84%), and about 50% of Wilms Tumours came too late to be resectable. In many African contexts, urology is still a tiny portion of the pediatric surgical workload compared to the 25% of European and American reports. There are also differences in the epidemiology of genitourinary conditions. A hidden burden of diseases may be presumed, remaining undiagnosed due to the shortage of specialist facilities.

Hypospadias is a common congenital malformation in pediatric urology with surgery being the only curative treatment. Although there are hundreds of surgical methods for hypospadias, no single method can treat all types, and there are still high rates of postoperative complications. We performed this study to investigate surgical procedure selection and perform risk factor analysis of postoperative complications in hypospadias repair.

Retrospective analysis was performed of complete clinical and follow-up data of children with hypospadias who were treated and followed up at 15 children's clinical centers in Mainland China from December 2018 to December 2019. Children were divided into groups according to Barcat classification and surgical methods in order to analyze the surgical choice for different types of hypospadias and the influencing factors of different surgical methods for complications.

In total, 1011 patients were followed up for 26 months. According to Barcat classification, there were 248 cases of distal type hypospadias, 214 of intermediate, and 549 of proximal type. Transverse preputial island flap urethroplasty (Duckett) and tubularized incised plate urethroplasty (TIP) were performed in 375 (37.1%) and 336 cases (33.2%), respectively. The postoperative complication rate of distal hypospadias was 23.4% (15.8-57.1%), mid shaft 29.0% (22.7-40.0%), and proximal 43.7% (30.2-52.9%). Among the 375 patients in Duckett group, 192 had complications. Multivariate logistic analysis showed that the length of prepuce island flap (OR = 3.506, 95% CI: 2.258-5.442) was an independent risk factor for complications after Duckett operation (P < 0.001). In TIP group, there were 336 cases with 84 complications. Multivariate logistic analysis showed that the width of urethral plate after longitudinal resection (OR = 0.836, 95% CI: 0.742-0.942) and glans width (OR = 0.851, 95% CI: 0.749-0.965) were independent risk factors for postoperative complications after TIP (P = 0.003, P = 0.012).

Several anatomical features play a role during the selection process among the different surgical approaches, including glans size, urethral plate width, and the meatal position. The width of the urethral plate and glans width were risk factors for postoperative complications after TIP. The length of prepuce island flap was a risk factor for complications after Duckett operation.

Hypospadias is one of the most common congenital diseases of the genitourinary system in children. The European Association of Urology (EAU) Guidelines recommend that children undergoing hypospadias surgery should be between 6 and 18 months. In China, where many children have hypospadias, it remains unknown whether clinical characteristics, socioeconomic factors and COVID-19 were associated with delayed surgery in children with hypospadias.

We retrospectively analyzed children with hypospadias who underwent primary surgery at the Department of Pediatric Urology in Guangzhou Women and Children's Medical Center between January 2010 and October 2021. Patients who had two-stage surgery or a second round of surgery due to complications were excluded to eliminate data duplication. The clinical characteristics and demographic information were collected. We defined delayed surgery as primary surgery performed after 18 months following the EAU Guidelines.

A total of 4439 children diagnosed with hypospadias were included in the study. The median age (29.1 ± 16.7 months) of surgery for hypospadias in our study was much higher than the recommended age reported in the EAU guidelines, and 76.6% of the children underwent surgery after the age of 18 months. Children without comorbidities including cryptorchidism (odds ratio [OR] = 1.562; 95% confidence interval [CI] 1.199-2.034; p = 0.001), prostatic cyst (OR = 2.613; 95% CI 1.579-4.324; p < 0.001), penile hypoplasia (OR = 1.778; 95% CI 1.225-2.580; p = 0.002), inguinal hernia (OR = 2.070; 95% CI 1.394-3.075; p < 0.001), and penoscrotal transposition (OR = 4.125; 95% CI 1.250-13.619; p = 0.020) were more likely to receive delayed surgery. Living in a low economic area (OR = 1.731; 95% CI 1.068-2.806; p = 0.026) or not close to a main medical center (OR = 1.580; 95% CI 1.370-1.824; p < 0.001) was highly associated with delayed surgery. The proportion of children undergoing delayed surgery and the median age of surgery during the COVID-19 pandemic were significantly higher than those before the COVID-19 pandemic (p = 0.004 and < 0.001, respectively).

Most children with hypospadias received delayed surgery (surgical age > 18 months). Comorbidities, living in a low economic area, too far from a main medical center and the COVID-19 pandemic were highly associated with delayed surgery. It is vital to improve the public awareness of hypospadias and strengthen the re-education of primary community doctors to reduce delayed surgery.

To evaluate the results and related factors of tubularized incised plate (TIP) urethroplasty at two institutions.

This was a prospective cohort analytical study conducted over a period of 12 months. All patients who underwent TIP urethroplasty in the specified period were studied. Quantitative and qualitative data of the intrinsic parameters of the penis were obtained and patients were followed up for an average period of 14.72±3.67 months (range 9-21months) after surgery.

One hundred twenty-nine patients (N = 129) were included in the study. The mean age at surgery was 50.93 months. The mean glans size and pre-incised urethral plate width were 14.34 mm and 8.38mm respectively. The post-operative results were satisfactory with the meatus in a glanular position in 122(94.6%) patients. Overall, 49 patients (38%) developed complications. Eighteen patients (14%) developed early complications whereas forty-two (32.6%) patients had late complications. UCF and Meatal stenosis occurred in 27 (20.9%) & 14 (10.9%) patients respectively. Seven patients developed recurrent hypospadias and dehiscence of glans occurred in eight patients (6.2%).

TIP can be used to repair for all types of hypospadias in the absence of severe penile curvature. It has more complications rate in proximal than distal hypospadias. Distal hypospadias was the most common type of hypospadias corrected with TIP. UCF and meatal stenosis were the most common complication followed by glans dehiscence and recurrent hypospadias. Glans size, age at surgery, plate width, location of meatus and stretched penile length were the most determinant factors for the outcome.

To report experience and technical refinements with the Koyanagi urethroplasty for proximal hypospadias.

A retrospective study was conducted between 2004 and 2017. Medical records of patients treated by the Koyanagi technique were investigated. The penile ventral skin was closed either with a Byars flap or using an "Ombredanne's chasuble" (OC). The cohort was divided chronologically into two groups of the same number of patients (early -E- and late experience group-LEG-). Demographics, anatomical findings and surgical outcomes were compared between groups using univariate analysis. A logistic regression was performed to assess factors associated with the occurrence of a postoperative urethrocutaneous fistula or urethroplasty dehiscence (UD).

The Koyanagi urethroplasty was performed in 67 patients, 33 in the EEG and 34 in the LEG. The overall surgical complication rate was 64.2% (n=43), including 42 fistula or UD, higher in the EEG (81.8%) than in the LEG (44.1%, P<0.01). However, in the LEG, patients underwent the surgery older and the use of OC more frequent. After multivariate analyses, factors associated with a postoperative fistula or UD were the year of surgery (OR=0.71 [0.53-0.96] P=0.02), the age at surgery (OR=1.11 [1.01-1.22], P=0.03); contrary to the skin coverage method or the stenting duration (P>0.05). An urethral stenosis occurred in 1 patient (1.5%). At last follow-up, 64.2% of patients required a further procedure and 80.6% of urethral meatus were glandular.

In this study the complication rate, particularly the urethrocutaneous fistula, remained high. Urethral stenosis, were rare but the follow-up was too short to clearly identified them. Considering as a 2 stage procedure koyanagi uretroplasty allows to obtain finally good results.

III.

Hypospadias is a congenital anomaly of male external genitalia and is the second most common congenital anomaly of male newborns after cryptorchidism. Although its prevalence in Afghanistan is unknown, anecdotal evidence shows that hypospadias is an important clinical and psychosocial problem in Afghanistan. There is no data available regarding the socio-demographic pattern of the disease and age at the time of surgery in Afghanistan context.

The aims of this study were to identify socio-demographic pattern of hypospadias among patients and to determine surgical outcomes of hypospadias patients operated in a tertiary care center in Kabul.

A descriptive case series design and non-probability consecutive sampling were used. Study population was 86 patients undergone urethroplasty during January 2018 to September 2019. A structured questionnaire was used to assess the dependent and independent variables. Follow up notes of patients' medical record files were reviewed to assess post-operative condition of the patients along with presence of any post-operative complications.

The mean age at the time of repair was 6.2 years. Family history was positive in 16.3% and consanguinity in almost 56% of the patients. The most common type of hypospadias was distal shaft type (30.2%), penile chordae was present in 25.6% and pinhole meatus in 32.6% of the cases. The most common associated anomaly was cryptorchidism (9.3%) followed by inguinal hernia (2.3%). Peno-scrotal transposition was the most common sexual differentiation disorder (11.6%), however majority didn't have any disorder of sexual differentiation (83.7%) The most common complication was urethral stricture and meatal stenosis (9.3% each) followed by urethra-cutaneous fistula (7%) in this study, although remarkable majority (74.4%) was complication free. Urethral advancement procedure was more practiced in distal type (94.7%) and 2-stage repair for the proximal type (70%) of hypospadias.

Findings showed that the age at repair for hypospadias in our center is much higher than the recommended age. Different than the literature, the most common complication was meatal stenosis. Majority of patients were result of consanguineous marriages. Procedure choice was different according to the type of hypospadias and preference of the surgeon.

We assessed self-reported outcomes after the onset of puberty in patients undergoing tubularized incised plate (TIP) repair in combination with preputial reconstruction (PR) or circumcision for primary distal hypospadias, in comparison to normal controls.

Out of 498 patients undergoing primary distal hypospadias repairs between 2001 and 2012, 83 underwent TIP repair associated with PR or circumcision before age 10-year, and had a Tanner stage ≥2 at study. Outcomes were assessed by online self-administration of validated questionnaires and additional non-validated questions. Results were compared between patients undergoing TIP repair vs. normal age-matched controls (healthy volunteers), and, between patients undergoing PR vs. circumcision.

Forty-one of the 83 eligible patients completed the study (response rate 49.3%). Of these, 26 had undergone PR. Median post-operative follow-up was 11.8 (range 6.5-16.2) years. The control group included 45 healthy boys. Comparing groups, there were no statistically significant differences in the cosmetic aspect of the penis, patient perception of penile appearance, and quality of life. Irrespective of preputial management, patients reported fear of being teased for the appearance of their genitalia much more commonly than controls (p = 0.02), but this did not affect their relationship patterns and quality of life.

Self-reported outcomes after the onset of puberty were not significantly different between patients undergoing primary TIP repair of distal hypospadias in childhood and normal controls. Patients were significantly more commonly feared of being teased for their genital appearance, but this did not interfere with their social skills. Preputial management, namely PR vs. circumcision, did not affect the outcome.

Health-related quality of life (HRQoL) is an important yet understudied construct for individuals with hypospadias (HS). An important barrier towards understanding HRQoL and integrating it into research and clinical care for people with HS is the absence of an underlying conceptual framework to illustrate this construct. We propose a conceptual framework for HS-specific HRQoL based upon a scoping review of the HS literature.

We conducted a literature review of articles published between 1989 and 2019. Our search in Embase and Pubmed used the keyword "hypospadias" in combination with "quality of life" and "patient-reported outcomes." We used thematic analysis of the resulting publications to identify core HRQOL domains. From these results and review of HRQoL literature in other pediatric populations, we developed a conceptual framework representing HS-specific HRQoL.

We identified five domains of HRQOL previously studied in research with youth and adults with HS: penile appearance, voiding, social interaction, sexual health, and psychological or behavioral function. We propose a model of HS-specific HRQoL comprised of these domains and their areas of overlap, based upon the findings and conceptual mapping of our literature review.

This novel conceptual framework provides a foundation for understanding disease-specific HRQoL in individuals with HS and may serve as a guide for the conduct of future qualitative studies of the HS population. The overlapping biopsychosocial domains illustrate the possible effects of HS on day-to-day life. This framework may guide future surgical, clinical, and behavioral interventions that aim to improve medical care and quality of life outcomes for HS patients.

There is growing recognition of the importance of patient-reported outcomes (PROs) in pediatric hypospadias. We have previously presented a conceptual framework for Hypospadias-Specific Health-Related Quality of Life (HRQoL), which posited 5 domains of HRQoL in this population. The framework components (domains) included penile appearance, voiding function, social function, psychological/behavioral function, and pubertal/sexual health. In this work, we investigated the established validity and relevance of PROs within each of these domains for patients with hypospadias.

We evaluated existing measures with published psychometric data, including validation data, in the hypospadias population. We also assessed the available data on each measure according to the guidelines of the Scientific Advisory Committee of the Medical Outcomes Trust (Table) in order to establish measure quality. We also examined the power of existing validation studies according to suggested guidelines for psychometric validation and factor analysis.

Available validated measures in the hypospadias population have focused primarily on penile appearance and to a lesser degree on pubertal development/sexual health. There were no validated disease-specific measures with dedicated evaluations of other key HRQoL domains including voiding-related sequelae, social function, or psychological function. In addition, no single measure addressed all of the quality guidelines posed by the Scientific Advisory Committee.

Current generalized measures for PROs lack relevance to the experience of hypospadias patients, and disease-specific assessments are often focused on penile appearance. Improving measure quality is necessary to optimize the value of our assessments and better help our patients with hypospadias.

To evaluate the surgical outcomes of meatal advancement and glanuloplasty incorporation (MAGPI) and tubularised incised plate (TIP) urethroplasty. We retrospectively reviewed the patient records who had hypospadias repairing surgery between March 2017 and March 2020 in terms of age, meatal position, operative and anaesthesia techniques and surgical outcomes. Hypospadias repair was performed in 85 patients. TIP urethroplasty was performed in 17 patients where MAGPI was performed in 63 patients. Preoperative meatal stenosis was present in 10. Coronal glanular and subcoronal hypospadias were present in 65 and 15 patients respectively. Mean operative time was 41.3 ± 28 min. The mean duration of the catheter was 7.56 days. A significant chordee was present in 35 patients where 45 patients had no chordee. The overall success rate was 93.75%. MAGPI ended up with an unfavourable result in 1 and TIP urethroplasty in 4. Postoperative urethral stenosis was seen in 1. Of those who ended up with an unfavourable result, 4 were with subglandular hypospadias. Urethral catheter required in 4 of those who failed, whereas 1 had no catheter following surgery. Therefore, MAGPI is a useful method in selected glandular and subglandular hypospadias repair with favourable surgical outcomes including no stent requirement.

To analyze a single-centre experience in feminizing genitoplasty in virilized girls with congenital adrenal hyperplasia (CAH).

Review of medical records of all 46, XX CAH patients undergoing single stage feminizing genitoplasty between 2003 and 2018 was performed.

A total of 31 girls aged from 4 months to 10 years were included in the study. The majority (n = 26/31, 84%) were operated before 2 years of age (median 8 months). External virilization was rated as Prader 3 (n = 7/31), Prader 4 (n = 21/31) and Prader 5 (n = 3/31). The urethrovaginal confluence location was low in 19 and high in 12 girls with a percentage distribution similar in Prader 4 and 5 (p > 0.05) but significantly different in Prader 3 (p = 0.017). The follow-up ranged from 12 months to 15 years. All parents assessed the cosmetic result as satisfactory. Perioperative complications occurred in two patients and included rectal injury (n = 1/31) and prolonged bleeding (n = 1/31). Three patients developed late complications including labial atheromas (n = 2/31) and vaginal stricture requiring surgical dilatation (n = 1/31). Low confluence did not decrease the risk of complications.

Early feminizing genitoplasty in girls with congenital adrenal hyperplasia, irrespective of virilization severity, gives satisfactory cosmetic results and is characterized by low and acceptable surgical risk. Nevertheless, the most important determinant of the effectiveness of such management would be future patients' satisfaction.

The objective of this study is to investigate the coping styles used by patients with hypospadias. Long-term hypospadias studies generally show satisfying outcomes, but some report a lower quality of life (QoL) or poorer psychosocial adjustment, particularly in patients with proximal hypospadias. A lower QoL or psychosocial adjustment was found to be associated with passive coping styles in other pediatric patient populations. Hypothetically, patients with hypospadias also develop different coping styles due to medical experiences in childhood, leading to the lower QoL outcomes on the long term. However, coping styles of patients with hypospadias have never been analyzed.

Adult men with hypospadias repair in childhood were recruited (n = 55; aged 19.9 [IQR 19.2-22.1]). Coping styles were determined with the Utrecht Coping List (UCL) and results compared with a reference group of male students (n = 55, age 20-30 years, no medical history). Sub analysis of coping styles of the hypospadias groups was done based on three items: severity of hypospadias, time of last hypospadias surgery and occurrence of postoperative complications.

Compared to the reference groups, patients with hypospadias had higher scores on Avoidance (P < 0.05), particularly patients who had ≥1 postoperative complication or proximal hypospadias. Patients with proximal hypospadias also had lower scores on Seeking Social Support compared to the reference group (P < 0.05). Within the hypospadias group, coping style scores did not differ based on severity of hypospadias, timing of surgery or postoperative complications.

Patients with hypospadias, particularly those with proximal hypospadias or a postoperative complication, more often use an avoiding coping style compared to a reference population. Attention to coping styles during the follow-up of patients with hypospadias might help to improve the QoL in these patients.

Patient-reported outcomes have the potential to provide invaluable information for evaluation of hypospadias patients, aid in decision-making, performance assessment, and improvement in quality of care. To appropriately measure patient-relevant outcomes, well-developed and validated patient-reported outcome (PRO) instruments are essential.

To identify and evaluate existing PRO instruments designed to measure quality of life and/or satisfaction of individuals with hypospadias that have been developed and validated in a hypospadias population.

A systematic search of MEDLINE, EMBASE, PsycINFO, CINAHL and Health and Psychosocial Instruments was conducted in April 2016. Two reviewers independently assessed studies and identified PRO instruments for inclusion. Data were extracted on study characteristics, instrument development and validation, and content domains.

A total of 32 studies were included that used or described five PRO instruments: Hypospadias Objective Scoring Evaluation (HOSE), Pediatric Penile Perception Score (PPPS), Penile Perception Score (PPS), Genital Perception Scale (GPS) for adults, and GPS for children/adolescents. Instrument development and validation was limited. The majority of identified instruments focused on postoperative cosmetic satisfaction, with only one instrument considering urinary function, and no instruments evaluating sexual function and psychosocial sequelae.

While many hypospadias studies have acknowledged the necessity of a patient-reported element, few have used validated PRO instruments developed in a hypospadias population. Existing instruments to measure patient-reported outcomes in hypospadias require improvement in both the breadth of content and in their development and validation methodology.

Psychosexual development is currently underrepresented in hypospadias outcome research. The aim of this study was to develop and validate a questionnaire addressing psychosexual long-term satisfaction, specifically of adolescent patients, after hypospadias repair.

In a multistep participative design we identified key interests of adolescent patients with hypospadias. Next, a questionnaire addressing specifically the psychosexual satisfaction of adolescents after hypospadias repair was established. A population of 109 former patients with hypospadias was then assessed using this questionnaire. Furthermore, functional and cosmetic aspects, behavioural anomalies, and sexual activity were investigated. Age-matched patients undergoing circumcision served as control patients. Possible influence factors on patient satisfaction were investigated. Clinical trial registry site: German Registry of Clinical Trials DRKS, Freiburg, Germany (Reference: DRKS00003432).

Key interests of adolescent patients were "normal appearance of the penis", "normal function of the penis regarding voiding and sexual activity", "no limitations regarding cosmetic appearance to others", "no limitations to sexual activity", and an "unimpaired masculine identity". The "Satisfaction In Genital Hypospadias Treatment" (SIGHT) questionnaire was developed using these items and using previously published evaluation systems. Nine questions address psychosexual aspects and two additional questions address current sexual activity. Internal consistency was high and retest reliability acceptable. The patient population showed a normal strength and difficulties score (SDQ). Overall satisfaction was high and similar to that of the control group. In a Spearman correlation a high SDQ value, erectile problems, and complications correlated negatively with satisfaction.

To date, few studies have examined patients' satisfaction and psychosexuality. To our knowledge, the SIGHT questionnaire is the first to be developed participatively and in a stepwise fashion in collaboration with a paediatric psychologist and an open approach to determine items specifically important for adolescents. The SIGHT questionnaire can thus offer a relevant assessment of patients' psychosexual satisfaction. It is most suitable to supplement current strategies that so far mostly neglect the impact on psychosexual wellbeing.

Long-term evaluation of the surgical outcome after puberty, particularly patient reported outcome is rare in the literature. The aims of this study were to investigation the patients' satisfaction with the long-term results and their views and memories of their childhood surgery and follow-up.

A modified version of previously used questionnaire was sent to 134 patients ages 18 years or older previously primarily repaired due to hypospadias by one of the authors (HS) between 1989 -2009.

Thirty-nine patients responded. Eighty-two per cent were satisfied with the appearance of their penis, 87% were satisfied with their ability to urinate and their sexual function and 92% were satisfied with the overall surgical results. Ninety per cent of patients were positive to the current duration of our post-pubertal follow-up program or would have preferred an even longer follow-up.

The majority of patients were satisfied with the long-term surgical results and the duration of follow-up. Despite having problems patients does not always contact the health care system spontaneously, which warrants long-term follow-up.

To retrospectively identify the individual risk factors for the urethrocutaneous fistula (UCF) in pediatric patients after hypospadias repair (HR) with onlay island flap urethroplasty.

A total of 167 patients who underwent primary HR at Nanjing Medical University Affiliated Children Hospital from January 2009 to December 2012 were enrolled. Clinical data including the patient' age at HR, hypospadias type and urethral defect length were documented.

Among 167 patients, 12.6% patients (n = 21) developed UCF after HR. Postoperative UCF occurred in 3.9% (3/76) cases at age of 0-2 years, 14.3% (9/63) at 2-4 years, 20.0% (2/10) at 4-6 years and 38.9% (7/18) at 6-12 years. The incidences of UCF were 12.0% (3/25), 11.4% (5/132) and 30.0% (3/10) for distal, middle and proximal types of hypospadias. As to the urethral defect length, the incidences of UCF were 8.2% (5/61) in patients with a length of ≤ 2 cm, 12.8% (9/70) in 2-3 cm, 22.6% (7/31) in 3-4 cm and 0% (0/5) in above 4 cm. Older age at HR was significantly associated with the high incidence of UCF formation (P = 0.004), while the hypospadias type and urethral defect length did not affect it (P = 0.264 and P = 0.312, respectively).

The patient' age at HR was a risk factor for the UCF formation after HR, and treatment of HR within two years old might be with the least incidence of UCF.

We present data on long-term functional and cosmetic results after hypospadias surgery.

Males older than 18 years with hypospadias treated in Sweden were asked to participate in the study, as well as age matched controls and circumcised men. All participants answered questionnaires, and a subgroup was examined during an outpatient visit. Relationships with outcome were analyzed using analysis of variance and regression analysis.

A total of 167 patients with a mean age of 34 years and 169 controls with a mean age of 33 years answered the questionnaire. Of the patients 63% had distal, 24% mid and 13% proximal hypospadias. A total of 46 patients and 49 controls presented for physical examination. Patients were significantly less satisfied with the penile cosmetic outcome regarding all parameters of the Penile Perception Score. There was a difference in penile length between patients and controls (mean 9.7 vs 11.6 cm, p <0.001). More patients than controls reported voiding dysfunction symptoms (p = 0.003). Patients had a lower maximum urinary flow rate than controls (p = 0.001). These differences were most prominent between patients with proximal hypospadias and controls.

Men operated on for hypospadias were less satisfied with the cosmetic result than controls, and had a shorter penile length. Patients presented with more symptoms of voiding dysfunction and displayed a lower maximum urinary flow rate. Patients with proximal hypospadias were more affected than those with milder hypospadias. Our results indicate that patients with hypospadias can be subgrouped and that those with severe phenotypes should be followed more closely during childhood as well as later in adulthood.

There is still much debate regarding the effect that age at hypospadias operation has on the rate of complications. The aim of this study was to evaluate whether patient age is a risk factor for surgical complications when using the tubularized incised plate (TIP) urethroplasty technique.

Between 2005 and 2011, 307 pediatric patients with distal or mid-penile hypospadias underwent the TIP procedure. Demographic and surgical data were evaluated.

The complications recorded across all age groups were: fistula, meatal stenosis, glans dehiscence and urethral stenosis. Fistula was the most frequent complication in 10-14 year olds compared to the younger patient groups, and this difference was statistically significant.

Age and surgical technique should be taken into consideration when planning hypospadias surgery, since the complication rate increases with patient age. The TIP technique is a safe procedure with a low rate of fistula formation in distal and mid-penile hypospadias repair for patients of any age during the prepubertal period.

The aim of the study was to evaluate health-related quality of life (HRQoL) of men with a corrected hypospadias in comparison to circumcised controls. Furthermore, determinants of HRQoL were examined.

In a cross-sectional study, HRQoL of 45 men with corrected hypospadias (mean age: 26.2 years +/- 5.1) was compared with a control group of 46 circumcised men (mean age: 25.5 years +/- 4.9). Participants answered three questionnaires: The Medical Outcomes Study Short Form-36 item questionnaire (SF-36), the International Index of Erectile Function (IIEF), and the Penile Perception Score (PPS).

Physical and mental dimensions of HRQoL were not impaired among men with corrected hypospadias if compared to circumcised men. Neither socio-demographic and medical variables nor erectile function (EF) predicted HRQoL. However, a negative genital self-perception (PPS) and a lower orgasmic function (OF) were risk factors for an impaired mental HRQoL.

Our data suggest that the HRQoL of adult hypospadias patients is comparable to that of circumcised men. However, patients should be supported in developing a positive genital self-perception, because poor genital self-perception correlated with an impaired mental HRQoL. Since studies with non-operated men suggest that some adapt well to their penile condition, further studies should also include non-operated hypospadias.

Failed hypospadias refers to any hypospadias repair that leads to complications or causes patient dissatisfaction. The complication rate after hypospadias repairs ranges from 5-70%, but the actual incidence of failed hypospadias is unknown as complications can become apparent many years after surgery and series with lifelong follow-up data do not exist. Moreover, little is known about uncomplicated repairs that fail in terms of patient satisfaction. Risk factors for complications include factors related to the hypospadias (severity of the condition and characteristics of the urethral plate), the patient (age at surgery, endocrine environment, and wound healing impairment), the surgeon (technique selection and surgeon expertise), and the procedure (technical details and postoperative management). The most important factors for preventing complications are surgeon expertise (number of cases treated per year), interposition of a barrier layer between the urethroplasty and the skin, and postoperative urinary drainage. Major complications associated with failed hypospadias include residual curvature, healing complications (preputial dehiscence, glans dehiscence, fistula formation, and urethral breakdown), urethral obstruction (meatal stenosis, urethral stricture, and functional obstruction), urethral diverticula, hairy urethra, and penile skin deficiency.

The ideal timing and nature of surgical reconstruction in individuals with Disorders of Sex Development (DSD) is highly controversial. Despite the increasing number of publications on this topic, evidence-based recommendations still cannot be made. However it is generally accepted that optimal care for DSD requires an experienced multidisciplinary team. This means that surgical decisions are now made within the context of a multidisciplinary team and all members of the team - and not just specialist surgeons - may be called upon to discuss choices for surgery with patients and parents. To do this well, every clinician in the team should have an understanding of the range of techniques available for genital surgery, the risks and benefits of procedures and the controversies surrounding timing of surgery. The aim of this paper is to give an overview of the variety of surgical procedures in current use and in what situation a particular technique would be indicated. The short-term risks and benefits are described and where available long-term outcome data is discussed. To date, discussions surrounding genital surgery have been led primarily by surgeons. Some non-surgical clinicians have expressed unease about decision making in genital surgery but have felt ill equipped to comment on an area with which they are unfamiliar. This review gives a detailed explanation of current surgical practice offered in a specialized center for DSD and such information should facilitate a more balanced discussion.

Hypospadias is one of the most common congenital malformations of the male genitalia. Severe cases present with associated curvature greater than 30° and the meatus opening proximally to the penoscrotal junction. The perioperative management of patients with primary severe hypospadias is variable. Systematic evaluation of the upper urinary tract and the search for enlarged prostatic utricles seem unnecessary in patients with isolated primary severe hypospadias, and should be limited to severe cases with associated extraurinary malformations. Detection of a disorder of sex development is key for gender assignment and prognosis, but the identification of cases warranting a full work-up and the influence of such a diagnosis on the success of hypospadias repair is controversial. Preoperative hormonal stimulation allows for penile growth irrespective of the administration route. Associated morbidity is minimal, but its influence on the success of surgery is still unknown. An age of 6-18 months is generally recommended for surgery, but no trial data support this policy. Second-layer coverage of the urethroplasty and postoperative urinary drainage seem to reduce the complications of surgery, whereas postoperative antibiotic prophylaxis and type of dressing have minimal impact on surgical success. Overall, most interventions are based on weak evidence, and their influence on the outcomes of repair is ill-defined. Clinicians should be made aware of the evidence supporting any single intervention in order to standardize their management policies. We hope the issues outlined here will prompt researchers to design new studies to address the clinically relevant questions.

Hypospadias surgery has been in continuous evolution for many years with steadily improving reported results. Despite this many unanswered questions on its cause, management and outcomes remain. Recent research has done little to clarify most matters.

There is increasing evidence of a balance of genetic and developmental factors in the development of hypospadias, but there is doubt whether the incidence of hypospadias is increasing or not. Many technical aspects of hypospadias repair and variations of perioperative management seem to have little effect on outcome, whereas the age at which surgery is performed may. Although efforts are being made to relate anatomical findings to outcome, a lack of objective assessment and standardization is a handicap. Severe hypospadias, particularly that associated with significant chordee, remains challenging although strategies for management are being developed. The future may lie in development of autologous tissue culture for these severe cases.

Further development of hypospadias surgery would benefit from objective, standardized methods of describing anatomy and outcome measures to facilitate effective comparison of techniques and procedures.