Degree Of Hypospadias Research Articles

Prevalence of disorders of sex development in patients with hypospadias and cryptorchidism

Hypospadias and undescended testicles (UDT) are common congenital conditions, affecting approximately 1 in 125 and 1 in 33 boys, respectively. There has been limited contemporary data regarding the prevalence of Disorders of Sex Development (DSD) in patients with a history of both hypospadias and UDT. Our objective is to determine the prevalence of DSD among patients presenting with hypospadias and UDT. Electronic medical records were reviewed, and a retrospective chart review was conducted on 177 patients that were evaluated at our institution from 2000 to 2021 with a diagnosis of hypospadias and UDT. The degree of hypospadias, presence of and palpability of UDT, and prevalence and type of DSD were recorded. The degree of hypospadias, presence of and palpability of UDT, and prevalence and type of DSD were recorded. Chi-squared and Fisher's exact tests were conducted for descriptive statistical analyses as appropriate. Logistic regression analyses were conducted, with adjusted models including demographic and relevant clinical data. 177 patients were identified with both hypospadias and UDT, with 17/111 (15.3%) diagnosed with DSD. The most common etiology of DSD was mixed gonadal dysgenesis (35.3%; n=6). Proximal hypospadias made up only 59.6% of those without DSD vs. 100% of those with confirmed DSD (p=0.0044). A significantly smaller proportion of DSD patients had bilaterally palpable gonads compared to those without DSD (29.4% vs. 79.8%, p<0.0001). Compared to those with palpable testes, patients with one non-palpable testicle had up to 26.67 times greater odds of DSD. To date, we present the largest cohort of patients that have undergone DSD work up for the combined presentation of hypospadias and UDT. Our findings highlight an increase in DSD diagnosis in proximal hypospadias patients with non-palpable UDTs, which offers further evidence and support for pursuing DSD diagnostic work-up in all proximal hypospadias patients with UDTs.

Hormone receptor expression in hypospadias

Sex hormone imbalance in utero is hypothesized to play an important role in the pathogenesis of hypospadias. Due to its easy accessibility, foreskin samples have been used to describe hormone receptor expression in rodents, and both adult and pediatric patients. In this study we conducted a systematic approach to assess hormone receptor expression in pediatric patients with hypospadias compared to healthy controls with a focus on age-matching and differences in severity and degree of hypospadias. Foreskin samples were collected from 35 children during hypospadias operations (18 distal and 17 proximal hypospadias) and compared with ventral foreskin samples of a control group of 32 children during circumcision (15 age-matched and 17 older boys). The samples were stained with H/E, androgen (AR), estrogen (ER) and progesterone receptors (PR). The receptor stainings were blindly evaluated. An Allred score was used to evaluate receptor expression in both the epithelium as well as stroma. AR was detected in all cases. AR expression in the stroma was more evident than in the epithelium. AR expression in the hypospadias groups was significantly less than the age matched controls (p<0.05). There was no significant difference between the two hypospadias groups nor between the two control groups. Older control group showed significantly elevated levels of AR expression compared to the hypospadias group (p<0.05). ER was also detected in all cases. The stroma showed more ER than in epithelium. PR was minimal or negative in all samples. Boys with hypospadias showed significantly weaker expression of androgen receptors than age matched controls. The severity of hypospadias did not influence hormone receptor distribution. AR expression is better observed in the stroma than in the epithelium. There was no difference in ER expression between the hypospadias group (distal or proximal) and age matched normal controls. ER was expressed in larger numbers in normal older preputial tissue. The foreskin of prepubertal boys shows little to no expression of PR.

Nueva técnica de fijación de dartos en la reparación de hipospadias

ObjectiveThis study investigates the impact of the use of dartos covering to augment the neourethra on functional and cosmetic results. To evaluate a novel technique demonstrating how to fix dartos flap to cover the neourethra as a barrier in hypospadias repair. Patients and methodsThis study comprised 204 male patients with different degree of hypospadias (DPH: 132, coronal hypospadias: 46, MPH: 26). Their ages ranged from 1-23 ys (mean age: 2ys). Penile chordee was in (DPH: 45, coronal hypospadias: 33, MPH: 26). All patients had abnormal downward directed urinary stream. Hypospadias repair was performed by the classic TIP technique in addition to our novel modification of dartos covering. Patients were submitted to 5 years of follow-up including clinical examination of the functional and cosmetic parameters. ResultsSuccess rates were reported in 200 patients, 3 patients had complications with subcoronal urethrocutaneous fistula and one patient with complete repair disruption. ConclusionTriple dartos fixation is a simple technique for all hypospadiologists to minimize urethrocutaneous fistula as common complication of hypospadias repair with good functional and cosmetic results.

Analysis in the influence factors of urethroplasty in DSD

BackgroundAt present, there is no specific research on the factors affecting the success rate of urethroplasty in patients with DSD. The purpose of this study is to explore the factors affecting the success of urethroplasty in DSD patients, and to provide some reference for the surgical treatment of DSD patients undergoing urethroplasty.MethodWe reviewed patients with DSD who underwent urethroplasty from January 2016 to December 2019 retrospectively. Patients were divided into four groups: the successful group, the urethrocutaneous fistula group, the urethral diverticulum group, and the urethral stricture group. Risk factors were determined from the following data included the DSD classification, the age of first operation, length of urethral defect, degree of hypospadias, cryptorchidism, micropenis, gonad type, hormone therapy before operation, transposition of penis and scrotum, surgical strategy, urethral covering material, and postoperative catheter removal time. We explored the difference of each factor between four groups through the comparative study of single factor and multifactor logistic regression analysis of related factors.Result122 cases were enrolled in this group (n = 122), 12 cases were lost to follow-up. Median follow-up was 28 months (12–55 months).We found the success rate of operation decreased with longer urethral defect (B = − 0.473, P = 0.005). The success rate of operation was higher in staged operation and TPIT (TPIT = Transverse Preputial Island Tube operation)-related operation than primary operation (B = 1.238, P = 0.006) and TPIT-nonrelated operation (B = 2.293, P = 0.001). Although there was a significant difference between the age of the first operation and the occurrence of urethrocutaneous fistula (P = 0.006 < 0.05), there was no significant difference in logistic regression analysis (P = 0.161 > 0.05). The incidence of urethrocutaneous fistula was lower in TPIT-related operation than in TPIT-nonrelated operation (B = − 2.507, P = 0.000). The incidence of postoperative urethral diverticulum was lower in staged operation than in primary operation (B = − 1.737, P = 0.015).ConclusionFor patients with disorder of sex development undergoing urethroplasty, the length of urethral defect is an independent risk factor affecting both the success rate of operation and the urethrocutaneous fistula. The age of the first operation has a statistically significant effect on the occurrence of postoperative urethrocutaneous fistula, but it is not an independent factor. Urethrocutaneous fistula is less found in TPIT-related operation in the study. Staged operation is an independent protective factor for postoperative urethral diverticulum compared with one-stage operation but isn’t related to urethrocutaneous fistula.

A novel triple dartous fixation in hypospadias repair

ObjectiveThis study investigates the impact of the use of dartos covering to augment the neourethra on functional and cosmetic results. To evaluate a novel technique demonstrating how to fix dartos flap to cover the neourethra as a barrier in hypospadias repair. Patients and methodsThis study comprised 204 male patients with different degree of hypospadias (DPH = 132, coronal hypospadias = 46, MPH = 26). Their ages ranged from 1 to 23 ys (mean age = 2 ys). Penile chordee was in (DPH = 45, coronal hypospadias = 33, MPH = 26). All patients had abnormal downward directed urinary stream. Hypospadias repair was performed by the classic TIP technique in addition to our novel modification of dartos covering. Patients were submitted to 5 years of follow-up including clinical examination of the functional and cosmetic parameters. ResultsSuccess rates were reported in 200 patients, 3 patients had complications with subcoronal urethrocutaneous fistula and one patient with complete repair disruption. ConclusionTriple dartos fixation is a simple technique for all hypospadiologists to minimize urethrocutaneous fistula as common complication of hypospadias repair with good functional and cosmetic results.

The Effect of the Duckett procedure on the Outcome and Prognosis of Children with Suburethral Cleft.

Background Hypospadias is one of the most common malformations of the male genitourinary system. In recent years, the incidence of hypospadias is increasing year by year, which seriously affects normal urination and sexual function. Repairing hypospadias has always been a challenge in paediatric urology, requiring a variety of surgical techniques and science and art that requires intensive study. Despite the availability of over 300 surgical procedures and continuous improvement, there is still a high level of surgical complications. It is crucial to choose an appropriate and effective surgical method for the treatment of hypospadias. Aims This study aimed to investigate the outcome and prognosis of children with hypospadias, using transverse cut foreskin island flap coiled urethroplasty (the Duckett procedure). Materials and Methods A retrospective study was conducted on 100 children with hypospadias who underwent surgery in our hospital from December 2018 to December 2021. Based on the degree of hypospadias and the degree of penile curvature both in line with the Duckett procedure, the comparison group was treated with a one-stage Duckett procedure and the treatment group was treated with a staged Duckett procedure. The differences in the surgical condition, inflammatory factor levels, and complications between the two groups of children were observed and compared. Results The length of hospital stay and VAS score in the treatment group were significantly lower than those in the control group, and the operation time and intraoperative bleeding were higher than those in the control group, with a statistical significance (P < 0.05). The success rate of one operation was higher than that of the comparison group, but the statistical comparison was not statistically significant (P > 0.05). There was no statistically significant difference in the inflammatory response between the two groups before surgery (P > 0.05), while the difference in CRP, IL-6, and calcitoninogen between the two groups after surgery was significant and lower in the comparison group than in the treatment group, which was statistically significant (P < 0.05). The clinical outcome of the children in both groups showed that the excellent rate of 92.00% in the treatment group was significantly higher than that of 74.00% in the comparison group, while the incidence of complications was significantly lower than that of the comparison group, and the difference was statistically significant (P < 0.05). Complications in children with poor surgical outcomes in both groups occurred mainly, early urethral stricture and cured by urethral dilatation or condition without improvement cured by urethrotomy. Conclusion A comparative study of hypospadias treated with the staged Duckett procedure was more effective in relieving postoperative pain and inflammatory reactions in children, reducing postoperative complications and improving healing efficiency, providing some reference value for hypospadias surgery.

Outcome of stented versus unstented mid-shaft to distal hypospadias repair

AimsWe compared the outcomes of unstented repair (UR) versus stented repair (SR) in patients with mid-shaft to coronal hypospadias (HS) to elucidate if SR has any advantage over the UR.Materials and Methods:We retrospectively studied our mid-shaft to coronal HS repair patients between January 2013 and January 2018. We recorded variables such as degree of HS, age at repair, surgeon, type of repair, suture used, stent usage, and standard early and late complications. Relative risk (RR) was calculated and P < 0.05 was considered significant.Results:We included 120 patients (63 UR, 57 SR). There was no statistically significant difference in any parameters in both the groups. All had either tubularized incised plate or Thiersch–Duplay procedure. Urethroplasty was done with PDS 6/0 in all cases. Trainees performed two-third of the repairs under variable supervision. Early complications included one UR patient having urinary retention needing insertion of urethral catheter, five SR patients having bleeding/swelling, and three UR having dysuria. All were managed conservatively. For late complications, 98 patients were available (UR: 51, SR: 47) with fistula in 17 (17.3%), UR 8 (15.6%) versus SR 9 (19.1%) (P = 0.5, RR = 1.2) meatal stenosis in 3, UR 3 versus SR 0 (P = 0.06, RR = 6.4) and glanular dehiscence 6, UR 4 versus SR 2 (P = 0.25, RR = 1.8).Conclusion:There were no statistically significant differences in the short-term complications between UR and SR for HS. In the long term, RR for meatal stenosis is higher in UR.

Topical Testosterone Therapy Prior to Hypospadias Repair (Our experience at Queen Rania Al-Abdullah Hospital for Children)

Objective: As hypospadias is one of the most common congenital male anomalies, furthermore, multiple approaches and methods of treatment were mentioned over the references, we tried to put our thumbs on one of the most preoperative steps which can be missed or has multiple debates during a discussion about it. Here in our study, we focused on the rule of topical testosterone treatment on multiple patients as an advantageous step to get a higher rate of success postoperatively side by side to achieve a good penile size intraoperatively. Deep detailed analysis of side effects of topical testosterone balanced with the duration of surgery, short and long-term outcome. Methodology: The study was carried out from January 2014 to January 2019 at Queen Rania Hospital for children, Amman, Jordan. The overall number was 182 patients. We divided them into two groups: (group A) 98 boys who did not receive the hormonal treatment and (group B) were referred to the patients who underwent the topical treatment and their number was 84. The age group was between 6 months and 6 years. Testosterone gel 1% was applied as a thin layer over the ventral aspect of the penis once daily for 3 weeks before surgery. The patient’s degree of hypospadias as glandular, coronal, distal shaft, and midshaft to illustrate the benefits on each group. The local and systemic side effects of the gel were documented. Concomitantly we measure the difference in penile length, glans width along with serum testosterone level before and after applying it. After that, the mean duration of surgery was measured. The common complications of surgery were listed as fistula, glans dehiscence, meatal stenosis and the number of each complication for each group was documented and analyzed. Results: There were good results related to the mean penile length and glans width no significant change related to the serum testosterone level. No valuable change upon the duration of surgery. Local skin pigmentation with ............

Narrow urethral plate augmentation in anterior and middle hypospadias repair: Onlay flap VS. Inlay graft. A prospective randomized comparative study

The measurement of the urethral plate width (as an objective parameter) and its effect of this width on the results of tubularized incised plate urethroplasty (TIPU) have been reported in two series and both authors reported that a urethral plate width<8mm is associated with higher complication rates. The augmentation of the urethral plate either by dorsal inlay graft urethroplasty (DIGU) or Onlay flap has been compared with the original TIPU in different degrees of hypospadias with better surgical results in augmented cases. To evaluate the surgical results of longitudinal Onlay preputial flap (LOF) vs (DIGU) techniques in augmentation of the narrow urethral plate. Primary anterior and middle hypospadias cases with narrow urethral plates were randomly allocated into two groups; group one operated by DIGU and group two operated by (LOF). The Success rate, individual complication rate, and operative times were compared. 39 cases completed the study. No significant differences in patients' characteristics were detected. In the DIGU group, one case (5.3%) complicated with fistula and glandular dehiscence wherein in the LOF group, 7 cases (35%) Complicated with 5 fistulae, 2 glanular dehiscences, one flap loss, one diverticulum (p= .02). No significant differences in the rates of individual complications. Variables affecting the success of hypospadias repairs are many. Urethral plate quality is an important variable among these variables. The definition of urethral plate quality is usually subjective. In the DIGU group, only 1/19 (5.3%) case had two complications, fistula and glandular dehiscence. Mouravas etal., in their comparative study between TIPU and G-TIP without mention of urethral plate width reported a significant reduction of the overall complications and urethral stenosis without significant reduction in fistula rate. In their G-TIP group, only 2 cases out of 24 (8.3%) were complicated with one fistula and one glandular dehiscence where in the TIPU group, 7 cases (30.4%) were complicated with one glandular dehiscence and 6 cases with urethral stenosis. In the LOF group, fistula in 5/20 cases (25%) was the commonest complication followed by glandular dehiscence in 2 cases (10%). Diverticulum and flap loss occurred in one case for each (5%). In the multivariate analysis of 474 patients' cohort by Spinoit etal., there were no significant differences between the risk of re-intervention after TIP and Onlay flap in anterior and middle hypospadias (25.8% vs. 18.8%), and (22.2% vs. 20%) respectively. Our data suggest that augmentation of the narrow urethral plate with DIGU has a better surgical outcome than with LOF. No significant difference in cosmetic outcomes detected.

PT271 - Endocrine and reproductive outcome of men born with various degrees of hypospadias

PT271 - Endocrine and reproductive outcome of men born with various degrees of hypospadias

Selective Criteria and a Modified Technique of Applying "URAGPI" Technique for More Proximal Degree of Hypospadias

Selective Criteria and a Modified Technique of Applying "URAGPI" Technique for More Proximal Degree of Hypospadias

Description of a novel variant in the MAMLD1 gene in isolated hypospadias

Introduction Hypospadias is a multifactorial malformation. Among previously described genes, the MAMLD1 has recently been identified in association with the development of hypospadias. So far, there are no studies in Latin America addressing this gene. The aim of this paper is to describe the presence of variants in exon 3 of the MAMLD1 in our population. Materials and Methods Using the Bio-Repository's database of the Human Genetics Institute, we identified 51 patients between 2008 and 2012 with varying degrees of isolated hypospadias. Exon 3 was sequenced looking for polymorphisms. These were assessed with in silico prediction algorithms to describe the effect of these changes on the function and structure of the protein. Results Four patients with 3 variants were identified. The p.S364P variant has not been previously described and has a deleterious effect. The other two, rs41313406 and rs61740566, have been previously described and are not considered variations with harmful effect on the protein function. The genotype-phenotype correlation varies in severity between the variables described. Conclusions For the first time a Latin American population is molecularly analyzed. The p.S364P variation has never been described before. Variations with different degrees of hypospadias are described.

The timing factors associated with urethroplasty in hypospadias population

Objective To analyze the relationship between clinical features and age of operation on children with hypospadias, and to explore and determine the factors for hypospadias associated with age at surgery. Methods Re-trospective study was conducted in children with hypospadias undergoing operation at the First Affiliated Hospital of Zhengzhou University between January 2011 to December 2016.The demographics, the degree of hypospadias, complications, age at first urethroplasty and the other factors in patients were recorded.t-test was used to determine the factors associated with surgical timing and the difference was significant when P<0.05. Results A total of 1 051 cases were included in the study.Among these patients with hypospadias 268 cases(25.5%) were of mild cases, 525 cases(50.0%) of moderate, and 258 cases(24.5%) of severe.Nine hundred and forty-six cases(90.0%) did not have complications, while 105 cases(10.0%) had complications.The median age at first urethroplasty was 5.3 years old and 66.22% of them were operated after 3 years, and 4.09% of them were after 18 years old.Insurance types, household income, race did not influence with surgical timing statistically (P>0.05). Multivariate analysis showed that the several factors were significantly associated with time of surgery: the severity of hypospadias (t=28.10, P=0.000), with or without complications (t=64.17, P=0.000), urban-rural gap (t=7.49, P=0.000), parental education level (t=36.90, P=0.000) and the age of receiving advice from pediatric surgeon (t=5.00, P=0.000). Conclusions The age of surgery in children with hypospadias were significantly affected by the severity of hypospadias, the presence of complications, differences between urban and rural areas, the degree of parental education, and the time of medical advice in the pediatric department of urology, and the education of hypospadias knowledge for children′s parents and related medical personnel at the grass-roots level will advance the age of surgery in children with hypospadias. Key words: Hypospadias; Timing of surgery; Urethroplasty

Single penile incision for combined hypospadias and inguinal surgery: A comparative study.

We sought to compare the surgical outcomes of hypospadias repair with correction of inguinal pathology using a single penile incision vs. conventional approach using two incisions. This is a retrospective study that reviewed all patients who underwent concurrent surgical repair for both hypospadias and inguinal pathologies between January 2003 and November 2015. Patients were classified into Group A, conventional (inguinal or scrotal and penile incision) approach; or Group B, single penile incision approach. Baseline characteristics, including age, degree of hypospadias, type and laterality of inguinal pathology, operative time, and surgical outcomes, were collected. Between groups, variable comparisons were analyzed using Mann-Whitney U-Test and Fisher-exact test. Statistical significant set at <0.05. Seventy-six patients (Group A: 40; Group B: 36) were eligible for study. Baseline characteristics of both groups were comparable, with no significant statistical difference. Overall mean operative time for Group A was 139.3 ± 56.2 minutes, while Group B was 107.8 ± 46.7 minutes (Z=2.6; U=470.5; p=0.009). Two patients in Group A and two patients in Group B had testicular ascension, all of which also had hypospadias-related complications (p=1.0). Hypospadias-related complications in Group A included seven urethrocutaneous fistulae and two repair dehiscence. Eight urethrocutaneous fistulae, one urethral stricture, and two repair dehiscence occurred in Group B (p=0.448). Surgical outcome appearance in both groups were comparable, with no statistically significant difference (p=0.466). Single penile incision for both hypospadias repair and correction of inguinal pathology is a feasible technique and comparable to the conventional approach, with similar surgical outcomes and shorter overall operative time.

Tubularized Incised Plate Urethroplasty for Primary Hypospadias Repair: Versatility versus Limitations

Introduction: Several techniques have been described for the repair of hypospadias but without any definitive privilege of one technique over the others. The choice of the repair remains largely dependent on the judgment and personal experience of the operating surgeon.Aim of Study: The aim of this study was to identify the most commonly encountered complications following tubularized incised plate (TIP) urethroplasty and their rate of occurrence in relation to different forms of hypospadias.Patients and Methods: This study was conducted on patients with different degrees of hypospadias (ranging from distal penile to scrotal hypospadias) who underwent primary TIP urethroplasty. All included cases were operated by the same surgeon (the author) at two tertiary centers for pediatric surgery during the period 2007 through 2016. Data analysis was performed in a retrospective manner based on retrieved medical records, in addition to saved digital photography documenting the preoperative phenotypic severity of the hypospadiac phallus, operative steps, appearance at follow-up visits, postoperative investigations, and reoperations.Results: We retrieved data of 193 patients with different degrees of hypospadias who underwent a primary TIP urethroplasty during the 10-year period of the study. All cases in the study completed their early postoperative follow-up (at 1 and 4 weeks). Approximately 40% of cases completed more than 1-year follow-up (mean: 2.3 years, median: 2 years). Rate of reoperation was 21.7% (20 cases for fistula, five recurrences of chordee, one meatal stenosis, and 16 skin refashioning and correction of penoscrotal interposition). All cases who returned for reoperation underwent urethral calibration under anesthesia±cystoscopy.Conclusion: TIP urethroplasty is a versatile technique that can be used for the repair of different degrees of hypospadias with low rate of complications. The main limitation is the presence of considerable chordee (moderate or severe) when urethral plate preservation and dorsal penile plication might be a suboptimal way of management that is liable for recurrence of the ventral curvature

Is approximated de-epithelized glanuloplasty beneficial for hypospadiologist?

ObjectiveFurther evaluation of the cosmetic and functional results of approximated de-epithelized glanuloplasty in different degree of hypospadias. Material and methodsThis study included 96 male patients (DPH=68 and MPH=28). Patients selected for repair with glans approximation should have wide urethral plate and grooved glans. All cases were repaired with the classic TIP and glans approximation technique. Follow up was for 1 year by clinical examination of the meatal shape, size and site, glans shape, skin covering, suture line, urethral catheter, edema and fistula in addition to parent satisfaction. ResultsMean operative time was 49±9min. As regards the functional and cosmetic outcomes, success was reported in 95.8%, while failure was in 4.16% in the form of glanular disruption in two patients and subcoronal urethrocutaneous fistula in another two patients. ConclusionGlans approximation has many advantages, good cosmetic and functional results, short operative time, less blood loss, no need for tourniquet.

¿Es la glanduloplastia con desepitelización beneficiosa para el hipospadiólogo?

ObjectiveFurther evaluation of the cosmetic and functional results of approximated de-epithelized glanuloplasty in different degree of hypospadias. Material and methodsThis study included 96 male patients (DPH=68 & MPH=28). Patients selected for repair with glans approximation should have wide urethral plate & grooved glans. All cases were repaired with the classic TIP and glans approximation technique. Follow up was for one year by clinical examination of the meatal shape, size & site, glans shape, skin covering, suture line, urethral catheter, edema & fistula in addition to parent satisfaction. ResultsMean operative time was 49±9minutes. As regards the functional and cosmetic outcomes, success was reported in 95.8%, while failure was in 4.16% in the form of glanular disruption in two patients and subcoronal urethrocutaneous fistula in another two patients. ConclusionGlans approximation has many advantages, good cosmetic and functional results, short operative time, less blood loss, no need for tourniquet. Study of a large number of cases and comparing glans approximation with the classic TIP technique.

Can separation of the scrotal sac in proximal hypospadias reliably predict the need for urethral plate transection?

One of the main challenges in proximal hypospadias repair is correcting curvature. The best technique to achieve this remains the object of debate. Indeed, some authors believe the urethral plate should be kept and used as often as possible. In some cases, however, even after extensive mobilization and dorsal plication, significant curvature remains and it is necessary to transect the urethral plate. Having a reliable pre-dissection marker of the need for urethral transection would be useful in choosing a technique.We wanted to determine if presence of marked separation of the scrotal sac (SSS), also referred to as bifid scrotum, could reliably predict the need for urethral plate transection. We prospectively enrolled a series of boys with proximal hypospadias. We noted age, degree of hypospadias, meatal position, presence of cryptorchidism, and presence or absence of SSS. During surgery we fully degloved the penile shaft, freeing all ventral tissues, and radically dissected the more proximal bulbar urethra. We then performed an erection test. If there was residual curvature <30° we performed a dorsal plication, if it was >30° we transected the urethral plate. Twenty-nine patients were included, of whom 18 presented SSS. The average age was comparable in both groups, as was type of hypospadias and meatal position. We estimated transection of the urethral plate to be necessary in 15 out of the 18 children with SSS, and 2 out of the 11 children without SSS. The relative risk for requiring urethral plate transection in case of SSS in this series was 4.58. Techniques that commit to urethral plate transection are criticized because they preclude using the urethral plate. In our study presence of SSS was predictive for the need to transect the plate. Obviously one can decide to keep the urethral plate at all cost, and mobilize it more than we did, or accept more residual curvature, but in reality our aim was to determine a preoperative marker allowing us to define a patient category. We believe presence of SSS is a marker of severity, and that this "severity" translates into "a less usable urethra". As recent studies caution us about the evolution of the reconstructed native urethra and the possibility that it may not grow as well as the other penile tissues, we believe this extra information could influence the surgeon's decision as to the most appropriate technique for each patient.

Phenotype and molecular characteristics in 45 Chinese children with 5α-reductase type 2 deficiency from South China.

Affected by steroid 5α-reductase type 2 deficiency (5α-RD2), 46, XY individuals present divergent phenotypes characterized by undervirilization of male external genitalia. To identify the disorder, mutational analysis of 5α-reductase type 2 gene (SRD5A2) is a reliable approach. The genotype-phenotype relationship has not been elucidated. To improve the diagnosis and expand the knowledge of the disease, we collected and analysed relevant data of clinical diagnosis, biological investigation and molecular determination in 45 children with the SRD5A2 gene mutations from South China in our centre. We studied a cohort of 45 Chinese children with SRD5A2 gene mutations. Isolated microphallus (35·6%) and microphallus associated with various degrees of hypospadias (55·6%) were frequent phenotype. Female external genitalia with clitoromegaly (8·9%) were rare. 16 of 18 (88·9%) cases had hCG-stimulated T/DHT ratio above 10. In 45 patients, we identified 15 different mutations. Five have never been described: p.His90ThrfsX31, p.Gly21Arg, p.Gly149Asp, p.Arg145Leu and p.Gly66Arg. The p.Arg227Gln mutation was detected in 41 (91·1%) patients. The p.Leu89Val polymorphism was found in 38 (84·4%) patients. Homozygous mutations were presented in 16 (35·6%) patients, compound heterozygous mutations in 20 (44·4%) patients, compound heterozygous mutations alone with the p.Leu89Val polymorphism in nine (20·0%) patients. Exons 1 and 4 were most affected, and the number of mutant alleles per exon was 78·1% and 12·2%, respectively. The study demonstrated a wide spectrum of phenotypes, biological profiles and genotypes in the children with 5α-RD2 from South China. The heterozygous mutation p.Arg227Gln is presumably a hot spot mutation and suggests a founder effect in the population of South China that may explain a moderate phenotype among our patients. Our report provides new insights into the molecular mechanism of 5α-RD2 and help to the diagnosis and treatment of this disease.

The Complication Rate after Hypospadias Repair and Correlated Preoperative Symptoms*

Aim: To assess the rate of complications following hypospadias repair in a consecutive series of boys and the correlations of those complications with their preoperative symptoms, degree of hypospadias and method of operation. This study was conducted to address the question of whether all boys with all degrees of hypospadias should undergo reconstruction. Methods: This was a prospective cohort study. We included every boy who underwent an operation for the primary repair of hypospadias between January 2011 and April 2014. The median follow-up time was 24 months. The study ended in October 2014. The main outcome measurements were the frequency of postoperative complications and their correlations with the degree of hypospadias, the preoperative symptoms and the operative intervention performed. Results: Among the 76 boys who underwent operations, 23 had degree 1, 47 had degree 2, and 6 had degree 3 hypospadias. Preoperatively, 43 of the boys had symptoms that motivated the operation, including stenosis (38), a curvature (10) or both (5). Forty-three boys underwent operations with the MAVIS technique, 28 underwent TIP repair, 1 underwent a Duckett procedure, and 4 underwent Byar two-stage procedures. There were complications requiring reoperations including fistulas or ruptures in 26 (34%) boys. There were no significant differences in the rates of complications with surgery, fistulas (P = 0.4775), ruptures (P = 0.2417) or other complications (P = 0.5165) between the groups with or without preoperative symptoms, those with different degrees of hypospadias or those who underwent different operative methods for repair. Conclusions: The complication rate in this series was high. The study was prospective, and no boy was lost during follow-up. Because the complication rate did not correlate with the degree of hypospadias nor the preoperative symptoms, there may be a group of boys with hypospadias without symptoms for whose operations are questionable. The preoperative symptoms should be reported in future reports of the results of hypospadias surgery.