Cases Of Hypospadias Research Articles

Vertical plication: A penile curvature correction technique that reduces the need for urethral plate transection in penoscrotal hypospadias

Penile curvature (PC) is a frequent component associated with hypospadias. Medial corporal rotation by interrupted suturing without incising the corporal bodies is well described in patients with epispadias and we think that it is an alternative technique for the management of patients with ventral PC, with or without hypospadias. We describe a PC correction technique which reduces the need for urethral plate transection in penoscrotal hypospadias. The main steps of "vertical plication" technique are following: The Buck's fascia at the maximum point of curvature was incised longitudinally at 12-o'clock position and then dissected from tunica albuginea from medial to lateral on each side. Minimal and precise dissection just enough to allow plication is essential to avoid injury to the neurovascular bundle. No incisions were made through the tunica albuginea. Corporal rotation was performed by approximating with polyester sutures which were placed 5mm apart at and around the point of maximum curvature. Three to 5 sutures were enough for full straightening of PC in our cases. Follow-up range was 1.5-4.5 years (mean: 3 years). 17 patients underwent this technique. 16 of them had a penoscrotal hypospadias and one patient had congenital PC without hypospadias. Full PC correction was achieved in 15 patients and residual curvature below 10° was seen in two patients. We were able to perform single-stage repair in 13 (81,25%) of our penoscrotal hypospadias cases without transection of urethral plate. High recurrence rates were reported in dorsal plication technique when compared to ventral corporal lengthening in patients with PC>30°. In the presence of high grade PC, a penile elongation technique is the preferred option for many authors. However, it requires incision of tunica albuginea. Although the variations of medial corporal rotation to correct ventral PC has been described in the past, they did not gain popularity. This technique allows the surgeon to proceed with single stage repair in patients with proximal hypospadias associated with high grade PC. Narrowing is the only disadvantages of our technique, which can easily be resolved by de-epithelialized flap coverage harvested from foreskin. No parents subjectively reported nor we detected narrowing, recurrence or shortening during follow-up.

Prenatal Diagnosis and Classification of Fetal Hypospadias: The Role and Value of Magnetic Resonance Imaging.

Prenatal diagnosis and classification of hypospadias are difficult and of value for management during perinatal and neonatal periods. The conventional approach for prenatal diagnosis of hypospadias is ultrasound; however, this technique may be inconclusive in certain cases, which prompts for further exploration with magnetic resonance imaging (MRI). To investigate the role of MRI in the prenatal diagnosis and classification of fetuses with hypospadias. Retrospective. Thirty-five fetuses (median gestational age=37, range 24-39 weeks) with possible hypospadias. Single-shot fast spin echo T2-weighted imaging, fast imaging employing steady-state acquisition (FIESTA), and three-dimensional FIESTA acquired at 1.5T. Diagnosis and classification of hypospadias using MRI were performed by three experienced radiologists based on MRI features, including a short penile shaft, abnormal penile tip, penile curvature, bifid scrotum, "tulip sign," and penoscrotal transposition. The accuracy of MRI in the diagnosis and classification of hypospadias was assessed in comparison to postnatal clinical diagnosis. The interobserver agreement between radiologists was also assessed. Kendall's W test was applied to assess the interobserver agreement between radiologists. Taking postnatal clinical diagnosis as the reference standard, the sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were calculated. Of the 35 fetuses, 24 cases were confirmed as hypospadias through postnatal clinical diagnosis. The interobserver agreement between radiologists was substantial (Kendall's W= 0.781, P< 0.001). Of the 24 confirmed cases (13 cases of severe hypospadias and 11 cases of mild hypospadias), 22 cases were correctly diagnosed by MRI. The accuracy of MRI in the diagnosis of hypospadias, severe hypospadias, and mild hypospadias was 85.71%, 82.86%, and 80.00%, respectively. MRI has good performance in the diagnosis of fetal hypospadias. In addition, MRI could help evaluate the severity of fetal hypospadias. 3 TECHNICAL EFFICACY: Stage 2.

Proximal hypospadias and 46XY disorder of sex development; which patient with hypospadias needs to be investigated?

Aim: This study examines the distribution of genital abnormalities based on physical examination of our patients with 46, XY disorders of sex development (46, XY DSD), and aims to define severity and frequency of hypospadias in 46, XY DSD. Hypospadias is a relatively prevalent congenital anomaly. Although genetic, environmental and hormonal factors are considered to be responsible, etiology is not clarified in several hypospadias cases. Materials and Methods: Clinical, laboratory and genetic records of all cases with 46, XY DSD, who were evaluated by the sex determination monitoring board were retrospectively reviewed. In the diagnosis, hypospadias cases were examined in terms of the place of hypospadias and coexisting other external genital findings. Results: There were 72 patients with 46, XY DSD. 5-α reductase deficiency [n=32 (44.4%)] was the most commonly encountered diagnosis followed by androgen insensitivity syndrome [n=26, (36.1%)]. Proximal hypospadias were presented in 44.4% (n: 32) of the cases and only 6 of them (18.8%) were isolated hypospadias. In 81.2% of these cases, at least one of the anomalies such as cordi, bifid scrotum, undescended testis and micropenis accompanied proximal hypospadias. None of the distal hypospadias cases were referral clinic finding. Conclusions: 46 XY DSD is a heterogeneous group of patients with a varying age of presentation and a diverse clinical profile. It can be stated that proximal hypospadias is the most common referral clinic finding of 46, XY DSD, and the risk of 46, XY DSD increases with the intensifying degree of hypospadias and the presence of coexisting genital abnormalities such as cordi, bifid scrotum, undescended testis and micropenis.

Adolescent and Adult Hypospadias Repair: A Retrospective Study

Introduction: There is a general consensus that results of hypospadias repair surgery, done on children, seem to have a better outcome when compared to the adults. Hypospadias cases in adults can present as primary or with complications resulting from an attempted repair. Aim: To assess any difference in outcome of surgeries done among the adolescent and adult population and to evaluate the use of a Tunica Vaginalis (TV) second layer barrier flap in preventing complications such as urethrocutaneous fistula. Materials and Methods: This was a single centre retrospective observational assessment of the adolescent and adult hypospadias surgeries conducted at a Tertiary Care Hospital from January 2016 to July 2019. The assessment was based on the presentation, site of hypospadias meatus, previous surgery and development of any complications or recurrence. Routine surgeries as per the hospital protocol were done in these patients. Follow-up was done for the next 12 months. For data analysis tables, columns and pie charts were used. Fisher’s-exact test was used to find the association and calculate the p-value with the help of SPSS 21.0 software. Results: Total 28 patients were assessed, of which 17 patients were adolescents (mean age 14.6±1.66 years) and 11 (mean age 22.7±3.51 years) were adult. There was no significant (p-value 0.581) difference in the final outcome of hypospadias repair surgery between the adolescent and adult population. Use of TV interposition barrier flap prevented development of complications. Urethrocutaneous fistula was the most common form of complication noted in both the groups overall rate of complication was 14.2%. Conclusion: When hypospadias repair in adolescents and adults is done in a systematic and meticulous fashion, the outcome was satisfactory and there was no significant difference in outcome among the two age groups. Use of TV flap as a second layer interposition can improve the outcome in such patients.

A rare case of female hypospadias associated with congenital heart disease

Female hypospadias is a rare lower urogenital female anomaly. Hypospadias is primarily a congenital defect of males in which the urethra opens on the underside (ventral aspect) of the penis and is one of the most common congenital anomalies in the USA, occurring in about one of every 125 live male births. The corresponding defect in females is opening of the urethra into the vagina, and is quite rare and often associated with other genitourinary anomalies such as Cloacal malformation, female pseudo hermaphrodism, nonneurogenic neurogenic bladder and urethral duplication. Early diagnosis requires a high index of suspicion. Early treatment plays an important role in reducing the psychological and psychosocial impacts, decreases indolent kidney damages and prevents urinary incontinence. Although, interestingly, sometimes, with conservative management and close follow-up (like in our case), symptoms and signs may get better spontaneously over time. The authors report on a 4-year-old girl with hypospadias presented by hydronephrosis and recurrent urinary tract infection in infancy associated with congenital heart disease who was closely observed until spontaneous resolution of her hypospadias.

Foreskin healing after distal hypospadias repair: Does stenting affect the outcome?

Distal penile hypospadias account for about 70% of all cases of hypospadias. There is a variety of operative techniques that could be performed when foreskin reconstruction is an option. The urethral stent is left in the urethra to prevent complications. To determine whether the duration of stenting influences the healing of foreskin after distal hypospadias repair. Data from 2 institutions was retrospectively analyzed. Inclusion criteria were as follows: 1) a modified meatal advancement glanuloplasty without tubularization of the urethral plate, 2) foreskin reconstruction and 3) follow-up - 12 months. All other types of reconstruction and re-do procedures were excluded. The period of urethral stenting was determined intraoperatively depending on the surgeon's preferences. Mean age at operation was 23.3 months. The cohort was divided into 3 groups. In Group I (G-I), no catheter was left or it was removed the next day after surgery. In Group II, the catheter was left for more than 5 days. In those 2 groups, the surgery was done by different surgeons. Group III consisted of 35 patients who had a stent for <2 days, and the procedure was performed by the same surgeon. The χ2 with Yates's correction and Pearson's χ2 tests were used for the statistical analysis. Overall, 11 patients had foreskin dehiscence and needed re-do surgery. None of the patients required operation because of foreskin stenosis. Complications occurred in 3 out of 33 patients (9%) in Group I, 2 out of 27 in Group II (7.4%) and 6 out of 35 in Group III (17%). There was no statistically significant difference between Groups I and II (p = 0.8144), nor between Groups I and III (p = 0.5344). In the non-parametric Pearson's χ2 test, no significant difference was found in such grouped data (p = 0.4239). Prolonged urethral stenting does not reduce the risk of a re-do foreskin surgery after hypospadias repair.

Is primary adult hypospadiatic prepuce different from healthy prepuce in terms of microvascular density and androgen-estrogen-progesterone receptors?

This study aims to determine the histological data such as microvessel density, nerve density, and the androgen, oestrogen and progesterone receptor density in the prepuce of primary distal hypospadias cases in adulthood, compared to that of healthy individuals in the same age group. Between 2014 and 2019, we prospectively evaluated adult hypospadias and adult circumcision patients. A total of 28 patients were included: Group 1 (18 patients) primary distal hypospadias and Group 2 (10 patients-control group) healthy individuals who had a previous circumcision request for social/religious reasons. The prepuce of healthy individuals that were excised after the circumcision and the excised parts excluded from the prepuce that was used in reconstruction during the repair of hypospadias, were shaped and stored to be able to perform a study by the pathology clinic. Histopathological findings on adult distal hypospadias cases showed that the microvascular density and inflammation in the prepuce with hypospadias were found to be increased compared to the healthy prepuce and the density of androgen and oestrogen receptors was similar in both groups. Unlike childhood studies, in this study with adults, the progesterone receptor was detected in both groups and found to be significantly lower in the hypospadias group.

Preoperative topical testosterone for penile hypospadias repair `a comparative study`

BackgroundAlthough the outcome of hypospadias repair has much improved, repair of a small-sized phallus is still challenging. Whether preoperative hormonal therapy is beneficial remains controversial.In this report, we prospectively conducted a clinical trial comparing two groups of patients regarding the effect of preoperative topical testosterone application on the outcome of primary hypospadias repair.ResultsThe study included 40 cases of primary hypospadias with a small glans. Their age ranged from 6 months to 3 years. Patients were divided into two equal groups. Group A were treated by local application of testosterone topical gel 1% for 30–40 days and stopped one month before operation, while cases in group B did not receive preoperative hormonal treatment. Genital skin biopsies were taken for histopathological examination. For group A, penile measurements were documented before and after hormonal therapy.There was a significant increase in all penile parameters after hormonal stimulation in group A. Interestingly, the ventral penile length proximal to the meatus showed more growth in response to hormonal stimulation compared to ventral penile length distal to meatus.We did not find significant difference between both groups regarding the rate of postoperative complications. Also, the cosmesis and parent satisfaction were ‘more or less’ similar. On the histopathological level, we could not detect a difference between both groups regarding micro-vessel density using CD31, which is a vascular marker indicating neovascularization supposed to play a fundamental role in the healing process.ConclusionThe use of preoperative topical testosterone significantly increases the size of the hypospadiac phallus. However, this increase in size appears to be disproportionate on the ventral penile surface and has not been reflected on improving the surgical outcome.Level of evidenceThis is a case control study (level III evidence).

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.

Long-Term Outcomes of Distal Hypospadias Repair: A Patients' Point Of View.

To assess the long-term outcomes of patients treated for distal hypospadias. Assessment of long-term follow-up for a homogeneous population with hypospadias is difficult and there has consequently been a paucity of publications in this regard. A retrospective review was carried out to compile cases of distal hypospadias operated at our center between 1990 and 1999 according to the MAGPI procedure. Four parameters were evaluated based on four validated questionnaires: Health-related quality of life (SF-36), Genital self-perception (PPS), Self-esteem (Rosenberg Self-Esteem Scale), and Erectile function (IIEF). A total of 77 patients who had undergone MAGPI surgery for hypospadias during the specified period were selected. Sufficient clinical data were available for 51 patients and only 15 of these patients were included, after a median follow-up of 22 years (20-26). Their outcomes were compared with those for a population of 15 matched circumcised men and 15 matched uncircumcised men. No significant difference was found between the patients and the control groups in terms of the score for quality of life (p=.29). There were, however, significant differences in the scores for self-perception of the penile cosmetic appearance (13.3 vs. 15.8; p<.01), self-esteem (30.6 vs. 35.8; p<.01), and erectile function (31.4 vs. 33.7; p=.04) between the patients and the controls. Lower self-esteem correlated with poor genital self-perception (r =.92). This study confirms that adult patients operated for distal hypospadias have poor genital self-perception. This poor genital perception correlated with lower self-esteem.

Expression of Mafb is down-regulated in the foreskin of children with hypospadias

Hypospadias is the second most common congenital malformation in males. Although the aetiology of hypospadias is not clear, it is generally thought to be affected by both genetic and environmental endocrine-disrupting factors that affect the development of the urethra, leading to deformity. To investigate the difference in expression of the transcription factor Mafb in hypospadias and normal penile tissues and to assess whether it is related to the occurrence of hypospadias. Penile tissue was obtained from children with hypospadias who underwent surgical repair at the Children's Hospital of Chongqing Medical University. Patients diagnosed with undescended testicles, intersex status or endocrine abnormalities were excluded from the study. Twenty-five cases with hypospadias (average 3.5 years old) and 15 cases with circumcisions (as control) (average 5 years old) were included in this study. Real-time quantitative polymerase chain reaction, Immunochemistry and Western blot were used to detect the expression of Mafb. Mafb mRNA expressions in the prepuce of cases with hypospadias was significantly reduced compared with that in the controls [(1.179±0.1275), (0.6652±0.07506), p<0.05)]. Hypospadias cases also showed decreased Mafb protein expression in the preputial subcutaneous mesenchymal cell layer. Mafb protein levels were significantly decreased in those with hypospadias compared with controls [(1.932±0.1139), (1.006±0.03312), p<0.05]. However, no such differences were found in Mafb expression between subjects with mild and severe hypospadias. Compared to the normal foreskin, expression of the Mafb gene was down-regulated at both mRNA and protein levels, which was consistent with our RNA-seq sequencing results in Diethylhexyl phthalate (DEHP)-induced hypospadias rats. This study is the first to report abnormal expression of Mafb in the preputial tissue of hypospadias cases. An in-depth study of the relationship between Mafb and cell proliferation, apoptosis, and urethra development may reveal the pathogenesis of hypospadias. Expression of the Mafb gene and protein in the foreskin of children with hypospadias is lower than that in normal foreskin. We postulate that such abnormal expression of the Mafb gene may be related to the occurrence of hypospadias and that this abnormal expression may affect the development of the urethra during the embryonic period.

10 year institutional experience of use of buccal mucosal grafts for complex urethral reconstruction for varied indications with its outcome

Background: The objective of the study was to evaluate long term efficacy and outcome of use of buccal mucosal graft (BMG) for urethral reconstruction in varied urologic conditions in children.Methods: We retrospectively reviewed the medical records of 41 patients from 2009 till 2019 in our institution in which BMG was used for urethral reconstruction. Clinical findings along with surgical techniques used were noted for these patients. Postoperative outcome and complications were evaluated.Results: Mean age was 6.8 years and mean follow up was for 4 years. Out of 41 patients, BMG for substitution urethroplasty was used in 25 cases of hypospadias, 4 cases of urethral stricture, 6 cases of 46 XY disorders of sexual disorders, 4 cases of Y-duplication of urethra, and 2 cases of redo-epispadias repair. 11 patients underwent one stage repairs with a success rate of 63% and 30 patients underwent two stage repair with a success rate of 66%. Analysis and comparison of the outcome in relation to the type of repair, meatal position and number of surgical procedures prior to BMG urethroplasty was statistically insignificant.Conclusions: Buccal mucosa is an ideal graft substitute for urethroplasty. Two stage reconstructions has a slightly higher success rate than one stage reconstruction but the choice of the technique must be based on patients characteristics and on surgeons preference.

Spatial analysis of hypospadias cases in northern France: taking clinical data into account

BackgroundStrong evidence for a causal role of environmental factors in a congenital anomaly is still difficult to produce. The collection of statistical data is crucial for gaining a better understanding of the epidemiology and pathophysiology of these anomalies. We aimed to evaluate spatial variations in hypospadias within our region and it’s association to socioeconomic and ecological factors, taking clinical data into account.MethodsAll boys with hypospadias born in northern France and seen in Lille University Medical Center (Lille, France) between 1999 and 2012 were included in the analysis. We retrospectively collected geographic data, clinical data (especially known confounding factors associated with an elevated risk of hypospadias), and demographic, socio-economic and ecological data. We analyzed the entire study population and subsequently the subset of boys lacking confounding factors.ResultsThe study sample of 975 cases of hypospadias over the 13-year period resulted in an incidence of 25.4/10,000 male births, and was characterized by significant spatial heterogeneity (p < 0.005) and autocorrelation (p < 0.001). We detected two high-incidence clusters that differed with regard to their land use. After the exclusion of 221 patients with confounding factors, two high-incidence clusters with significant disease risks (1.65 and 1.75, respectively; p < 0.001) and a significant difference in land use (p < 0.001) again appeared. The first cluster contained a higher median [interquartile range] proportion of artificialized land (0.40 [0.22;0.47]) than the remaining “neutral areas” (0.19 [0.08;0.53]) did (p < 0.001). Conversely, the second cluster contained a higher median proportion of rural land (0.90 [0.78;0.96]) than the “neutral areas” (0.81 [0.47;0.92]) did (p < 0.001). The median deprivation index was significantly lower in the urban cluster (0.47 [0.42;0.55]) and significantly higher in the rural cluster (0.69 [0.56;0.73]) (p < 0.001).ConclusionsOur results evidenced the heterogeneous spatial distribution of cases of hypospadias in northern France. We identified two clusters with different environmental and social patterns – even after the exclusion of known confounding factors.

Association between prenatal exposure to ambient particulate matter and risk of hypospadias in offspring: A systematic review and meta-analysis

BackgroundEpidemiological studies on whether prenatal exposure to PM2.5, PM2.5-10, and PM10 increases the risk of hypospadias are limited and the results are inconsistent. ObjectivesWe conducted this systematic review and meta-analysis to evaluate these associations. MethodsRelevant studies were identified by searching the PubMed and Embase databases up to the end of February 2020. Observational studies that investigated the relationships between prenatal exposure to PM10, PM2.5, and PM2.5-10 and the risk of hypospadias in offspring were eligible for the review. The risk of bias was evaluated using the Newcastle-Ottawa scale. Random-effects models were used to estimate pooled relative risks (RRs) and 95% confidence intervals (CIs) for the associations. ResultsNine studies (two cohort and seven case-control studies) with a total of 2806 hypospadias cases were included. Eight studies were at low risk of bias and only one study was at high risk of bias. Five studies reported the association between per interquartile range increase in PM2.5 exposure within the first trimester and the risk of hypospadias. The pooled RR was 1.17 (95% CI: 1.00, 1.36). Two studies reported the per interquartile range increase in PM2.5 exposure during 1 month before pregnancy associated with the risk of hypospadias, with a pooled RR of 1.25 (95% CI: 1.03, 1.51). No association was observed between PM2.5-10 and PM10 exposure during pregnancy and the risk of hypospadias in offspring. ConclusionThis study suggested a modest association between prenatal PM2.5 exposure during 1 month before pregnancy or within the first trimester and the risk of hypospadias in offspring. Further large-scale cohort studies are required to verify this association.

The feasibility of urethral plate preservation in proximal and mid-penile hypospadias: sequential and anatomical approach to the repair

BackgroundThe assessment of a child having hypospadias with chordee/ventral curvature (VC) and meatal position that appears to be proximal/mid-penile can be challenging with regard to the choice of chordee correction and type of urethroplasty. The feasibility of preservation of native urethral plate is dependent on these choices. Classically, urethral plate resection of the corpora is performed to correct severe VC, whenever VC is presumed to be due to short urethral plate. But VC is seen to persist in many such cases, in spite of this maneuver that commits the patient to a more complex reconstruction. The aim of the study was to analyze the variation in preoperative and intraoperative assessment of proximal and mid-penile hypospadias, with respect to severity and possible surgical options, in patients treated by a standard protocol of sequential and anatomical approach to repair. The other objective was to ascertain if “extensive ventral urethral detethering” (in all cases) and “urethral plate augmentation” (in select cases) could facilitate more anatomical repair in these cases, with urethral plate preservation and utilization for repair wherever feasible.ResultsFifty-two children who underwent the specific protocol of repair during the 5-year period from January 2014 to December 2018 at a tertiary teaching institution were included in the study. The essential principles adopted for the surgery were as follows: (i) urethral plate preservation in all possible situations; (ii) “extensive ventral urethral detethering” up to the bulb, as a preliminary step, (iii) sequential correction of VC with urethral plate augmentation (in select cases); and (iv) selecting the optimal repair based on the following principles: (a) urethral plate tubularization, (b) urethral plate augmentation, and (c) urethral plate substitution. There was considerable difference in the preoperative and intraoperative assessment of meatal position, with 12 of the apparently mid-penile hypospadias being reclassified as distal penile after preliminary dissection. Three of the penoscrotal variants were regrouped as proximal penile and 6 of the proximal penile were regrouped as mid-penile type. Majority of cases which were deemed to require complex reconstruction on preoperative assessment could be treated by a single-stage procedure.ConclusionThe meatal position is seen to shift distally in select cases of proximal or mid-penile hypospadias after thorough degloving, dartos release, and detethering of Buck’s fascia over ventral urethra. Urethral plate can be preserved for utilization in repair in many of these cases by augmenting the urethral plate, enabling a more anatomical neourethral reconstruction. Resection of urethral plate can be thus restricted to the most severe cases of VC.

Comparison of Two Tubularized Incised Plate Urethroplasty Techniques in Hypospadias Reconstructive Surgery

BACKGROUNDHypospadias repair is a challenging type of urogenital reconstructive surgery for which different techniques are currently used. The purpose of this study is to determine the outcomes of distal, mid-shaft and proximal hypospadias repair using two new variations of tubularized incised plate (TIP) urethroplasty (TIP-δ and TIP-ελ) and to compare their complication rates with other already known operative techniques made from the same surgical team.METHODSThis study included 269 boys with hypospadias. The preoperative meatal site was distal in 179 patients, mid-shaft in 44 and proximal in 46. The average age at the operation was 17 months. The technique applied in distal hypospadias was Mathieu in 77 patients, Snodgrass in 28 and (TIP)-δ in 74. The technique applied in mid-shaft hypospadias was a tubularized island flap (TIF) in 12 patients, onlay island flap (OIF) in 5 and TIP-ελ in 27. The operative technique for proximal hypospadias was TIF in 15 patients, OIF in 10 and TIP-ελ in 21. TIP-δ and TIP-ελ are two new variants of TIP operation that we have used in our clinic since 2010. Postoperative complications were recorded, and we compared the outcomes obtained by applying the techniques.RESULTSThe use of TIP-δ in the distal hypospadias and long TIP-ελ in the mid-shaft and proximal hypospadias resulted in significantly fewer complications than the other surgical methods across all cases of hypospadias (p<0.05).CONCLUSIONThe type of tissue used for neourethral coverage seems to play an important role in the outcome of hypospadias surgery.

Case Report: Disorder of Sex Development 46, XY with Proximal Hypospadias in a 10-Year-Old Twin Boy: Disorder of Sex Development 46, XY with Proximal Hypospadias

Hypospadias is a congenital defect in which the opening of the urethra is located on the ventral side of the penis proximal to the tip of the glans penis. The cause of hypospadias is still not clear and believed to have several factors. Hypospadias occurs due to disruption of urethral fold fusion at the 8 th – 15 th week in the uterus which is influenced by dihydrotestosterone. Hypospadias can cause a disorder of sex development according to the conditions. A case of proximal hypospadias was reported to occur in a 10-year-old twin boy accompanied by undescended testis causing a disorder of sex development. For this case, various examinations were carried out along with evaluations to establish the diagnosis, the cause, the treatment plan, and the necessary evaluation. Keywords : hypospadias, disorder of sex development, undescended testis DOI: 10.7176/JHMN/80-01 Publication date: September 30 th 2020

Altered mechanisms of genital development identified through integration of DNA methylation and genomic measures in hypospadias

Hypospadias is a common birth defect where the urethral opening forms on the ventral side of the penis. We performed integrative methylomic, genomic, and transcriptomic analyses to characterize sites of DNA methylation that influence genital development. In case–control and case-only epigenome-wide association studies (EWAS) of preputial tissue we identified 25 CpGs associated with hypospadias characteristics and used one-sample two stage least squares Mendelian randomization (2SLS MR) to show a causal relationship for 21 of the CpGs. The largest difference was 15.7% lower beta-value at cg14436889 among hypospadias cases than controls (EWAS P = 5.4e−7) and is likely causal (2SLS MR P = 9.8e−15). Integrative annotation using two-sample Mendelian randomization of these methylation regions highlight potentially causal roles of genes involved in germ layer differentiation (WDHD1, DNM1L, TULP3), beta-catenin signaling (PKP2, UBE2R2, TNKS), androgens (CYP4A11, CYP4A22, CYP4B1, CYP4X1, CYP4Z2P, EPHX1, CD33/SIGLEC3, SIGLEC5, SIGLEC7, KLK5, KLK7, KLK10, KLK13, KLK14), and reproductive traits (ACAA1, PLCD1, EFCAB4B, GMCL1, MKRN2, DNM1L, TEAD4, TSPAN9, KLK family). This study identified CpGs that remained differentially methylated after urogenital development and used the most relevant tissue sample available to study hypospadias. We identified multiple methylation sites and candidate genes that can be further evaluated for their roles in regulating urogenital development.

Single-stage urethroplasty: An eight-year single-centre experience and its associated factors for urethrocutaneous fistula

Introduction: This study aimed to describe single-stage urethroplasty and to determine factors associated with urethrocutaneous fistula after the procedure at our institution. Methods: All hypospadias patients without any prior surgery who underwent single-stage urethroplasty from July 2010 to January 2018 were included. In total, 179 patients were followed for at least one year postoperatively. Information on types of hypospadias, operation techniques, chordee degree, preoperative pyuria (urine white blood cell count &gt;5/HPF), urethral defect length and urethrocutaneous fistula formation was collected. Results: We obtained 103 cases of posterior (57.5%), middle (57 cases) and anterior (19 cases) hypospadias on whom onlay preputial island flap (71 cases), tubularized incised plate (46 cases) and Duckett’s transverse preputial island flap (35 cases) were conducted. After 47.1±25.8 months of follow-up, urethrocutaneous fistula were found in 23 (12.8%) patients associated with posterior hypospadias occurrence ( p=0.025), longer urethral defect ( p=0.007) and preoperative pyuria ( p&lt;0.001). Chordee degree ( p=0.886) and age ( p=0.187) were not significant factors associated with urethrocutaneous fistula formation. Conclusion: Single-stage urethroplasty is a versatile procedure for various hypospadias cases, with a urethrocutaneous fistula rate of 12.8%. Posterior hypospadias, preoperative pyuria and longer urethral defect were associated with urethrocutaneous fistula formation. Level of evidence Level 2B.

Maternal hypertensive disorders and subtypes of hypospadias: A Dutch case-control study.

BackgroundHypospadias is a frequently occurring congenital anomaly in male infants, in which the opening of the urethra is located along the ventral side of the penis. Although various studies attempted to identify its causes, the aetiology of the majority of hypospadias cases remains poorly understood. Maternal hypertensive disorders are believed to be associated with hypospadias, but the results of previous studies are not consistent, especially for subtypes of hypospadias.ObjectivesTo investigate the associations between maternal hypertensive disorders, stratified by pharmacological treatment, and the occurrence of hypospadias divided into subtypes in a large population‐based case‐control study.MethodsWe included 887 hypospadias cases and 1005 male controls from the AGORA data‐ and biobank. Cases and controls were born in the periods 1975‐2016 and 1990‐2011, respectively. All data were collected in the period 2004‐2018. Maternal questionnaires were used to obtain information on hypertensive disorders during pregnancy, antihypertensive medication treatment, and potential confounders. Adjusted odds ratios (aORs) with 95% confidence intervals (CIs) for the associations between hypertensive disorders and hypospadias were estimated using logistic regression.ResultsHypertensive disorders were reported by 15.3% of the women in this study. Maternal hypertensive disorders in general, chronic hypertension, and gestational hypertension were not associated with hypospadias or its subtypes. Preeclampsia was associated with posterior hypospadias (aOR 3.09, 95% CI 1.49, 6.43), whether it was untreated (aOR 2.81, 95% CI 1.24, 6.38) or pharmacologically treated preeclampsia (aOR 4.96, 95% CI 1.08, 22.80).ConclusionsOur findings indicate that preeclampsia is associated with posterior hypospadias, irrespective of pharmacological treatment. This result supports the hypothesis of aetiological heterogeneity among the subtypes of hypospadias, with pregnancy‐related risk factors being associated with the more severe types of hypospadias.