Triphallia: the first cadaveric description of internal penile triplication: a case report.

INTRODUCTION: Triphallia, a rare congenital anomaly describing the presence of three distinct penile shafts, has been reported only once in the literature. This case report, based on an extensive literature review, describes the serendipitous discovery during cadaveric dissection of the second reported human case of triphallia, distinctly morphologically different from the previous case. CASE PRESENTATION: Despite the normal appearance of external genitalia on examination, the dissection of a 78-year-old white male revealed a remarkable anatomical variation: two small supernumerary penises stacked in a sagittal orientation posteroinferiorly to the primary penis. Each penile shaft displayed its own corpora cavernosa and glans penis. The primary penis and largest and most superficial of the supernumerary penises shared a single urethra, which coursed through the secondary penis prior to its passage through the primary penis. A urethra-like structure was absent from the smallest supernumerary penis. CONCLUSION: This case report provides a comprehensive description of the anatomical features of triphallia in a cadaver, shedding light on the morphology, embryology, and clinical implications of this anomaly. Without dissection, this anatomical variation would have remained undiscovered, suggesting the prevalence of polyphallia may be greater than expected. The single tortuous urethra present in this case, as well as the supernumerary and blind ending urethras present in many cases of penile duplication, may pose significant risk of infection, sexual dysfunction, subfertility, and traumatic catheterization. SIGNIFICANCE: These findings underscore the importance of meticulous anatomical dissections and may act as a resource for anatomists and those studying genitourinary anomalies. Although we can only speculate as to which functional implications this patient may have experienced, understanding such anatomical variations contributes to both knowledge of human anatomy and clinical management should the condition be encountered in living individuals.

Penoscrotal transposition: Long-term outcome in 29 patients.

OBJECTIVE: Penoscrotal transposition (PST) is a rare anomaly of the external genitalia characterized by malposition of the penis in relation to the scrotum. This transposition may be partial or complete and may be associated with hypospadias, chordee, and other anomalies. We have reviewed our experience with the surgical repair of PST utilizing a modified Glenn-Anderson technique. MATERIALS AND METHODS: Twenty-nine patients with a median age of 5.6 years (8 months -15 years) underwent surgical repair of PST at our institution between 2004-2022. Of those, 20 (69%) had complete PST, while 9 (31%) had partial PST. All children were divided into three groups. In the first group of 8 (28%) children, repair of PST was an integral part of one-stage male genitoplasty; in the second group of 18 (62%) children, repair of PST was an isolated last stage of the staged hypospadias repair and the remaining 3 (10%) children underwent PST repair without the presence of hypospadias. All patients underwent modification of the Glenn-Anderson technique involving utilization of bilateral rotational advancement scrotal flap, complete de-tethering of the testis from the internal part of the scrotum when indicated, and relocation of the scrotal compartment in a normal dependent position. The follow-up ranged from 6 months to 18 years. RESULTS: In the first group, five children (62%) underwent Onlay Prepucial Island Pedicle Flap (OIF) hypospadias repair, and three (38%) underwent Long Tubularized Incised Plate Repair (TIP). In the second group, 8 (44%) underwent OIF hypospadias repair, 2 (12%) had Long TIP repair, and the remaining 8 (44%) underwent staged hypospadias repair. Post-operative Clavien Dindo grade III presented among three patients in group I and only one patient in group II. In the third group, no postoperative complications were observed. CONCLUSION: Our data show that penoscrotal transposition correction utilizing the Glenn-Anderson technique is a reliable and durable surgery in the pediatric population. These children require careful monitoring till adolescence to ensure that no re-operation is needed.

Functional outcome of the one-stage scrotal tunnel + ventral inverted V incision + inverted Y anastomosis technique to treat penile paraffinoma: A single center retrospective study.

INTRODUCTION AND OBJECTIVE: Penis enlargement through substance injection is common in many countries of Southeast Asia and Eastern Europe. The definitive therapy involves removing the entire skin and the subcutaneous tissue and resurfacing the penile shaft via a single-stage or multi-staged procedure. This study aimed to report the functional outcome and esthetics of treating penile paraffinoma patients using the scrotal tunnel + ventral inverted V incision + anastomosis inverted Y technique. MATERIAL AND METHODS: This study was a single-center retrospective descriptive analysis of patients who underwent one-stage scrotal tunnel + ventral inverted V incision and inverted Y-shaped anastomosis procedures from January 2013 to December 2023. The following data were collected: chief complaint, reason for the injection, type of fluid injected, duration of surgery, duration of hospitalization, length of follow-up, and results after surgery. RESULTS: Of the 32 patients included in the study, 78% injected liquids in the form of oil and the goal of the majority of patients was penis enlargement (71%). The average age was 36.84 years, and the main complaint was pain in the penis during erection (53%). The average operation time was 130 minutes, hospitalization duration was 2.21 days, primary wound healing was 91%, patient satisfaction level was 97%, and Scale 4 erection hardness was 91%. CONCLUSION: One-stage surgery for penile paraffinoma produced promising results when the granuloma was limited to the penis and healthy scrotal skin was available to cover the penis.

The Emerging Role of Artificial Intelligence and Automated Platforms for the Assessment of Penile Curvature: A Scoping Review.

PURPOSE OF THE REVIEW: The estimation of penile curvature is an essential component in the assessment of both Peyronie's disease and hypospadias-associated congenital penile curvature, as the degree of curvature can significantly impact treatment decision-making. However, there is a lack of standardization in curvature assessment and current methodologies are prone to inaccuracies. With the rise of artificial intelligence (AI) in urology, new research has explored its applications in penile curvature assessment. This review aims to evaluate the current uses of AI and other automated platforms for assessing penile curvature. RECENT FINDINGS: Several novel and promising tools have been developed to estimate penile curvature, some utilizing AI-driven models and others employing automated computational models. These platforms aim to improve curvature assessment in various settings, including at-home evaluation of Peyronie's disease, in-office assessments using three-dimensional (3D) methodologies, and preoperative evaluations for hypospadias repair. In general, these new platforms produce highly accurate and reproducible angle estimates in non-clinical studies, however their effectiveness and relation to patient outcomes has had limited evaluation in clinical settings. Significant advancements have been made in the assessment and estimation of penile curvature in both Peyronie's and pediatric patients, largely driven by AI and other automated platforms. Continued research is needed to validate these findings in clinical studies, confirm their efficacy, and assess their feasibility for real-world applications.

Creation of a Novel Classification System (PTNM) for Peyronie's Disease and Penile Curvature Using Evidence-Based Criteria.

PURPOSE: Our goal was to identify new Peyronie's disease (PD) subtypes, non-PD penile curvature classifications, and define active (acute) vs stable (chronic) phases of disease using evidence-based analyses. MATERIALS AND METHODS: A retrospective review was performed of 1098 men who presented with penile deformity, including subjective standardized and nonstandardized questionnaires and objective measures. A second cohort of 719 men who were sent a mailed survey was also utilized for the relapsing/remitting subtype. Statistical analyses were performed to identify clusters of disease characteristics representative of distinct PD and non-PD categorizations, including sensitivity/specificity analyses and subtype comparisons. RESULTS: Comparative analyses identified 4 distinct subtypes of PD: (1) classical and nonclassical, (2) calcifying-moderate/severe calcification, (3) progressive-subjective worsening following disease onset, and (4) relapsing/remitting-reactivation following ≥ 6 months of stability. Additional, non-PD categorizations included congenital (lifelong), maturational (developed around puberty), and trauma induced. Statistical analyses demonstrated unique profiles among each category. Penile pain was not found to be a reliable predictor for disease progression or stability. Stable phase disease (historically "chronic") was variably defined by subtype: classical (≥3 months); progressive, calcifying, or trauma induced (≥12 months + ≥3 months stable OR ≥6 months stable). Similarly, PD subtypes may be assigned at ≥ 3 months following disease onset. A PTNM staging system is proposed to help communicate disease states, in which P = PD component (Ca-calcifying, Cl-classical, P-progressive, R-relapsing/remitting, U-undifferentiated), T = trauma component (0-absent, 1-present), N = non-PD component (C-congenital, M-maturational, U-undifferentiated), and M = mode (0-stable, 1-active); for example, PClT1N0M0 = stable classical PD with prior trauma. CONCLUSIONS: The current study provides an evidence-based proposal for the establishment of new PD subtypes and non-PD curvature categorizations as well as a standardized definition for active vs stable phases of disease.

Technical Considerations and Outcomes for Panniculectomy in the Setting of Buried Penis Patients: A Systematic Review and Database Analysis.

BACKGROUND: Often secondary to obesity, adult-acquired buried penis (AABP) is an increasingly common condition. AABP is often detrimental to urinary and sexual function, psychological well-being, and quality of life. Surgical treatment involves resection of excess soft tissue, with adjunct procedures, including a panniculectomy. However, few studies have been conducted investigating the risks of panniculectomy in the context of AABP surgical repair. METHODS: A systematic review of PubMed, Embase, and Cochrane databases was performed, following the PRISMA 2020 guidelines. Descriptive statistics regarding patient demographics, complications, and surgical technique were conducted. After this, an analysis of AABP patients within the American College of Surgeons National Surgical Quality Improvement Program (NSQIP) database was conducted. RESULTS: Four studies including 57 patients reported panniculectomy as part of buried penis repair (PBPR). Surgical approaches included a modified trapezoid and traditional transverse incision. All authors utilized postoperative drains. Dehiscence and wound infection were the most frequent complications. Univariate NSQIP analysis revealed that PBPR patients had higher body mass index, more comorbidities, and greater wound complication rates. Multivariate analysis revealed that PBPR did not significantly increase 30-day complications compared to isolated BPR ( P > 0.05), while body mass index remained a significant predictor. CONCLUSIONS: Surgical repair of AABP can greatly improve patient quality of life. The available literature and NSQIP-based analysis reveal that concurrent panniculectomy in AABP repair has a comparable complication profile. Future studies are necessary to better characterize the long-term outcomes of this PBPR.

Characteristic external genitalia in male neonates with 5α-reductase deficiency.

There have been no reports comparing neonatal external genitalia of 5α-reductase deficiency (5αRD) with those of other 46,XY differences of sex differentiation (DSD). This study enrolled 31 Japanese cases of 46,XY DSD whose external genitalia was examined during the neonatal period; four were diagnosed as 5αRD and 15 were defined as non-5αRD by genetic analysis of SRD5A2 or urinary steroid metabolites. We compared the following characteristics between 5αRD and non-5αRD groups, adjusting the severity of undermasculinization of the external genitalia: stretched penile length (SPL), glans width, location of the external urethral opening, and proportion of undescended testis. The external genitalia of all the 5αRD cases were Quigley classification grade 2 or 3. We compared the phenotypes between the four 5αRD cases and 11 non-5αRD cases with grade 2 or 3. The median (range) of SPL in the 5αRD group (14 mm [11-16]) was significantly lower than that in the non-5αRD group (22 mm [15-29]) (p = 0.003). An SPL cut-off value of <15 mm yielded a sensitivity of 50% (95% confidence interval [CI]; 7-93%) and specificity of 100% (95% CI, 72-100%) for discriminating between the groups. The median glans width, location of the external urethral opening, and proportion of undescended testis were not significantly different between the groups. The SPL of 5αRD in Quigley classification grade 2 or 3 was significantly shorter than that of other 46,XY DSDs with the equivalent grade.

Technical nuances of the concealed penis repair.

Circumcision is commonly performed but anatomic variants occur and can affect outcomes if not addressed properly. The combination of concealed penis and penoscrotal webbing is fairly common and presents across a spectrum of severity. If not repaired, this can result in a buried penis that can cause penile adhesions, wound healing concerns, and make the penis appear shorter secondary to a retracted position. We present our technique that is reproducible and highly successful in addressing both of these concerns. The paraphimotic band approach is performed more commonly and is able to reliably correct webbing and concealment without an incision at the penoscrotal junction. When more severe defect is present, a Y shaped incision is made at the penoscrotal junction to mobilize skin flaps to correct the deficit. In our cohort of 885 patients, 736 were corrected using the paraphimotic band technique while 149 underwent a complex scrotoplasty. None of the patients required a secondary surgery for complications.

[Efficacy of oral testosterone undecanoate in children with androgen insensitivity syndrome].

Objective: To investigate the efficacy and safety of oral testosterone therapy in individuals diagnosed with androgen insensitivity syndrome (AIS). Methods: A self-controlled study design was utilized, focusing on individuals with AIS who were genetically diagnosed at the Department of Endocrinology, Genetics, and Metabolism of Beijing Children's Hospital between 2009 and 2021. These patients underwent treatment involving the administration of testosterone. The primary observed indexes include the measurement of penis length, which should meet the minimal surgical standard (penis length≥2.5 cm) or greater than or equal to -2.5 s (lower limit of normal). Secondary observed indexes include penile length standard deviation score (PL-SDS), an increase in penis longitude (ΔPL), medication dosage, the course of therapy, and safety indicators, among others. There were 4 courses of treatment. After each course, patients were evaluated to determine whether termination of treatment was appropriate. Patients who exhibited inadequate post-treatment penile length growth were advised to continue with further treatment. The statistical methodology included t-test, and a Wilcoxon rank sum test to describe efficacy and safety. The patients were followed up until 2023. Results: The study comprised a total of 51 individuals with AIS, comprising 33 males and 18 females (gender of registered permanent residence). Among these patients, 10 were diagnosed with complete androgen insensitivity syndrome (CAIS) and 41 were diagnosed with partial androgen insensitive syndrome (PAIS). There were 2 children with CAIS were diagnosed by doctors and prescribed testosterone undecanoate, but the children did not really take medicine.The penile length of CAIS patients could not be measured (penile length<0.5 cm) before and after treatment. For PAIS patients, baseline penile length and PL-SDS were (2.3±0.6) cm and -3.7±1.3, respectively. The measurements for penile length and PL-SDS after each treatment course were recorded as follows: (2.7±0.8), (2.8±0.6), (2.6±0.4), (2.6±0.4) cm and -2.8±1.6, 2.5±1.6, 2.9±1.2, -3.2±0.9, respectively. Both penile length and PL-SDS interventions showed statistically significant gains when compared to the baseline performance of the 4 courses (t=4.05、3.56、2.55、2.23 and 3.88、3.50、2.50、2.19, all P<0.05). Before treatment, 13 PAIS patients (32%) reached 2.5 cm and seven (17%) reached greater than or equal to -2.5 s. Following the initial, subsequent, third, and fourth therapeutic interventions, 18 cases (44%), 24 cases (59%), 25 cases (61%), and 26 cases (63%) reached 2.5 cm, respectively. Additionally, A total of 12 cases (29%), 15 cases (37%), 20 cases (49%), and 21 cases (51%), respectively, were found to reach greater than or equal to -2.5 s. The study involved the longitudinal monitoring of patients with the highest recorded age being 13.7 years. The weight, height, body mass index, bone age/age, cholesterol, hemoglobin and so on were all within the normal range and the difference were not statistically significant (all P>0.05). All 49 patients were no abnormalities in blood electrolyte, liver and kidney function and thyroid function and no changes in precocious puberty, pubic hair growth, aggressive behavior, vulvar skin darkening, diarrhea or other conditions. Conclusions: Testosterone undecanote in children with CAIS was no effective. The initial course of treatment for patients with PAIS demonstrates observable enhancements in penile length and PL-SDS. For patients with inadequate penile length growth, continued treatment in subsequent courses (such as the second, third, and fourth courses) is recommended toenhance outcomes gradually. Testosterone undecanoate was safe and effective for the majority of individuals with PAIS patients, with few adverse effects and good treatment tolerance.

New classification of the penoscrotal positional anomalies.

BACKGROUND: The aspect of sexual differentiation and the mechanism controlling the position of genitalia, which represents one of the most substantial differences between the sexes, is still poorly understood. Minor cases and some variants of penoscrotal transposition (PST) are unreported, and obvious cases were classified broadly and confused with other unrelated anomalies. METHODOLOGY: Relevant literature published till 2022 were reviewed then organized, recapitulated, and presented in comparison with the findings and data of 65 child diagnosed with PST. So, an integrated comprehensive approach to this uncommon condition enabled a new classification including few unreported variant cases, which were complemented. RESULTS: PST is classified herein into a cephalic or caudal scrotal migration, the cephalic type subdivided into major and minor subtypes the latter type subdivided into bilateral, unilateral or central subtypes. Cases of caudal scrotal regression is an unreported anomaly in which the scrotum located caudally, as constant association with epispadias/exstrophy anomalies leaving a wide distance between the fixed penis and the scrotal sacs. CONCLUSION: PST is not rare as it was believed, it occurs in two directions; cephalic and caudal directions. Scrotal caudal regression anomaly was not described before, as well the PST presented as an inguinal hernia.

Hypogonadotropic hypogonadism with Zinner syndrome: a coincidence or a consequence?

This case report describes a man in his 20s presenting with bilateral crypto-orchidism, micropenis and underdeveloped secondary sexual characteristics. The patient also exhibited hyposmia, eunuchoid stature and gynecomastia. Biochemical investigations revealed low levels of testosterone, luteinising hormone and follicle-stimulating hormone. Hence, he was diagnosed with Kallmann syndrome. Imaging studies showed an absent right kidney and cystic dilatation of the distal ureteric bud, seminal vesicle and absent/hypoplastic ejaculatory duct. The association of hypogonadotropic hypogonadism with Zinner syndrome, a rare condition characterised by renal agenesis, seminal vesicle cyst and ejaculatory duct obstruction, was noted.

A Child With Diphallia, Duplicate Bladder, Bladder Exstrophy, and Anorectal Malformation.

Congenital true diphallia, complete duplicate bladder, bladder exstrophy, and anorectal malformation in a child are uncommon. Here, we present the case of a 3-year-old boy with multiple genitourinary malformation, including true diphallia, complete duplicate bladder, bladder exstrophy, epispadias, and anorectal malformation. Multi-departmental collaborative treatment for complex conditions ultimately achieved an ideal appearance for this patient. All vital signs were stable after the surgery and they remained consistent during follow-up. In such cases, surgical correction is individualized to achieve adequate urinary continence and erection with adequate esthetics.

Comparison of hypospadias phenotype pixel segmentation to GMS score.

BACKGROUND: Hypospadias phenotype assessment determines if the anatomy is favorable for reconstruction. Glans-Urethral Meatus-Shaft (GMS) has been adopted in an effort to standardize hypospadias classification. While extremely subjective, GMS has been widely used to classify the severity of the phenotype to predict surgical outcomes. The use of digital image analysis has proven to be feasible and prior efforts by our team have demonstrated that machine learning algorithms can emulate an expert's assessment of the phenotype. Nonetheless, the creation of these image recognition algorithms is highly subjective. In order to reduce a subjective input in the evaluation of the phenotype, we propose a novel approach to analyze the anatomy using digital image pixel analysis and to compare the results using the GMS score. Our hypothesis is that pixel cluster segmentation can discriminate between favorable and unfavorable anatomy. OBJECTIVE: To evaluate whether image segmentation and digital pixel analysis are able to analyze favorable vs unfavorable hypospadias anatomy in a less subjective manner than GMS score. METHODS: A total of 148 patients with different types of hypospadias were classified by 1 of 5 independent experts following the GMS score into "favorable" (GG), "moderately favorable" (GM) and "unfavorable" (GP) glans. From there, 592 images were generated using digital image segmentation. 584 were included for final analysis due to certain images being excluded for poor image quality or inadequate capture of target anatomy. For each image, the region of interest was segmented separately by two evaluators into "glans," "urethral plate," "foreskin" and "periurethral plate". The values obtained for each segmented region using machine-learning statistical pixel k-means cluster analysis were analyzed and compared to the GMS score given to that image using an ANOVA analysis. RESULTS: Analysis of image segmentation demonstrated that k-means pixel cluster analysis discriminated "favorable" vs "unfavorable" urethral plates. There was a significant difference between scores when comparing the GG and GM groups (p = 0.03) and GG and GP groups (p = 0.05). Pixel cluster analysis could not discriminate between "moderately favorable" and "unfavorable" urethral plates. CONCLUSIONS: Through our analysis, we found significant pairwise difference for different tissue qualities. Digital image segmentation and statistical k-means cluster analysis can discriminate anatomical features in a similar way to the GMS score. Future research can target discerning between different tissue qualities in an effort to predict surgical outcomes for hypospadias repair.

Penile Enhancement Using Biodegradable Scaffolds Covered with Platelet-rich Plasma-Fibrin Glue, Mesenchymal Stem Cells for Micropenis.

PURPOSE: Evaluation of preliminary cosmetic and functional outcomes of biodegradable scaffolds covered with platelet-rich plasma in penile girth augmentation. MATERIALS AND METHODS: Between June 2016 and June 2018, 36 males who had a mean age of 28.91 years (range 20 - 48 years) with micropenis underwent this procedure. A mixture of platelets-fibrin glue and mesenchymal cells obtained from dermal fat tissue were prepared. Then the mixture was seeded on the pretreated tube-shaped poly lactic-co-glycolic acid scaffold and underwent a whole day of incubation. Following penile degloving, scaffolds were surgically implanted within the interface region of dartos and Buck's fascia. The 5-point Likert scoring scale was used to evaluate the patients' satisfaction with surgery. RESULTS: Patients followed up for 6-12 (8 ± 2.86) months. The penile length in an erected state before surgery was 6.5 - 12.5 cm (9.08 ± 1.6) which enhanced to 7 - 14 cm (10.59 ± 1.71) after surgery (P < .0001). The penile girth before and after surgery were 8.49 ± 1.53 and 10.91 ± 1.96 cm, respectively (P < .0001). An augment in penile length and girth of 1.5 and 2.6 cm were achieved, respectively. Patients appraised surgical intervention on a rating of one to five. The highest possible score (5) was assigned by 27 %, 33 % expressed a very good mark (4), and 19 % gave a good mark (3). CONCLUSION: Covering the scaffold with a mixture of Platelets-Fibrin glue and mesenchymal cells seems a safe and feasible method for penile reconstruction surgery. More studies should be done to determine the effect of platelets- fibrin glue and mesenchymal cells for treating micropenis.

The GUDplay technique: A shift of paradigm in glans reconstruction for midshaft and penoscrotal hypospadias with moderate chordee. A proposal of a new approach.

INTRODUCTION: After 5 years experience with the GUD (glandular urethral disassembly) technique for distal hypospadias, we present the GUDplay technique, incorporating Thiersch-Duplay tubularization of the plate till the coronal area, disassembling the glans aggressively and refurbishing the glans. METHODS: We defined the urethral plate and designed an inverted Y incision to open the glans in two wings. The glans was entirely detached from the corpora to gain a great mobility that allowed minor cranial mobilization of the urethra and caudal rotation of the wings. In sequence, there are well-known steps: Duplay urethroplasty, spongioblasts and a Dartos flap to cover the neourethra. The glans was connected to the urethra by 6.0 PDS sutures except in the ventral meatus and the glans wings are joined in the midline. RESULTS: The 5-year-old patient had midshaft hypospadias without previous surgery. The catheter was removed after a week and the healing appears to be good. DISCUSSION: We combined principles of total glans deconstruction in association to Duplay tubularization and then lifted it up to the tip of the glans divided in two wide and mobile wings. We have treated a small series of 6 cases without complications and mean follow-up of 6.2 months.

The "V-I penoscrotal reconfiguration": A simple technique for the surgical treatment of congenital webbed penis.

OBJECTIVES: To describe a new penoscrotal reconfiguration technique, named "V-I penoscrotal reconfiguration" for the surgical reconstruction of a congenital webbed penis (CWP). METHODS: Twenty-one patients who underwent the "V-I penoscrotal reconfiguration technique" were included in this retrospective study. The CWP severity was assessed according to El-Koutby's classification. Demographic and clinical data, surgical data, and postoperative outcomes were scheduled and analyzed. Specifically, the postoperative follow-up included both physical and psychological assessments at 2 weeks, 1, 6, and 12 months after surgery. Parents' satisfaction degree was quantified by the Likert scale. RESULTS: CWP was grade 3 in 11 (52%) patients, 2 in five (24%), and 1 (24%) in five. Five (24%) CWP were isolated malformations, 11 (52%) were associated with phimosis, three (14%) with hypospadias, and two (10%) with hypospadias and phimosis. There were no postoperative complications and no cases of redo surgery. The cosmetic outcomes were excellent in all cases: the parents' satisfaction score was 4 in 17 (81%) cases and 3 (9%) in the other four cases. CONCLUSIONS: CWP may cause psychological distress and functional problems, especially during sexual intercourse. Its correction in childhood is advocated to prevent psychological and sexual issues. The "V-I reconfiguration technique" is simple, and easy with excellent cosmetic and functional outcomes.

Penile reconstruction after complete bladder exstrophy repair.

INTRODUCTION: The decreased penile length in patients born with bladder exstrophy (BE) results partly from pubic symphysis diastasis and the separation of the corporal bodies. Also, intrinsic shortening of anterior corporal compartment, residual penile dorsal curvature, and postsurgical scarred skin share in creation of short penile length. OBJECTIVE: The goal of this study was to look at whether adult men who had bladder exstrophy (BE) surgery as babies needed and benefited from penile reconstruction, which included penile lengthening and repair of any redo penile pathology that was present at the same time. STUDY DESIGN: We reviewed the records of 31 repaired BE patients with mean age of 21.4 ± 3.7 years. The patients complained of their dissatisfaction with short penile length, residual dorsal penile curvature, distal dorsal or hypospadiac urethral opening and scared penopubic skin. The penile lengthening was performed by sub-periosteal detachment of the corporal bodies from the pubic rami in all cases. In 8 patients full thickness dermal grafts were used to penile resurfacing after its lengthening. Twelve patients underwent coronal or glanular urethroplasty. Phalloplasty was performed in one patient using forearm free graft. RESULTS: Subjective evaluation by the patient reported satisfactory results in 25/31 (80.6 %). The degree of penile lengthening measured at 6 months and one year postoperatively showed increased length which varied between 50 % and 150 % of the preoperative penile length. DISCUSSION: We hypothesize that congenital causes, such as short anterior corporeal compartment, wide pubic rami diastasis, and short penile urethra, as well as iatrogenic causes, such as post-surgical peno-pubic scars, could account for the coexisting multifactorial causes of penile shortening in male adults with BE. Penile lengthening is permitted, in our opinion, provided that the crura from the pubic rami is carefully and partially mobilized. CONCLUSIONS: The short phallus, residual dorsal chordee and distal urethroplasty can be corrected successfully in the majority of patients. Adult males with BE may have short penis that requires another reconstructive stage. The short phallus, residual chordee and distal urethroplasty can be corrected` successfully in the majority of patients.

Classes and predictors of reversal in male patients with congenital hypogonadotropic hypogonadism: a cross-sectional study of six international referral centres.

BACKGROUND: Although some male patients with congenital hypogonadotropic hypogonadism (CHH) undergo spontaneous reversal following treatment, predictors of reversal remain elusive. We aimed to assemble the largest cohort of male patients with CHH reversal to date and identify distinct classes of reversal. METHODS: This multicentre cross-sectional study was conducted in six international CHH referral centres in Brazil, Finland, France, Italy, the UK, and the USA. Adult men with CHH (ie, absent or incomplete spontaneous puberty by age 18 years, low serum testosterone concentrations, and no identifiable cause of hypothalamic-pituitary-gonadal [HPG] axis dysfunction) were eligible for inclusion. CHH reversal was defined as spontaneous recovery of HPG axis function off treatment. Centres provided common data elements on patient phenotype, clinical assessment, and genetics using a structured, harmonised data collection form developed by COST Action BM1105. Latent class mixture modelling (LCMM) was applied to establish whether at least two distinct classes of reversal could be identified and differentially predicted, and results were compared with a cohort of patients without CHH reversal to identify potential predictors of reversal. The primary outcome was the presence of at least two distinct classes of reversal. FINDINGS: A total of 87 male patients with CHH reversal and 108 without CHH reversal were included in the analyses. LCMM identified two distinct reversal classes (75 [86%] in class 1 and 12 [14%] in class 2) on the basis of mean testicular volume, micropenis, and serum follicle-stimulating hormone (FSH) concentration. Classification probabilities were robust (0·998 for class 1 and 0·838 for class 2) and modelling uncertainty was low (entropy 0·90). Compared with class 1, patients in class 2 had significantly larger testicular volume (p<0·0001), no micropenis, and higher serum FSH concentrations (p=0·041), consistent with the Pasqualini syndrome (fertile eunuch) subtype of CHH. Patients without CHH reversal were more likely to have anosmia (p=0·016), cryptorchidism (p=0·0012), complete absence of puberty (testicular volume <4 cm³; p=0·0016), and two or more rare genetic variants (ie, oligogenicity; p=0·0001). Among patients who underwent genetic testing, no patients (of 75) with CHH reversal had a rare pathogenic ANOS1 variant compared with ten (11%) of 95 patients without CHH reversal. Individuals with CHH reversal had a significantly higher rate of rare variants in GNRHR than did those without reversal (nine [12%] of 75 vs three [3%] of 95; p=0·025). INTERPRETATION: Applying LCMM to a large cohort of male patients with CHH reversal uncovered two distinct classes of reversal. Genetic investigation combined with careful clinical phenotyping could help surveillance of reversal after withdrawing treatment, representing the first tailored management approach for male patients with this rare endocrine disorder. FUNDING: National Institutes of Health National Center for Advancing Translational Sciences; Ministry of Health, Rome, Italy; Ministry of University, Rome, Italy; National Institutes of Health Eunice Kennedy Shriver National Institute of Child Health and Human Development; and the Josiah Macy Jr Foundation. TRANSLATION: For the Italian translation of the abstract see Supplementary Materials section.