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.

Evaluation of Z-plasty versus Heineke-Mikulicz scrotoplasty in the management of penoscrotal web in pediatric age group.

BACKGROUND: The penoscrotal web may be congenital or acquired following excessive ventral skin removal during circumcision. Several surgical techniques were described for the treatment of congenital webbed penis without a clear comparison between their outcomes. This prospective study aimed at comparing the surgical results of Z-scrotoplasty and Heineke-Mikulicz scrotoplasty in the treatment of congenital webbed penis in uncircumcised pediatric patients. METHODS: Our study included 40 uncircumcised patients who were divided randomly into two groups; Group A included 20 patients who were treated by Z-scrotoplasty and Group B included the other 20 patients who were treated by Heineke-Mikulicz scrotoplasty. All patients were circumcised at the end of the procedure. RESULTS: The surgical outcome was good without a significant difference between the two groups in 36 patients. Recurrent webbing developed in one patient of Group A and in three patients of Group B (FE p = 0.605) The only significant difference between the two groups was the operative duration which was shorter in Group B than in Group A (P < 0.001*). CONCLUSIONS: Treatment of congenital penoscrotal web in the pediatric age group could be done with either Z-scrotoplasty or Heineke-Mikulicz scrotoplasty with satisfactory results, however, without significant difference in the surgical outcomes. TRIAL REGISTRATION: • Registration Number: ClinicalTrials.gov ID: NCT05817760. • Registration release date: April 5, 2023.

Surgical correction of congenital megaprepuce: a pilot study.

OBJECTIVE: Congenital megaprepuce (CMP) is a rare penile deformity that usually requires surgical correction. This study was performed to examine the efficacy of the modified Sugita procedure for repairing CMP in pediatric patients. METHODS: We retrospectively analyzed the clinical data of pediatric patients with CMP treated by a surgeon using the modified Sugita procedure in our hospital from January 2019 to April 2021. RESULTS: Twenty patients were enrolled, and their median age at surgery was 70.5 months (range, 60-96 months). All surgeries were successful, and no complications occurred during the operation. The postoperative foreskin had moderate edema in five patients, and soaking in 10% hypertonic saline resulted in disappearance of the edema within 4 to 8 weeks. The follow-up duration was 6 to 20 months (median, 10 months). No other complications occurred, such as dehiscence or hematoma. CONCLUSIONS: The modified Sugita procedure for correction of CMP produces excellent cosmesis and a low complication rate. Our study indicates that the modified Sugita procedure is a safe and feasible treatment option.

Individualized care for patients with intersex (differences of sex development): Diagnosis and treatment of aphallia.

This review discusses issues and concerns in the management of aphallia, updating status of a post-pubertal individual who required further surgery after having initial surgery for aphallia as an infant. Through this case, which discusses an 18-year-old young adult who had penile agenesis, who desired further phalloplasty involving glanuloplasty and implantation of an erectile device, we highlight the importance of periodic evaluation and close follow up. Surgery during infancy or early childhood to create a penis is important for gender development in a boy, especially if there were functional testes during fetal life, even if this surgery would only be the first stage. There is a strong probability of subsequent surgery after initial phalloplasty before puberty, even with the use of currently refined techniques. Here we discuss the changing techniques that document the ongoing, continued refinement of these procedures, highlighting that further outcome data are needed to identify ways to further optimize current techniques.

Shaeer's corporal rotation IV: length-preserving correction of congenital ventral penile curvature.

BACKGROUND: For cases with severe penile curvature, the loss in length with shortening techniques or the loss in rigidity with incision grafting can compromise the results, hence the advent of Shaeer's corporal rotation III technique, which corrects the most severe degrees of ventral penile curvature without loss in length, though with a certain degree of narrowing. AIM: We sought to describe Shaeer's corporal rotation IV, aiming at correction of moderate-to-severe ventral penile curvature with minimal shortening, minimal narrowing, and minimal mobilization of the neurovascular bundle, among other improvements such as using thicker suture material to decease recurrence while inverting the surgical knots. METHODS: Forty-two patients with congenital ventral penile curvature were selected for the study with a curve ranging from 60° to 90°. Patients were randomized into 1 of the 2 groups: Shaeer's corporal rotation III (SCR-III) and SCR-IV. In SCR-IV, rotation is used to correct the main brunt of curvature, up to 70% to 80% of the curve. Plication is used to correct the residual curvature up to 100% straightness. This keeps the rotation points fewer and closer to the midline, thereby minimizing narrowing and mobilization of the neurovascular bundle. OUTCOMES: Both groups were compared with regard to intraoperative erection angle, length, and girth, before and after rotation, as well as subjectively reported postoperative recurrence, penile sensitivity, satisfaction, and IIEF. RESULTS: The postcorrection angle was zero for all cases in the 2 groups. Dorsal length decreased by 3% in the SCR-IV group compared with 0.5% in the SCR-III group (2.5% difference). The difference in circumference between the narrowest and widest points was 2% in the SCR-IV group vs 9.3% in the SCR-III group (7.3% difference). The average operative time was 19.2% shorter with SCR-IV. Girth asymmetry was reported in 1 (4.8%) of 21 patients in the SCR-IV group compared with 15 (71.4%) of 21 in the SCR-III group. Partial hyposensitivity of the penis was reported in 9.5% in the SCR-IV group compared with 19% in the SCR-III group. CLINICAL IMPLICATIONS: SCR-IV is an improvement over former versions of the technique, with higher patient satisfaction. STRENGTHS AND LIMITATIONS: A strength of the study is the long follow-up period. Limitations include being a single-center study and reliance on patient reporting to evaluate recurrence and satisfaction. CONCLUSION: The SCR-IV technique corrects moderate and severe degrees of congenital ventral penile curvature, with little or no compromise in penile length, girth, or sensitivity.

The factors that cause penile shortening after plication surgery in patients with congenital penile curvature: [[es]]Los factores causantes del acortamiento de pene después de la cirugía con plicatura en pacientes con incurvación peneana congénita.

OBJECTIVE: Congenital penile curvature is defined as the non-straightness of the penis without any urethral or penile pathology. We aimed to evaluate the factors that cause penile shortening after plication surgery in patients with congenital penile curvature. METHODS: Between November 2010 and December 2020, we retrospectively reviewed patients with CPC undergoing tunica albuginea plication surgery. Before the procedure, patients' age, curvature location and degrees, as well as penile length were recorded. After the treatment, penile lengths were measured and recorded again. Early and late period results were recorded. RESULTS: Plication surgery was performed in 130 patients. The median age was 24 years. Seventy-six patients had ventral curvature, 22 had dorsal curvature, 32 had lateral curvature. Average shortening of penile length in patients with curvature below 30° was: ventral 8-16 mm, dorsal 6-13 mm, lateral 5-12 mm. Patients with curvatures above 30° were: ventral 12-22 mm, dorsal 8-20 mm, lateral 2-12 mm. CONCLUSION: Penile length shortening after plication is inevitable. Curvature degree and direction are factors affecting penile length after surgery. Therefore, patients and relatives should be informed in more detail about this complication.

Scrotal flap phalloplasty as temporary neophallus in infants and children with penile agenesis: Multi-institutional experience and long-term follow-up.

INTRODUCTION: Aphallia is a rare congenital disorder pertaining to genotypic males. Early surgical creation of a neophallus is recommended to reinforce the child's male gender-identity, favoring proper psychosexual development. Modern microsurgical techniques used to create a neophallus in adults are not recommended in children due to the invasiveness and complexity of the procedures, along with high complication rates. Scrotal flap phalloplasty is a simple and reproducible technique to create a temporary neophallus in prepubertal boys with aphallia. OBJECTIVE: We present a multi-institutional experience, ten years after the initial description of the scrotal flap phalloplasty (SFP) technique, in which a flap from the well-developed scrotum is used to build a temporary neophallus, without obvious scars in patients with aphallia. STUDY DESIGN: The records of surgical neophalloplasty for aphallia patients from 4 centers between 2011 and 2021 were reviewed. All patients had at least one year follow-up to assess for short and long-term complications. Age at initial operation, associated anomalies, and other related surgical procedures were analyzed. RESULTS: The post-operative aesthetic result in all patients was satisfactory and has been maintained in the long-term follow-up, with all patients presenting a cylindrical structure resembling an uncircumcised penis, without evidence of significant contraction or loss of length. (Summary Figure) DISCUSSION: Non-microsurgical neophalloplasty techniques in patients with penile agenesis are temporary procedures that help to establish the body image and preserve the psychosexual development of the patient with aphallia. These techniques do not involve tissue transplant from a distant region, and are simpler to perform, with less scarring at the donor sites. Due to significant donor scars and considerable morbidity and complexity associated with the definitive phalloplasty techniques, we created a simple, reproducible and straightforward procedure to serve as a temporary neophallus in young boys with aphallia. As affected patients usually have a well-formed scrotum with normal and orthotopic testicles, it is the ideal donor site for a temporary neo-phallus in childhood. Furthermore, other donor sites are preserved for a definitive phalloplasty. There are limitations to this study, as quality of life could not be assessed and psychological or gender-identity investigations have not been carried out. None of these children have reached puberty, and hence decision and outcomes of definitive neophallus reconstruction has not been considered to date. CONCLUSION: Scrotal flap phalloplasty is a minimally invasive, simple and reproducible technique used to create a temporary neophallus in boys with aphallia, while waiting for definitive reconstructive surgery after puberty.

Fasciocutaneous foreskin twin flaps in hypospadias repair: Reconstructing a functional penis with freestyle design.

BACKGROUND: Hypospadias is the most common congenital urological deformity in males; however, there is still no perfect solution or surgical method for one-stage operations to fix proximal hypospadias with ventral penile curvature (VPC). We developed a fasciocutaneous foreskin twin flap technique that can provide freestyle flap designs in hypospadias repair. METHODS: A retrospective study including 26 patients was performed between January 2017 and June 2020. The surgical procedures included VPC correction, skin flap design, transfer and reconstruction. The urethra and shaft were reconstructed by twin flaps in one stage. All patients were followed up until 12 months. RESULTS: All VPC cases were corrected after the operation. The fistula rate was 11.5% (3/26). No meatal stenosis, urethral diverticulum, or stricture was observed. A total of 92.3% (24/26) of patients were satisfied with their penis appearance. CONCLUSIONS: The fasciocutaneous foreskin twin flaps provide freestyle designs in hypospadias repair, which can achieve a one-stage operation for proximal hypospadias with low fistula rates.

Quantification of the Androgen and Estrogen Receptors in the Penile Tissues of Hypospadias in Comparison with Normal Children.

INTRODUCTION: Hypospadias is a common congenital abnormality typified by a proximally placed ectopic urethral meatus along the ventral surface of the penis. Androgen receptor (AR) and estrogen receptor (ER) expression in the hypospadias tissues may be altered in hypospadias. METHODOLOGY: We evaluated by immunohistochemistry the AR and ER expression in 75 tissues from hypospadias repair, and compared this expression to that of tissue from 75 patients undergoing circumcision. We also compared the intensity of AR and ER expression between different severities of hypospadias. RESULTS: AR quantitative grading score decreased with severity of hypospadias, while the ER score increased as the hypospadias worsened, which was statistically significant (p-value <0.05). CONCLUSION: The penile tissue AR expression is decreased and ER expression is increased with increasing severity of hypospadias.

CONGENITAL SCAPHOID MEGALOURETHRA: REVISITING A RARE ANOMALY.

Megalourethra is an infrequent malformation of the anterior urethra that is caused by the lack of corpus sponigosum and in severe cases is accompanied by the lack of corpora cavernosa as well. We report a five-year-old boy presented to us with scaphoid variety of megalourethra having complaints of ballooning of urethra during voiding. He was investigated with urethrogram and cystoscopy and subsequently repaired with Nesbitt Longitudinal Reduction Urethroplasty. He had smooth recovery postoperatively with normal voiding stream on follow up.

Buried penis secondary to ectopic scrotum. / Pene oculto secundario a ectopia escrotal.

INTRODUCTION: Ectopic scrotum is a rare clinical entity, potentially associated with other congenital abnormalities. We present the case of a patient with buried penis secondary to ectopic scrotum. The surgical technique was described, and a literature review was carried out. CLINICAL CASE: 1-year-old patient with ectopic right hemiscrotum and the testes within the scrotal sac. A double Z-plasty was performed with two flaps - one above the penis, surrounding the ectopic scrotum, and the other one at the scrotum to modify the bifid scrotum. The upper flap was rotated downwards, which allowed ectopy to be repaired, and the lower flap was used to repair bifidity. No postoperative complications were recorded. Follow-time was 6 months, with good final cosmetic results. DISCUSSION: Ectopic scrotum is an infrequent congenital malformation. Cutaneous rotation flaps with Z-plasties are a valid treatment option, with good long-term cosmetic results.

INTRODUCCION: La ectopia escrotal constituye una entidad clínica rara, que puede asociar otras anomalías congénitas. Presentamos el caso de un paciente con un pene oculto secundario a una ectopia escrotal, con descripción de la técnica quirúrgica y revisión de la literatura. CASO CLINICO: Paciente de un año de vida que presentaba un hemiescroto derecho ectópico con testes en bolsa. Se diseñó una doble Z-plastia con realización de dos colgajos, uno suprapeneano rodeando el escroto ectópico y otro escrotal para modificar el escroto bífido. El colgajo superior se rotó hacia abajo corrigiendo la ectopia y el colgajo inferior corregió la bifidez. No se produjeron complicaciones posoperatorias. El tiempo de seguimiento fue de seis meses con buen aspecto estético final. COMENTARIOS: El escroto ectópico es una malformación congénita infrecuente. Los colgajos de rotación cutáneos con Z-plastias son una opción válida de tratamiento con buenos resultados estéticos a largo plazo.

An original mininvasive corporoplasty technique for penile curvature without circumcision.

OBJECTIVE: We describe an original minimally invasive penile plication technique with scrotal or infrapubic access, not requiring circumcision, for penile curvature of different severity and types. This technique can be used to correct both congenital and acquired curvatures, mono or bidirectional deformities. MATERIALS AND METHODS: Between 2012 and 2018 we treated 134 patients suffering from congenital curvature (33) and acquired curvature from Peyronie's disease (101). The average curvature was 62.2° (± 30.4°). Preoperative evaluation included prostaglandin E1 injection with photographic documentation and measurement of penile angulation, administration of IIEF- 15, vasoactive penile Doppler ultrasound, analysis of thermal and vibratory sensitivity with Genito-Sensory-Analyzer (GSA) and assessment of nocturnal penile stiffness with Rigiscan, performed twice, for a detailed evaluation of patient's erectile function. Scrotal access was performed in patients with dorsal and/or lateral penile curvature; the infrapubic access was performed in patients with ventral curvature. After preparation and incision of Colles' fascia, penis was partially degloved and an original plication technique called "binary corporoplasty" was performed at the site or sites established at preoperative assessment, with non-resorbable synthetic multifilament (Premicron®) suture. RESULTS: Complete correction of penile curvature was achieved in 96.8 % of patients. No major complications were reported, and no patients suffered worsening in erectile function or in penile sensitivity. The average shortening of convex side was 1.65 cm (± 0.7 cm) and all patients report easy intercourse after correction. The average time of surgery was 46 minutes (± 11 min) and all procedures were performed as a day-hospital or ambulatory settings, with local anesthesia and light sedation. Overall satisfaction rate is 96%. CONCLUSIONS: This is a simple and rapid technique that perfectly corrects even the most severe and complex penile curvatures. In comparison to traditional techniques, such as Nesbit procedure, this technique is associated with low morbidity, a very low recurrence rate and a great aesthetic results. Aesthetic and functional patients' satisfaction was excellent.

Total corpora mobilization for penile reconstruction.

PURPOSE: Total corpora mobilization (TCM) is a novel technique that is used for penile reconstruction in cases of micropenis and penile amputation. Its principle is based on Kelly's procedure for bladder exstrophy (1). In contrast to the Kelly procedure, TCM is performed entirely through the perineum with the patient in the lithotomy position. MATERIALS AND METHODS: TCM was performed on three patients. The first was a boy who suffered trauma from a dog bite at an age of eight months. At 23 years old he underwent TCM. The second patient had genital self-amputation induced by psychiatric disorder. After treatment, at 27 years old, he desired surgery for penile reconstruction. The third patient had partial androgen insensitivity syndrome (PAIS) with a micropenis and at 23 years old had TCM procedure. The patients were placed in the lithotomy position with a perineal incision in the midline. A subperiosteal incision was made and the corpora cavernosa were detached from the pubic arch and the ischial rami. The periosteum and the neurovascular bundles were preserved. Subsequently the corpora cavernosa was mobilized upward and the periosteum that was left attached to them was sutured to the pubis. RESULTS: At twenty-four, nine, and six months, respectively, in the follow-up process, all patients expressed satisfaction with the final cosmetic appearance, penile length, and erectile function. CONCLUSION: TCM may prove to be an alternative for patients with a functional disturbance because of small penile length, though a higher number of cases and a more extended follow-up are needed to draw a more definitive conclusion.

An objective hypospadias classification system.

OBJECTIVE: Current approaches to classifying hypospadias severity are based on meatus position. These classification tools lack precise reflection on the degree of urethral hypoplasia and extent of urethral underdevelopment hence are not good representative of hypospadias severity. Here, an alternative classification system is introduced that objectively reflects the developmental pathology underlying this condition. The study goal was to appraise location of bifurcation in the corpus spongiosum (BCS) relative to the penile shaft as an indicator of hypospadias severity. PATIENTS AND METHODS: Patients aged <18 years with primary hypospadias were included in the study. Urethral defect ratio (UDR) was calculated by dividing the extent of urethral defect (distance between the glandular knobs and BCS) relative to stretched penile length (SPL). Hypospadias severity was then categorized into three distinct grades (UDR <0.5, 0.5-0.99, ≥1.0). The Inter-Class Correlation (ICC) was evaluated to assess the intra- and inter-rater agreement between the reviewers of UDC ratio. Linear regression analysis was performed to estimate the correlation between UDC ratios and either plate objective scoring tool (POST) and Curvature degrees. RESULTS: A total of 67 patients aged 12.3 ± 3.7 months with primary hypospadias were enrolled. UDR ranged between 0.2 and 1.3. A significant difference in hypospadias level was observed between UDR grades, which further correlated degree of curvature (P < 0.0001), urethral plate quality (P < 0.0001), and associated anomalies (P < 0.05). The Inter-Class Correlation (ICC) value to examine the intra- and inter-rater agreement between the two reviewers in UDC ratio was 0.998 (95% CI 0.998, 0.999). Regression analysis revealed that UDR and both POST and Curvature degree were significantly associated (P < 0.001). CONCLUSIONS: A hypospadias severity scoring system based on embryological etiology and urethral hypoplasia and assessed relative to the penile shaft represents an objective, feasible, and consistent tool. These results clearly indicate that the reviewers have had excellent consistency/reliability across their consecutive readings. This new system can facilitate objective description of hypospadias-spectrum anomalies and thus support precise communication between individual surgeons and centers.

Diphallia: literature review and proposed surgical classification system.

BACKGROUND: Diphallia occurs once in 5-6 million births, with no two patients presenting with the same anatomical variation. Here we discuss a review of diphallia case reports, as well as present a new surgical classification system based on the soft tissue composition of the two phalluses, the anatomy of the urethra present within the most normal phallus and the bladder configuration. METHODS: Eighty-seven diphallia case reports were collected and analysed, excluding those presented in animals and articles that were non-English, with the results compiled to provide an in-depth reference of the specific anatomy found in diphallia patients and the associated abnormalities. RESULTS: Our proposed classification system was then applied to each patient and the most common configuration base on our classification system presented, along with commonly seen associated anomalies. CONCLUSION: The reviewed cases represent a subset of the most unique diphallia patients; thus, several cases may be left unreported. Future reports can then be categorized, aiding as a reference, and potentially building on the classification, should the patient not fit into a specific group, leading to an expansion of the classification system.

[Effectiveness of penis up transfer method for penoscrotal transposition in children].

Objective: To investigate the effectiveness of penile up transfer method in the treatment of penoscrotal transposition (PST) in children. Methods: A clinical data of 46 children with PST admitted between January 2015 and March 2021 and met selective criteria was retrospectively analyzed. The M-shaped scrotal flap method was adopted in 21 cases (group A), and the penile up transfer method was adopted in 25 cases (group B). There was no significant difference in age and PST typing between the two groups ( t=0.491, P=0.626; χ 2=0.710, P=0.790). The operation time and postoperative complications of the two groups were recorded and compared, and the correction effect of PST was evaluated. Results: All operations were successfully completed, and no urethral injury occurred during operation. The operation time was significantly less in group B than in group A [(70.36±9.76) minutes vs. (96.62±13.18) minutes; t=7.553, P=0.000]. All children were followed up 6-33 months (mean, 13.2 months). There were 5 cases (23.8%) of skin necrosis and 6 cases (28.6%) of scar hyperplasia in group A, and 1 case (4.0%) of skin necrosis and 1 case (4.0%) of scar hyperplasia in group B. The incidence of complication was significantly lower in group B than in group A ( χ 2=3.949, P=0.047; χ 2=5.341, P=0.021). In the evaluation of PST correction at 6 months after operation, there were 15 cases of complete correction and 6 cases of incomplete correction in group A, 24 cases of complete correction and 1 case of incomplete correction in group B, and the difference was significant ( χ 2=5.341, P=0.021). Conclusion: The penis up transfer method is not only easy to correct PST, but also can correct scrotal separation at the same time, with fewer postoperative complications and satisfactory correction effect of penile scrotal appearance.

Correlation Analysis of Genotypes and Phenotypes in Chinese Male Pediatric Patients With Congenital Hypogonadotropic Hypogonadism.

Congenital hypogonadotropic hypogonadism (CHH) can be divided into Kallmann syndrome (KS) and normosmic HH (nHH). The clinical and genetic characteristics of CHH have been studied in adults, but less in pre-adults. The medical records of patients with CHH in our gonad disease database from 2008 to 2020 were evaluated. In total, 125 patients aged 0 to 18 years were enrolled in our study. KS patients had a higher incidence of micropenis compared with nHH (86.2% vs. 65.8%, p=0.009), and 7 patients (5.6%) had hypospadias. Among the 39 patients with traceable family history, delayed puberty, KS/nHH, and olfactory abnormalities accounted for 56.4%, 17.9%, and 15.4%, respectively. In total, 65 patients completed the hCG prolongation test after undergoing the standard hCG test, and the testosterone levels of 24 patients (22.9%) were still lower than 100 ng/dL. In 77 patients, 25 CHH-related genes were identified, including digenic and trigenic mutations in 23 and 3 patients, respectively. The proportion of oligogenic mutations was significantly higher than that in our previous study (27.7% vs. 9.8%). The most common pathogenic genes were FGFR1, PROKR2, CHD7 and ANOS1. The incidence rate of the genes named above was 21.3%, 18.1%, 12.8% and 11.7%, respectively; all were higher than those in adults (<10%). Most mutations in CHH probands were private, except for W178S in PROKR2, V560I in ANOS1, H63D in HS6ST1, and P191L and S671L in IL17RD. By analyzing family history and genes, we found that both PROKR2 and KISS1R may also be shared between constitutional delay of growth and puberty (CDGP) and CHH. L173R of PROKR2 accounts for 40% of the CHH population in Europe and the United States; W178S of PROKR2 accounts for 58.8% of Chinese CHH patients. Micropenis and cryptorchidism are important cues for CHH in children. They are more common in pediatric patients than in adult patients. It is not rare of Leydig cell dysfunction (dual CHH), neither of oligogenic mutations diagnosed CHH in children. Both PROKR2 and KISS1R maybe the potential shared pathogenic genes of CDGP and CHH, and W178S in PROKR2 may be a founder mutation in Chinese CHH patients.

Prenatal diagnosis of diphallia in association with bladder exstrophy: a case report.

BACKGROUND: Penile duplication or diphallia is a rare congenital anomaly with unclear pathophysiological cause. Most cases of diphallia are reported postnatally; however, today with the use of a high-resolution ultrasound device, in-uterine diagnosis of many congenital anomalies is possible. CASE PRESENTATION: Herein we report a multiparous mother at 25 weeks of gestation who referred due to an abnormal cystic structure protruding from a large abdominal wall defect located below the umbilicus that was noted during a routine exam. Target scan revealed two penile-like protrusions with an empty scrotal sac and double bladder in an otherwise normal fetus, which was confirmed postnatally. Neonatal microarray study and karyotype were normal. CONCLUSION: Diphallia could be detected prenatally as an isolated anomaly, associated with caudal duplication syndrome, or as an exstrophy-epispadias complex. As this is a rare congenital anomaly, all sonographers should be familiar with prenatal ultrasound features and associated anomalies, an important issue in prenatal counseling with parents, delivery planning, psychological support of the family, and postnatal management.

Complete diphallia: How to proceed?

INTRODUCTION: Diphallia is a rare anomaly. It has a range of appearances from a small accessory penile to complete duplication. METHODS: We present a 2 year-old boy with complete penile duplication. The left penile was the largest. NMR (Nuclear Magnetic Resonance) suggested one corporal body for each penile and VCUG (Voiding Cystourethrogram) showed a normal urethra in the right penile and stricture at glandular and mid penile urethra of the left penis. A Y confluence to bulbar urethra was observed confirming only one prostate and bladder. RESULTS: The cystoscopy through the right penile identified the urethral confluence in the bulbar area. We performed a meatotomy in the left penile to insert the cystoscope and confirmed the blind ending urethra. We decided to remove this penile. The penile was degloved entirely and clamped and took out the corpora at the base. DISCUSSION: Diphallia can have three presentations: only glans duplication, bifid diphallia and complete diphallia (two corpora cavernosa and a corpus spongiosum for each penile). In our case, each penile presented only one corpora cavernosa and the decision taken was based on urethral patency. CONCLUSION: The treatment should always be planned individually whereas associated anomalies with the goal of attaining satisfactory functional and cosmetic results.

Surgical Correction of Adult Congenital Penile Curvature: A Systematic Review.

BACKGROUND: Congenital penile curvature (CPC) is corrected surgically by various corporoplasty or tunica albuginea plication techniques, but the optimal surgical approach is not well-defined. AIM: To provide a comprehensive evaluation of the published literature pertaining to outcomes with penile plication and corporoplasty techniques for surgical management of CPC. To determine if plication or corporoplasty offers superior outcomes in surgical correction of CPC. METHODS: A systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis Checklist. The following databases were queried from inception to March 18, 2020 to search for studies describing surgical treatment of CPC: Ovid MEDLINE(R) and Epub Ahead of Print, In-Process & Other Non-Indexed Citations and Daily, Ovid Embase, Ovid Cochrane Central Register of Controlled Trials, Ovid Cochrane Database of Systematic Reviews, and Scopus. OUTCOMES: Objective and subjective postoperative outcomes including penile straightening, shortening, penile sensory changes, and reoperation rates for both corporoplasty and tunica albuginea plication were summarized. RESULTS: Fifty-five articles comprising 2,956 patients with CPC who underwent a plication procedure (n = 1,375) or corporoplasty (n = 1,580) were included. The definition of "treatment success" varied widely and most often involved subjective patient reporting (22 studies; 40%) or objective assessment (15 studies; 27%). We considered curvature correction to be satisfactory if there was self-reported patient satisfaction or residual curvature after correction of <20˚. Reported rates of successful straightening ranged from 75 to 100% and 73 to 100% for plication and corporoplasty, respectively. A comprehensive and accurate assessment of surgical outcomes for CPC correction, such as satisfactory penile straightening, reoperation rates, glans sensory changes, and other complications was limited by significant inter-study heterogeneity with respect to the reporting of treatment outcomes. CLINICAL IMPLICATIONS: While both plication and corporoplasty appear to be safe and effective options in the treatment of CPC, definitive conclusions cannot be drawn with respect to treatment superiority due to low-quality study design, methodology flaws, and significant heterogeneity in reporting. STRENGTH & LIMITATIONS: This report represents the most comprehensive review of CPC surgical management. However, there is a significant lack of standardization in the reporting of treatment outcomes for CPC, thereby limiting the reliability of the published data summarization encompassed by our review. CONCLUSION: Both plication and corporoplasty demonstrate high success rates and relatively low complication rates in the treatment of CPC, albeit with low-level evidence available in most research publications. Robust comparison of the surgical techniques used to correct CPC is limited by significant variation in reporting methods used in the literature. C. J. Britton, F. A. Jefferson, B. L. Findlay, et al. Surgical Correction of Adult Congenital Penile Curvature: A Systematic Review. J Sex Med 2022;19:364-376.