Penoscrotal Transposition Research Articles

Penoscrotal transposition: Long-term outcome in 29 patients.

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. 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. 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. 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.

New classification of the penoscrotal positional anomalies.

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. 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. 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. 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.

An epidemiologic study of penoscrotal transposition by maternal characteristics using data from the Texas birth defects registry.

Penoscrotal transposition (PST) is an uncommon urogenital malformation in which the penis is mal-positioned to be inferior to the scrotum. The purpose of this study was to explore PST risk by maternal characteristics and to describe co-occurring congenital abnormalities in the Texas Birth Defects Registry (TBDR). We conducted a population-based descriptive study examining occurrence of PST in the TBDR between 1999 and 2019. The primary outcome variable was PST diagnosis during infancy. Descriptive variables included maternal age, education, and race/ethnicity. Prevalence ratios (PRs) were calculated within each maternal variable category using Poisson regression. Counts and percentages of cases with select co-occurring congenital abnormalities were also calculated. Overall, 251 infants had PST, providing a prevalence of 0.61/10,000 live male births (95% CI: 0.53-0.68). PST prevalence was significantly lower among infants of mothers who had lower educational attainment (<high school vs. >high school), who were younger (<25 vs. 25-34), and who were Hispanic (vs. non-Hispanic White) and was significantly higher among older mothers (35+ vs. 25-39). Hypospadias was the most common co-occurring genitourinary anomaly, affecting close to 70% of cases. To our knowledge, this is the first investigation exploring the prevalence of PST in a population-based birth defects registry. Our findings help to understand the risk for PST among select maternal demographic characteristics and may assist in generating hypotheses about the underlying etiology of this condition for future work.

Surgical treatment of perineal hypospadias with complete penoscrotal transposition in DSD patients: Our experience with a “three-staged” reconstructive procedure

Surgical treatment of perineal hypospadias with complete penoscrotal transposition in DSD patients: Our experience with a “three-staged” reconstructive procedure

Patient Perspectives and Outcomes in Adult Hypospadias Repair.

Background Hypospadias is a common congenital anomaly that needs repair at an early age (six months to one year). Ironically, many cases in India present late due to a lack of healthcare facilities, poverty, and illiteracy. Adult patients are different from children as they are aware of their genitalia. They are concerned with the aesthetics and, predominantly, the potency. In this study, we present the perspectives and outcomes of 111 adult cases of hypospadias. Methodology In this retrospective study conducted between January 2010 and December 2020, 111 patients aged more than 14 years who were diagnosed with hypospadias of any level with or without mild-to-moderate chordee were included. Hypospadias repair using a tubularized incised plate (TIP) urethroplasty technique was performed, and patients after surgery were followed up at three months, six months, and 12 months for any complications with physical examination, uroflowmetry, and patient-related outcomes (PROs). Results Age varied from 14 years to 32 years (mean = 19.88 years, SD = 5.93). The most frequent meatus positions after chordee adjustment were distal (n = 64, 57.65%), middle (n = 25, 22.52%), and proximal (n = 22, 19.82%). Among these, four patients had penoscrotal transposition. Chordee was present in 65.7% (n = 73) of the cases. (<30° in 38.7%, n = 43; 30°-60° in 23.4%, n = 26, and >60° in 3.6%, n = 4). Chordee was corrected using many techniques, including ventral corporotomies. Urethroplasty was done using TIP and spongioplasty in 89% (n = 99), and one patient underwent inner preputial onlay flap urethroplasty. Snodgraft was used to augment the urethral plate in 10 cases. The success rate of one-stage surgery was 74.77% in our series, which significantly correlated with PROs. Uroflow varied from 12 mL/second to 18 mL/second, and in the majority of the cases, the flow rate improved over time. The most common complication was urethrocutaneous fistula in 11 (11.8%) patients, followed by glanular dehiscence in nine (8.1%) patients. Conclusions Adult patients undergoing primary hypospadias repair generally show good outcomes. Patients can have an acceptable mild degree of residual chordee and torsion, which correlate well with PROs. In our series, hypospadias fistula was the most common complication of hypospadias surgery, followed by glanular dehiscence.

Klinefelter syndrome with penoscrotal transposition and diphallia: A case study.

The authors declare no conflict of interest. Table S1. Klinefelter syndrome complicated by penoscrotal transposition; literature review. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

The Role of Tissue Expanders in the Reconstruction of Proximal Hypospadias

OBJECTIVETo report the technique and outcomes of tissue expansion (TE) for generating additional penile skin for urethroplasty and/or skin coverage during primary or redo hypospadias repair in penoscrotal transposition (PST) patients with a proximal hypospadias. METHODSProximal hypospadias and PST patients with a lack of penile skin, congenitally or after failed repair, who underwent TE assisted reconstruction were reviewed. TE were placed under the penile shaft and expanded skin was used for tubularized incised plate repair. Success was defined as urethral advancement to the corona or more distal with tension free skin coverage. RESULTSA total of 24 patients underwent reconstruction including 16 as part of primary repair and for redo repairs in 8. Nine patients experienced expander extrusion and posturethroplasty complications occurred in 43.8% of primary repairs and 75.0% of redo repairs; a urethrocutaneous fistula was most common (n = 8). Overall, success was achieved in 87.5% of patients with postoperative meatal locations almost all coronal (45.8%) or glanular (50.0%). CONCLUSIONProximal hypospadias reconstruction is challenging, and complications are not infrequent. TE is a useful alternative for complex patients with a skin paucity such that cutaneous coverage would be difficult following urethroplasty. Although the complication rate was 43.8% for primary repair, TE generated sufficient residual skin for success after additional reconstruction. For redo repair early use is most beneficial as there is more expandable skin. The pseudocapsules provide vascularized coverage to reinforce the urethra while there is sufficient skin to minimize the need for a skin graft for penile coverage.

Evaluation of tunica vaginalis flap-covering combined with modified Glenn-Anderson in one-stage repair of proximal hypospadias with incomplete penoscrotal transposition.

To explore a novel repair method for proximal hypospadias with incomplete penoscrotal transposition in children and evaluate its safety and outcomes. A retrospective analysis of clinical data was conducted for 86 children with severe proximal hypospadias with incomplete penoscrotal transposition who were hospitalized in our department between June 2018 and February 2021. In total, 42 patients (Group A) underwent repair following a one-stage method in which tunica vaginalis flap-covering was combined with a modified Glenn-Anderson procedure, while 44 patients (Group B) underwent a two-step repair consisting of tunica vaginalis flap-covering using the Duplay technique and the modified Glenn-Anderson procedure. The two groups were compared on operation time, length of postoperative hospital stay, postoperative complications, and associated costs. All operations were successful in both groups. No statistical difference was observed between the two groups in incidence of stenosis of the urinary meatus (2.38% vs. 4.54%, P = 0.279), urethral stricture (2.38% vs. 2.27%, P = 0.948), urinary fistula (7.14% vs. 6.82%, P = 0.907), or urinary infection (7.14% vs. 4.55%, P = 0.309). Additionally, there was no statistical difference between the groups in operation time (63.21 ± 5.20 vs. 62.07 ± 4.47 min, P = 0.059), postoperative off-bed time (7.02 ± 1.32 vs. 6.84 ± 1.20 days, P = 0.456), or duration of hospitalization (10.55 ± 1.15 vs. 10.15 ± 1.45 days, P = 0.092). However, Group B patients underwent an additional second-stage operation, incurring extra costs. Three months after surgery, Group A were judged more positively on the PPPS (specifically receiving higher scores on shaft skin and general appearance) by both the parents (shaft skin: 2.10 ± 0.82 vs. 1.93 ± 0.62, P = 0.024; general appearance: 2.16 ± 0.91 vs. 1.93 ± 0.72, P = 0.042) and the surgeon (shaft skin: 2.42 ± 0.70 vs. 2.25 ± 0.58, P = 0.025; general appearance: 2.38 ± 0.69 vs. 2.29 ± 0.51, P = 0.041). In most cases, the parents and surgeon were satisfied with the appearance of the genitals after one-stage repair. The advantages of the novel repair technique include use of a single-stage operation, producing a better appearance at a lower cost. The tunica vaginalis flap-covering method is not only demonstrated to be safe and effective, but it is also a simpler method than the conventional operation.

OR05-3 Utility of Genomic work-up for 46XY Disorder of Sex Development Patients with Severe Hypospadias

Abstract Background Hypospadias is a common human congenital anomaly occurring in ∼1: 200 to 1: 300 live male births in the USA, with an unexplained doubling in incidence over previous decades. Historically, 46XY Differences of Sex Development (DSD) patients, with severe hypospadias have lacked a genetic diagnosis. With the advent of commercially available DSD genomic panels, this is now changing (GeneDX. Neonatal 46 2021, In-vitae 2021). The goal of this study is to review the anatomical characteristics of severe hypospadias that can accurately define patients that have 46XY DSD and to determine the practical utility of performing a genomic workup in these rare 46XY DSD patients with severe hypospadias.Patients: 14 patients seen in disorders of sexual development clinic between 2018–2020 with severe hypospadias defined by an ectopic urethral meatus typically in the scrotum or perineum, an abnormally small glans penis size (&amp;lt;14 mm at maximal width in the first year of life), severe curvature and associated foreskin fusion or penoscrotal transposition were enrolled in the study. Methods Patients who met the criteria for 46XY disorder of sexual development and severe hypospadias were offered a genomic work-up in consultation with pediatric genetics. Patients were identified through chart review. Data extracted included karyotype, hypospadias phenotype including stretched penile length at diagnosis, age at genetic diagnosis/genomic workup, gene defect/ pathologic variant, gender identity, and clinical course.Main Outcome Measurements: The main outcome measured was the presence of a genetic mutation that can be linked to the disorder of sexual development. Secondary outcomes measured included gender identity, clinical course, and histological findings if a biopsy was obtained. Results Of the 14 patients that underwent genomic analysis, 8 had documented pathologic variants known to cause hypospadias. Pathologic genetic variants include defects in the AR (3), SRD5A2 (1), NR5A1 (2), WT1 (1), and ARTX (1). Five had no evidence of pathologic variants based on the Invitae and GeneDx commercial 46XY DSD panel (see Table 2 for genes tested). One had a genetic variant of unknown significance, FREM2. The patient with WT1 was consequently found to have a Wilms tumor and the patients with SRD5A2 are now undergoing adrenal insufficiency surveillance after their genetic testing. Conclusions Patients with 46XY DSD and severe hypospadias can benefit from genetic evaluation as their underlying disorders may unveil mutations that could have potentially life-altering consequences and change surveillance and monitoring. Presentation: Saturday, June 11, 2022 12:00 p.m. - 12:15 p.m.

Incomplete penoscrotal transposition as a phenotype of 49XXXXY Klinefelter variant and its surgical management

The 49XXXXY Klinefelter variant is rare and chromosomal analysis is required for diagnosis. We discuss the surgical management of incomplete penoscrotal transposition and consider this characteristic as an associated genital anomaly. We report the case of a patient who initially presented with a poor urinary stream. Physical examination revealed an incomplete penoscrotal transposition and a subcoronal hypospadias. Investigations included blood tests, abdominal Ultrasound and a chromosomal analysis. A multidisciplinary team (MDT) approach facilitated diagnosis of the 49 XXXXY Klinefelter variant. At MDT, medical and surgical management were discussed with the parent. We opted for delayed surgical correction of the incomplete penoscrotal transposition in the pre-pubertal period due to unavailability of topical hormone therapy. Operative technique aimed to relocate the scrotum in a dependent position. This was achieved via an inverted omega incision to create scrotal flaps that were rotated inferomedially for midline closure. Post-operatively, the patient can now urinate standing up with a good stream and has improved cosmesis. Incomplete penoscrotal transposition can be surgically corrected in the pre pubertal period with a good functional outcome. Chromosomal analysis is necessary in evaluating this associated genital anomaly and for diagnosis of 49XXXXY Klinefelter variant. • The 49XXXXY Klinefelter variant is rare and diagnosed by chromosomal analysis. • Incomplete penoscrotal transposition (PST) may be an associated phenotype. • Incomplete PST can be surgically corrected in the pre pubertal period.

SINGLE STAGE HYPOSPADIAS REPAIR IN PRIMARY CASES AND THEIR PATIENT RELATED OUTCOMES – TERTIARY CARE CENTER EXPERIENCE

Objective: This study aims to study whether hypospadias repair in primary cases can be done in a single stage. We present patients’ perspective and their outcomes in 268 primary hypospadias cases among which around 90% of patients (n=240) had their hypospadias surgery after recommended age of repair (18 months). All primary cases were managed in a single stage. Material &amp; Methods: From January 2010 – December 2019, 377 patients were included and outcomes were documented. Patients with prior hypospadias with complications (n=48), chordee without hypospadias (n=16), isolated penile torsion (n=12), and 33 patients who were lost to follow-up were excluded. The age at surgery, location of the meatus, chordee, associated anomalies, techniques used for correction, postoperative complications with overall success rate and patient related outcomes were evaluated. Results: After orthoplasty, distal hypospadias was seen in 59% (n=158), middle in 22.8 % (n=61) &amp; proximal in 13.1% (n=35) patients. Fourteen patients (5.2%) had penoscrotal transposition. Chordee was present in 71.2% (n=192) cases. Urethral closure was done using TIP alone in 18 cases, TIP, and spongioplasty (n=233). The success rate of one-stage surgery was 73.5% in our series which correlated with PRO with high significance. Conclusion: The rate of complications increased with the length of urethral tube reconstruction. Hypospadias fistula was the most common, followed by glanular dehiscence. The use of the TIP in primary repairs, spongioplasty &amp; additional buffering layers reduced the complication rates. Patients can have acceptable residual chordee and torsion and their surgical results correlated well with patient related outcomes(PRO).

Effectiveness of penis up transfer method for penoscrotal transposition in children

To investigate the effectiveness of penile up transfer method in the treatment of penoscrotal transposition (PST) in children. 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. 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). 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.

46 XY disorder of sex development (DSD) due to 5 alpha (SRD5A2) deficiency – Experience from a multidisciplinary Pediatric Gender Clinic

SRD5A2 deficiency leads to incomplete masculinization of individuals with a 46 XY karyotype. A definitive diagnosis in early infancy facilitates decisions concerning choice of sex of rearing and management. To review the clinical presentation, diagnosis, treatment and outcome of children with 46 XY DSD due to SRD5A2 deficiency at a Paediatric Gender Clinic. Retrospective review of cases of SRD5A2 deficiency (2000-15) managed with a standard protocol at a multidisciplinary clinic. Demographic data, clinical presentation, physical findings, investigations (hormonal profile, imaging, genitoscopy), psychological evaluation (child, family), medical and surgical management, outcome and follow up were collated and analyzed. There were 12 cases aged 3 days-14 years at presentation, 3 had parental consanguinity. Eight were reared as males and 4 as females. Specialist referral was sought for hypospadias (5), atypical genitalia (5) or incongruent pubertal masculinization (2). All had chordee, symmetrical inguinoscrotal gonads, rugose labioscrotum and proximal hypospadias (perineoscrotal -9, perineal -3). Both pubertal cases had significant masculinization and no gynecomastia. The median testosterone/dihydrotestosterone ratio was 22.1(IQR-8.6-55.7). Despite a classical phenotype, four (2 prepubertal, 2 pubertal) had a ratio <10. Genitoscopy showed urogenital sinus remnant (4) and hypoplastic verumontanum (5). Sex reassignment was done in 4. Surgical management was staged and completed by 4 years in those with infantile presentation. Besides correction of chordee and urethroplasty in 11, other procedures included orchidopexy (5), excision of a urogenital sinus remnant (4) and correction of penoscrotal transposition (4). The urethroplasty was single staged in 3. All operated cases were followed up (mean age at last follow up - 10.63 years, mean follow up period - 7.25 years). The overall cosmetic result was satisfactory, but the phallic structure remained relatively small across prepubertal period. Uroflowmetry curves were normal in 9. All showed penile tumescence/erection and two peripubertal cases had typical secondary sexual characters. All cases, including those with sex reassignment, have a well-adjusted male psyche. The diagnosis, management and longitudinal follow up of cases of SRD5A2 deficiency at a multidisciplinary gender clinic is presented. Diagnostic dilemmas with low T/DHT ratios remained in a third of cases. Most were diagnosed in infancy and assigned a male sex of rearing, all underwent staged masculinizing genitoplasty. Those with sex reassignment also fared well with comprehensive management after family counseling.

Congenital perineal defect with rectal duplication and extreme hypospadias\u2014a rare association

BackgroundCongenital perineal hernia is an extremely rare entity with only 10 cases having been reported in literature. We report a newborn with exposed bowel loops coming out through a defect in the perineum to the right of the median raphe and propose the term perineoschisis for this abnormality. The exposed bowel loops were a sequestered segment of colon. The baby also had extreme perineal hypospadias and left undescended testis.Case presentationA term neonate, born of caesarean section, was noted at birth to have exposed bowel loops visible in the perineum. The bowel loops were not covered by any membrane. To avoid injury to the exposed bowel loops and setting in of infection, the baby was taken up for emergency surgery. At laparotomy, the bowel loops were traced from duodeno–jejunal junction to ileocecal junction and sigmoid colon and rectum. No loops were seen herniating outside from the abdomen through the perineum opening. The extruded bowel loops were sequestered and ending blindly close to the rectum. The sequestered bowel loops were excised completely and perineum reconstruction was done.ConclusionThe extruded bowel loops would appear to be a form of colonic duplication with the duplicated colonic loops herniating outside through an opening in the perineum. Since the bowel loops were lying to the right of median raphe and were not covered by hernia sac or any other membrane we propose the term perineoschisis for this abnormality. The baby also had extreme hypospadias with a perineal meatus, bifid scrotum and penoscrotal transposition. This association of herniated duplicated colon with extreme hypospadias is hitherto unreported to the best of our knowledge.

Bilateral Fetal Hydronephrosis presenting with Complete Penoscrotal Transposition at Birth: What to tell the Prospective Parents?

Bilateral Fetal Hydronephrosis presenting with Complete Penoscrotal Transposition at Birth: What to tell the Prospective Parents?

Hypospadias

Hypospadias

V03-08 TRUE DIPHALLIA: EVALUATION AND SURGICAL CORRECTION OF A RARE CASE OF COMPLETE DUPLICATED PENIS

You have accessJournal of UrologyPediatrics (V03)1 Sep 2021V03-08 TRUE DIPHALLIA: EVALUATION AND SURGICAL CORRECTION OF A RARE CASE OF COMPLETE DUPLICATED PENIS Amr A. Elbakry, Chad Crigger, and Osama Al-Omar Amr A. ElbakryAmr A. Elbakry More articles by this author , Chad CriggerChad Crigger More articles by this author , and Osama Al-OmarOsama Al-Omar More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000001991.08AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: We are presenting a rare case of true diphallia. In this video, we will present our evaluation and demonstrate the surgical technique for management of a patient with complete duplicated penis. METHODS: Our patient is an 11-month-old male who presented initially as 28-day-old that was referred for evaluation of abnormal male genitalia. Physical examination showed duplicated penis with penoscrotal transposition and large scrotal lipoma. VCUG was done and showed Y-shaped duplicated urethra, with normal right urethra and atretic narrow left urethra. Diagnosis was confirmed with US and MRI, that showed complete duplication of the penis with separate two corpora cavernous and a corpus spongiosum and urethra in each penis. We decided to proceed with surgical correction with amputation of the left penis as right penis has the normal urethra evidenced by the VCUG. We started our procedure with performing a cystourethroscopy that confirmed normal right urethra that in continuation with prostatic urethra and bladder neck. Next, bilateral sub-coronal circumferential incisions were made, and complete deglovig of both penises was completed. The lipoma of the scrotum was dissected excised. The left penis was amputated, and the stump was oversewn. Next, we turned our attention to correction of pent-scrotal transposition. The two hemiscrotums were mobilized at the level of the peso-scrotal junction, paying attention to maintain penile skin attached at peno-pubic junction to maintain blood supply. The scrotal flaps were brought ventrally and approximated in the midline in 3 layers. penile skin was trimmed and redistributed for penile coverage. penile skin was approximated ventrally in the midline and was sutured to the foreskin of the left penis. A catheter was left place and dressing was applied. RESULTS: Our procedure was successful with total operative time of 4 hours, minimal blood loss and the patient was discharged home on the same day. Urethral catheter was removed at POD7. There was no reported Complications, no fistula, no wound dehiscence or discharge. Pathology confirmed the diagnosis of accessory penis with normal left and right corpora cavernosum and atretic urethra. CONCLUSIONS: We showed a case of a complete duplicated penis which is a rare congenital anomaly of the genitalia. We demonstrated a surgical technique for correction and reconstruction of the male genitalia in this rare case. Source of Funding: None © 2021 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 206Issue Supplement 3September 2021Page: e224-e224 Advertisement Copyright & Permissions© 2021 by American Urological Association Education and Research, Inc.MetricsAuthor Information Amr A. Elbakry More articles by this author Chad Crigger More articles by this author Osama Al-Omar More articles by this author Expand All Advertisement Loading ...

Surgical Correction of True Diphallia in a Newborn Male

Diphallia is an exceedingly rare anomaly characterized by partial or complete duplication of the phallus. Approximately 100 cases have been reported worldwide since its initial documentation, and incidence is estimated at 1 in 5 to 6 million live births. Therapeutic management is dependent on the extent of the anomaly, ranging from phallic excision to complex reconstructive procedures in cases of broader systemic involvement. We present the case of congenital true diphallia with associated penoscrotal transposition, bifid scrotum, partial urethral duplication, ventral chordee, large scrotal lipoma and sacral dimple. We further present a review of available literature pertaining to diphallia.

Proximal Hypospadias and Acquired Cryptorchidism: Incidence, Morphology and Potential Clinical Implications.

We identified the incidence of acquired cryptorchidism among patients with proximal and mid shaft hypospadias, predictors of acquired cryptorchidism, and the prevalence of testis-epididymis nonfusion with ascended testes. We hypothesized that proximal hypospadias would be associated with higher incidence of acquired cryptorchidism than mid shaft hypospadias, and that ascended testes would exhibit increased prevalence of testis-epididymis nonfusion similar to anatomical findings in an undescended testis. A retrospective cohort study of patients who underwent primary proximal and mid shaft hypospadias repair from 2010 to 2016 was conducted. Clinical and operative notes were reviewed. Patients with congenitally undescended testes or differences of sex development were excluded. A total of 175 patients were identified. Those with proximal hypospadias (14/104, 13%) were more likely than those with mid shaft hypospadias (1/71, 1%) to develop acquired cryptorchidism (p=0.04). Among proximal hypospadias patients, increased risk of acquired cryptorchidism was associated with pre-term birth (p <0.01) and penoscrotal transposition (p=0.01) but not with testis position on initial examination (p >0.99). In the 14 proximal hypospadias patients with acquired cryptorchidism, 21 ascended testes underwent orchiopexy. Operative notes adequately described testis-epididymis anatomy for 8/21 ascended testes. Testis-epididymis nonfusion was described in 6/8 ascended testes. Risk of acquired cryptorchidism is increased among patients with proximal hypospadias. Operative notes revealed a high rate of epididymal nonfusion with ascended testes, suggesting these testes morphologically resemble undescended testes. Close followup of testis position is needed in these patients, and the threshold to perform orchiopexy may need to be lower in select patients.

Surgical outcomes and socio-demographic pattern of hypospadias patients treated in a tertiary care center in Kabul, Afghanistan

Hypospadias is a congenital anomaly of male external genitalia and is the second most common congenital anomaly of male newborns after cryptorchidism. Although its prevalence in Afghanistan is unknown, anecdotal evidence shows that hypospadias is an important clinical and psychosocial problem in Afghanistan. There is no data available regarding the socio-demographic pattern of the disease and age at the time of surgery in Afghanistan context. The aims of this study were to identify socio-demographic pattern of hypospadias among patients and to determine surgical outcomes of hypospadias patients operated in a tertiary care center in Kabul. A descriptive case series design and non-probability consecutive sampling were used. Study population was 86 patients undergone urethroplasty during January 2018 to September 2019. A structured questionnaire was used to assess the dependent and independent variables. Follow up notes of patients' medical record files were reviewed to assess post-operative condition of the patients along with presence of any post-operative complications. The mean age at the time of repair was 6.2 years. Family history was positive in 16.3% and consanguinity in almost 56% of the patients. The most common type of hypospadias was distal shaft type (30.2%), penile chordae was present in 25.6% and pinhole meatus in 32.6% of the cases. The most common associated anomaly was cryptorchidism (9.3%) followed by inguinal hernia (2.3%). Peno-scrotal transposition was the most common sexual differentiation disorder (11.6%), however majority didn't have any disorder of sexual differentiation (83.7%) The most common complication was urethral stricture and meatal stenosis (9.3% each) followed by urethra-cutaneous fistula (7%) in this study, although remarkable majority (74.4%) was complication free. Urethral advancement procedure was more practiced in distal type (94.7%) and 2-stage repair for the proximal type (70%) of hypospadias. Findings showed that the age at repair for hypospadias in our center is much higher than the recommended age. Different than the literature, the most common complication was meatal stenosis. Majority of patients were result of consanguineous marriages. Procedure choice was different according to the type of hypospadias and preference of the surgeon.