Enlarged prostatic utricle and vagina masculinus in boys with disorders of sex development.

PURPOSE: To determine the prevalence, radiological characteristics, and clinical symptomatology of enlarged prostatic utricles and vagina masculinus in boys with disorders of sex development. METHODS: Over 10 years (from February 2012 to March 2022), 102 boys with severe hypospadias underwent voiding cystourethrography. All patients presented with disorders of sex development and Y material in the karyotype (46,XY karyotype, 46,XY/45,X mosaic, etc.). The age of the patients at the first examination ranged from 4 days to 27 months (mean age 2.7 months). RESULTS: Voiding cystourethrography revealed the presence of a cyst posterior to the urethra in 66 patients (64.7% of cases). There were 15 cases of "vagina masculinus" and 51 of enlarged prostatic utricles. These dilations were classified according to the Ikoma classification and cyst size. In more than 2/3 of cases, the cysts were small (less than 20 mm), and in less than 8% of cases, these cysts were large. In addition, retrograde opacification revealed the presence of vesicoureteral reflux in 20% of boys with a male vagina. The most severe hypospadias with a scrotal or perineal meatus are most at risk of developing an enlarged prostatic utricle, and 80% of patients with Ikoma Grade III had a scrotal or perineal meatus. CONCLUSION: This study shows that the prevalence of enlarged prostatic utricles and vagina masculinus is high in patients with severe hypospadias. Therefore, their search should be systematic, and for clinical and therapeutic interest, the enlarged prostatic utricles should be classified according to cyst size.

Anatomical Explanations of the Pathogenesis of Proximal Hypospadias.

AIMS: The aim of this study was to try to explain the pathogenesis of proximal hypospadias based on anatomical and histological findings. METHODS: During 9 years, we performed systematic biopsies (in the lateral areas of the urethral plate, as well as under this plate) in 81 patients treated for proximal hypospadias. The histological study was performed by routine coloring, hematoxylin and eosin, and Masson's trichrome, which colors the collagen fibers in blue, and monoclonal antibody against alpha-smooth muscle actin. RESULTS: There is a fibrosis tissue abnormally present on the ventral side of the penis. This tissue consists of a mixture of fibrous connective tissue, nerve nets, short vessels, and smooth muscle fibers. The penis' dartos does not contain smooth muscle fibers. These fibers can come from a blood vessel or spongy tissue which existed during the neonatal period in the distal part of the penis before disappearing. CONCLUSIONS: The proximal hypospadias is due presumably to avascular necrosis of the distal part poorly vascularized of the corpus spongiosum.

Can We Preserve the Urethral Plate in Proximal Hypospadias Repair?

PURPOSE: To know the limits of the preservation of the urethral plate in the correction of the curvature associated with proximal hypospadias. METHODS: From January 2002 to January 2015, we treated 224 patients of proximal hypospadias. In 182 cases, we used of the lateral pictures taken during the successive saline erection tests to measure the correction obtained after each stage. RESULTS: The analysis of the lateral pictures taken at the successive saline erection tests clearly demonstrated that release of the skin and dartos fascia provides an important correction of the chordee. This correction is complete of all curvature lower than 45 degrees and in 3 fourths of cases for curvatures between 45 and 90 degrees. However, for angles 90 degree and more, the release of the skin and dartos fascia was insufficient in more than 73.7% of cases. And the mobilization of the urethral plate with resection of the underlying fibrous tissue realized gives a very low correction of the chordee (0-20 degrees). CONCLUSIONS: The essential factor responsible of curvature associated with proximal hypopsadias is the fibrosis tissue present on the ventral side of the penis. However, in the severe forms, a short urethral plate must be transected to obtain a complete correction of the chordee.

Penile strangulation by hair.

PURPOSE: To know the causes and results of treatment of complications from a penile strangulation by hair in seven boys. METHODS: From April 2000 to December 2012, seven boys presenting serious penile complications by hair strangulation have been operated in two centers. All seven boys had transection of the urethra at the coronal level. And none of our patients were at the stage of necrosis or amputation of the glans. The age of the patients at surgery ranged from 34 to 134 months (mean 96 months). RESULTS: Four children presented urethrocutaneous fistulas (57% of cases), and the innervation and vascularization of the glans remained poor after repair in three patients. CONCLUSION: The penile strangulation by hair was due to a simple accident. However, several predisposing factors were discovered in our patients: lack of cleanliness; a moist environment with nocturnal enuresis; the presence of pubic hair in young children reported in the four children, and a coronal sulcus not covered by the foreskin in circumcised children.

The Use of Urogenital Mobilization Extended by Two Flaps From the Redundant Distal Part of the Urethra in the Repair of the Severe Form of Distal Vaginal Agenesis.

OBJECTIVE: To describe and present the results of the use of urogenital mobilization extended by two flaps from the redundant distal part of the urethra in the correction of a severe form of distal vaginal agenesis. METHODS: During the past 4years (from December 2018 to March 2023), we have performed 2 vaginoplasties using the described procedure. The age of the patients at surgery was 13years. Two girls presented with primary amenorrhea associated with cyclic pelvic pain evolving over several months. Clinical examination revealed a blind vagina and an anterior pelvic mass. Ultrasound and magnetic resonance imaging showed a severe form of distal vaginal agenesis. One patient had a 47,XX+mark karyotype and right vesicoureteral reflux. Two patients underwent urogenital mobilization, guided by a tube introduced into the upper part of the vagina through an abdominal approach, and the use of two flaps from the redundant distal part of the urethra to extend the neovagina to the perineum. RESULTS: The esthetic results were very satisfactory with the disappearance of pelvic pain, and the restoration of a regular flow of menstrual blood without pain. Urinary continence was preserved in two patients. CONCLUSION: This is the first report of the use of urogenital mobilization extended two flaps from the redundant distal part of the urethra in the correction of a severe form of distal vaginal agenesis. This procedure yielded a very satisfactory esthetic and functional results.

Prevalence and causes of meatal stenosis in circumcised boys.

INTRODUCTION: The prevalence of meatal stenosis after circumcision remains unclear, and its causes are unknown. OBJECTIVE: To know the prevalence and the causes of meatal stenosis after circumcision in boys. STUDY DESIGN: Between October 2018 and April 2019, we carried out a prospective cross-sectional study on 1031 circumcised boys, aged 5-8 years (mean age 6.1 years ± 0.3 years), enrolled in the first level of primary school. All enrolled children underwent genitourinary examination. Moreover, an anonymous questionnaire was filled-in by one of the parents. RESULTS: The screening revealed the presence of meatal stenosis in 185 children, representing a prevalence of 17.9% of cases (95% CI = 15.6-20.3). Analysis of the results using both the univariate and multivariate mode brought out some common risk factors such as a foreskin that completely adheres to the glans with forceful retraction of the prepuce and the use of a healing product: Beta-sitosterol and Hydrocotyl (Centella Asiatica), rarely Trolamine. In addition, this study showed that boys circumcised during their first week of life are twice as likely to develop meatal stenosis than those circumcised between 7 and 12 months (OR = 2.08; 95% CI = 1.10-3.92, p = 0.021). DISCUSSION: We believe that when the foreskin is fully attached to the glans, forced retraction of the prepuce most often causes a loss of the mucous membrane that covers the glans, making the glans less resistant to chemical attack, and therefore may play an important role in the development of meatal stenosis. However, This study is limited by the absence of a cohort of uncircumcised boys for comparison to see if the small diameter of the meatus is also present in this group. CONCLUSIONS: This study showed that stenosis of the urethral meatus is a frequent complication of circumcision. Circumcision in the first week of life, complete adhesion of the foreskin to the glans, and the use of a healing product were associated with the risk of stenosis development.

Comparative study of two techniques used in distal hypospadias repair: tubularized incised plate (Snodgrass) and tubularized urethral plate (Duplay).

OBJECTIVE: The aim of this study was to compare the results of the tubularized incised plate (the Snodgrass technique) and tubularized urethral plate (the Duplay technique) in distal hypospadias repair. MATERIAL AND METHODS: Between April 2000 and September 2008, 245 distal hypospadias was corrected by a single surgeon: 132 patients underwent repair by tubularized incised plate and 113 by tubularized urethral plate. The age of the patients ranged from 16 to 48 months (mean 27 months). RESULTS: Mean follow-up was 84 months (21-120 months). With the tubularized incised plate, the meatus was vertically oriented and expanded to the apex of the glans, and the rate of fistulae formation was low (3%) but that of neourethral stenosis was high (22%). The Duplay technique led to fistulae in nine patients (8%) and stenoses in only eight (7%). CONCLUSIONS: The tubularized incised plate gave an excellent cosmetic result with fewer fistulae; however, more stenoses occurred than with the tubularized urethral plate technique.

Koyanagi- Snow-Hayashi Urethroplasty in Severe Hypospadias Repair: Between Hope and Reality.

OBJECTIVE: To present the results of Koyanagi-Snow-Hayashi urethroplasty in severe hypospadias repair. METHODS: From June 2015 to December 2019, 43 patients with severe hypospadias associated with curvature underwent Koyanagi- Snow-Hayashi urethroplasty. All patients were treated for the first time and by a single surgeon. Thirty-five patients included in this study presented a form of disorders of sex development. The age of the patients at surgery varied from 12 to 120 months (mean age 21 months ± 6 months). The median follow-up was 28 months (range, 5-65 months). RESULTS: Functional and esthetic results were satisfactory with a straight and excellent appearance of the penis, without any complications in only ten patients (23%). While 30 of 43 patients (70 %) required additional procedures. Postoperative complications encountered in our patients were dominated by fistulas which were found in 17 of 43 patients (39.5%). Among them, 3 fistulas appeared in the penile shaft between 11 and 28 days and disappeared spontaneously. While proximal fistulas have been required surgical repair. Diverticulum seemed to be a significant complication of this technique, it was found in 4 of 43 (9%). This study also revealed the presence of a peculiar complication for this procedure, urethral meatus in the shape of fish lips. CONCLUSION: The use of Koyanagi-Snow-Hayashi urethroplasty in severe hypospadias repair gives a complete penile straightening in all cases. However, Despite the modifications added by Snow and Hayashi to Koyanagi urethroplasty to improve the vascular supply of the flap, the rate of complications encountered with this technique remains high.

Complete penile disassembly in epispadias repair.

PURPOSE: To report current results of complete penile disassembly technique in epispadias repair. METHODS: In ten years, we have preformed 31 complete penile disassembly for proximal epispadias repair. Twenty-four patients had epispadias after primary repair of bladder exstrophy and 7 isolated penopubic epispadias. The age of the patients ranged from 10 months to 6 years (median 3 years). RESULTS: The shortening of urethral plate was found in 30 patients (97% of cases), and this shortening varied between 6 and 16 mm. However, in one patient we found a lengthening of the urethral plate of 8 mm. The narrowing of urethral plate was found in all patients, and this narrowing varied between 30 and 50% of the width of the plate. Postoperative complications encountered in our patients were dominated by fistulas and dehiscence, particularly in patients who had bladder exstrophy-epispadias complex. After dehiscence and fistulas repair, the cosmetic results were satisfying in 25 patients (80.5% of cases) with conical glans and meatus in orthotopic position without any necrosis of the glans. However, the urinary continence ≥ 1 h was observed in 6 patients (19% of cases) and only 3 patients (9.7% of cases) had a urinary continence ≥ 3 h. The mean follow-up was 61 months. CONCLUSIONS: The complete penile disassembly remains one of the best techniques for epispadias repair. However, we noticed a reappearance of the dorsal curvature of the penis in a large number of patients treated for isolated epispadias and the impact of this technique on urinary incontinence remains uncertain.

Vaginoplasty and creating labia minora in children with disorders of sex development.

PURPOSE: To report current results of vaginoplasty using the mucosa of the prepuce, and creating labia minora by penile skin in children with disorders of sex development (DSD). METHODS: In 10 years, we have performed 22 vaginoplasties using the described technique of vaginoplasty, 21 patients with 46, XX DSD and 1 ovotesticular DSD. The assessment of the results of this technique of vaginoplasty was undertaken in several stages: (a) The evaluation of the cosmetic result. (b) The research for a urinary incontinence and urethrovaginal fistulas. (c) The research for a vaginal stenosis by the introduction of a lubricated feeding tube into the vaginal cavity. The labia minora was evaluated by three criteria: its skin should be thin and very supple, it should have a free edge which partially or totally covers the clitoris, urethral meatus, and vaginal orifice; and it is preferable that its color be darker than the rest of the skin. RESULTS: The cosmetic outcome was considered by parents and the surgeon as very satisfactory in 11 patients (50% of cases), satisfactory in 4 patients (18.2%), and unsatisfactory in 7 patients (31.8%). The postoperative complications were five cases of proximal stenosis (22.7%), one distal stenosis (stenosis of introitus) and two necrosis of the preputial flap. No urethrovaginal fistula and urinary incontinence were reported. CONCLUSIONS: In infant and young child, when it is difficult to make use of complete urogenital mobilization, the mucosa of the prepuce can be an alternative to create a neovagina, its histological constitution is identical to a vaginal wall, and it does not prevent to have a good labia minora.

What is the pathogenesis of proximal hypospadias?

OBJECTIVE: To report the information concerning the pathogenesis of proximal hypospadias and causes of curvature associated with it. MATERIAL AND METHODS: From January 2009 to December 2015, 74 patients underwent repair of proximal hypospadias. In 70 patients we performed a systematic biopsies in the lateral areas of the urethral plate, as well as under this plate. The study of the histological structure of these areas was performed using routine staining with hematoxylin and eosin, and the Masson's trichrome which color the collagen fibers in blue and monoclonal antibody against alpha-smooth muscle actin. RESULTS: This prospective study shows that the fibrotic tissue abnormally present on the ventral side of the penis consists of a mixture of fibrous connective tissue, nerve nets, short vessels, and smooth muscle fibers. In contrast to the scrotal dartos, penile dartos fascia does not contain smooth muscle fibers. Therefore, these fibers may come from a blood vessel or spongy tissue which existed during neonatal period in the distal part of the penis before disappearing. In addition, in 13 cases, the presence downstream of the urethral meatus, of a bifurcation of corpus spongiosum into two branches supposes that the corpus spongiosum is form by fusion around the urethra of two mesenchymal bodies. The arterial supply of this purely masculine formation originates from a new vascularization and it is probably, developed under secretion of androgens (angiogenic substances in target tissues). CONCLUSION: These findings allow us to suggest that the proximal hypospadias is due to avascular necrosis of the distal part ie. poorly vascularized part of the corpus spongiosum.

Is there a shortening of the urethral plate in complete penile disassembly used in epispadias repair, and what is its impact on the final outcomes?

PURPOSE: To assess the importance of shortening of the urethral plate that occurred with complete penile disassembly technique in epispadias repair and its impact on cosmetic and functional results (on urinary incontinence). METHODS: From January 2009 to December 2016, 26 boys underwent complete penile disassembly technique for proximal epispadias repair. Twenty-one patients had epispadias after primary repair of bladder exstrophy, and 5 patients had isolated penopubic epispadias. The age of the patients ranged from 11 months to 6 years (median 3 years). RESULTS: After disassembling the penis in three parts, the shortening and narrowing of urethral plate were found in all patients; the shortening varied between 2 and 16 mm. However, in isolated epispadias, the urethral plate is easily extensible. The cosmetic results (after dehiscence and fistulas repair) were found to be satisfactory in 24 patients with conical glans and meatus in the orthotopic position without any necrosis of the glans. However, 18 patients (81.8% of cases) who initially had a bladder exstrophy presented a dehiscence or fistula. The urinary continence ≥ 1 h was observed in 5 patients (19% of cases), and only 3 patients (11.5% of cases) had a urinary continence ≥ 3 h. CONCLUSIONS: The complete penile disassembly procedure restores the normal anatomy of the penis. Despite the shortening and narrowing of the urethral plate, the cosmetic results were good in the majority of patients. However, its functional outcomes on urinary incontinence, particularly for epispadias with bladder exstrophy, remain uncertain.