Expression of Rab25 is down-regulated in the foreskin of children with hypospadias.

BACKGROUND: Hypospadias, a congenital malformation of the penis, is one of the newborns' most common developmental defects. The incidence of hypospadias is increasing yearly, and its pathogenesis is closely related to genetic susceptibility and environmental exposure to endocrine disruptors. Exploring the hypospadias' key molecular regulatory mechanism is crucial to reducing its incidence. OBJECTIVE: To examine the differential expression of Rab25 in hypospadias and normal penile tissue and to identify whether it is a candidate gene for exploring the mechanism of hypospadias. STUDY DESIGN: This study included 18 children aged 1-6 years undergoing hypospadias repair surgery at the Children's Hospital of Chongqing Medical University, and foreskin samples were collected. Children diagnosed with cryptorchidism, intersex status, or endocrine abnormalities were excluded from this study. Another 18 children aged 3-8 years with phimosis were included in the control group. The specimens were used for immunohistochemistry, western blotting, immunofluorescence, and polymerase chain reaction to assess the expression of Rab25. RESULTS: Rab25 protein expression was lower in the hypospadias group than in the control group [ (2.101 ± 0.1845), (0.7506 ± 0.1779), p = 0.0008 < 0.05). The hypospadias group showed decreased expression of Rab25 protein in the epithelial cell layer. Rab25 mRNA levels were downregulated in the foreskin of children with hypospadias compared with controls [(1.697 ± 0.2005), (0.7687 ± 0.2130), p = 0.0053 < 0.05)]. DISCUSSION: Rab25 mRNA and protein expressions in the hypospadias group were significantly downregulated compared with the control group. This was consistent with the results of single-cell sequencing of fetal mice reproductive nodules at 15.5 days of gestation (Zhang Z, Liu Z, Zhang Q, et al., unpublished observations). Our study represents the first report of abnormal Rab25 expression in the foreskin tissue of patients with hypospadias. More detailed research on the relationship between Rab25 and urethral development could be conducted to reveal the molecular mechanism of hypospadias. CONCLUSION: The expression of Rab25 in foreskin tissue was lower in the hypospadias group than in the control group. Rab25 is involved in the formation of the urethral seam and the occurrence of hypospadias. The potential mechanism by which Rab25 affects the canalization of the urethral plate needs to be further investigated.

Megaprepuce: A New Surgical Technique with Excellent Cosmetic and Functional Results.

Background: Congenital megaprepuce (CMP) is a malformation consisting of redundant inner foreskin, normal penile shaft, and severe phimosis. The excess inner prepuce pushes the penile shaft deeper causing the appearance of a buried penis. We describe a novel surgical technique using dartos fascial flaps to reconstruct the prepuce giving excellent cosmetic and functional results. Patients and Methods: Penile reconstruction was performed by a single surgeon in 07 cases of CMP between January 2018 and December 2019. In all cases, the described surgical technique was used. Following surgery, cosmetic and functional outcomes were reviewed as well as the incidence of complications. The patients' ages ranged from 15 to 27 months (mean = 19). Mean hospital stay was 9 hours (range = 7-12). Operating time was between 50 and 85 minutes. Results: Postoperative appearance of the penis was satisfactory for all the parents in our group. Swelling of the penile shaft was seen in all of our patients but settled within one week of surgery. Scrotal hematoma was seen in only one of our patients, but that also did not need any intervention and settled spontaneously. During the follow-up period (mean = 14.5 months, range 6-24) no patient underwent revision surgery. In all our patients, the final appearance was of a circumcised penis. The final similarity to a normal circumcised penis was excellent in all of our patients. Correction of penoscrotal transposition was done in all patients achieving an elongated penile shaft and almost invisible postoperative scarring. Conclusion: Our modified surgical procedure to correct CMP is a safe and simple technique, providing good cosmetic results with the appearance of a standard circumcised penis. This also provides a good functional outcome with complication rate quite low. This technique is also easy to reproduce and teach compared with other complex procedures.

Androgen receptor expression in preputial dartos tissue correlates with physiological androgen exposure in congenital malformations of the penis and in controls.

INTRODUCTION: The androgen receptor (AR) plays an important role in the development of male genitalia, and impaired androgen signalling has been hypothesised to underlie congenital penile malformations (CPM) such as hypospadias. Previous studies exploring the role of AR expression in the development of CPM have yielded conflicting results. OBJECTIVES: To assess AR expression in human foreskin of boys/men born with hypospadias, buried penis versus controls. STUDY DESIGN: Foreskin samples of 428 boys and men undergoing primary penile surgery (198 controls, 197 hypospadias, and 33 buried penis) were collected between October 2013 and July 2018. AR staining was performed in all samples and semi-quantitatively scored by two researchers independently, using a modified quick score (mQuicks) that assesses the proportion and intensity of AR staining in smooth muscle fibres. RESULTS: The interobserver variability of the mQuicks had a high level of agreement for the total score, as well as for the subscores. Two phases of high AR expression were observed in all groups, the first following the postnatal gonadotropin surge (i.e., mini-puberty) and the second in (pre-) puberty. No differences in AR expression were found in hypospadias or buried penis cases as compared to controls matched for age at time of surgery. DISCUSSION: This study describes the physiological evolution in AR expression in the human foreskin of boys with CPM and explains the cause of the previously reported, conflicting results. Despite the very large cohort, the limitations of this study are the low number of cases younger than six months at the time of surgery and the lack of Tanner stages to correlate with the mQuicks in adolescents. CONCLUSIONS: The mQuicks is a straightforward and informative tool to semi-quantitatively assess AR expression in the dartos tissue. In this study, AR expression in human foreskin shows a bimodal distribution in boys with CMP and controls, following physiological androgen exposure. No statistically significant difference in AR expression could be found between both groups. Whether other local mechanisms are affected by these physiological changes is currently unclear. However, strict age-matching should be considered when exploring the mechanisms underlying disturbed penile and urethral development in CMP.

Megaprepuce: presentation of a modified surgical technique with excellent cosmetic and functional results.

OBJECTIVE: Congenital megaprepuce is a malformation consisting of a great redundancy of the inner preputial skin over a penis with normal shaft and glans and is combined with a severe phimosis. Patients suffer from difficulties in voiding because the urine is trapped in the large dome-shaped megaprepuce. We describe a modification of the surgical technique of reconstructing a megaprepuce initially presented by Leao et al. PATIENTS AND METHODS: We retrospectively reviewed 7 patients aged 6-53 months (mean age 17 months, 6 were younger than 18 months) who underwent congenital megaprepuce repair between 02/2014 and 05/2018 in our institution. All these otherwise healthy children suffering from difficulties in voiding and reporting genital ballooning during micturition and urinary retention were referred to our hospital. In all cases, parents needed to express the trapped urine. Four of these patients additionally showed a glanular hypospadias, another one a distal penile hypospadias. In addition to the repair of the megaprepuce, six patients needed correction of a penile curvature, five of whom needed correction of the chordee and one a corporoplasty (Schröder-Essed). The patient showing the distal penile hypospadias additionally underwent hypospadias repair. During the follow-up, we evaluated the cosmetic result and complications such as secondary concealed penis, difficulties in voiding, urinary retention, and urinary infections. RESULTS: Mean follow-up was 18 months. All patients following surgery showed normal voiding without urinary retention or urinary infections and good cosmetic results resembling a circumcised penis in appearance without reconcealment. No intraoperative complications occurred. One patient had a scrotal hematoma postoperatively. Mild transient edema of the penis was seen in all patients, which disappeared spontaneously within one week after surgery. CONCLUSION: Our surgical approach is a safe and relatively simple procedure with a low rate of complications, good cosmetic results, and functional outcome. Whether the hypospadias associated with ventral curvature was a coincidence or part of the disease pattern remains unclear but will probably be the object of further investigations.

Zebras in Foreskin Dermatopathology: A Review.

OBJECTIVES: The aim of the study was to review uncommon foreskin dermatopathology conditions clinically and pathologically. METHODS: A database search of PubMed and Google Scholar were extracted between March 1, 2009, and March 1, 2019, using the search terms "foreskin," "prepuce," "penis," "pathology," "dermatology," and "rare." The search was limited to "humans" and "dermatopathology." Full article texts were reviewed. Reference lists were screened for additional articles. Patient details (diagnosis, dermatopathology, treatment, and follow-up if available) were extracted. We excluded articles written in the non-English language, unusual variants of common conditions, and cases of common dermatologic conditions. RESULTS: A list of 369 articles was identified and another screening identified 30 articles for rare foreskin pathologies. Those are divided into categories based on the following etiologies: (a) benign, including congenital (e.g., aposthia), infectious (graft versus host disease and histoplasma), autoimmune (Crohn's disease and pyoderma gangrenosum), and benign neoplasms (neurofibroma, apocrine hidrocystoma, verruciform xanthoma, porokeratosis, penile cutaneous horn, localized amyloidosis) and (b) malignancies, including primary (myeloid sarcoma, basal cell carcinoma, Kaposi's sarcoma, mucosal-associated lymphoid tissue lymphoma), and metastasis. CONCLUSIONS: We reviewed and discussed unusual benign and malignant dermatopathology conditions that can affect the foreskin.

Mid- to long-term outcomes of the 'anatomical approach' to congenital megaprepuce repair.

INTRODUCTION: The understanding of the aetiopathogenesis and the long-term outcomes of correctional repair of congenital megaprepuce (CMP) remains unestablished. Different techniques have been described; however, optimum results have been difficult to achieve. OBJECTIVE: The aim of our study was to review our experience of the 'anatomical approach' to the correction of CMP. MATERIAL AND METHODS: This is a retrospective review of boys who underwent CMP repair between 2003 and 2014. All of them had the anatomical approach: A curved incision is made between the scrotal and penile skin. Circumferential dissection superficial to Buck's fascia frees the penis and allows the scrotum to assume a more caudal position. The dissection distally lifts the preputial sac that is opened ventrally. The redundant inner preputial skin is excised. After full degloving, the dorsal flap of skin is thinned. The base of the flap is anchored, and the rectangle of skin is wrapped around the shaft. The remaining diamond-shaped incision is closed in a vertical line. Postoperatively, foam dressings and urethral catheter are left in place. Symptomatology, hospital stay, postoperative complications, redosurgery, voiding and cosmesis were evaluated. RESULTS: Sixty-one patients had surgery at 5-151 months (median 17 months). Median hospital stay was 24 h (12-168 h). Foam dressings and urethral catheter were used in 47 boys and removed in outpatient clinic after 3-7 days. There were two immediate postoperative complications requiring surgery. One due to severe oedema and constriction ring, and the other had wound infection with wound breakdown, both in patients with no dressings (P < 0.05). Follow-up was available for 58 patients, with a median of 22 months (3-79 months). Two required redosurgery (3.5%), one with poor cosmetic result and one recurrence. In all patients, a normal circumcised appearance had been achieved and in 47, cosmetic result was excellent (81%). Five patients have residual suprapubic fat, and four, excess ventral skin. One with residual suprapubic fat has reached puberty and it has resolved. All declared good stream. The same technique was used for six patients with poor cosmetic result after surgery elsewhere. The outcome is excellent for all, with median follow-up of 20.5 months (5-31 months). CONCLUSION: The 'anatomical' repair provides the ability to create the appearance of a standard circumcision, which is generally accepted as normality. Apart from the penis itself, this technique facilitates correction of the penoscrotal transposition, giving the external genitalia the best appearance. It seems to provide good functional and cosmetic outcomes, and it holds true at mid- to long-term follow-up. Dressings and catheter can potentially prevent postoperative complications. However, a prospective study with standardised parameters is required to confirm these findings.

Histological and morphological characteristics of the prepuce of penis skin structure in different age groups.

BACKGROUND: Hypospadias is one of the most common congenital abnormalities in childhood. The number of cases has rapidly grown in recent years. OBJECTIVES: The purpose of this research was to analyze the histological and morphological differences of the foreskin samples taken from boys in three age groups. STUDY DESIGN: A total of 30 Asian patients participated in the research. Clinical materials obtained via biopsy were divided into three age groups. The first group included 10 biopsy materials of preputial skin taken from boys aged <3 years. The second included 10 similar biopsy materials from boys aged 3-5 years. The third included 10 biopsy materials taken from boys aged 5-7 years. The skin areas were taken from the dorsal, two lateral and the ventral surfaces (closer to the bridle) with dimensions of 1.0 × 1.0 cm. All removed foreskins underwent histological examination. RESULTS: Obtained results showed that the number of vein clusters in the prepuce and the cases of vessel wall fibrosis grew with age. It is worth noting that no such discoveries were made in younger boys (aged <3 years). Sample analysis showed that the number of nerve, vessel, and collagen fibers increased with age. DISCUSSION: It is believed that it is important to continue investigating the prepuce in hypospadias, in order to gain a better understanding of the abnormality depending on type. CONCLUSION: Peculiarities of prepuce in hypospadias discovered in different age groups allowed a full understanding of the pathology development processes.

[Circumcision versus the foreskin-deglove plus shaft-fix procedure for phimosis or redundant prepuce in obese adult patients].

OBJECTIVE: To compare the clinical effects of circumcision and the foreskin-deglove plus shaft-fix (FDSF) procedure in the treatment of phimosis or redundant prepuce in obese adult males (body mass index [BMI] ≥ 28 kg/m²). METHODS: Forty-four obese adult men with phimosis or redundant prepuce underwent circumcision (n = 24) or FDSF (n = 20) according to their own wishes. The patients in the circumcision and FDSF groups were aged (26.38 ± 4.24) and (26.90 ± 3.14) years, with BMIs of (27.77 ± 0.77) and (28.07 ± 2.28) kg/m² and penis lengths of (3.51 ± 0.46) and (3.50 ± 0.59) cm, respectively. The operations were performed under local anesthesia with lidocaine plus ropivacaine mesylate. RESULTS: The operation time of circumcision was (28.04 ± 2.65) min and that of FDSF was (45.45 ± 3.49) min. At 6 months after surgery, normal penile erection was found in all the patients, the penis length was significantly longer in the FDSF than in the circumcision group ([5.01 ± 0.73] vs [3.70 ± 0.47] cm) , and the rate of satisfaction with penile appearance was markedly higher in the former than in the latter group (3.25 ± 0.71 vs 2.83 ± 0.56). CONCLUSION: The foreskin-deglove plus shaft-fix procedure under local anesthesia with lidocaine and ropivacaine mesylate may achieve desirable penile erection and appearance in the treatment of phimosis or redundant prepuce in obese adult patients.

Plasmocitoma extramedular em bulbo peniano de cão: relato de caso / Extramedullary plasmacytoma in the penile bulb of a dog: case report

O objetivo deste relato de caso é descrever a ocorrência de plasmocitoma em bulbo peniano de um cão, classificado como uma doença extramedular não cutânea de localização rara e casuística inédita. Um cão, sem raça definida, com sete anos de idade e pesando 15kg, não castrado, apresentou histórico clínico de anorexia, vômitos, anúria e constipação. Ao exame específico da genitália externa, foi encontrada uma massa em bulbo peniano durante a inspeção do prepúcio, aderida à pele e encapsulada, extremamente firme e arredondada, medindo cerca de 6cm de diâmetro. Por meio da ultrassonografia dessa estrutura, foi observado aumento do volume regional com ecotextura heterogênea e ecogenicidade mista, além de neovascularização tecidual ao Doppler colorido. Foi realizada biópsia da massa, sendo verificada a presença de neoplasia de células redondas. A caracterização do tumor foi realizada pela imuno-histoquímica, e as células neoplásicas foram imunoexpressas para CD79a e MUM1, indicando o diagnóstico de plasmocitoma extramedular. Embora os tumores penianos em cães sejam os predominantemente venéreos transmissíveis (TVT), e os plasmocitomas sejam neoplasias raras nessa localização, este relato de caso fornece com ineditismo a ocorrência de plasmocitoma extramedular em bulbo peniano de cão, condição ainda não descrita em veterinária.

Non-cutaneous extramedullary plasmacytomas are relatively rare in dogs, affecting mainly the oral cavity and bowel loops. The involvement of the penile bulb has not been described, a fact of great importance for obstetric and veterinary oncology. The aim of this case report is to describe the occurrence of plasmacytoma in a dog's penile bulb, classified as a non-cutaneous extramedullary disease of rare location and unpublished casuistry. A non castrated dog of undefined breed, with seven years of age and weighing 15kg, presented clinical history of anorexia, vomiting, anuria and constipation. By specific examination of the external genitalia, a penile bulb mass was found in the preputial inspection, which was adhered to the encapsulated skin, extremely firm and rounded, measuring approximately 6 cm in diameter. By ultrasound evaluation of the structure in the penile bulb an increase of regional volume with heterogeneous echotexture and mixed echogenicity and tissue neovascularization upon color Doppler was observed. Incisional biopsy of the mass was performed and showed the presence of neoplasia of round cells. The characterization of the tumor was performed by immunohistochemistry and the neoplastic immuno cells were expressed CD79a and MUM1, indicating the diagnosis of extramedullary plasmacytoma. Although the penile tumors in dogs are predominantly transmissible venereal tumors (TVT) and plasmocytomas are rare neoplasms in this location, this case report provides a novel occurrence of extramedullary plasmacytoma in the penile bulb of a dog, a condition not yet described in veterinary.

Limited Panniculectomy for Adult Buried Penis Repair.

Patients with buried or hidden penis may be unable to carry out normal hygiene, void with a directable urine stream, or be sexually active as a result of the condition. Although these patients are nearly always obese, weight loss often does not reverse the problem, as the mons pannus may remain after weight loss. Furthermore, associated penile skin changes such as lichen sclerosus or stenosis of the penile shaft skin are often irreversible. Treatment includes removal of the diseased shaft skin surrounding the penis, in combination with a limited panniculectomy. The authors present their technique for this procedure in a typical patient with buried penis that prevented him from voiding effectively.

A new surgical technique for concealed penis using an advanced musculocutaneous scrotal flap.

BACKGROUND: Until recently, no single, universally accepted surgical method has existed for all types of concealed penis repairs. We describe a new surgical technique for repairing concealed penis by using an advanced musculocutaneous scrotal flap. METHODS: From January 2010 to June 2014, we evaluated 12 patients (12-40 years old) with concealed penises who were surgically treated with an advanced musculocutaneous scrotal flap technique after degloving through a ventral approach. All the patients were scheduled for regular follow-up at 6, 12, and 24 weeks postoperatively. The satisfaction grade for penile size, morphology, and voiding status were evaluated using a questionnaire preoperatively and at all of the follow-ups. Information regarding complications was obtained during the postoperative hospital stay and at all follow-ups. RESULTS: The patients' satisfaction grades, which included the penile size, morphology, and voiding status, improved postoperatively compared to those preoperatively. All patients had penile lymphedema postoperatively; however, this disappeared within 6 weeks. There were no complications such as skin necrosis and contracture, voiding difficulty, or erectile dysfunction. CONCLUSIONS: Our advanced musculocutaneous scrotal flap technique for concealed penis repair is technically easy and safe. In addition, it provides a good cosmetic appearance, functional outcomes and excellent postoperative satisfaction grades. Lastly, it seems applicable in any type of concealed penis, including cases in which the ventral skin defect is difficult to cover.

[Outward versus inward placement in Shang Ring circumcision for phimosis and redundant prepuce in adult men: analysis of 527 cases].

OBJECTIVE: To observe the clinical effects of two different circumcision procedures with the Shang Ring and compare their advantages and disadvantages. METHODS: A total of 527 adult males with phimosis or redundant prepuce underwent Shang Ring circumcision by conventional outward replacement (n = 254) and inward placement (n = 273), respectively. We observed the in-ring nocturnal pain, complications, ring-removal pain, degree of edema, recovery time, and patients' satisfaction after surgery, and compared them between the two groups. RESULTS: Compared with the conventional outward placement (5.9%) of the Shang Ring, the inward placement method showed the advantages of mild in-ring nocturnal pain, a low complication rate, significantly reduced ring-removal pain, and mild edema, but exhibited longer healing time. CONCLUSION: In Shang Ring circumcision for phimosis and redundant prepuce in adult males, each of the outward and inward placement methods has advantages and disadvantages of its own, but the latter is more advantageous and feasible.

[Shang Ring circumcision by transverse incision in the distal penis foreskin and pull-up of the interior board for short frenulum praeputii].

OBJECTIVE: To investigate the effectiveness of surgical strategies for Shang Ring circumcision in the treatment of short frenulum praeputii in patients with redundant prepuce or phimosis. METHODS: Totally, 130 cases of short frenulum praeputii with redundant prepuce or phimosis were randomly assigned to an experimental group and a control group of equal number to receive Shang Ring circumcision, the former by transverse incision in the distal penis foreskin and pull-up of the interior board, and the latter by conventional transverse incision and longitudinal suture of the frenulum praeputii. Comparisons were made between the two groups in the surgical duration, intraoperative blood loss, 24 h postoperative pain visual analog score (VAS), postoperative complications, satisfaction with the penile appearance, and the quality of sexual life. RESULTS: The surgical duration, intraoperative blood loss, 24 h postoperative VAS, postoperative sexual satisfaction, and satisfaction with penile appearance were (4.60 +/- 1.20) min, (2.61 +/- 1.81) ml, 1.73 +/- 0.76, 98.5%, and 98.5%, respectively, in the experimental group, as compared with (21.60 +/- 6.30) min, (11.10 +/- 3.40) ml, 5.37 +/- 1.84, 70.3% and 69.8% in the control, with statistically significant differences between the two groups (P < 0.05). The incidence rates of such major complications as wound dehiscence, infection, and moderate to severe edema were 1.5% (1/65), 3.1% (2/65), and 4.6% (3/65), respectively, in the experimental group in comparison with 12.3% (8/65), 15.3% (10/65), and 30.7% (20/65) in the control, with statistically significant differences between the two groups (P < 0.05). None of patients had any serious complications. CONCLUSION: Shang Ring circumcision by transverse incision in the distal penis foreskin and pull-up of the interior board, with its advantages of shorter operation time, less blood loss, mild pain, fewer complications, and higher satisfaction and acceptance of the patients, can be used as an safe and effective approach to the treatment of short frenulum praeputii.

Double-V scrotoplasty for repair of congenital penoscrotal webbing: a hidden scar technique.

OBJECTIVE: Penoscrotal webbing (PSW) is a common reason for deferral of neonatal circumcision. Reports of successful procedures and outcomes in the literature are sparse. We have performed double-V scrotoplasty (DVS), a modification of a V-Y technique, in 138 patients with excellent results. PATIENTS AND METHODS: We retrospectively reviewed the charts of boys who had undergone DVS for PSW since January 2009 by a single surgeon (S.A.A.). The indications, intraoperative findings, concomitant procedures, outcomes, and complications were recorded. RESULTS: A total of 138 DVSs were performed. Concomitant genital surgeries included 81 hidden penis repairs and 10 other (hernia, hypospadias, chordee, orchidopexy). The median age at the time of surgery was 9.6 months (6.1 months-9.8 years). Patients were evaluated about 1 month postoperatively. In seven cases (5%), minor skin separation occurred at the penoscrotal junction but all healed completely. Superficial skin infection occurred in one patient. None required reoperation and cosmetic results were subjectively excellent. CONCLUSIONS: PSW has been corrected in 138 patients without significant complications and with excellent results. This is the largest known peer-reviewed series evaluating a surgical technique for congenital PSW repair. We believe our technique is simple, reproducible, and, with no diverging suture lines lateral to the median raphe, improves cosmesis.

DOuble LOngitudinal Megapreputium Incision TEchnique: the DOLOMITE.

OBJECTIVE: To present a straightforward, reproducible technique with the basic principle of preserving all available outer penile shaft skin and using this to cover the dorsal side of the penis. The DOuble LOngitudinal Megapreputium Incision TEchnique is presented in a step-by-step fashion. MATERIALS AND METHODS: Six consecutive patients with a mean age of 13.3 months (range, 7-25) underwent this reconstruction between 2006 and 2011. The technique starts with 2 longitudinal incisions, the first on the ventral side, and the second on the dorsal side of the penis. After hinging the penile skin to the dorsal side, redundant inner preputial tissue is resected using diagonal incisions. Dartos is spared. A comparison with previously reported techniques and a concise review of existing literature are provided. RESULTS: The final cosmetic results, after an initial period of edematous swelling of the ventral aspect of the penis, were good in all patients. The bilateral diagonal scars on the penile shaft became inconspicuous over time. Voiding normalized. No complications occurred. CONCLUSION: The DOuble LOngitudinal Megapreputium Incision TEchnique provides a straightforward and reproducible surgical correction for congenital megaprepuce.

Vaginoplasty using the inner surface or mucosa of the prepuce in children with congenital adrenal hyperplasia.

OBJECTIVE: To describe and present the results of the use of the inner surface or mucosa of the prepuce in vaginoplasty for ambiguous genitalia. PATIENTS AND METHODS: This technique can be used alone in low vaginal insertion or associated with a total urogenital mobilization in cases of high vagina. During the past 7 years, we have performed 18 vaginoplasties using the described technique in 46, XX DSD (female pseudohermaphroditism). All patients had 21-hydroxylase deficiency; four had the simple virilizing form and 14 had additional salt loss. The age of the patients at surgery varied from 4 to 28 months (average 11.2). RESULTS: The final cosmetic results were considered good by the parents and surgeon for the majority of our patients, and satisfactory in three cases. Clinical examination revealed stenosis of vaginal introitus in only one patient (which responded very well to one dilatation) and one urethra-vaginal fistula. CONCLUSION: This technique of vaginoplasty, simple in its realization and answering the purpose of treatment with few complications, can be used in the feminizing genitoplasty of young children with 46,XX DSD, ovotesticular DSD (true hermaphroditism), mixed gonadal dysgenesis and dysgenetic male pseudohermaphroditism.

Congenital absence of preputial foreskin: an extremely uncommon anomaly.

The part of the penile skin that covers the glans penis is named prepuce or foreskin. The embryologic development of the prepuce and urethra is related. Several congenital anomalies of the prepuce have been previously reported, but the absence of the prepuce with a normal development of the urethra is a very rare association. We report a sporadic case with absence of the prepuce and normal urethral development.

Congenital Megaprepuce: surgical approach.

OBJECTIVE: To describe surgical procedure and results of Congenital Megaprepuce (CMP) that consists in phimosis and megaprepuce; glans and corpora trapped into pre-pubic fat; incomplete micturition with typical "ballooning" aspect. METHODS: Genitoplasty with penoscrotal separation (GPS) was performed at Pediatric Surgery Department of Hospital Nord - Marseille, between July and December 2010, in 5 uncircumcised children with CMP, by the same surgeon. Transversal incision at the ventral penoscrotal junction was used, with shaft bipartition and Buck's ventral fascia exposition. The inner layer of prepuce was resected while the outer layer was preserved for coverture of the penile shaft. The dorsal foreskin was medially and longitudinally incised to obtain adequate length of the dorsal shaft. The first middle dorsal suture was made using PDS 6.0 and completed in ventral direction with interrupted stitches. The urinary catheter was placed and hydrocolloid duoderm dressing used. Perioperative prophylactic antibiotic was given and pain control achieved with paracetamol and codeine. RESULTS: All patients presented urinary troubles before surgery. Skin penile coverage was complete in all patients with cosmetically and functionally satisfying results; no complication was observed at 12 months mean follow-up. CONCLUSION: CMP is a specific entity of "inconsistent penis." Surgical treatment should be discussed at diagnosis, resolving micturition troubles. GPS is a simple and reproducible procedure by experienced surgeons. Excellent cosmetic and functional results are obtained at mean follow-up, even if long-term results are required. GPS can be useful in other malformations of the penile shaft.