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J P Gearhart

Publications and source records attributed to J P Gearhart.

At least 19 recordsLinked to original sources

Reconstructive lower urinary tract surgery in incontinent adolescents with exstrophy/epispadias complex.

OBJECTIVES: To outline the management strategies applied to the adolescent patient population with exstrophy/epispadias and incontinence at our institution. These patients present a difficult management problem. At the same time they are dealing with difficult issues, including body image and sexual awareness, the added burden of ongoing incontinence causes major anxieties and lifestyle restrictions. In many, incontinence has persisted despite numerous operations. METHODS: A total of 25 (19 male and 6 female) patients who remained incontinent into adolescence or early adulthood were reviewed. Of the 25 patients, 19 had exstrophy, 4 had cloacal exstrophy, and 1 male and 1 female had epispadias. Six patients had undergone eight prior attempts at continent reconstruction, one had undergone cutaneous diversion, and one had problems after ureterosigmoidostomy. RESULTS: The mean age at continence surgery was 12.9 years. Of the 25 patients, 18 underwent bladder augmentation, with a continent stoma in 17 and an artificial sphincter in 1. An additional 5 patients underwent bladder neck transection, with a new continent stoma in 3. The ureterosigmoidostomy was converted to a Mainz II pouch. One patient underwent continent neobladder formation. Nine patients (36%) developed complications during follow-up. Three required stoma revision for stenosis and one for prolapse. Pouch stones occurred in 4 patients, and vesicocutaneous fistula developed in 1. All achieved full urinary continence. The mean follow-up was 72.4 months. CONCLUSIONS: Some children with exstrophy/epispadias reach adolescence and remain incontinent. For these patients, modern reconstructive techniques provide hope of continence. With careful preoperative assessment, exact surgical precision, and regular follow-up, a successful outcome can be expected in virtually all cases without the need for external urine collection devices.

Adolescent↗

The use of combined bladder and epispadias repair in boys with classic bladder exstrophy: outcomes, complications and consequences.

PURPOSE: Despite widespread use of modern staged reconstruction for classic bladder exstrophy, there remains a role for combined bladder closure and epispadias repair when primary closure is delayed or initial reconstruction has failed. The principle of combining bladder and urethral closure in 1 operation was first proposed more than 40 years ago, and represents a demanding technical procedure. We recount our experience to date with this approach. MATERIALS AND METHODS: A total of 38 boys underwent combined bladder and epispadias repair using pelvic osteotomies. Five cases were delayed primary closures owing to a bladder template unsuitable for newborn closure. A total of 30 cases were previous failed bladder closures, with concurrent epispadias repair in 6. The 3 remaining cases were staged closures where the epispadias repair failed, leading to bladder prolapse via the posterior urethra. Overall, there was a major bladder prolapse in 25 cases, and separation of the pubic symphysis with dehiscence of anterior abdominal wall structures and bladder in 8. RESULTS: Mean age at surgery was 26.5 months for the 33 reclosures. A total of 10 boys had development of a urethrocutaneous fistula and 4 had development of strictures. A total of 19 patients required additional procedures of the bladder neck (endoscopic), urethra or penis. A later bladder neck reconstruction was undertaken in 19 boys, of whom 12 are continent. Seven boys underwent continent diversion and 5 are considering the procedure. A total of 14 boys are awaiting adequate capacity for bladder neck reconstruction. Ureteral reimplantation was performed in 22 patients, and no patient was rendered hypospadiac. CONCLUSIONS: When combined closure is applied to failed previous exstrophy repair one can expect at best a 50% continence rate without continent diversion. Furthermore, based on the experience at this institution the majority of patients require ureteral reimplantation, and many require additional surgery to the penis or urethra. However, with the application of modern reconstructive techniques continence and a cosmetically pleasing phallus can be expected in most cases, although at the expense of multiple surgical procedures.

Bladder Exstrophy↗

Polyps in the exstrophic bladder. A cause for concern?

PURPOSE: The role of environmental injury in carcinogenesis is widely recognized. Malignancy in exstrophic bladders has been reported most frequently in untreated adults and those undergoing surgical treatments which involve the mixing of fecal and urinary streams. The question of whether the closed exstrophic bladder has a similar potential for malignancy has not been resolved. The polypoid appearance of the exstrophic bladder template raises the concern of premalignant lesions. We characterized the histology of these lesions and analyzed their microscopic features with particular reference to predisposition for dysplasia. In doing so, we attempt to address the aforementioned question and set the stage for definitive quantification of the risk of malignancy in these patients with careful, long-term followup. MATERIALS AND METHODS: Under institutional board review, the slides of 38 patients with classic bladder exstrophy who had polyps excised at the time of closure were reviewed by a single genitourinary pathologist (JIE). The most common findings were reported for polyps resected at primary and secondary closure, respectively, and a comparative analysis was performed. RESULTS: Of the 38 cases 24 were primary closures and 14 were secondary closures. Six of the primary closures were delayed by 6 weeks or greater. The 2 basic types of polyps observed were fibrotic and edematous. Both types were associated with overlying reactive squamous metaplasia in approximately 50% of cases. Varying degrees of fixed on file Brunn's nests, cystitis cystica and cystitis glandularis were noted. Cystitis glandularis was observed in a significantly greater percentage of secondary closures (p = 0.0014). CONCLUSIONS: Although no dysplasia was noted, cystitis glandularis is associated with the development of adenocarcinoma of the bladder. The finding of cystitis glandularis suggests a more severe epithelial injury and it follows that the significant majority of these cases (10 of 14, 71.4%) were observed with polyps resected during secondary closure. These patients warrant future surveillance with urine cytology and cystoscopy as they enter adult life.

Bladder Exstrophy↗

Attitudes of adult 46, XY intersex persons to clinical management policies.

PURPOSE: We surveyed a clinic sample of adult 46,XY intersex patients regarding attitudes to clinical management policies. MATERIALS AND METHODS: All adult former patients of 1 pediatric endocrine clinic in the eastern United States whose addresses could be obtained and who consented to participation were surveyed by a comprehensive written followup questionnaire. Three questions on attitudes concerning the desirability of a third gender category and the age at which genital surgery should be done were presented in the context of ratings of satisfaction with gender, genital status and sexual functioning. RESULTS: A total of 72 English speaking patients with 46,XY, including 32 men and 40 women 18 to 60 years old, completed the questionnaire. The majority of respondents stated that they were mainly satisfied with being the assigned gender, did not have a time in life when they felt unsure about gender, did not agree to a third gender policy, did not think that the genitals looked unusual (although the majority of men rated their penis as too small), were somewhat or mainly satisfied with sexual functioning, did not agree that corrective genital surgery should be postponed to adulthood and stated that their genital surgeries should have been performed before adulthood, although there were some significant and important differences among subgroups. CONCLUSIONS: The majority of adult patients with intersexuality appeared to be satisfied with gender and genital status, and did not support major changes in the prevailing policy. However, a significant minority was dissatisfied and endorsed policy changes.

Adolescent↗

Loss of the penile glans and/or corpora following primary repair of bladder exstrophy using the complete penile disassembly technique.

PURPOSE: The technique of penile disassembly is increasingly being used for the surgical repair of classical bladder exstrophy. We describe the complications and discuss the possible etiologies of genital injuries we have seen following this operation. MATERIALS AND METHODS: A review of the records of patients evaluated for management of genital complications following complete repair of bladder exstrophy from 1996 to 2003 was performed. RESULTS: Nine patients were evaluated for genital injuries following complete repair of bladder exstrophy using the penile disassembly technique. Injuries included the loss of 1 hemiglans and penile urethra in 2 cases; loss of 1 hemiglans and distal corpora in 2; loss of bilateral glans, distal corpora and penile urethra in 2; loss of 1 hemiglans, 1 corporal body, urethral plate and penile shaft skin in 1; loss of 1 hemiglans, distal corporal body and a portion of the urethra in 1; and loss of 1 hemiglans in 1. The exact etiology of these complications is unknown, and possibilities include, a technical mishap, induction of venous congestion/arterial spasm or disruption of a congenitally abnormal blood supply. CONCLUSIONS: The finding that primary repair of bladder exstrophy using the penile disassembly technique is associated with the risk of partial or complete penile loss dampens our enthusiasm for this procedure. The association of genital injuries with penile disassembly has resulted in a modification of this surgical technique that would hopefully lessen its risk.

Bladder Exstrophy↗

Coping with bladder exstrophy: diverse results from early attempts at functional urinary tract surgery.

OBJECTIVES: To report the long-term surgical outcome in a group of patients with bladder exstrophy treated from 1960 to 1982, and to assess physical health, social integration and sexual function, as attempts at functional closure during the development of this surgery resulted in patients with differing surgical status of the genitourinary organs. PATIENTS AND METHODS: A review of medical record archives revealed 36 patients; of these, seven had died, six were untraceable and six declined to participate, leaving 15 evaluable subjects (seven men and eight women, mean age at follow-up, 35 years). Data were collected from medical records and direct interviews were conducted using a semi-structured questionnaire, after obtaining informed consent. The Short Form-36 (SF-36) v2 Health Survey instrument was used to assess health and well-being. RESULTS: Three patients have retained the use of their bladder, and 11 had initial bladder closure but required (at a mean age of 3.1 years) various urinary diversions. Eight patients had a pelvic osteotomy. Of 27 functioning renal units, 25 are in reasonable to good condition. Six men and six women had genital reconstruction. Four men are capable of penetrative intercourse and ejaculate; six women manage penetrative intercourse and five have orgasms. All patients attended mainstream school and 13 achieved examination success. Nine patients took vocational training and 11 work full-time. Five patients are married and five are in long-term relationships. Two men have achieved three pregnancies and one women has had a child. The mean total SF-36 score (maximum 3600) was 2763 in men and 2235 in women. CONCLUSIONS: Surgery for bladder exstrophy has been developing for more than 40 years and the legacy of early attempts at functional closure is a population of adults who have a diversity of lower urinary tracts, good preservation of renal function overall and acceptable sexual function. They are usually robust, healthy and well-adjusted individuals functioning well in society, often in full-time employment and long-term relationships. Adolescent follow-up must be clearly focused, incorporating a multidisciplinary team approach to facilitate a seamless transition into adulthood.

Adaptation, Psychological↗

Urogynaecological and obstetric issues in women with the exstrophy-epispadias complex.

OBJECTIVE: To review of the sexual and urogynaecological issues faced by a large cohort of women with the exstrophy-epispadias complex (EEC). PATIENTS AND METHODS: The study comprised 83 women and girls with EEC; a confidential survey was mailed to identify their social and sexual concerns. Fifty-six women had classical bladder exstrophy (CBE), 13 had female epispadias (FE) and 14 had cloacal exstrophy (CE). Data on the initial method of reconstruction and urogynaecological problems were obtained from a review of the hospital records. Information on continence, infection and sexual function was obtained from 34 completed surveys. RESULTS: The bladder was closed in 51 patients with CBE and 13 with CE. Urinary calculi developed in 10 patients with CBE, two with FE and three with CE. Vaginal and uterine prolapse occurred an earlier age in patients with EEC. Eight women had 13 pregnancies, eight of which resulted in normal healthy children. Overall continence was achieved in 85% of the women surveyed. Urinary tract infections remained a frequent problem for women with EEC; only 27% of respondents indicated that they were infection-free. Women aged > 18 years (24) who responded indicated that they had appropriate sexual desire; 16 were sexually active and the mean age for commencing sexual activity was 19.9 years. Six patients had dyspareunia and 10 indicated that they had orgasms. However, five additional patients indicated that they had restricted intercourse, as they were dissatisfied with the cosmesis of their external genitalia. CONCLUSIONS: Sexual and gynaecological issues become increasingly important in patients with EEC as they become adults. Understanding these issues faced by patients with EEC as they mature will permit better counselling of future patients.

Adolescent↗

Simulated ischemia induces renal tubular cell apoptosis through a nuclear factor-kappaB dependent mechanism.

PURPOSE: Ischemia-reperfusion injury is a relatively common cause of renal tubular cell death and acute renal failure. While nuclear factor-kappaB has been implicated in the pathophysiology of renal ischemia-reperfusion injury, the effect of nuclear factor-kappaB inhibition on ischemia induced renal tubular cell death remains unknown. MATERIALS AND METHODS: Renal tubular cells (LLC-PK1) were exposed to simulated ischemia in the presence or absence of 10 microM. pyrrolidine dithiocarbamate (nuclear factor-kappaB inhibitor). Nuclear factor-kappaB activation (electrophoretic mobility shift assay and immunohistochemistry) and the effect of pyrrolidine dithiocarbamate on nuclear factor-kappaB activation (electrophoretic mobility shift assay) and ischemia induced apoptosis (terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphate nick end-labeling) were determined. RESULTS: Simulated ischemia induced nuclear factor-kappaB activation and renal tubular cell apoptosis versus controls (mean plus or minus standard error of mean 62 +/- 5.2 versus 0.4 +/- 0.3 apoptotic nuclei per high power field, p <0.05). In contrast, previous cellular exposure to pyrrolidine dithiocarbamate effectively inhibited nuclear factor-kappaB activation and prevented ischemia induced apoptosis (mean 14 +/- 6 apoptotic nuclei per high power field). CONCLUSIONS: Simulated ischemia induces nuclear factor-kappaB intranuclear translocation and activation in renal tubular cells. Furthermore, nuclear factor-kappaB mediates ischemia induced renal tubular cell apoptosis. Further elucidation of the complex role of nuclear factor-kappaB in inflammatory injury may lead to the development of targeted therapeutic strategies that ameliorate ischemic renal injury.

Animals↗

Hugh Hampton Young: a pioneer in pediatric urology.

PURPOSE: Hugh Hampton Young (1870-1945) is regarded as a major force in urology during the first half of the 20th century. While he is acknowledged for many advances in open and transurethral prostatic surgery, his contributions to pediatric urology have been less well recognized. MATERIALS AND METHODS: The manuscripts and autobiography of Hugh Hampton Young were reviewed and major contributions to pediatric urology were identified. Patient records corresponding to these areas were then obtained from the Alan Mason Chesney Medical Archives and reviewed. RESULTS: Hugh Hampton Young was the first clinically to recognize and surgically treat posterior urethral valves, the first to develop a procedure to correct incontinence in patients with the epispadias-exstrophy complex and the first to describe bilateral subtotal adrenalectomy for virilization secondary to congenital adrenal hyperplasia. He was successful in his endeavors and his techniques are still in use today or have become the building blocks of future modifications. CONCLUSIONS: The contributions of Hugh Hampton Young to pediatric urology were numerous. In addition to his accomplishments in academic and adult urology, Young should be recognized as a true pioneer in pediatric urology.

Bladder Exstrophy↗

Combined bladder neck reconstruction and epispadias repair for exstrophy-epispadias complex.

PURPOSE: The achievement of satisfactory continence in the management of classic bladder exstrophy remains a surgical challenge. During the last 20 years a staged approach to the management of the exstrophy-epispadias complex has been used at many exstrophy centers to attain this goal. In select cases repairs can be combined to reduce the number of mandatory operations to achieve continence. We retrospectively review our experience with, and long-term results and complications of combined bladder neck reconstruction and epispadias repair. MATERIALS AND METHODS: A total of 19 boys with classic bladder exstrophy (17) and complete male epispadias (2) underwent combined bladder neck reconstruction and epispadias repair between 1982 and 1999. Primary closure was performed elsewhere in 16 cases and osteotomy was performed at primary closure in 8 (42%). All patients have undergone modified Cantwell-Ransley epispadias repair except for 2 who underwent a Young procedure. RESULTS: At the time of combined bladder neck and epispadias repair mean patient age was 5.2 years (range 2.5 to 10). Mean bladder capacity was 119 ml. (range 60 to 250). Of the 19 patients 13 (69%) are completely continent, and 2 (11%) are partially continent and 1 remained incontinent. Three patients did not gain satisfactory functional bladder capacity after combined repair, and underwent bladder augmentation and a continent diversion procedures. CONCLUSIONS: Combined bladder neck and epispadias repair is applicable in experienced hands but careful patient selection and long-term followup are the most important issues to develop criteria to select those best to undergo this procedure.

Bladder Exstrophy↗

Complete repair of bladder exstrophy in the newborn: complications and management.

PURPOSE: Complete repair of bladder exstrophy in the newborn includes bladder, and posterior and penile urethral closure, along with epispadias repair and abdominal wall closure without bladder neck reconstruction. While reports have shown the need for early reimplantation of the ureters in 50% of patients and repair of hypospadias in those in whom the urethral plate does not reach the tip of the glans, to my knowledge none has described immediate postoperative complications in these patients. MATERIALS AND METHODS: Six males and 1 female were referred after "complete repair" procedure in the newborn period without osteotomy. Complete dehiscence with loss of the proximal urethra and a significant amount of penile skin occurred in 2 male patients. Bladder prolapse with loss of the urethra well onto the penis occurred in 2 newborns, of whom 1 had undergone 2 closures before referral and 1 had partial loss of the glans on 1 side. One patient had only minor bladder prolapse but lost most of the urethra and penile skin. The final male patient lost most of the urethra and had separation of the pubis but the bladder maintained a subcutaneous position and did not prolapse. The female patient had major prolapse of the bladder and loss of the urethrovaginal septum. RESULTS: The 2 patients with complete dehiscence underwent reclosure with bilateral innominate and vertical iliac osteotomy with replacement of the urethral plate with a skin graft and await epispadias repair. The patient with major prolapse and 2 prior closures was treated with reclosure, bladder augmentation and continent stoma reconstruction, and awaits epispadias repair. In the male patient with marked prolapse and partial loss of the glans has undergone reclosure with bilateral innominate and vertical iliac osteotomy was done and he awaits epispadias repair. The patient with minor prolapse was treated with reclosure with bilateral innominate and vertical iliac osteotomy, and reclosure with epispadias repair under testosterone stimulation. The patient in whom the pubis was separated and the urethra was lost underwent reclosure, epispadias repair and combined osteotomy under testosterone stimulation. The female patient was treated with reclosure with combined bilateral osteotomy and mobilization of an anterior bladder tube to use as a urethra with no attempt to establish continence. CONCLUSIONS: Complete repair of bladder exstrophy in the newborn period requires experience with the exstrophy condition, appropriate pelvic and patient immobilization, and excellent postoperative care. Complications with this repair are similar to those with modern versions of the staged repair, and its failures are similar. However, penile skin loss with dehiscence or prolapse is particularly difficult because of the paucity of skin already due to the exstrophy condition, for which skin grafting and testosterone stimulation are valuable adjuncts. Other than the patient who underwent augmentation and continent stoma construction, none has undergone any type of continence procedure, all are awaiting an increase in bladder capacity and followup is short. Complete repair of bladder exstrophy, whether in the newborn or older child, is a formidable task and not for the occasional exstrophy surgeon. While earlier reports of this procedure seem encouraging, complications are real and difficult to manage, even in experienced hands.

Bladder Exstrophy↗

Modified Young-Dees-Leadbetter bladder neck reconstruction in patients with successful primary bladder closure elsewhere: a single institution experience.

PURPOSE: Achievement of urinary continence in patients with the exstrophy-epispadias complex remains a challenge. We reviewed our experience with the modified Young-Dees-Leadbetter bladder neck repair in patients with bladder exstrophy who underwent primary bladder closure elsewhere. MATERIALS AND METHODS: We retrospectively reviewed exstrophy charts and database of 57 male and 11 female with classic bladder exstrophy who underwent bladder neck repair at our institute and successful primary bladder closure elsewhere during the last 2 decades. Osteotomy was performed at primary closure in 14 (20%) cases and 9 (13%) patients at bladder neck repair in 9 (13%) to aid in stabilizing the urethra and pelvic ring, and to help reapproximate the pelvic floor musculature facilitating urinary continence. RESULTS: Primary closure was done within 72 hours of life elsewhere in 41 (60%) patients, and between ages 72 hours and 5 years (most during the first month of life) in 27. Paraexstrophy skin flaps were used in 33 (48%) cases, and the most common complication was bladder outlet obstruction of the posterior urethra secondary to the skin flaps. Of the 68 patients 57 (83%) are continent and voiding per urethra without need for augmentation or clean intermittent catheterization, 9 (13%) required clean intermittent catheterization including 7 who underwent continent urinary diversion after failed bladder neck repair, and 2 are still incontinent due to a severe posterior urethral stricture. Urinary retention was the most common symptom after bladder neck repair which resolved following catheter dilation or prolonged suprapubic catheter drainage. CONCLUSIONS: Successful early primary closure of a good bladder template is the most important determinant of eventual bladder capacity and compliance.

Adolescent↗

Determinants of continence in the bladder exstrophy population: predictors of success?

OBJECTIVES: To delineate factors that may predict eventual urinary continence after bladder neck reconstruction (BNR) in the bladder exstrophy population. METHODS: The records of 65 patients who underwent all phases of bladder exstrophy reconstruction at our institution between 1975 and 1997 with greater than 1-year follow-up were reviewed and data analyzed. RESULTS: Fifty patients (77%) are continent day and night and voiding per urethra without need for augmentation or intermittent catheterization. Nine (14%) patients have social continence, dry for more than 3 hours during the day. Two patients required continent diversion for continence after failed BNR. Four patients are completely incontinent. The mean age of BNR was 4 years with a mean and median capacity of 93 and 85 cc (range 45 to 175). Analysis of bladder capacity measurements prior to BNR revealed that patients with a bladder capacity greater than 85 cc median capacity at the time of BNR had better outcomes. No correlation was found between the age of BNR and obtaining eventual continence. The mean time to daytime continence was 14 months (range 4 to 21) and the mean time to nighttime continence was 22 months (range 11 to 33). CONCLUSIONS: Determinants of continence in the bladder exstrophy population are multifactorial. In our experience, 77% of patients are completely dry, day and night, and 91% can achieve social continence, being dry for at least 3 hours. However, with careful evaluation of bladder capacity and bladder growth, urinary continence may be improved in this population with better patient selection.

Bladder Exstrophy↗

Metanephric stromal tumor: a rare benign pediatric renal mass.

We report a rare case and description of a benign pediatric renal mass. To our knowledge, this tumor has never been described in urologic published reports. It is possible that the identification of this renal tumor could spare a child the toxic adjuvant chemotherapy that would be administered if confused with histologically similar tumors such as clear cell sarcoma of the kidney.

Adenofibroma↗

Evaluation of the bony pelvis in classic bladder exstrophy by using 3D-CT: further insights.

OBJECTIVES: To provide a complete look at the bony pelvis in children with classic bladder exstrophy: dimensions, orientation, and relationships. METHODS: Three-dimensional computed tomography was used in 6 boys and 1 girl, 5 of whom underwent primary closure and 2 who underwent reclosure at 4 and 8 months. These exstrophy pelves (intrapelvic angles and osseous dimensions) were compared with 26 age and sex-matched controls. RESULTS: The iliac wing angle was 11.4 degrees larger in the classic bladder exstrophy cases. The sacroiliac joint angle was 9.9 degrees more externally rotated in the exstrophy cases. The pelvis was rotated 14.7 degrees in the superoinferior plane in the exstrophy cases. The mean pubic diastasis was 4.2 cm (0.6 cm in controls). The inter-triradiate distance in the patients with classic bladder exstrophy averaged 6.0 cm (4.2 cm in controls). CONCLUSIONS: These new findings provide a better understanding of the bony pelvis, especially its posterior portion, in patients with classic bladder exstrophy. The results of this study revealed the orientation of the sacroiliac joints to be more externally oriented than previously thought and the pelvis to be rotated inferiorly, a previously unknown observation. Both of these factors will be important in the planning of newer osteotomies and pelvic reconstruction.

Adult↗

Congenital micropenis: long-term medical, surgical and psychosexual follow-up of individuals raised male or female.

OBJECTIVES: To document long-term medical, surgical and psychosexual outcome of individuals with congenital micropenis (13 males, 5 females). METHODS: Physical measurements from childhood were collected retrospectively from medical records and at adulthood by physical examination. An adult psychosexual assessment was conducted with a written questionnaire and oral discussion. RESULTS: Adult penile length was below the normal mean in all men. Three women had vaginoplasty resulting in normal length. All men reported good or fair erections but 50% were dissatisfied with their genitalia. Dissatisfaction with body image resulted from having a small penis (66%), inadequate body hair (50%), gynecomastia (33%) and youthful appearance (33%). Ten men were heterosexual, 1 homosexual and 2 bisexual. Among women, 4 (80%) were dissatisfied with their genitalia. Three women reported average libido with orgasm and were also heterosexual. Two women had no sexual interest or experience. Finally, males were masculine and females feminine in their gender-role identity, and both groups were satisfied with their sex of rearing. CONCLUSIONS: Regarding choice of gender, male sex of rearing can result in satisfactory genito-sexual function. Female gender can also result in success, however it requires extensive feminizing surgery.

Adolescent↗