PURPOSE: Feminizing genitoplasty is the surgical management after female gender assignment for intersex patients. The surgical outcome and complications of 20 cases of feminizing genitoplasty were analyzed. MATERIALS AND METHODS: Between January 1988 and December 2003, 20 patients surgically treated by feminizing genitoplasty, were retrospectively reviewed. The mean ages at the time of diagnosis and surgical treatment were 6.25 and 7.35 years, respectively. The preoperative evaluations included history taking, physical examination, and chromosomal, hormonal, and radiological studies. All patients underwent feminizing genitoplasty, including at least one of clitoral reconstruction, vaginoplasty or labial reconstruction. The 20 patients were analyzed according to their karyotype, phenotype, gender of rearing, ages at diagnosis and operation, surgical procedures, complications and follow up. RESULTS: Of the 20 cases, there were 10 female pseudohermaphroditism, 6 male pseudo- hermaphroditism, 3 gonadal dysgenesis, and 1 Mayer- Rokitanski-Kuster syndrome. Within these cases, 14, 10 and 9 clitoral reconstructions, vaginoplasties and labial reconstructions were performed. The streak gonad was removed in all patients with gonadal dysgenesis. Postoperative cosmetic and functional effects were successful, with few complications. CONCLUSIONS: Considering our surgical outcomes, feminizing genitoplasty for intersex patients, who are determined to a female gender assignment, is a good surgical procedure.
46, XX Disorders of Sex Development ; Diagnosis ; Disorders of Sex Development ; Female ; Follow-Up Studies ; Gonadal Dysgenesis ; Gonads ; Humans ; Karyotype ; Male ; Phenotype ; Physical Examination ; Retrospective Studies ; Surgery, Plastic

OBJECTIVETo investigate the diagnosis and treatment of ovotesticular disorders of sex development (DSD) in children.

METHODSWe reviewed the clinical data of 9 cases of ovotesticular DSD admitted in our department from 1988 to 2007.

RESULTSThe patients ranged in age from 9 months to 9 years, 7 raised as males and 2 as females. As for the karyotype, 4 cases were 46,XX, 2 were 46,XX/46,XY, 1 was 46,XY, and the other 2 had no karyotype data. All of them presented with obscure external genitalia: perineal or penoscrotal hypospadias with or without cryptorchidism in males and hypertrophy of the clitoris in females. They were diagnosed with ovotesticular DSD by gonad biopsy and underwent genitoplasty.

CONCLUSIONThe gender assignment of the ovotesticular DSD patient was chiefly based on the development of external genitalia, dominant gonad, karyotype and the parent's will. Laparoscopic technology is recommended in gonad biopsy and orchiopexy during the treatment of ovotesticular DSD.

Child ; Child, Preschool ; Disorders of Sex Development ; diagnosis ; surgery ; Female ; Humans ; Infant ; Laparoscopy ; Male ; Retrospective Studies ; Sexual Development

Child ; Chromosomes, Human, X ; Cryptorchidism ; etiology ; surgery ; Disorders of Sex Development ; Gonadal Steroid Hormones ; blood ; Humans ; Hypospadias ; etiology ; surgery ; Male ; Penis ; surgery ; Sex Chromosome Disorders ; surgery ; Testis ; growth & development ; Urethra ; surgery

OBJECTIVETo evaluate the therapeutic effect of laparoendoscopic single-site surgery (LESS) for treatment of male pseudohermaphroditism.

METHODSA 17-year-old patient with male pseudohermaphroditism and a female social sex was admitted. According to the request by the patient and the relatives for a female gender, LESS vaginoplasty and cryptorchidectomy were performed using a single multilumen port inserted through a 2.5 cm incision below the umbilicus, followed by reconstruction of the perineal region by open surgery.

RESULTSThe total operative time was 7 h, and the LESS procedure lasted for about 3.5 h. No other port incision was needed. The estimated intraoperative blood loss was 400 ml. No electrolyte or metabolic acid-base balance disorders were observed perioperatively. In the follow-up examination at 6 months after the operation, the reconstructed vagina healed smoothly without obvious contraction or fixation failure, and the perineal region showed good appearance.

CONCLUSIONWith minimal invasiveness, LESS surgery produces good cosmetic effect and allows rapid postoperative recovery, thus may become a promising alternative to the management of pseudohermaphroditism.

46, XY Disorders of Sex Development ; surgery ; Adolescent ; Female ; Humans ; Laparoscopy ; methods ; Male ; Reconstructive Surgical Procedures ; methods ; Vagina ; surgery

We evaluated the long-term results of feminizing genital reconstruction in patients with genital ambiguity with high vaginal confluence. The medical records of 10 consecutive patients with ambiguous genitalia and high vaginal confluence who underwent feminizing genital reconstruction from 1996 to 2007 were reviewed. Seven patients had congenital adrenal hyperplasia, one had mixed gonadal dysgenesis, one had partial androgen insensitivity, and one had 5-alpha reductase deficiency syndrome. Median age at operation was 21 months (range, 2-47 months). Median follow up was 7.7 yr. Of the six patients who underwent feminizing genital reconstruction with the Gonzalez method, three had good results. Of the other three patients, one had a urethrovaginal fistula and underwent fistula repair 9 yr after, one had distal vaginal stenosis and underwent revision vaginoplasty 9 yr after, and one had a urethrovaginal fistula and urethral stricture. The patient with urogenital mobilization had persistent urogenital sinus. Feminizing genitoplasty using the Gonzalez method showed good long-term results in patients with ambiguous genitalia and Congenital adrenal hyperplasia. The procedure is less invasive than other approaches and results in excellent cosmetic outcomes; and complications can be corrected by relatively simple procedures.
Child, Preschool ; Disorders of Sex Development/*surgery ; Female ; Feminization/*surgery ; Follow-Up Studies ; Humans ; Infant ; Male ; Penis/abnormalities ; *Reconstructive Surgical Procedures ; Urethral Stricture/surgery ; Urogenital System/surgery ; Vagina/*surgery

Persistent Muellerian duct syndrome (PMDS) is a rare form of male pseudohermaphrodism without the feature of ambiguous genitalia. We present a case of PMDS with transverse testicular ectopia (TTE).
Abnormalities, Multiple ; Adult ; Disorders of Sex Development ; pathology ; surgery ; Hernia, Inguinal ; surgery ; Humans ; Male ; Mullerian Ducts ; abnormalities ; surgery ; Testicular Hydrocele ; surgery ; Testis ; abnormalities ; surgery

46, XY Disorders of Sex Development ; complications ; genetics ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Middle Aged ; Ovarian Neoplasms ; pathology ; surgery ; Sex Chromosomes ; Sex Cord-Gonadal Stromal Tumors ; pathology ; surgery

Adult ; Cell Transformation, Neoplastic ; metabolism ; pathology ; Humans ; Immunohistochemistry ; Keratin-10 ; metabolism ; Male ; Ovotesticular Disorders of Sex Development ; metabolism ; pathology ; surgery ; Teratoma ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

OBJECTIVETo investigate the characteristics, diagnosis, and treatment of ovotesticular disorder of sex development (OT-DSD).

METHODSWe retrospectively analyzed 2 cases of OT-DSD treated in our hospital. The patients were 19 and 15 years old, respectively, and both received systematic physical examination and examinations of the karyotype, sex hormone, adrenocorticotropic hormone (ACTH), color Doppler ultrasonography, urethrocystoscopy, and human chorionic gonadotropin (HCG) test. Under the laparoscope, we performed surgical gonad exploration, gonadectomy, and vulvar orthopedics. Intraoperative exploration and pathology confirmed true hermaphroditism in both cases, with sex selection as female. One underwent laparoscopic resection of the ovotestis, and the other removal of the testis with the ovarian tissue reserved.

RESULTSThe patients were followed up for 12 months postoperatively, which found no abnormality in either the vulvas or the genital glands.

CONCLUSIONSurgical exploration of the gonad is the only method for the diagnosis of OT-DSD and sex selection is the key to treatment. Laparoscopic surgical exploration of the gonad and vulvar orthopedics are the first treatment options.

Adolescent ; Chorionic Gonadotropin ; Female ; Gonadal Steroid Hormones ; Humans ; Karyotype ; Laparoscopy ; Male ; Ovary ; Ovotesticular Disorders of Sex Development ; diagnosis ; surgery ; Retrospective Studies ; Testis ; surgery ; Young Adult

Adolescent ; Diagnosis, Differential ; Disorders of Sex Development ; Female ; Humans ; Male ; Neoplasms, Germ Cell and Embryonal ; pathology ; radiotherapy ; surgery ; Seminoma ; pathology ; Testicular Neoplasms ; pathology ; radiotherapy ; surgery