Clinical effect of clitoroplasty with preservation of glans clitoris and dorsal neurovascular pedicle / 中华医学美学美容杂志

Objective To evaluate the clinical effect of clitoroplasty with preservation of the glans clitoris and the dorsal neurovascular pedicle in patients with clitoral hypertrophy. Methods From July 2003 to June 2009, cunnus feminization operations with preservation of the glans clitoris and the dorsal neurovascular pedicle and labioplasty were performed on 8 patients with female pseudohermaphroditism. Results All 8 cases were primary healing. The shaping glans of clitoris was completely viable with satisfactory size, location, contour and touching sensitivity, and the cunnuses presented the appearances of female external genital organs. Conclusions Clitoroplasty with preservation of the glans clitoris and the dorsal neurovascular can improve the appearance of clitoris and labia, preserving the functions of sensation and erection, reconstruting a new clitoris which is aesthetically appealing and functional, and it is suitable for various kinds of cunnus feminization operations.

Meta-analysis of 342 Female Pseudohermaphroditism / 中国医科大学学报

Objective To summarize the clinical characteristics of female pseudohermaphroditism (FPH) in the publication from 1994 to 2009 and the cases diagnosed and treated in our hospital from 2000.Methods We employed Chinese and English name of female pseudohermaphroditism as a key word separately to retrieve published articles of FPH during 1994 to 2009.A meta-analysis was performed together with the ten cases in our hospital.Results Among 342 cases involved in this study,only twenty-seven children were diagnosed immediately after birth and the other 315 cases at the age of 1 month to 59 years with median age of 29.54 years.The etiological factors were mostly congenital adrenal hyperplasia (70.47%).Clinical manifestations were advanced bone age (95.52%),clitoromegaly (84.14%) and early virilization (77.24%).The treatment mainly included drug and operation.Early diagnosis and available therapy were very important for the prognosis.Conclusion FPH is not rare,so we should improve our knowledge for its early diagnosis,good intervention and final better prognosis.

Diagnosis and treatment of female pseudohermaphroditism caused by congenital adrenal hyperplasia (report of 4 cases) / 中华泌尿外科杂志

Objective To report the effect of hormone replacement plus orthopedic surgery for 4 patients with female pseudohermaphroditism caused by congenital adrenal hyperplasia. Methods Four patients with female pseudohermaphroditism caused by congenital adrenal hyperplasia were included.On admission their ages were 11,15,14 and 20 years,respectively.The diagnoses were confirmed by medical histories,ultrasonography,serum levels of hormones and chromosome analysis.They all underwent clitoroplasty and vaginoplasty with perineal skin flap. Results After operation the patients received estrogen and progestogen for a short time to adjust the menstrual cycle and hydrocortisone replacement(10 to 20 mg,twice a day) for their lifetime.Normal menstruation and well-developed breasts were observed in all the patients.No constriction occurred in the reconstructed vaginas. Conclusions This disease can be easily misdiagnosed as hypospadias with bilateral cryptorchidism.Therefore,in addition to careful physical examination,the measurement of serum hormones,ultrasonography of the abdomen,and chromosome analysis are necessary.Proper utilization of the perineal skin flap for reconstruction of the clitoris and vagina as well as careful perioperative managements in combination with hormone replacement therapy can greatly contribute to satisfactory results.

A Case of Female Pseudohermaphroditism with m llerian agenesis, urinary tract malformations, and imperforate anus / 대한산부인과학회잡지

Special idiopathic female pseudohermaphroditism with urinary tract, m llerian duct, and lower gastrointestinal tract anomalies, in addition to masculinization of the external genitalia, is rare phenomenon. Masculinization of external genitalia and other anomalies occur in the absence of a recognized exposure to androgen or other teratogenic factors. We report a case of a female pseudohermaphroditism with m llerian agenesis, bilateral multicystic dysplastic kidney, urethral agenesis, left double ureter, rectovesical fistula, imperforate anus, single umbilical artery, detected after therapeutic abortion at 23+5 weeks of gestational age. The final diagnosis is based on autopsy.

Three cases of nonadrenal (nonprogressive) female pseudohermaphroditism / 대한비뇨기과학회지

Female pseudohermaphrodities are 46XX genetic females with normal Mullerian derivatives, but have various degrees of ambiguous external genitalia. While most of them are commonly associated with adrenogenital syndrome, some of them have been occasionally associated with maternal ingestion of testosterone or synthetic progestational agent, maternal virilizing tumor or rarely idiopathic. Herein, we present three cases of female pseudohermaphroditism which is unrelated with adrenogenital syndrome. One case is resulted from maternal ingestion of progestational agent during the first trimester of pregnancy, and the other idiopathic.