Sexuality in Males With Congenital Adrenal Hyperplasia Resulting From 21-Hydroxylase Deficiency.

PURPOSE: Although sexuality has been reported to be impaired in females with congenital adrenal hyperplasia (CAH) resulting from 21-hydroxylase deficiency, sexuality in males with CAH so far has remained largely unconsidered. PATIENTS: One of the largest European male cohorts of patients with CAH in which sexuality in male patients with CAH was assessed. METHODS: Sexuality was evaluated in 91 sexually active male patients with CAH using questionnaires investigating sexual orientation, age at sexual initiation, sexual activity, satisfaction with sex life, and sexual problems, such as fears or dislike of sexual activity, lack or excessive sexual desire, difficulties getting aroused or reaching an orgasm, premature ejaculation, and no or incomplete erection. RESULTS: Sexuality in male patients with CAH was similar to European reference populations. If sexuality problems were present, they were less frequently reported by the most severely affected CAH males. Adducing a holistic perspective, sexual problems showed substantial association to psychological problems, such as anxiety and depression. CONCLUSIONS: Sexuality in male patients with CAH in general was unaffected and sexuality problems seemed to be associated in particular with psychological problems. Because sexual health is a key factor of general health, we recommend that sexuality as well as psychological issues explicitly should be addressed in health care of patients with a CAH diagnosis, independent of sex.

Reconstructive surgical management of cryptomenorrhea because of complex uterovaginal malformations with duplicate uterus and complete vaginal agenesis.

OBJECTIVE: To describe the surgical reconstructive options in cases of cryptomenorrhea because of an obstructed functioning uterus with upper and lower vaginal agenesis. DESIGN: Assessment of operative, anatomic, and functional results after operative reconstruction over a 1-year follow-up period. SETTING: University hospital and referral center for pediatric and adolescent gynecology. PATIENT(S): Three adolescent girls, aged 15 to 18 years with primary amenorrhea, cyclic abdominal pain, hematometra, and complete vaginal agenesis. INTERVENTION(S): A combinative vaginal and laparoscopic approach to reconstruct a neovagina by human amniotic membranes and establish the uterovaginal continuity. MAIN OUTCOME MEASURE(S): Anatomic success was defined by a vaginal length ≥8 cm, and a width allowing the introduction of two fingers. Functional success was evaluated according to the restoration of menstrual cycle resolving of pain and patients'sexual satisfaction. RESULT(S): Neovaginal length was 9 to 12 cm and adequately wide. In all patients the uterovaginal continuity could successfully be restored. Satisfactory anatomic and functional results could be achieved, with no operative morbidity. Regular menstruation resumed with normal ultrasound findings during follow-up. CONCLUSION(S): In rare cases of an obstructed functioning uterus by complete vaginal agenesis, a combinative laparoscopic and vaginal surgical approach effectively restores anatomy and function, by an overall minimal operative morbidity. Human amnion provides excellent results in neovaginal reconstruction.

Functional and anatomic results of amnion vaginoplasty in young women with Mayer-Rokitansky-Küster-Hauser syndrome.

OBJECTIVE: To evaluate the surgical outcome and the long-term anatomic and functional results in young women with Mayer-Rokitansky-Küster-Hauser Syndrome (MRKH) undergoing neovaginal creation with amniotic membranes. DESIGN: Evaluation of surgical and functional outcome according to clinical records and validated questionnaires about sexuality (Female Sexual Function Index [FSFI]) over a 1.5-year follow-up period. SETTING: University hospital and referral center for pediatric and adolescent gynecology. PATIENT(S): Seven patients with congenital vaginal aplasia with a mean age of 20.86 +/- 3.56 years (range 17-26 years). INTERVENTION(S): McIndoe procedure modified by the application of human freeze-dried amniotic membranes. MAIN OUTCOME MEASURE(S): Anatomic success was defined by a vaginal length >or=8 cm, and a width allowing the easy introduction of two fingers. FSFI scores were applied to define functional results. RESULT(S): Mean neovaginal length was 9.3 cm (range 4-12 cm). The mean FSFI score was 30.0 +/- 6.9. Major operative complications occurred in one patient. In six out of seven patients satisfactory anatomic and functional results were achieved. CONCLUSION(S): The surgical dissection of the vesicorectal space and the application of human amnion over a vaginal mold to create a neovagina results in satisfying anatomic and functional outcome with low perioperative morbidity in MRKH patients.