Please cite this paper as: Michala L, Aslam N, Conway G, Creighton S. The clandestine uterus: or how the uterus escapes detection prior to puberty. BJOG 2010;117:212–215. Uterine agenesis is one of the differential diagnoses in adolescent girls with delayed menstruation. It may also be suspected earlier in childhood during investigations for other genitourinary conditions. However, accurate confirmation that the uterus is absent can be extremely difficult before puberty because of its small size. We describe ten girls referred to a specialist centre with a presumed diagnosis of an absent uterus which was later found to be incorrect. We conclude that imaging should be undertaken by clinicians with experience in management of this age group and in some girls it may be necessary to delay final diagnosis until after puberty.
A 22-year-old woman with mosaic Turner syndrome (TS) (45,X 86%, 47,XXX 11%, 46,XX 3%) had been attending our well-established and dedicated tertiary-level TS clinic annually since the age of 5 years. She was initially referred with short stature, and was subsequently diagnosed with mosaic TS, and received growth hormone treatment until the age of 15 years. Her final height was 150 cm with a body mass index of 21. Puberty developed spontaneously and she reached menarche at 13 years; she had a regular menstrual cycle every 28 days. She was normotensive, and had normal thyroid and kidney function tests, bone densitometry scan and echocardiography. She was not yet in a stable relationship, but hoped to have a family in the future, and wanted to discuss the option of fertility preservation owing to the risk of premature menopause. The possibility of controlled ovarian hyperstimulation (COH), followed by transvaginal oocyte retrieval and cryopreservation, was raised. After she had been fully counselled about the risks of pregnancy in TS, the genetic risks and the risks related to COH, such as poor response and cycle cancellation, she was keen to proceed. She therefore had an ovarian reserve assessment as part of the investigations, and was found to have a follicle-stimulating hormone level of 4.6 iu/ml, anti-mullerian hormone (AMH) level of 8.52 pmol/l and estradiol level of <44 pmol/l. Pelvic ultrasound revealed a normal uterus and the ovaries had an antral follicular count of seven. Controlled ovarian hyperstimulation was started using a short stimulation protocol from day 3 of menstruation, initially with 375 iu of human menopausal gonadotrophins (Menopur, Ferring, Berkshire, UK), which was then increased to 450 iu on day 5 of stimulation when the estradiol level was 651 pmol/l. After 11 days of COH and serial ultrasound monitoring, 10 000 units of human chorionic gonadotrophin were administered with an estradiol level of 5956 pmol/l. Eight oocytes were collected under transvaginal ultrasound-guidance 36 h later, and all the oocytes obtained were subsequently vitrified for future use, as described previously. 1 DiscussionFertility in women with TS is a rare phenomenon, as most suffer from gonadal dysgenesis, with only 2% of pregnancies a result of spontaneous conception, 2 and the remaining pregnancies resulting from oocyte donation which, to date, has been the only reproductive option. The accelerated loss of ovarian primordial follicles from the 18th week of fetal life characterises classical TS. However, in women with mosaic TS, follicular development can persist beyond puberty, leading to spontaneous pubertal development, regular menses and pregnancy before the onset of premature menopause.3 Indeed, even spontaneous monozygotic monochorionic twin pregnancy has been reported in such patients. 4 The possibility of fertility preservation in young women with mosaic TS was first raised by Abir et al. 5 Novel methods of fertility preservation in these women have been reported recently, including ovarian tissue cryopr...
Measurements of clitoral length and clitoral to urethral distance were made and analysed for a relationship in a group of 19 women with complete androgen insensitivity syndrome (CAIS) attending a specialist clinic for adult women with disorders of sexual development. These were compared with a control group of 50 women attending hospital for a gynaecological procedure. There was a positive correlation between clitoral length and clitoral to urethral distance for women with CAIS. In contrast, a negative correlation was seen between clitoral length and clitoral to urethral distance for women in the control group. Women with CAIS had a reduced mean clitoral length compared with controls (P = 0.001), but no difference was observed for the clitoral to urethral distance between the two groups (P = 0.116).Keywords Androgen, androgen insensitivity syndrome, clitoris, female genitalia, urethra.Please cite this paper as: Crouch N, Michala L, Creighton S, Conway G. Androgen-dependent measurements of female genitalia in women with complete androgen insensitivity syndrome. BJOG 2011;118:84-87.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.