Objective To establish the spectrum of presentation, natural history and gynaecological outcomes in women with Swyer syndrome.Design Retrospective notes review.Setting Tertiary referral centre for disorders of sex development.Population A total of 29 adult women with Swyer syndrome.Methods Information was collected on age at diagnosis, biometric characteristics, timing of gonadectomy, histology of gonad, bone mineral density, uterine size and fertility.Main outcome measures Age at diagnosis, risk of gonadal malignancy, bone mineral density, uterine size.Results With regard to presentation, 26/29 (90%) women in this series presented with delayed puberty, and the median age at diagnosis was 17.2 years (range 0-55 years). The median age at gonadectomy was 18 years (range 9-33 years). Histology of the gonad was available in 22 women and demonstrated streak gonads with no evidence of malignancy in 12, dysgerminoma in 7 and gonadoblastoma in 3. The youngest patient diagnosed with dysgerminoma was 10 years old. The median height of the women was 1.73 m (range 1.54-1.95 m). Twelve out of the 20 (60%) women had evidence of osteopenia on dual energy X-ray absorptiometry scan. The uterine size and shape was assessed in eight women after completion of induction of puberty, and the uterine cross-section was found to be significantly lower than that in normal controls. Fertility was achieved with ovum donation in three women, all of whom had live births and one subsequently had a second successful pregnancy.Conclusion Early diagnosis of Swyer syndrome is necessary in view of the risk of dysgerminoma that can develop at an early age. Adequate hormone replacement is required to maintain bone mineral density and may improve the uterine size and shape.
Objective To assess clinical characteristics and expectations in well women requesting elective labial reduction surgery.Design Prospective study of women attending an outpatient gynaecology clinic.Setting General gynaecology clinic at a Central London teaching hospital.Sample Women requesting labial reduction surgery and referred by their general practitioner.Methods The labia minora width and length were measured for all participants for comparison with published normal values. The presenting complaint was recorded, along with demographic details, expectations of surgery and sources of information regarding appearance of the labia.Main outcome measures Labial measurements, reported symptoms and expectations of surgery.Results The labia of all participants were within normal published limits, with a mean (SD) of 26.9 (12.8) mm (right labia), and 24.8 (13.1) mm (left labia). The majority of complaints were regarding appearance or discomfort. Expectations were to alter the appearance with surgery.Conclusions All women seeking surgery had normal-sized labia minora. Clear guidance is needed for clinicians on how best to care for the worried well woman seeking surgery.
Objective To examine the management and long-term outcomes of transverse vaginal septae.Design Observational study with cross-sectional and retrospective arms.Setting Tertiary referral centre specialising in M€ ullerian anomalies.Population Forty-six girls and women with a transverse vaginal septum.Methods Data from medical records of all cases (1998-2013) of transverse vaginal septae were collected and reviewed. Patients over 16 years of age also completed a questionnaire.Main outcome measures Presentation, examination findings, investigations, surgery, and long-term reproductive outcomes.Results The septae in the study were described as follows: 61% (95% CI 0.46-0.74) were imperforate, and presented with obstructed menstruation; 39% (95% CI 0.26-0.54) were perforate, and presented with a variety of concerns; 72% (95% CI 0.57-0.83) were low, 22% (95% CI 0.12-0.36) were mid-vaginal, and 6% (95% CI 0.02-0.18) were high; 33% were managed via an abdominoperineal approach, 59% were managed via a vaginal approach, and 6% had laparoscopic resection (one patient did not have surgery); 11% (95% CI 0.05-0.23) of patients presented with reobstruction, all following abdominoperineal vaginoplasty; 7% presented with vaginal stenosis, two following vaginal resection and one following the abdominoperineal approach; 61% of questionnaires were returned. These results showed that 22/23 patients were menstruating and one had a hysterectomy, 74% had been sexually active, 35% had dyspareunia, and 36% complained of dysmenorrhoea. There were seven pregnancies, with one termination and six live births, all following the vaginal excision of a transverse vaginal septum.Conclusions Transverse vaginal septae resected vaginally or laparoscopically have low complication rates and good long-term outcomes. Complex septae require more extensive surgery, with an increased risk of complications.
This review investigates the quality and content of published reports relating to labial surgery for well women. Electronic databases were searched for relevant articles between 1950 and April 2009. Forty articles were identified, 18 of which included patient data. The specification of the study design was unavailable in 15 of the 18 papers; the remaining three were retrospective reports. No prospective, randomised or controlled studies were found. All reports claimed high levels of patient satisfaction and contained anecdotes pertaining to success. Medically nonessential surgery to the labia minora is being promoted as an effective treatment for women's complaints, but no data on clinical effectiveness exist.
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