Transverse vaginal septae: management and long-term outcomes.

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 Müllerian 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.

Developing a comprehensive, effective patient-friendly website to enhance decision making in predictive testing for Huntington disease.

PURPOSE: Predictive testing for Huntington disease is a complex decision, requiring in-depth counseling, education, and evaluation. Despite the growth in Web-based decision aids and educational resources, such tools for those considering Huntington disease testing are not available. The main objective of this project was to develop a patient-friendly, comprehensive, accessible Web-based tool to provide accurate information about testing for Huntington disease. METHODS: A semistructured interview study was conducted to determine the informational, educational, and support needs of those considering Huntington disease testing. A dedicated predictive testing website was subsequently developed and pilot tested. RESULTS: The interview study revealed that an effective website should include interactive diagrams, video documentaries, and personal stories of others who had considered testing. The pilot test revealed that the multidimensional site was easy to navigate and understand and provided an accurate, unbiased overview of the important factors to be considered before undergoing predictive testing. CONCLUSION: This project demonstrates the use of a mixed-method approach to develop the first tailored website dedicated to predictive testing for Huntington disease. Such an approach enabled the development of a comprehensive, accurate, and effective educational tool that supports informed decision making for people considering predictive testing for Huntington disease in an accessible, nonthreatening manner.

A comparison of genital sensory and motor innervation in women with pelvic organ prolapse and normal controls including a pilot study on the effect of vaginal prolapse surgery on genital sensation: a prospective study.

OBJECTIVE: To study genital sensory and motor innervation in women with pelvic organ prolapse and to determine the effect of vaginal prolapse surgery on genital sensation. DESIGN: A prospective observational study. SETTING: A tertiary referral unit in northwest England. POPULATION: Twenty women complaining of prolapse symptoms (including seven undergoing vaginal prolapse repair) and a control group of ten healthy women. METHODS: Women attended a research clinic where genital sensory thresholds were determined by quantitative sensory testing and motor innervation was assessed by concentric needle electromyography (EMG) of the pelvic floor muscles. Women undergoing surgery were assessed preoperatively and postoperatively at 6 months. MAIN OUTCOME MEASURES: Primary outcome measure was change in genital vibration threshold and the percentage of polyphasic potentials on EMG. RESULTS: Healthy control women had normal vibration detection thresholds at the vagina and clitoris. Thresholds in the majority of women with prolapse were abnormal and in all women with prolapse over the age of 50 years. Women with prolapse had a significantly larger percentage of polyphasic potentials of the left pubococcygeus but not the right. There was no significant change in genital sensory thresholds at 6 months postoperatively following vaginal repair. CONCLUSIONS: The majority of women with prolapse have abnormal genital vibration detection, which is particularly evident after the age of 50 years. Impaired sensory function does not correlate with EMG markers of partial motor denervation. In women with abnormal sensory thresholds, no additional effect was detected following vaginal prolapse repair.

Adult recall of childhood female genital cutting and perceptions of its effects: a pilot study for service improvement and research feasibility.

This study aimed to gather information from service users of an African Women's Clinic for the purposes of planning service improvement and estimating research feasibility. The report is based on 17 interviews with Somali speaking women who had experienced female genital cutting in childhood. With language barriers removed, a high percentage of clinic attendees responded positively to the invitation to participate in research. They willingly discussed their experiences of FGM and expressed their negative viewpoints about the practice of FGM, suggesting that psychosocial and psychosexual research may be feasibly carried out in specialist contexts. The results also point to the need for psychological and educational input for service improvement.

The normal vulva in medical textbooks.

When a healthy woman expresses concerns about her vulva, the doctor's response should be informed by clinical knowledge. For many doctors, accumulation of such knowledge would have begun with undergraduate teaching and medical textbooks. The aim of this study is to examine the information on female genital morphology in medical textbooks. A total of 59 gynaecology and anatomy textbooks were searched for information on the dimensions of vulval constitutent parts. No textbook gave measurements for all vulval structures. Vaginal length was reported in 21/59 textbooks, clitoral size in 15/59 and labia minora in 1/59. Where measurements appear, they suggest narrower ranges than recent reports. Information of vulval morphology is scanty and inaccurate in medical textbooks. The general lack of professional resources means that doctors may consciously or non-consciously rely upon personal experiences and popular culture to form their opinions, as do their patients.

Quality of obstetric and midwifery care for pregnant women who have undergone female genital mutilation.

Despite the availability of professional guidelines for the pregnancy management of women affected by female genital mutilation (FGM), this study demonstrated major deficits in identification, management and safeguarding.

False-positive pregnancy tests following enterocystoplasty.

People with major congenital urological or neurological malformations invariably require bladder reconstruction with enterocystoplasty in early childhood. The improvement of the surgical management of these children has reflected significantly on their life expectancy. As a result, more young people with enterocystoplasty are being transitioned to adolescent clinics where they receive the usual counselling about sexual health and pregnancy risks. However, the possibility of false-positive urinary pregnancy tests in these young women remains an overlooked but essential message. The lack of awareness about this fact can result in significant patient anxiety and the potential for unnecessary interventions as exemplified by the three cases we have encountered.

Androgen-dependent measurements of female genitalia in women with complete androgen insensitivity syndrome.

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).

Clinical characteristics of well women seeking labial reduction surgery: a prospective study.

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.

Labial surgery for well women: a review of the literature.

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.

The clandestine uterus: or how the uterus escapes detection prior to puberty.

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.

Service users' experiences of obtaining and giving information about disorders of sex development.

OBJECTIVE: To quantify participants' experiences of obtaining and giving information about disorders of sex development (DSD). DESIGN: Cross-sectional survey study that asked people about their current and past experiences relating to DSD disclosure. SETTING: A large tertiary referral centre for DSD management in the UK. POPULATION: One hundred of 126 people with a confirmed diagnosis of DSD who were invited to participate in the study formed the usable sample. METHODS: All people who attended clinic for follow-up during the study period and members of a patient support group whose annual meeting fell within the study period were asked to complete the Middlesex Communication Survey. MAIN OUTCOME MEASURES: The Middlesex Communication Survey. RESULTS: Younger participants were more likely to report having been appropriately informed about their diagnosis than older people. Nearly half of the former had been fully informed about their diagnosis by age 15 years, compared with 0% of the older age group. In terms of information sharing, mothers were most likely to be the person with whom the participant had shared (almost/all) DSD information (74%), followed by current partners (71%). Information relating to genital surgery, presence of testes and clitoral anomalies were the least likely aspects to have been unambiguously shared with even the most informed person. CONCLUSIONS: Our results suggest that difficulties in obtaining DSD information from care providers were common, and that communication has improved for younger participants. The study also confirmed that many people with DSD continue to struggle with confiding, even in those closest to them, about aspects of their diagnosis. Care protocol needs to centralise psychological adaptation, which should also be a primary focus for future research.

Minimal access surgery in adolescent gynaecology.

The benefits of a minimally invasive approach are now well documented in adult women, and thus surgeons have embraced the notion of expanding such expertise in adolescence with measured enthusiasm and a great sense of responsibility. Faster recovery is likely to have a positive impact on schooling, while less adhesion formation may reduce future fertility issues. Gynaecologists performing minimally invasive procedures in adolescents ought to be aware of the steep learning curve required for achieving proficiency with complex laparoscopic surgery. In the group of rare congenital anomalies and advanced endometriosis, the best surgical results can only be achieved after careful preoperative planning by a multidisciplinary team.

Augmentation vaginoplasty with buccal mucosa for the surgical revision of postreconstructive vaginal stenosis: a case series.

BACKGROUND: Vaginal stenosis is a common consequence of vaginal reconstruction in childhood. Significant scarring can make dilation ineffective, and there maybe a paucity of skin to create perineal skin flaps. Numerous vaginoplasty techniques exist, including perineal skin flaps for distal stenosis and intestinal vaginoplasty requiring laparotomy and bowel anastomosis. Buccal mucosa graft is widely used in urethroplasty and has been reported in neovaginal construction. It is easily accessible with minimal graft site morbidity and provides a close functional replica to vaginal mucosa. AIM: The aim was to describe the use of buccal mucosa grafts for the surgical revision of postreconstructive vaginal stenosis. METHOD: The study setting was a tertiary centre for congenital gynaecological anomalies. Buccal mucosa grafting was provided to women with vaginal stenosis unsuitable for perineal skin flaps and in whom intestinal vaginoplasty was the next option. Four cases have been performed, and the medical notes were reviewed for clinical data. RESULTS: Mean age at buccal mucosa vaginoplasty was 21 years (range 18-26 years). Two patients had a cloacal anomaly, one had mosaic Turner syndrome and one had postradiation vaginal stenosis. All four had undergone previous vaginal reconstruction. In all cases, there was a tight band of vaginal stenosis either too proximal or with insufficient perineal tissue to allow a perineal flap vaginoplasty. There were no immediate complications. Mean clinic follow-up was 16 months (3 months-4 years). Two patients were able to have penetrative sexual intercourse, and two were using vaginal dilators successfully. Patients have an email contact for the team nurse specialist and therefore are able to contact before clinical review if they develop concerns. DISCUSSION: The use of buccal mucosal grafts for vaginoplasty is increasingly reported. This is the first detailed case series describing its use for augmentation vaginoplasty with postreconstructive/stenosis. CONCLUSIONS: All patients had a successful outcome with a normal capacity vagina, and two were able to have penetrative intercourse. This suggests that buccal mucosal graft vaginoplasty is a safe and effective alternative for women with previous vaginal reconstruction requiring surgery for vaginal stenosis.

Swyer syndrome: presentation and outcomes.

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.

Obstructive mullerian anomalies and modern laparoscopic management.

STUDY OBJECTIVE: Congenital uterine anomalies are common, although the majority are asymptomatic. When an obstructed system exists, women may present with abdominal pain, or dysmenorrhea. Removal of the obstructed horn may be required in the symptomatic patient. In the past, surgical treatment necessitated a laparotomy. DESIGN: After preoperative diagnosis and planning using magnetic resonance imaging, laparoscopic removal of the obstructed uterine horn and tube was performed. Morcellation of tissue was used to permit removal through a 15mm port. SETTING: A central London tertiary referral teaching hospital. PARTICIPANTS: 15 women aged between 13 and 41. INTERVENTIONS: Between 1999 and 2005, all women underwent laparoscopic removal of the obstructed uterine horn and tube. MAIN OUTCOME MEASURES: Recovery, hospital stay, length of operation. RESULTS: All women recovered well, with an operation time of 80 to 300 minutes and an average hospital stay of 5 days. CONCLUSIONS: A laparoscopic approach is a safe and appropriate technique for the removal of an obstructed uterine horn.

Laparoscopic treatment of endometriosis in teenagers.

OBJECTIVES: To determine the frequency and severity of endometriosis in adolescent and teenager girls with chronic pelvic pain (CPP) who fail to respond to medical treatment and to evaluate the outcome of radical laparoscopic surgery for severe endometriosis. DESIGN: Retrospective review of case records of all girls under the age of 21 years who underwent diagnostic and/or operative laparoscopy for CPP unresponsive to medical treatment between January 2001 and December 2003. The operative findings and the response to surgery were retrospectively reviewed. RESULTS: Thirty-one girls were referred. No pelvic abnormalities were detected in 11 patients (35.5%). Endometriosis was detected in 11 (35.5%). Six had severe endometriosis. Other diagnoses included: non-functional non-endometriotic ovarian cyst (4 patients), functional ovarian cyst (1 patient), hydrosalpinx (bilateral, 1 patient; unilateral, 1 patient) and obstructed uterine horn (2 patients). Of those with severe disease all six were treated laparoscopically without complications. Five were rendered pain free and one had an improvement in symptoms. CONCLUSIONS: Endometriosis can occur in adolescent and teenager girls. Laparoscopy should be carried out in all adolescents and teenagers with CPP unresponsive to medical treatment. This the first study reporting the outcome of radical excision treatment for severe endometriosis in this age group and early results are encouraging.

Functional evidence for nitrergic neurotransmission in a human clitoral corpus cavernosum: a case study.

Although the presence of neuronal nitric oxide synthase (nNOS) has been demonstrated in human clitoral corpus cavernosum, functional evidence for the nitrergic neurotransmission as a nonadrenergic noncholinergic (NANC) transmitter has been limited to animal studies. Here we show that electrical field stimulation elicited reproducible NANC relaxation responses in a clitoral corpus cavernosum, obtained from a 38-y-old woman undergoing clitoral reduction surgery. These relaxation responses were abolished by an inhibitor of NO synthase or tetrodotoxin proving that they were nitrergic in nature and neuronal in origin. Large and small calibre nitrergic nerves were demonstrated with immunohistochemistry using nNOS antibody, which were also immunostained with cholinergic nerve markers. nNOS expression was confirmed using Western blotting. This is the first report to show that NO produced by nNOS within the cholinergic-nitrergic nerves is responsible for the NANC relaxation responses in a human clitoral corpus cavernosum.

Experiences of surgical continence management approaches for cloacal anomalies: a qualitative analysis based on 6 women.

STUDY OBJECTIVE: The aim of this qualitative study was to gain insight into health care experiences of young women diagnosed with cloacal anomalies, with a special focus on continence management. DESIGN: Qualitative analysis of one-to-one interviews. SETTING: A tertiary center for congenital anomalies of the urogenital tract in London. PARTICIPANTS: Six women aged 16 to 24 with cloacal anomalies. INTERVENTIONS: Tape-recorded one-to-one semi-stuctured interviews with a skilled interviewer. MAIN OUTCOME MEASURE(S): The taped interviews were transcribed and analyzed verbatim using interpretative phenomenological analysis according to the research question. Organizing themes across all of the accounts were identified. RESULTS: Two organizing themes concerning our research interests are summarized. The first theme Personal Agency in the Hands of Experts focuses on the interviewees' appreciation of their life-saving surgical care and their involvement in treatment decisions. The second theme Compromises and Trade-Offs focuses on what it was like to live with the more traditional versus the more advanced continence methods. Reliability emerged as a key priority in terms of continence treatment outcome. Gratitude may have interfered with the women's honest communications during treatment decision and evaluation consultations. CONCLUSIONS: A more developed approach to communication about the complex interventions proposed, founded on a nuanced understanding of users perspectives, can enhance informed decision making about continence management approaches. Despite these specific gaps, the interviewees were appreciative of their care and optimistic about life.