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.

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.

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.

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.

Persistent unexplained congenital clitoromegaly in females born extremely prematurely.

OBJECTIVE: Unexplained clitoromegaly is a rare but well recognised feature in girls born premature. Although detected at birth, girls may re-present during childhood to paediatric urologists and gynaecologists who should be aware of this condition. The aim of the study was to describe the clinical findings and management of a series of girls presenting with persistent congenital clitoromegaly associated with prematurity. MATERIALS AND METHODS: This was a retrospective notes review set in a tertiary referral centre for Paediatric and Adolescent Gynaecology (PAG). RESULTS: Eight girls with a mean age of 6 years were seen over an eight year period. In all cases a Disorder of Sex Development (DSD) had been previously excluded. The main symptoms were discomfort or concern about appearance. On examination five girls had excess skin over the clitoris and three had enlarged corporal tissue. Management included reassurance and simple measures to ease discomfort. In two cases the parents requested referral to a paediatric urologist to consider clitoral surgery. CONCLUSION: As survival rates for extreme prematurity improve, paediatric urologists and gynaecologists are likely to see more of these cases. Clinicians must be familiar with this condition to ensure children are managed appropriately.

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.

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.

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.

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.

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.

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).

Management of vaginal hypoplasia in disorders of sexual development: surgical and non-surgical options.

Patients with disorders of sexual development (DSD) requiring vaginal reconstruction are complex and varied in their presentation. Enlargement procedures for vaginal hypoplasia include self-dilation therapy or surgical vaginoplasty. There are many vaginoplasty techniques described, and each method has different risks and benefits. Reviewing the literature on management options for vaginal hypoplasia, the results show a number of techniques available for the creation of a neovagina. Studies are difficult to compare due to their heterogeneity, and the indications for surgery are not always clear. Psychological support improves outcomes. There is a paucity of evidence to inform management regarding the optimum surgical technique to use, and long-term data on success is lacking, particularly with respect to sexual function. In conclusion, vaginal dilators remain the cornerstone of treatment of women with vaginal hypoplasia and should be used as the first-line technique. Surgical vaginoplasty has a role in complex patients with previous failed dilation and surgical intervention, particularly those cases where there is significant scarring from previous surgery. Regardless of the vaginal reconstruction technique, patients should be managed in a multidisciplinary team where there is adequate emotional and psychological support available.

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.

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.

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.

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.

Comparison of bone mineral density and body proportions between women with complete androgen insensitivity syndrome and women with gonadal dysgenesis.

OBJECTIVES: To compare bone mineral density (BMD) and body proportions between women with complete androgen insensitivity syndrome (CAIS) and with gonadal dysgenesis (GD). SETTING: Adult Disorders of Sexual Development and Ovarian Failure Clinics at University College London Hospitals. DESIGN: Retrospective cross-sectional study of three groups of women aged 17-58 years with varying degrees of exposure to sex hormones and different combinations of sex chromosomes. Forty-six subjects had CAIS, 18 had GD and 46,XY (GD(XY)), and 25 had GD and 46,XX (GD(XX)). In addition, body proportions of subgroups of these women were analysed. OUTCOME MEASURES: Oestrogen therapy, karyotype, anthropometry and BMD. RESULTS: Height differed between groups (F ratio 5.2, P=0.007)), with GD(XX) women being the shortest (mean+/-s.d.: 1.66+/-0.10 m), GD(XY) women the tallest (1.74+/-0.09 m) and CAIS women were in-between (1.70+/-0.07 m). Delayed gonadectomy resulted in taller stature in CAIS women (P=0.011). The ratio of lower to upper body length in GD(XY) women was significantly (P=0.001) greater than that of CAIS women. Multivariate logistic regression analysis (adjusted for age and height) showed that among women with XY karyotype, GD(XY) women were 5.2 times (95% confidence interval (CI): 1.3-20.1, P=0.018) more likely than CAIS women to have a low hip BMD. This difference was not evident among women with GD of different karyotypes (P=0.938). Spinal BMD did not differ between subject groups. Further adjustment for oestrogen replacement did not alter these relationships. CONCLUSIONS: Taller stature in late gonadectomised CAIS women suggests an oestrogen deficiency in these women prior to gonadectomy. Increased lower to upper body ratio in GD(XY) women compared with the other groups implies that these subjects have the greatest degree of oestrogen deficiency in puberty. Androgen rather than sex chromosomes may play an important role in cortical bone mineralisation in CAIS women, probably via estrogen receptor-alpha either directly or via aromatisation during critical periods of growth prior to gonadectomy.