Management of vaginal agenesis.

PURPOSE OF REVIEW: Mayer-Rokitansky-Küster-Hauser syndrome is undergoing new research outcomes involving genetics and management. RECENT FINDINGS: Recent literature supports a polygenic multifactorial genetic basis for the syndrome. Management is now predominantly by vaginal dilators and nonsurgical, but holistic. The future of uterine transplantation is discussed. SUMMARY: New developments open new possibilities for understanding the genetic basis of the disease, and research in this area will continue. Management in terms of fertility may have an added dimension if uterine transplantation and successful pregnancy outcome can be proven.

Mayer-Rokitansky-Küster-Hauser syndrome: a review of 245 consecutive cases managed by a multidisciplinary approach with vaginal dilators.

OBJECTIVE: To understand the efficacy of vaginal dilators in the management of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. DESIGN: Retrospective sequential study. SETTING: Hospital. PATIENT(S): 245 women. INTERVENTION(S): Vaginal dilators. MAIN OUTCOME MEASURE(S): Functional vaginal length and sexual satisfaction. RESULT(S): Of the patients who completed the program, 232 (94.9%) achieved a successful vaginal length (defined as greater than 6 cm in length and maximum width throughout the vagina and especially at the apex) and sexual function. When the program was completed by all patients, 100% of patients were successful. CONCLUSION(S): Vaginal dilator therapy is the treatment of first choice for creation of the vagina in MRKH syndrome, and the success rates suggest that surgery is rarely, if ever, required.

Psychological distress in women with uterovaginal agenesis (Mayer-Rokitansky-Kuster-Hauser Syndrome, MRKH).

BACKGROUND: Uterovaginal agenesis (Mayer-Rokitansky-Küster-Hauser Syndrome; MRKH) is a congenital nonformation of the vagina and the uterus, but with normal ovaries. OBJECTIVE: The authors investigated the psychological impact of this disorder, about which very little is known. METHOD: A group of 66 women with MRKH were compared with 31 control-group women on a range of self-rating scales assessing psychological distress and self-esteem. RESULTS: Women with MRKH had significantly more pathological scores on some of the scales and subscales, such as phobic anxiety and psychoticism (interpersonal alienation), with a similar trend for subscales measuring depression and anxiety. CONCLUSION: MRKH has a lasting negative impact on affected women's level of psychological distress and self-esteem.

A cognitive behavioural model and therapy for utero-vaginal agenesis (Mayer-Rokitansky-Küster-Hauser syndrome: MRKH).

BACKGROUND: Utero-vaginal agenesis, also called the Mayer-Rokitansky-Küster-Hauser Syndrome (MRKH), is a congenital abnormality of the female genital tract, characterized by the non-formation of the vagina and the uterus. It is a common cause of primary amenorrhoea. Little is known about the psychological impact and management of this condition. METHOD: We describe a specific model of the core negative psychological impact of diagnosis and medical treatment of MRKH and a cognitive-behavioural therapy of MRKH based on the model (CBT-MRKH). The Medical Research Council's (2002) framework for the development and evaluation of complex health interventions was used for intervention development and evaluation. RESULTS: Evidence from a recent cross-sectional study and a small randomized controlled trial (RCT) provides preliminary support for the model and treatment (Heller-Boersma, Schmidt and Edmonds, in press; Heller-Boersma, Schmidt and Edmonds, 2007), and this is further validated by extensive qualitative material gathered over the course of the RCT from participants. CONCLUSIONS: The model and treatment described may also be applicable to a number of other congenital or acquired gynaecological conditions such as premature ovarian failure, breast cancer, early onset endometrial cancer, female genital mutilation, Turner's Syndrome, ovarian dys/agenesis or, Complete Androgen Insensitivity Syndrome, all of which have a psychological impact not dissimilar to MRKH in terms of these women's sense of self and femininity.

Preconception transabdominal cervicoisthmic cerclage.

OBJECTIVE: The purpose of this study was to report pregnancy outcome and complication rates for women with recurrent late pregnancy loss who were treated with preconception transabdominal cervicoisthmic cerclage. STUDY DESIGN: This was a case note review of 19 women at high risk for second trimester loss and early preterm delivery who were treated with preconception transabdominal cervicoisthmic cerclage at Queen Charlotte's and Chelsea Hospital from 1994 to 2003. RESULTS: Preconception transabdominal cervicoisthmic cerclage was associated with a postoperative fetal survival rate of 100% for pregnancies that reached >12 weeks of gestation, compared with a preoperative fetal survival rate of 12%. There were no significant intraoperative, antenatal, intrapartum or neonatal complications. CONCLUSION: Within this case series, preconception transabdominal cervicoisthmic cerclage was a safe alternative to transabdominal cervicoisthmic cerclage that was performed in pregnancy with no risk to a fetus. It should be considered in appropriate cases in women seen for prepregnancy counseling.

Congenital malformations of the genital tract and their management.

While congenital malformations of the genital tract are not common, the sequelae of their presence can be serious. The practising gynaecologist must be aware of the range of congenital abnormalities that may occur and the symptoms that may result from them. Failure to manage these patients correctly may have long-term sequelae for their psychological, sexual and reproductive health. The involvement of a multi-disciplinary team in dealing with these patients is imperative, and preparation for surgery-particularly in congenital malformations of the vulva and the vagina-is imperative if the long-term sexual function in these patients is to be fulfilled. Surgical correction of vulval abnormalities in adolescence is related solely to sexual function as most of the reconstructive surgery is done in childhood. For the management of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome, the recommendation is now that passive dilatation by Franks' technique is the treatment of first choice and only if that fails should surgical approaches be embarked upon. The results of the surgery are similar in all techniques and the particular surgical centre will have its own preference of which technique it adopts. Congenital absence of the cervix is a complex surgical problem and should be dealt with solely in centres with expertise.

Pediatric and adolescent gynecology--the UK experience.

This review looks at the history of the establishment of pediatric and adolescent gynecology in the United Kingdom and, having addressed the chronology, sets the service in the context of the current health care system. The National Health Service does not provide an environment that encourages the delivery of this care in a structured way. There are some specialist services that are provided by central government, one of which addresses one part of pediatric and adolescent gynecology, but currently the services are relatively haphazard and delivered by individuals who have acquired knowledge and skills over time rather than in a structured training way. The need for improved services for adolescents and the training of doctors to provide this service is emphasized.