Syndrome Mayer-Rokitansky-Küster-Hauser - uterine and vaginal agenesis: current knowledge and therapeutic options.

OBJECTIVE: Evaluation of existing knowledge of etiopathogenesis, clinical manifestations and treatment options to increase quality of life in women with Mayer-Rokitansky-Küster-Hauser syndrome (MRKH). DESIGN: Review article. SETTING: Department of Obstetrics and Gynaecology, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague. METHODS: Literature search using the Web of Science, Google Scholar and Medline databases with keywords (absolute uterine infertility factor, AUFI, Mayer-Rokitansky-Küster-Hauser syndrome, MRKH, uterine transplantation) and analysis of articles published in impact and reviewed journals. RESULTS: MRKH syndrome is defined as congenital agenesis of the upper two-thirds of vagina and uterus in women with normal secondary sexual characteristics and female karyotype (46, XX). The incidence of the syndrome is 1 : 4500 births of female sex children. It is the second most common cause of primary amenorrhea. Recent research has focused on elucidating the genetic origin of the disease, focusing on the research of candidate genes that could be participating in the genesis of Müllerian ducts and their derivatives. CONCLUSION: MRKH syndrome now appears as a multifactorial congenital developmental defect based on a combination of genetic predisposition and environmental factors. Modern medicine can help girls with MRKH syndrome to a quality sexual life. It is also able to offer different possibilities of achieving motherhood. In the future, however, further research is needed, in particular on the etiology and pathogenesis of this syndrome to detect a possible genetic basis of the disease.

The use of estradiol metered-dose transdermal spray in clinical practice.

OBJECTIVE: The aim of this study was to evaluate the use of novel estradiol metered-dose transdermal spray (EMDTS) in the treatment of acute climacteric syndrome. METHODS: A multicenter open-label trial was conducted with a 24-week intervention. EMDTS 1.53 mg was given to symptomatic menopausal women. The Menopause Rating Scale (MRS) was used to assess the climacteric syndrome severity. The Friedman non-parametric test and a post-hoc test with Bonferroni correction were used for statistical evaluation. RESULTS: A total of 132 women were enrolled in 20 centers, of whom 123 (93.2%) completed the study. The average age of patients was 53.8 years (37-65 years). The study was discontinued by 6.8% of women. The patients were checked at the beginning of the study, and after 12 and 24 weeks. There was a statistically significant drop (p < 0.001) in MRS values both after 12 and 24 weeks of therapy. The average MRS values improved by 66.2% between the first and the third visits. The most significant improvement was manifested in patients with initial moderate climacteric syndrome (70.9%). CONCLUSION: This study confirms that application of EMDTS offers a novel treatment option for climacteric symptoms.

[Pregnancy in women with solid-organ transplants]. / Tehotenství u zen po transplantacích solidních orgánu.

OBJECTIVE: Evaluation of pregnancies and deliveries in women after solid organ transplantations with respect to the maternal and fetal risks. DESIGN: Overview article. SETTING: Department of Obstetrics and Gynaecology, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague. METHODS: Literature search using the Web of Science, Ovid, Cochrane, Google Scholar and Medline databases with keywords (transplantation, pregnancy, immunosuppression) and analysis of articles published in impact and reviewed journals from 1958 to 2017. RESULTS: Pregnancy in patients after solid organ transplantions is associated with the higher risk of complications, particularly preeclampsia, and high incidence of comorbidities. Women after transplantation have an increased risk of premature deliveries and low birth weight newborns. The highest risk of complications is documented after lung transplantation. For immunosuppression in pregnancy inhibitors of calcineurin, azathioprine and prednisone are used. CONCLUSION: Pregnancies and deliveries in women after solid organ transplants are in a high risk. With early transplantation, adequate patient health compensation, properly planned pregnancy, adequate immunosuppressive therapy and specialized prenatal and obstetric care, women can give birth to healthy newborns after transplantation.

[What is the risk of pelvic organ prolapse recurrence after vaginal hysterectomy with colporrhaphy?] / Jaké je riziko recidivy sestupu pánevních orgánu po vaginální hysterektomii s posevními plastikami?

OBJECTIVE: To determine the risk of prolapse recurrence in patients after vaginal hysterectomy with colporrhaphy. DESIGN: Retrospective clinical study. SETTING: Department of Obstetrics and Gynaecology, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague. METHODS: The trial involved 220 women who underwent vaginal hysterectomy with anterior and posterior kolporrhaphy at our department for uterine prolapse at least grade 2. It was the first vaginal surgery in all of the patients. Subjective feeling of pressure in the vagina and/or palpable mass in the vagina or in front of the vaginal entrance and/or objective finding decline of the vaginal walls or fundus at lest the second degree or repeated surgery for prolapse were considered as a prolapse recurrence. RESULTS: The mean age at the time of vaginal hysterectomy was 66.7 years (range 37-88). Only 11 patients were premenopausal (5%), the others were postmenopausal, a mean time of posmenopause was 16.9 years. The average parity rate of the study group was 2.1. The average duration of the surgery was 68 minutes. Postoperative urinary retention was observed in one patient (0.45%). 156 women were enrolled in the study. The average length of follow-up was 47 months (minimum 24 months). Recurrence of prolapse based on subjective assessment occured in 24 patients (15.4 %) with pressure and resistance in the vaginal introitus. According to objective criteria, the recurrence of prolapse was diagnosed in 33 (21.2%) patients, of which 16 had prolapse of the anterior vaginal wall, three of the posterior vaginal wall (two rectocoele, one rectoenterocoele), eight combination prolapse of anterior and posterior vaginal wall and vaginal vault prolapse was diagnosed in six women. Eight patients (5. 1%) underwent surgery because of prolapse recurrence. The mean interval from primary surgery to the time of reoperation was 24.4 months (range 6-62). CONCLUSION: Patients have to be preoperatively informed about the risk of the prolapse recurrence with the recommendation of appropriate preventive arrangements.

[Female sexual desire disorders--prevalence, classification and treatment possibilities]. / Poruchy zenské sexuální touhy--prevalence, klasifikace a moznosti terapie.

OBJECTIVE: Description of female Hypoactive Sexual Desire Disorder (HSDD) and its prevalence, information on possible ways of diagnosis, definition of models of female sexual behaviour, classification of female sexual dysfunctions and perspective on treatment possibilities. SUBJECT: Systematic review. SETTING: GONA, Private Sexuology Centre, Prague. SUBJECT AND METHODS: Current literature review. CONCLUSION: Low sexual desire is present in more than 40% of the female population. 10-16% of women report personal distress associated with low sexual desire and which is characteristic of HSDD. Standardized questionnaires are used for HSDD diagnosis. Current treatment modalities do not provide satisfactory results and require further research.

[Abdominal sacrocolpopexy--simple and still actual method in the treatment of vaginal vault prolapse?]. / Abdominální sakrokolpopexe--jednoduchá a stále aktuální metoda 1écby prolapsu posevního pahýlu?

OBJECTIVE: To evaluate the effectiveness and complications of abdominal sacrocolpopexy in the treatment of vaginal vault prolapse. DESIGN: Review article. SETTING: Obstetrics and Gynecology Department, Charles University 2nd Medical Faculty and Teaching Hospital Motol, Prague. METHODS: Review from biomedical database Medline from 1960 till 2006. The articles were found under the key words: vaginal vault prolaps, abdominal sacrocolpopexy, effectiveness, complications, urinary incontinence, female sexual dysfunctions. RESULTS: The effectiveness of surgery is getting near to 100 percent in the smaller groups between 11 and 21 women. The larger groups are presenting greater success rate variability from 85 to 97 percent. The studies with up to 2 years of follow up are giving representative results of the effectiveness of surgery. Prolapse recurrence and cystocele, rectocele, enterocele and stress urinary incontinence occurrance belong among the characteristic failure signs. Studies evaluating abdominal sacrocolpopexy argue low incidence of complications. Complications connected with surgery can be divided to intraoperative and early and late postoperative. Bleeding from injured medial sacral artery, cystotomy, enterotomy and ureterotomy are the most common intraoperative complications. Wound infection, ileus and urinary tract infection are the most often early postoperative complications. Stress urinary incontinence, anterior or posterior vaginal wall descent, recurrence of vaginal vault prolapse and mesh erosion through vaginal wall are characteristic late postoperative complications. CONCLUSIONS: Abdominal sacrocolpopexy can be a method of choice in the treatment of vaginal vault prolapse, mainly because of the low morbidity and high success rate. Polypropylene mesh is the prefered suspension structure but the accurate surgical technique is the most important for the abdominal sacrocolpopexy success and safety.

[Abdominal hysterectomy--risk factor in development of urinary incontinence? Results of a questionnaire study]. / Abdominální hysterektomie--rizikový faktor vzniku mocové inkontinence? Výsledky dotazníkové studie.

OBJECTIVE: To evaluate the risk of urinary incontinence development after abdominal hysterectomy for benign gynecological disease. DESIGN: Retrospective questionnaire study. SETTING: Obstetrics and Gynecology Department, Charles University 2nd Medical Faculty and Teaching Hospital Motol, Prague. METHODS: Questionnaire study of women in which abdominal hysterectomy for benign gynecological disease was performed from January till December 2001 at our Department of Gynecology and Obstetrics. The questionnaire included questions about symptoms of stress, urge and mixed urinary incontinence. They were evaluated in connection with age, parity, hormonal status and body mass index. Student t-test was used for the statistical analysis. RESULTS: 114 (59.7%) from all sent-out questionnaires were obtained. In 15.8% cases (18 from 114) de novo urinary incontinence after surgery developed. 77.8% (14 from 18) from these patients suffered from genuine stress incontinence. The rest (4 from 18) of the women had symptoms of mixed incontinence. Study results draw attention to 13.1% women (15 from 114) in which preoperative stress incontinence persisted even after hysterectomy. Preoperative stress incontinence was cured in 6.1% (7 from 114) cases after hysterectomy. Age, parity, hormonal status and body mass index were not statistically different in all groups after surgery. CONCLUSIONS: The results of the study show relatively high risk of urinary incontinence development after abdominal hysterectomy in previously continent women. The results also show high persistence of the stress incontinence symptoms in women who did not inform about their incontinence even if they were asked about it. Higher quality of further health education of the women with drawing their attention to both the possibility of surgical treatment of urinary incontinence simultaneously with hysterectomy are the most important aspects how to make the current gynecological surgical therapy more effective.

[Prevalence of the female urinary incontinence. Results from a questionnaire study]. / Prevalence zenské mocové inkontinence. Výsledky dotazníkové studie.

OBJECTIVE: To evaluate the prevalence of urinary incontinence in patients of gynecological practise aged 31-60. DESIGN: Prospective questionnaire study. SETTING: Obstetric and Gynecologic Department, Charles University 2nd Medical Faculty and Teaching Hospital Motol, Prague. METHODS: Questionnaire study of 561 women aged 31-60 examined with gynecological problems (not for the symptoms of urinary incontinence) from November 2001 till October 2002 in standard gynecological practise. The questionnaire included history, evaluation of urinary continence, lasting of the symptoms, body mass index, obesity, age, parity. Stress, urgent and mixed incontinence and influence on the sexual life were also evaluated. Cochran Mantel-Haenszel test and chi2 test were used for the statistical analysis. RESULTS: The incontinence rate in the group of 533 evaluated patients (95% completed questionnaires from 561) of gynecological practise was 23.8%. 81.1% of incontinent patients in the study suffered from stress urinary incontinence. For an easy survey and analysis the patients were divided into three age groups (31-40, 41-50, 51-60). Prevalence of the urinary incontinence rised with age. Statistically significant lower prevalence of urinary incontinence was in the age group 31-40 (p<0.0005). Influence of parity on the prevalence of incontinence was statistically significant only in the age group 31-40 (p=0.002). Obesity had no statistical impact on prevalence of urinary incontinence (p=0.79). 5.5% of incontinent women suffered from negative effect of urinary incontinence on sexuality; the differencies among the age groups were not statistically significant. CONCLUSIONS: The results of the study show high prevalence of urinary incontinence in population of healthy women of gynecological practise. Low interest for the treatment is in contrast with high prevalence of this symptom. Higher quality of the enlightenment with attention to the prevention and therapy of urinary incontinence in population is the way how to improve quality of lives of the afflicted women.

[Abdominal sacral colpopexy in the treatment of posthysterectomy vaginal vault prolapse]. / Abdominální sakrokolpopexe v lécbe prolapsu posevního pahýlu po hysterektomii.

OBJECTIVE: To evaluate the safety and efficacy of the abdominal sacral colpopexy in the treatment of posthysterectomy vault prolapse. DESIGN: Retrospective clinical trial. SETTING: Obstetric and Gynecologic Department, The Charles University 2nd Medical School and Faculty Hospital Motol, Prague. METHODS: A group of 18 patients with vaginal vault prolapse after hysterectomy who underwent abdominal sacral colpopexy between July 2001 and June 2003 was studied. Ten women had undergone previous abdominal and 8 previous vaginal hysterectomy. All patients were operated on by one surgeon. Preoperative evaluation consisted of physical examination, age, parity, body mass index, history of previous pelvic surgery and hormonal status. The cure rate and complications were evaluated in follow-up. RESULTS: The mean age was 59.5 (41-72) years, the median parity was 2.05 (1-5). Seven (38.9%) patients were slightly overweight (BMI 25-30 kg/m2), one (5.6%) patient was obese (BMI 33.4 kg/m2) and 10 (55.5%) had normal weight (20-25 kg/m2). The average period of follow-up was 14.9 months (6-24). The cure rate was 94.4% (17 of 18 patients). No serious intraoperative complications occurred. Postoperative complications occurred in 2 (11.2%) cases (one urgent and one stress urinary incontinence). The complications were resolved and the patients were free of negative postoperative symptoms in the time of the last follow-up visit. CONCLUSIONS: Vaginal vault prolapse is an infrequent complication after both vaginal and abdominal hysterectomy. The study shows that abdominal sacral colpopexy is an effective and safe surgical procedure in the treatment of posthysterectomy vault prolapse. The operative technique is simple but the surgery should be performed by experienced pelvic surgeons able to resolve intraoperative complications.

[Female sexual dysfunction]. / Zenské sexuální dysfunkce.

OBJECTIVE: Comprehensive information about female sexual dysfunctions and possibilities of their treatment. DESIGN: Review article. SETTING: Institute for Postgraduate Medical Education, Prague. METHODS: Analysis of the literary data (texts in medical journals, monographies, textbooks, internet--"Medline") and author's clinical experience. CONCLUSIONS: Female sexual dysfunctions are highly prevalent but not well defined and understood. Existing definitions and classifications of female sexual dysfunctions are reevaluated and revised at present. Contemporary classification was expanded to include psychogenic and organic causes of desire, arousal, orgasm and sexual pain disorders. The female sexual response cycle is initiated by neurotransmitters which increase pelvic blood flow, vaginal lubrication, clitoral and vaginal reactivity. The effect of the therapy consists in the stabilization of the hormonal levels. New drugs with vasocongestive effect on genital are discussed.

[Importance of correct interpretation of incidental detection of intraocular administration of silicone oil on the CT image]. / Význam správné interpretace náhodne zachycených CT obrazu intraokulárne aplikovaného silikonového oleje.

Liquid silicone is used in ophthalmology as one of the possible substitutes of the vitreous body and for internal tamponade of the vitreous space in pars plana vitrectomy (PPV). The intraocular presence of silicone causes marked changes of the CT image of the bulbus. As the number of patients treated by PPV is steadily increasing (this applies in particular to patients with diabetes and AIDS), the probability increases that the radiologist will encounter this image. Typical changes in the CT image of the bulbus after PPV using a silicone cerclage strip with administration of silicone oil (SO) into the vitreous space: 1. Disfiguration of the bulbus after application of the cerclage strip, 2. the area of the vitreous body is visualized as a hyperdense area with a slit-like hypodense space between SO and the bulbar wall. Knowledge of these changes will avoid errors in the interpretation of accidentally recorded CT images.