Long Term Findings Concerning the Mental and Physical Condition, Quality of Life and Sexuality after Laparoscopically Assisted Creation of a Neovagina (Modified Vecchietti Technique) in Young MRKHS (Mayer-Rokitansky-Küster-Hauser-Syndrome) Patients

The Mayer-Rokitansky-Küster-Hauser-syndrome (MRKHS) is characterized by a congenital uterine and vaginal aplasia. A large body of literature reports that a diagnosis of MRKHS has a variety of psychological effects on patients and doubts about female identity. The aim of the underlying study was to detect the patient-reported physical and mental health and sexual function before and after laparoscopically assisted creation of a neovagina. 160 women with MRKHS who underwent this type of surgery between September 2009 and December 2015 were invited to complete the questionnaires. Packages consisting of six questionnaires were handed out before surgery, six and 12 months after surgery. Data from 82 patients could be included in the study. Patients had a mean age of 19.9 years at inclusion in the study. We detected an impairment of the health-related mental quality of life. There was no higher risk for psychological disorders. MRKHS patients show similar self-acceptance and normal body image compared to the general population. The sexual function is limited before surgery and normalizes after surgery. Useful factors for coping with the disease are an interdisciplinary approach in diagnostics and treatment, psychosocial adaptation as well as a supportive social environment.

Possibilities of physiotherapy in Mayer–Rokitansky–Kuester–Hauser syndrome

BACKGROUND: MayerRokitanskyKuesterHauser syndrome occurs in 1 of 40005000 newborn girls. The first-line treatment of aplasia of the vagina is considered a vaginal dilation. The use of physiotherapy capabilities in the practice of obstetricians and gynecologists is quite widespread and has firmly established itself in clinical practice. AIMS: To evaluate the effectiveness and tolerability of dilation in combination and without physical effects in the formation of vagina in adolescent girls. MATERIALS AND METHODS: A prospective cohort study of 64 adolescent girls 15 to 18 y with a first-time diagnosis of vaginal and uterine aplasia was conducted. Their psychophysiological features were analyzed with testing according to the questionnaire well-being, activity, mood (SAN), physical and sexual development, a gynecological examination was performed to determine the depth of the vaginal fossa. Teenage girls were randomized into 2 groups: 1 (n=36) to create an artificial vagina, with the method of dilation. The second group of patients (n=28) underwent preformed physiotherapy with subsequent dilation. All the girls daily made a graph of the increase in the length of the vagina and determined the intensity of pain using a visual-analog scale (VAS). After the treatment was completed, the patients were re-tested according to the SAN method. RESULTS: A significant increase in the length of the neovagal space was noted in the group of complex treatment with physiotherapy already at the 8th procedure, with an increase after the completion of 20 procedures. In group 2, the dynamics of pain intensity significantly decreased in comparison with the 1st group. The psychological status of the patients according to the SAN before the start of treatment was characterized by an unfavorable state. CONCLUSION: Conducting course procedures of complex dilation with the use of heat-magneto-vibration in girls with vaginal aplasia has a significant reduction in pain and allowed to achieve the required anatomical length in a shorter time compared to monomethodics.

Double Pedicle Artery Rotation Sigmoid Vaginoplasty for Vaginal Aplasia Management

Sigmoid vaginoplasty has been popular for neovagina reconstruction in vaginal aplasia. The most common surgical complication was vaginal stenosis caused by inadequate vascularization and tension because of graft length. Therefore, ischemia ensued and disrupted wound healing. The selection of double pedicle artery rotation sigmoid vaginoplasty is expected to reduce this problem. Five patients from April to December 2016 were diagnosed with vaginal aplasia; 4 had history of neovagina stenosis. These patients underwent sigmoid vaginoplasty with double pedicle artery rotation. No complications occurred during or after the procedure. Assessment postsurgery was conducted at 1 year. These results suggest that double pedicle artery rotation sigmoid vaginoplasty is a safe and acceptable technique for management of vaginal aplasia. The procedure decreased tension inside vascular pedicles as a result of maintaining abundant vascularization supply. Consequently, this procedure could avert graft necrosis, leakage, and severe stenosis. All of the patients exhibited regular menstrual cycle and satisfactory sexual activity. The outcomes were excellent with remarkable anatomical and functional results.

MRKH syndrome: a review of literature

Primary amenorrhea is defined as failure to achieve menarche till age of 14 years in absence of normal secondary sexual characters or till 16 years irrespective of secondary sexual characters. The most common cause of primary amenorrhea is gonadal pathology followed by Mayer-Rokitansky-Küster-Hauser syndrome (MRKH syndrome). MRKH syndrome is a rare congenital disorder characterised by uterine and vaginal aplasia. It occurs due to failure of development of Müllerian duct. Its incidence is 1 per 4500 female births. Mostly girls present with primary amenorrhea. It is characterised by presence of normal secondary sexual characteristics, normal 46 XX genotype, normal ovarian function in most of the cases and absent or underdeveloped uterus and upper part (2/3) of vagina. It is of two types: type A is isolated type while type B is associated with other renal/skeletal/cardiac anomalies. Treatment includes psychological counselling and vaginoplasty. Vaginoplasty can be done by various non-surgical and surgical techniques. The authors hereby review the literature of MRKH syndrome regarding its embryology, etiopathogenesis, approach to work up and management.

UTERUS AND VAGINAL APLASIA AND PELVIC KIDNEY - MANAGEMENT AND SURGICAL POSSIBILITY IN CONGENITAL ANOMALY CORRECTION

Objective. To clarify the possibilities and peculiarities of neovagival creation among the patients with MRKX syndrome and pelvic kidney. Subject and methods. Examination and surgical treatment were conducted in 3 patients with MRKX syndrome and pelvic kidney, including total laparoscopic colpopoiesis in 2 patients. Results. After surgical correction of vaginal and uterus aplasia in patiets with MRKX syndrome and pelvic kidney neovagina were created. In one patients neovagina were restore after previous surgery by vaginal approach because of extensive adhesions and high risk of laparoscopic surgery. In 2 patients - total laparoscopic colpopoiesis were perform without complications. Conclusion. Our clinical observations of surgical treatment in patients with MRKX syndrome and pelvic kidney shows possible surgical approaches of neovaginal creation depending of previous surgery and pelvic kidney localization, established during laparoscopy. Improvement of endoscopic technique and new modifications of neovaginal construction let us to increase the indications and possibilities of neovaginoplasty in patients with MRKX syndrome and pelvic kidney.