Mayer-Rokitansky-Küster-Hauser Syndrome Research Articles

Gender Role, Gender Identity and Sexual Orientation in CAIS (“XY-Women”) Compared With Subfertile and Infertile 46,XX Women

The perception of gender development of individuals with complete androgen insensitivity syndrome (CAIS) as unambiguously female has recently been challenged in both qualitative data and case reports of male gender identity. The aim of the mixed-method study presented was to examine the self-perception of CAIS individuals regarding different aspects of gender and to identify commonalities and differences in comparison with subfertile and infertile XX-chromosomal women with diagnoses of Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) and polycystic ovary syndrome (PCOS). The study sample comprised 11 participants with CAIS, 49 with MRKHS, and 55 with PCOS. Gender identity was assessed by means of a multidimensional instrument, which showed significant differences between the CAIS group and the XX-chromosomal women. Other-than-female gender roles and neither-female-nor-male sexes/genders were reported only by individuals with CAIS. The percentage with a not exclusively androphile sexual orientation was unexceptionally high in the CAIS group compared to the prevalence in “normative” women and the clinical groups. The findings support the assumption made by Meyer-Bahlburg (2010) that gender outcome in people with CAIS is more variable than generally stated. Parents and professionals should thus be open to courses of gender development other than typically female in individuals with CAIS.

Customized Silicone Vaginal Stent.

Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome, a congenital anomaly of the female genital tract, is characterized by vaginal agenesis, a rudimentary to absent uterus compromising the normal functions of the genital tract, thereby causing psychological trauma. This syndrome is estimated to occur in approximately 1 in every 4500 females [1]. Patients with vaginal agenesis in MRKH syndrome can be treated by both surgical and non-surgical procedures. However irrespective of the procedure opted, prosthetic vaginal dilators or long-term vaginal stents are required to prevent the possible contraction of the reconstructed vagina and to maintain vaginal width and depth to avoid vaginal stenosis. The use of prosthetic vaginal dilators for non-surgical procedure for the creation of neovagina for patients with complete Mullerian agenesis is usually considered the first line of treatment if suitable. Vaginal dilators (Hegar candles) are indicated only when the vaginal dimple is deep enough (2–4 cm). Due to its elastic nature, the vaginal tissue has the tendency to expand during the insertion of a dilator. To accomplish the tissue expansion, customized stents are made, with increasing measurements in its length and diameter over a period of time. However, vaginal stents are used postoperatively to maintain vaginal width and depth and to prevent contraction or shrinkage and stricture of neovagina, and it also serves as hemostat. Failure to wear a stent, even if it is inconvenient, is the major cause of failure. Several prefabricated or customized stents have been described for postoperative maintenance after vaginoplasty, such as ORFIT “S” vaginal stent, tissue expander, simple syringe, vacuum expandable condom mold, inflatable stents, and acrylic, hollow acrylic or acrylic stents lined with silicones [2, 3]. However, prefabricated stents made from medical grade plastics and acrylic are hard, costly, and are not suitable for all clinical situations owing to the varying anatomies of the defect. So far, the literature review has not revealed much information about complete silicone vaginal stent and fabrication of the same. In this case report, we have made a vaginal stent using silicone which not only maintains the shape but also provides comfort to the patient due to its soft nature. A thorough search has revealed that not much information has been reported in the literature regarding silicone vaginal stent. This case report describes the advantages, disadvantages, and procedure of fabrication of the customized silicone vaginal stents.

Primary amenorrhoea – a single centre experience of 38 cases

Introduction: Primary amenorrhoea is defined as absence of menstruation by the age of 14 in absence of secondary sexual characteristics & by age 16 regardless of the presence or absence of secondary sexual characteristics. It occurs in around 1-4% of women in reproductive age group. The common causes of primary amenorrhoea include outflow tract disorders or uterine abnormalities, ovarian disorders, pituitary dysfunction, and hypothalamic dysfunction. The data of primaryamenorrhoea from our country is limited due to poor reporting and frequent loss to follow up. Hence we undertook this prospective study to determine the etiology for primary amenorrhoeabased on clinical examination and laboratory investigations. Methodology: This prospective study was done in Gynecologic Clinic of Sunrise Hospital between August 2013 to May 2015. The work up of primary amenorrhoea patients comprised of 1) History taking 2) Physical examination 3) Laboratory investigations. Patients were classified into 5 groups based on the compartment of organs involved.I- End organ failure/ outflow tract obstruction, II- Gonadal failure, III- Pituitary cause, IV- Hypothalamic cause, V- Other causes. Results: In our study, the 2 most common etiologic factors of primary amenorrhoeawere mullerian agenesis (65.78%) and gonadal dysgenesis (21.05%). Hypogonadotrophichypogonadism was noted in 10.52% of cases. Range of average age of the patients when they first consulted the physician was 14 to 33 years. Conclusion: Prompt reporting and awareness of available treatment options based on the etiology can make a huge difference in this often underreported disorder.

Associations of Polymorphisms in WNT9B and PBX1 with Mayer-Rokitansky-Küster-Hauser Syndrome in Chinese Han.

BackgroundMayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare syndrome that is characterized by congenital aplasia of the uterus and the upper portion (2/3) of the vagina. Previous attempts to identify causal mutations of MRKH syndrome have primarily resulted in negative outcomes. We investigated whether these reported variants are associated with MRKH syndrome (types I and II) in a relatively large sample size of Chinese Han patients, and whether any gene-gene epistatic interactions exist among these variants.MethodsThis study included 182 unrelated Chinese women with MRKH syndrome (155 with type I and 27 with type II) and 228 randomized female controls. Seventeen candidate loci in the AMH, PBX1, WNT4, WNT7A, WNT9B, HOXA10, HOXA11, LHXA1 and GALT genes were genotyped using the Sequenom MassARRAY iPLEX platform. Single-marker association, additive effects and multifactor interactions were investigated.ResultsThe gene frequency distributions of MRKH type 1 and type 2 were similar. Rs34072914 in WNT9B was found to be associated with MRKH syndrome (P = 0.024, OR = 2.65, 95%CI = 1.14–6.17). The dominant models of rs34072914 and rs2275558 in WNT9B and PBX1, respectively, were significantly associated with MRKH syndrome risk in the Chinese Han patients. Additive gene-gene interaction analyses indicated a significant synergetic interaction between WNT9B and PBX1 (RERI = 1.397, AP = 0.493, SI = 4.204). Multifactor dimensionality reduction (MDR) analysis revealed novel dimensional epistatic four-gene effects (AMH, PBX1, WNT7A and WNT9B) in MRKH syndrome.ConclusionsThis association study successfully identified two susceptibility SNPs (WNT9B and PBX1) associated with MRKH syndrome risk, both separately and interactively. The discovery of a four-gene epistatic effect (AMH, PBX1, WNT7A and WNT9B) in MRKH syndrome provides novel information for the elucidation of the genetic mechanism underlying the etiology of MRKH syndrome.

Síndrome de Mayer-Rokitansky: Presentación de seis casos y revisión de la literatura

La digenesia de los conductos de Müller o agenesia del útero y la vagina, conocido como síndrome de Mayer-Rokitansky-küster-Hauser, es un trastorno del aparato reproductor femenino caracterizado por amenorrea primaria y asociado a anomalías del sistema urogenital. Presentamos 6 casos de pacientes con síndrome de Mayer-Rokitansky, describimos el cuadro clínico, manejo y hacemos una revisión de la literatura.

Use of novel laparoscopic vaginoplasty in MRKH syndrome

Use of novel laparoscopic vaginoplasty in MRKH syndrome

ANOMALIAS DEL APARATO GENITAL FEMENINO

Se realizó un estudio de 58 pacientes con anomalías del aparato genital, vistas entre 1970 y 1984. Se registró un caso por cada 555 mujeres hospitalizadas. Las anomalías genitales más frecuentes fueron tabique vaginal (25.9%), himen imporforado (17.2%), agenesia vaginal (12%), y quiste de Gartner (12%). También se halló agenesia uterina (8.8%), útero bicorne (6.9 %), útero didelfo (5.2%), hipoplasia uterina (5.2%), útero tabicado (1.7%) , hipertrofia del cérvix (1.7%), síndrome de Rokitansky (1.7%) y pseudohermafroditismo (1.7%). Como procedimientos de diagnóstico se efectuó laparotomías, laparoscopias, neumopelvigrafías, ecografías, urografías excretorias, histerosalpingografías, uretrografía, vaginografía, y estudios de cromatina sexual. El tratamiento quirúrgico se efectuó en 44 casos. Las complicaciones post-operatorias fueron estenosis vaginal (2 casos), hemorragia vaginal (2 casos), y eventración abdominal (1 caso).

The Swedish uterus transplantation project: the story behind the Swedish uterus transplantation project.

The Swedish uterus transplantation project: the story behind the Swedish uterus transplantation project.

Laparoscopic vaginoplasty using a single peritoneal flap: 10 years of experience in the creation of a neovagina in patients with Mayer-Rokitansky-Küster-Hauser syndrome

To assess anatomical and functional outcomes of a novel laparoscopic vaginoplasty technique using a single peritoneal flap (SPF) in patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. Prospective follow-up study. University-based tertiary-care hospital. Patients with MRKH syndrome (n = 83) and randomly selected frequency-matched age-comparable healthy women serving as controls (n = 85). From March 2004 to March 2014, a total of 83 patients with MRKH syndrome underwent laparoscopic vaginoplasty using an SPF. Intraoperative parameters, postoperative parameters, and anatomical outcomes were recorded. The functional results of patients who became sexually active were assessed using the Female Sexual Function Index (FSFI) questionnaire and were compared with those of the controls. Laparoscopic vaginoplasty using an SPF was successfully performed in all 83 patients, with no intraoperative complications. The mean operative time and intraoperative blood loss were, respectively, 71.2 ± 18.9minutes and 88.5 ± 57.2ml. The mean length and width of the neovagina at the 6-month follow-up examination were, respectively, 8.2 ± 0.8cm and 3.0 ± 0.6cm. Anatomical success was achieved in all patients. At 12months after surgery, functional success, as assessed by the FSFI questionnaire, was achieved in 95.3% of patients. The FSFI scores did not differ significantly between patients with MRKH and healthy women in a control group. Laparoscopic vaginoplasty using an SPF may be a feasible and effective approach that has satisfactory anatomical and functional outcomes for patients with MRKH syndrome.

Sexual and Psychosocial Functioning in Women with MRKHS after Neovaginoplasty According to Wharton-Sheares-George: A Case Control Study.

BackgroundMayer-Rokitansky-Küster-Hauser syndrome (MRKHS) has a major impact on a woman’s psychological and sexual well-being. In most of the studies that addressed treatment techniques, postoperative sexual function was reported to be satisfactory; however, comparatively few studies have additionally provided an accurate analysis of psychosocial functions in MRKHS patients following different kinds of neovaginal treatment. This study is to evaluate sexual and psychosocial functioning after creation of a neovagina according to Wharton-Sheares-George in women with MRKHS.MethodsWe performed a case-control-study using multiple measures to assess sexual and psychosocial functioning. Ten MRKHS patients and 20 controls of a University hospital and tertiary center for pediatric and adolescent gynecology were assessed. The follow-up assessment comprised 6 standardized questionnaires (Female Sexuality Function Index, FSFI; Patient Health Questionnaire, PHQ; Brief Symptom Inventory, BSI; World Health Organization Quality of Life Assessment, WHOQoL-BREF; Parental Bonding Instrument, PBI; and a German questionnaire on body image). The main outcome measures were sexual function, psychological status, quality of life, body image, and parental bonding styles.FindingsSexual function, psychological status (including depressive and somatic symptoms), quality of life, and own-body experience were at least as good in operated MRKHS patients as in controls. In some measures (FSFI, PHQ-15, psychological domain of the WHOQoL-BREF, and BSI Positive Symptom Total), patients scored significantly better than controls. The results of the PBI indicated a close and sustainable mother-daughter-relationship in MRKHS patients.ConclusionsWe found no evidence for an impairment of sexual or psychosocial functioning in patients after neovaginoplasty according to Wharton-Sheares-George. MRKHS may not necessarily compromise sexual and psychological well-being, provided that the syndrome is properly managed by a multidisciplinary team of health professionals.

MAYER-ROKITANSKY-KUSTER-HAUSER TYPE I SYNDROME - A CASE REPORT

Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome is not uncommon as this is a part of the Mullerian agenesis. This is usually diagnosed during adolescent period when signs and symptoms of the ongoing changes in female body type are delayed. We present 17 years old adolescent female who presented with primary amenorrhoea as the normal menstruation did not start at puberty. She was diagnosed as MRKH Type I Syndrome on the basis of radiological investigations. Key words: MRKH Syndrome, Mullerian agenesis, amenorrhoea, MRI

Self-Reported Psychopathological Symptoms in Young Females with Mayer-Rokitansky-Küster-Hauser Syndrome and Polycystic Ovary Syndrome: Findings from a Cross-sectional Study

Objective: The Mayer-Rokitansky-Kuster-Hauser syndrome (MRKHS) and the polycystic ovary syndrome (PCOS) have been associated with disturbances in youths' psychological functioning and psychosexual development. We examined self-reported psychopathological symptoms in young females with MRKHS and those with PCOS at the time of diagnosis, compared with healthy adolescents. Methods: Self-reported questionnaires to measure depression, anxiety and general psychopathological symptoms were obtained from 70 young females aged 11-20 years. Of the total sample, 24 adolescents presented with MRKHS (mean age ± SD: 17.2 ± 1.5), 22 with PCOS (mean age ± SD: 16.9±2.00) and 24 subjects were recruited as healthy age-matched controls (mean age ± SD: 17.3 ±2.2). Results: The MRKHS group showed significantly higher levels of anxiety symptoms compared with the control group. Also, older MRKHS patients (18-20 years old) presented significantly higher levels of depression, anxiety symptoms, phobic anxiety symptoms, and hostility than PCOS patients of the same age group. Older PCOS patients (18-20 years old) reported significantly less attention problems and more somatic complaints compared with MRKHS patients and controls of the same age group. Conclusion: Young females with PCOS and especially with MRKHS appear to be a psychologically vulnerable group. A biopsychosocial approach should emphasize the assessment and treatment of the psychopathological symptoms of these patients alongside somatic treatments.

Full-Thickness Skin Grafts for Neovaginal Construction in Mayer–Rokitansky–Küster–Hauser Syndrome

Abstract Background: Mayer–Rokitansky–Kuster–Hauser (MRKH) syndrome is characterized by vaginal agenesis, the treatment of which typically involves neovaginal construction using split-thickness skin grafts. While successful in many patients, this method may result in vaginal contracture or foreshortening. Neovaginal construction using full-thickness skin grafts (FTSGs) is an underutilized surgical technique associated with decreased rate of contracture and improved functional outcomes. Cases: FTSGs were used for neovaginal construction in 5 patients with MRKH syndrome. This report describes the surgical technique and the current authors' experience when using it. There was a mean follow-up time of 39.4 months (range: 9–111 months). Results: All 5 grafts have remained patent, and all of the patients who desired vaginal intercourse are now capable of achieving this. None of the 5 patients had vaginal contraction or foreshortening. Conclusions: Neovaginal construction using FTSGs is an underutilized and unde...

Human Uterus Transplantation: Have We Opened a Pandora's Box?

The only untreatable subgroup of female infertility is absolute uterine factor infertility (AUFI), which is due to congenital or surgical absence of a uterus or presence of a non-functional uterus. The solitary option for a woman with AUFI to become a biological mother today is through a gestational surrogacy, a procedure that is outlawed in many countries globally. Human uterus transplantation is a potential future treatment of AUFI. Akar et al. from Turkey reported the first clinical pregnancy in 2013 after uterus transplantation in a 23-year-old Mayer-Rokitansky-Kuster-Hauser syndrome patient with previous vaginal reconstruction and uterus transplantation [2]. 18 months after the transplant, the endometrium was prepared for transfer of the thawed embryos. The first ET cycle with a single, day-3 thawed embryo resulted in a biochemical pregnancy. The second ET cycle resulted in a clinical pregnancy confirmed with transvaginal ultrasound visualization of an intrauterine gestational sac with decidualization. This pregnancy unfortunately did not grow further. Uterine transplantation requires extensive evaluation of the recipient and donor by an experienced multidisciplinary transplantation team both pre- and post-operatively [3]. Eleven human uterine transplantation attempts had been done worldwide but no live birth had yet been reported till 2014. In 2013, a 35-year-old woman with congenital absence of the uterus (MRKH syndrome) underwent transplantation of the uterus in Sahlgrenska University Hospital, Gothenburg, Sweden [4]. The uterus was donated from a living, 61-year-old, parous donor. In vitro fertilization treatment of the recipient had been done before transplantation, from which 11 embryos were vitrified. The recipient and the donor had essentially uneventful post-operative recoveries. The recipient’s first menstruation occurred 43 days after transplantation, and she continued to menstruate at median intervals of 32 days. 1 year after transplantation, the recipient underwent her first single embryo transfer, which resulted in pregnancy. She was then given triple immunosuppression (Tacrolimus, Azathioprine, and Corticosteroids), which was continued throughout pregnancy. She had three episodes of mild rejection, one of which occurred during pregnancy. These episodes were all reversed by corticosteroid treatment. Fetal growth parameters and blood flows of the uterine arteries and umbilical cord were normal throughout pregnancy. The patient was admitted with pre-eclampsia at 31 full weeks and 5 days, and 16 h later a cesarean section was done because of an abnormal cardiotocogram. A male child with a normal birth weight for gestational age (1775 grams) and with APGAR scores 9, 9, and 10 was born. Brannstrom et al. thus described the first live birth after human uterus transplantation [4].

Restoration of a total vaginal closure following McIndoe vaginoplasty

Vaginal aplasia is a rare congenital anomaly, and McIndoe technique is the most preferred for this anomaly. Here, the authors present an unusual case with a total vaginal closure following McIndoe vaginoplasty, its management, and surgical tips and reviewed the related literature.

A rare association of schizophrenia and Mayer-Rokitansky-Küster-Hauser syndrome.

Sir, Schizophrenia is known to be associated with the various congenital disorders such as craniofacial anomalies, DiGeorge syndrome but its association with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome has rarely been reported.[12] MRKH syndrome is characterized by congenital aplasia of the uterus and the upper part. (2/3) of the vagina in women showing the normal development of secondary sexual characteristics and a normal 46, XX karyotype.[3] To the best of our knowledge, till date, there has only been one published case report of the coexistence of these two disorders.[4] We report another case of a 24-year-old unmarried woman Ms. A, with MRKH syndrome and schizophrenia for its interesting phenomenology and management. She presented with worries related to primary amenorrhea and infertility since adolescence, though detailed evaluation had not been done before. This was followed after 3 years by suspiciousness, the delusion of control and impulsive behavior. She believed that a certain unseen man staying abroad was controlling her mind and wants to marry her. She would hear voices of that man and converse with him. She would experience sensations in her vaginal area that she would attribute to sexual intercourse with that man, possible through some scientific technology devised by him. In addition, she would get distressed due to hearing voices of some other unknown persons, discussing about her and commanding her to do certain activities. Those unknown people would know her thoughts and control her actions through a transmitter device fitted in her ear. Ms. A would become irritable, aggressive, and violent toward family members whenever confronted regarding her beliefs. On psychiatric assessment, the psychopathology was confirmed. She had hallucinatory behavior, fearful affect, thought broadcast, the delusion of control, second and third person auditory hallucinations, a haptic hallucination of sexual content, impaired judgment with absent insight on mental status examination. She received a diagnosis of schizophrenia, paranoid type with MRKH syndrome. Diagnosis of MRKH was established by the presence of primary amenorrhea, normal development of secondary sexual characteristics, external genitalia, and ultrasonography finding of absence of the uterine structure between the bladder and rectum. The patient was started on oral olanzapine 10 mg/day, which was later, changed to oral quetiapine up to 600 mg/day with a satisfactory response. She was counseled for the vaginal atresia and vaginoplasty was recommended just before marriage after the consultation with the gynecologist. Future treatment options such as in vitro fertilization and embryo transfer was also discussed with the patient and family members. MRKH syndrome is associated with a number of neuropsychiatric manifestations such as distortions of body image, anxiety, depression, suicide, inter-personal sensitivity, and high psychological distress.[3567] Recent genetic research shows the deletion 17q12 is a recurrent,. pathogenic copy number variants that confers a very high risk for schizophrenia,[8] similarly 17q12 complex micro deletion is also found to be associated with the MRKH syndrome[910] suggestive of common genetic link between the two disorders. Both, psychiatrists and gynecologists need to be aware of this association and more research is required to test the link between these two disorders. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. Acknowledgement We acknowledge the help of the department of obstetrics and gynecology in helping us to manage the patient.

Mutational Analysis of the LHX1 Gene in a French National Cohort of Patients with Mayer-Rokitansky-Küster-Hauser Syndrome

Mutational Analysis of the LHX1 Gene in a French National Cohort of Patients with Mayer-Rokitansky-Kuster-Hauser Syndrome Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH syndrome) is characterized by congenital aplasia of the uterus and the upper part of the vagina in women showing normal secondary sexual characteristics development and a normal 46, XX karyotype. MRKH syndrome is a cause of primary amenorrhea with an incidence of approximately 1 in 5,000 newborn girls. Most cases of MRKH syndrome are sporadic, but some familial cases have been reported, suggesting a genetic cause. The genetic basis of MRKH syndrome remains largely unknown and seems heterogeneous. The LHX1 gene has been suggested to be a strong candidate, because (i) inactivation of the ortholog gene (Lim1) in the mouse model causes a Mullerian aplasia, and (ii) it is located in the chromosomal region 17q12, where deletions including this gene have been identified in approximately 5% of MRKH patients. Recently, three relevant heterozygous mutations of the LHX1 gene have been reported in association with the syndrome.

Mayer-Rokitansky-Küster-Hauser Syndrome with Rare Findings of Crossed Fused Renal Ectopia and Ovarian Serous Cystadenoma

ABSTRACT The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome occurs in one of 4,000 to 5,000 female births and is characterized by normal development of secondary sexual, congenital aplasia of uterus and upper part (2/3) of vagina, and normal fallopian tubes and ovaries. Therefore, individuals with MRKH syndrome are at normal risk of developing ovarian neoplasms. We report a case of 19 years old unmarried female who was admitted to the hospital with pain in lower abdomen for 7 months. She was having primary amenorrhea. Gynecological examination revealed a blind vaginal pouch. Rectal examination revealed a large left-sided 8 cm cystic mass which was mobile with a smooth surface. Uterus was not palpable. Phenotypic sex was female. Breast development, axillar and pubic hair development were normal. Abdominopelvic ultrasonography showed left-sided crossed fused renal ectopia and a heterogeneous hypoechoic cystic mass with dense internal echoes on left-side. Uterus was not visualized. Intravenous pyelography also confirmed the finding of left-sided crossed fused renal ectopia. At laparotomy, a 10 × 8 cm cystic hemorrhagic ovarian mass with a single twist of pedicle was found, with a normal right ovary and bilateral tubes. A small hypoplastic unicornuate uterus was present. Cervix was absent. Left-sided Salpingo-oophorectomy was done and tissue was sent for histopathology, which revealed ovarian serous cyst adenoma. All patients with vaginal agenesis must be regarded primarily as a MRKH syndrome and they must be investigated to establish whether there were any associated congenital anomalies. How to cite this article Asnani M, Srivastava K, Inam L, Kunwar S. Mayer-Rokitansky-Küster-Hauser Syndrome with Rare Findings of Crossed Fused Renal Ectopia and Ovarian Serous Cystadenoma. J South Asian Feder Obst Gynae 2015;7(3):220-222.

Personality traits and coping styles in women with Mayer-Rokitansky-Küster-Hauser syndrome.

IntroductionThe purpose of the study was to determine whether women with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome differ from healthy women as regards personality traits and coping styles.Material and methodsPersonality (the Big Five) and coping styles (problem-, emotion- and avoidance-focused coping) were analyzed in a group of 46 women with MRKH syndrome (age: M = 23.48; SD = 4.88), and in a group of 44 healthy women (age: M = 25.95; SD = 5.24), based on Polish versions of the NEO-FFI and CISS inventories.ResultsResults of analysis of covariance (ANCOVA, age, education and having a partner controlled) revealed significant differences in neuroticism level (F1.86 = 7.01, p < 0.01) and problem (F1.86 = 7.88, p < 0.01) and emotion-focused (F1.86 = 4.98, p < 0.05) coping styles. Differences in the level of neuroticism and instrumental coping was also revealed in analyses which took into account the time for which individuals have been aware of their illness: in women who have been aware of the MRKH diagnosis for more than 6 years, the level of neuroticism was higher (F1.85 = 3.97, p < 0.05) and the problem-focused coping style was at a lower level (F1.85 = 3.93, p < 0.05) than in healthy women.ConclusionsThe results obtained make it possible to identify basic psychological problems related to MRKH syndrome, and to formulate proposals on psychological interventions addressed to this group of women. This study showed that with time negative psychological consequences become more severe, and thus those women should be provided with specialist help from the moment the diagnosis is made.

Family case of Mayer—Rokitansky—Kuster—Hauser syndrome and literature review

Family case of Mayer—Rokitansky—Kuster—Hauser syndrome and literature review