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Objective: To explore how women with Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome experience dilation or surgical vaginal lengthening treatment, and their current sexual well‐being. Design: A qualitative interview study. Setting: Denmark. Population: Women aged ≥25 years diagnosed with MRKH syndrome. Methods: Semi‐structured video interviews were conducted with 18 women. Interviews lasted a median of 92 min and were digitally recorded, transcribed and anonymised. Data were analysed using thematic analysis. Main Outcome Measures: A qualitative analysis of women's experiences. Results: The analysis identified three themes. Firstly, Experiences with dilation treatment revealed dilation as an awkward routine, especially for adolescents living with parents and yet to sexually debut. While some experienced successful vaginal lengthening, others faced treatment failure leading to frustration and self‐blame. Secondly, Experiences with neovaginal surgery described the procedure as extremely painful but resulting in a 'normal size' vagina. Some women felt that the procedure had negatively impacted their self‐confidence, and all underscored the importance of maturity before opting for surgery. Lastly, Current sex life and sexual well‐being indicated a well‐functioning sex life for many women, but with reported low sexual confidence and genital self‐image due to the perceived 'deviance' of their genitalia. Conclusions: For women with MRKH syndrome, vaginal lengthening treatment, whether through dilation or surgery, may result in a 'normal size' vagina. However, according to the women's experiences, vaginal lengthening treatment does not adequately foster positive sexual esteem and genital self‐image. This article includes Author Insights, a video abstract available at: https://1drv.ms/v/c/426d88d9eb0a10fb/ERqbdfeTFeFLkp24xVb9n7ABTZ5VS3f6yxoHlXT7BUPs6A?e=y41xwc. [ABSTRACT FROM AUTHOR]

Background Objectives Search Strategy Selection Criteria Data Collection and Analysis Main Results Conclusions Congenital uterine anomalies include a wide diversity of uterine malformations that can compromise reproductive potential. Uterus transplantation (UTx) proposes an innovative treatment for absolute uterine factor infertility; however, there is a lack of standardized protocols to guide clinical management among this population.To describe recipient and donor characteristics and obstetric outcomes in patients undergoing UTx.We performed a literature search using the PubMed database to retrieve available scientific articles. We analyzed the references of included articles to assess additional articles that could be eligible to be included in the review. Likewise, we identified further studies using other methods, including Google Scholar.Titles and abstracts were screened in duplicate to select original reports with information available for the outcomes of interest.This review assessed the advantages and disadvantages of the techniques used, patient characteristics, obstetric and non‐obstetric complications, functional duration of the organ, and neonatal outcomes.Among the 36 reports included in this review we found 55 pregnancies and 38 live births following UTx and a higher success rate for in vivo uterine donations. The most common obstetric complications reported included miscarriage, pre‐eclampsia, and gestational hypertension. The most common non‐obstetric complications reported include episodes of rejection, acute kidney injury, anemia, and cholestasis. Living donors required a comprehensive preoperative workup, decreasing organ rejection, infection, and vascular complications.More studies are needed to standardize the UTx procedure and improve obstetric, fetal, and neonatal outcomes. Further understanding of which recipient and donor characteristics minimize complications will significantly decrease the risk of adverse outcomes. [ABSTRACT FROM AUTHOR]

Laparoscopy was introduced more than 100 years ago. However, in some fields its use still meets resistance. Technology such as laparoscopy may help to identify rare and complex disorders, even in very ordinary procedures, such as inguinal hernia repair. This report highlighted the importance of early diagnosis of a complex condition using commonly available technology. To the best of our knowledge, there has not been a similar reported case in such a young patient during laparoscopic inguinal hernia repair. [ABSTRACT FROM AUTHOR]

Congenital uterine anomalies (CUA) may have major impacts on the health and social well‐being of affected individuals. Their expressivity is variable, with the most severe end of the spectrum being the absence of any fully or unilaterally developed uterus (aplastic uterus), which is a major feature in Mayer‐Rokitansky‐Kuster‐Hauser syndrome (MRKH). So far, etiologies of CUA remain largely unknown. As reports of familial occurrences argue for strong genetic contributors in some cases, we performed whole exome sequencing in nine multiplex families with recurrence of uterine and kidney malformations, a condition called hereditary urogenital adysplasia. Heterozygous likely causative variants in the gene GREB1L were identified in four of these families, confirming GREB1L as an important gene for proper uterine and kidney development. The apparent mode of inheritance was autosomal dominant with incomplete penetrance. The four families included fetuses with uterovaginal aplasia and bilateral renal agenesis, highlighting the importance to investigate GREB1L in such phenotypes. Subsequent sequencing of the gene in a cohort of 68 individuals with MRKH syndrome or uterine malformation (mostly sporadic cases) identified six additional variants of unknown significance. We therefore conclude that heterozygous GREB1L variants contribute to MRKH syndrome and this probably requires additional genetic or environmental factors for full penetrance. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the feasibility of laparoscopic metroplasty for the treatment of unicornuate uterus with a functional noncommunicating rudimentary horn.Methods: Laparoscopic metroplasty was performed in one patient using traditional laparoscopy and four patients using robot-assisted laparoscopy from December 2013 to December 2017 at the Obstetrics and Gynecology Hospital of Fudan University. The records of the five patients were analyzed retrospectively.Results: In all five patients the unicornuate uterus and functional noncommunicating rudimentary horn were unified into a single cavity without intraoperative or postoperative complications. Average operative time was 281 minutes (range, 204-330 minutes) and average blood loss was 180 mL (range, 100-300 mL). Postoperative hospital stay was 7 days (range, 5-11 days) and there was no re-admission. All patients were relieved of pain and had regular menstruation after surgery. Average follow-up time was 44 months (range, 22-70 months). One patient conceived by in vitro fertilization and embryo transfer 2 years after the operation and delivered twins by cesarean at 33 weeks.Conclusion: Laparoscopic metroplasty, with or without robotic assistance, is an acceptable alternative to resection for a noncommunicating rudimentary horn with a functional endometrium. [ABSTRACT FROM AUTHOR]

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital disorder of still unknown etiology, characterized by uterovaginal agenesis and can be associated with renal, skeletal and cardiac malformations. Most cases are sporadic. We report on a familial case of two female cousins with MRKH syndrome and unilateral renal agenesis. Family history revealed two cases of renal agenesis in male relatives and ultrasonographic (US) examination of healthy relatives diagnosed an uncle with multiple renal cysts. We have reviewed the literature on familial occurrence of MRKH syndrome and its associated anomalies and collected a total of 67 familial cases. We found familial cases to share the same associated anomalies as sporadic cases and we discuss the necessity of US examination of healthy relatives. © 2014 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]