Arizona woman born without a vagina fundraises for surgery
Kaylee Moats, from Gilbert, Arizona, suffers from Mayer Rokitansky Küster Hauser syndrome - which means she has no cervix, uterus or vaginal opening.
M üllerian agenesis or MRKH occurs in 1 in ∼5,000 females. Currently, the worldwide experience of women with MRKH with regard to vaginal lengthening treatments (VL) and genitourinary (GU) symptoms is unknown, however, anecdotal data suggests that people with MRKH may experience a variety of genitou rinary (GU) symptoms. We conducted this study to describe the GU experiences of people with MRKH in the international community, specifically to identify trends in VL experience, prevalence of GU symptoms, and how symptoms may vary by VL treatment.
We present a series of three women with MRKH and inability to achieve satisfactory vaginal depth with dilation due to pain.
Mayer-rokitansky-kuster-hauser (MRKH) syndrome is a congenital condition affecting 1 in 5000 females that results in vaginal agenesis. Mullerian anomalies such as those found in MRKH are also associated with MURCS (M üllerian duct aplasia–renal agenesis–cervicothoracic somite dysplasia) which is phenotypically similar to VACTERL (vertebral defects, anal atresia, cardiac defects, TE fistula, renal dysplasia, limb anomalies). All three of these conditions are believed to be multifactorial in nature, with pati ents having a typical chromosome complement.
Approximately 1:4000 women are diagnosed with M üllerian agenesis, typically when they are evaluated for primary amenorrhea. The most common conditions associated with Müllerian agenesis are Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) and Androgen Insensitivity Syndrome (AIS). A serum testosterone and karyotype should be performed to differ entiate between these 46,XX and 46,XY conditions. With the advent of direct-to-consumer genetic testing companies, patients may discover they have a 46,XY karyotype without genetic counseling to explain the implications.
This study aimed to evaluate the reproductive potential of patients with Mayer-Rokitansky-K üster-Hauser syndrome (MRKHS) who were candidates for uterus transplantation (UTx) before inclusion in the experimental trial and to summarize the existing experience with posttransplant embryo transfers in functionally successful cases.
ConclusionMRKH patients typically display bilateral uterine rudiments combined with a fibrous band and normally located ovaries. The uterine rudiments are generally small with only one-layer differentiation, a subset of which might be large and exhibited other atypical presentations, including two- or three-layer differentiation or even hematometra. Abnormally located ovaries are not rare.Key Points• Morphologically, MRKH patients typically displayed bilateral uterine rudiments combined with a fibrous band.• Typically, the uterine rudiments (84.4%) were small and displayed only one-layer differentiation.• Ab...
Insufficient penile skin is common during vaginoplasty for male-to-female transition. This issue may be compensated via a scrotal skin flap, with the drawback of hair growth . In recent studies, Nile tilapia skin was successfully used for surgical management of Mayer-Rokitansky-K üster-Hauser syndrome [2,3] and vaginal stenosis [4,5]. The present study aims to describe a novel technique for primary vaginoplasty in male-to-female gender-affirming surgery using Nile tilapia skin as a biocompatible graft to assure adequate vaginal depth.
Authors: Dural Ö, Poyrazoğlu Ş Abstract In patients with Mayer-Rokitansky-Küster-Hauser syndrome and complete androgen insensitivity syndrome (CAIS), management of vaginal hypoplasia includes non-surgical or surgical vaginal elongation techniques. For these patients, primary vaginal dilation is considered a first-line option to avoid the risks of having surgery and complications that may occur due to these procedures. Non-surgical dilation is a highly successful treatment if treatment is initiated when the patient is emotionally mature and ready. Here, we present a case of CAIS with vaginal hypoplasia m...
CONCLUSIONS: Until a few years ago, patients with an absolute uterine factor of infertility, including women with MRKH syndrome, had a real choice of only two equally controversial options giving a chance for motherhood - surrogacy and adoption. However, modern transplantation has shown that a third option - a uterine transplant - exists and is available. PMID: 32016956 [PubMed - in process]
Publication date: 2020Source: European Journal of Radiology Open, Volume 7Author(s): Ramezan Jafari, Mohammad Javanbakht, Fatemeh DehghanpoorAbstractThe incidence rate of ovarian herniation in the inguinal canal is 3 %. Nevertheless, the herniation of ovary with uterus is extremely scarce. Most of these cases are accompanied with congenital genitourinary malformations such as Mayer-Rakitansky-Kuster-Hauser (MRKH) syndrome and Mullerian duct malformations. Herein, a 13-year-old girl presented with type2 MRKH syndrome together with left groin swelling and pain. Sonography reports ovarian herniation into left inguinal canal a...