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Vaginal agenesis

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https://www.readbyqxmd.com/read/27846665/menstrual-sexual-and-obstetrical-outcomes-after-vaginal-replacement-for-vaginal-atresia-associated-with-anorectal-malformation
#1
Clare Skerritt, Alejandra Vilanova Sánchez, Victoria Alison Lane, Richard J Wood, Geri D Hewitt, Lesley L Breech, Marc Levitt
The authors of this article became aware of significant differences in the management of two females with a rectovestibular fistula and associated vaginal agenesis. In one patient, a sigmoid colovaginoplasty was performed at the time of the posterior sagittal anorectoplasty (PSARP), and the other underwent repair of the rectovestibular fistula with a primary PSARP, but the surgeons elected to delay the timing of vaginal reconstruction. We decided to review the literature, to establish if recommendations could be made to optimize the management of these children based on current evidence...
November 15, 2016: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27843659/rare-case-of-leiomyoma-and-adenomyosis-in-mayer-rokitansky-kuster-hauser-syndrome
#2
P S Hoo, A R Norhaslinda, J N Shah Reza
We report a case of adenomyosis which developed from a hypoplastic uterus and leiomyoma in a patient with MRKH syndrome. A 45-year-old Malay female with primary amenorrhoea and primary infertility presented with abdominal mass and abdominal pain. She is phenotypically female, has well developed secondary sexual characteristics, and has normal female external genitalia with shallow vagina dimple. Transabdominal ultrasonography showed a homogenous adnexal mass of 10 × 8 cm, uterus sized 5 × 4 cm, and normal kidneys...
2016: Case Reports in Obstetrics and Gynecology
https://www.readbyqxmd.com/read/27693935/distribution-of-female-genital-tract-anomalies-in-two-classifications
#3
Pentti K Heinonen
OBJECTIVE: This study assessed the distribution of Müllerian duct anomalies in two verified classifications of female genital tract malformations, and the presence of associated renal defects. STUDY DESIGN: 621 women with confirmed female genital tract anomalies were retrospectively grouped under the European (ESHRE/ESGE) and the American (AFS) classification. The diagnosis of uterine malformation was based on findings in hysterosalpingography, two-dimensional ultrasonography, endoscopies, laparotomy, cesarean section and magnetic resonance imaging in 97...
September 20, 2016: European Journal of Obstetrics, Gynecology, and Reproductive Biology
https://www.readbyqxmd.com/read/27678038/using-the-wharton-sheares-george-method-to-create-a-neovagina-in%C3%A2-patients-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-a-step-by-step-video-tutorial
#4
Lorenz Kuessel, René Wenzl, Marie-Louise Marschalek, Georg Slavka, Daniela Doerfler, Heinrich Husslein
OBJECTIVE: To provide a review of the literature regarding this technique as well as a step-by-step description with the goal of increasing its use as a safe surgical option. Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is characterized by vaginal agenesis and a variety of mullerian duct anomalies. To date, a variety of procedures have been described for creating a neovagina, but the best treatment remains debated. The Wharton-Sheares-George method, a minimally invasive surgical approach for the creation of a neovagina, is remarkably simple to perform...
September 24, 2016: Fertility and Sterility
https://www.readbyqxmd.com/read/27567309/vaginal-agenesis-and-rectovestibular-fistula-experience-utilizing-distal-ileum-for-the-vaginal-replacement-in-these-patients-preserving-the-natural-fecal-reservoir
#5
Luis De la Torre, Kimberly Cogley, Juan L Calisto, Karla Santos, Alejandro Ruiz, María Zornoza
BACKGROUND: The association of rectovestibular fistula (RVF) and vaginal agenesis (VA) presents a diagnostic and management challenge. The vaginal replacement is usually performed with rectum or sigmoid, which are the natural fecal reservoirs; thus, the fecal control could be affected. We present our experience utilizing ileum to preserve the rectum and sigmoid. METHODS: We performed a retrospective study of eight patients with RVF and VA treated from May 2011 to June 2015 at two colorectal centers, at Pittsburgh and Mexico...
November 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27555476/a-case-of-distal-vaginal-agenesis-presenting-with-recurrent-urinary-tract-infection-and-pyuria-in-a-prepubertal-girl
#6
Ozlem Dural, Funda Gungor Ugurlucan, Cenk Yasa, Ercan Bastu, Hulya Eren, Bahar Yuksel, Serdal Celik, Suleyman Engin Akhan
BACKGROUND: Isolated distal vaginal agenesis is a rare anomaly and mostly becomes symptomatic after menarche. We describe an unusual presentation of this anomaly in a prepubertal girl. CASE: An 11-year-old prepubertal girl presented with recurrent urinary tract infection, pyuria, and right-sided renal agenesis. The findings of perineal inspection, ultrasonography, and magnetic resonance imaging were consistent with a distal vaginal agenesis with pyometrocolpos. Discharging pyometrocolpos with dissection of the atretic portion and a pull-through vaginoplasty were performed...
August 20, 2016: Journal of Pediatric and Adolescent Gynecology
https://www.readbyqxmd.com/read/27552172/uterus-transplantation-in-france-for-which-patients
#7
S Huet, A Tardieu, M Filloux, M Essig, N Pichon, J F Therme, P Piver, Y Aubard, J M Ayoubi, O Garbin, P Collinet, A Agostini, V Lavoue, A Piccardo, T Gauthier
OBJECTIVE: Uterine infertility (UI), which can be caused by a variety of congenital or acquired factors, affects several thousand women in Europe. Uterus transplantation (UTx), at the current stage of research, offers hope for these women to be both the biological mother and the carrier of their child. However, the indications of UTx still need to be defined. The main aim of the study was to describe the different etiologies of UI and other data as marital and parental status from women requesting UTx who contacted us in the framework of a UTx clinical trial...
October 2016: European Journal of Obstetrics, Gynecology, and Reproductive Biology
https://www.readbyqxmd.com/read/27521064/vaginoplasty-with-full-thickness-mesh-skin-graft-for-vaginal-agenesis
#8
Guilherme Lang Motta, Patric Machado Tavares, Lucas Medeiros Burttet, Milton Berger, Brasil Silva Neto, Tiago Elias Rosito
OBJECTIVE: To present a novel technique in reconstructive urology for congenital vaginal agenesis using a full-thickness mesh skin graft and to evaluate the functional capacity for maintenance of satisfactory intercourse. METHODS: From January 2009 to August 2015, seven patients diagnosed with vaginal agenesis underwent vaginoplasty using a full-thickness mesh graft from lower abdominal skin. Herein, the authors describe the technique and initial results in adult patients...
August 9, 2016: Urology
https://www.readbyqxmd.com/read/27507534/diagnostic-imaging-and-cataloguing-of-female-genital-malformations
#9
REVIEW
Pedro Acién, Maribel Acién
UNLABELLED: To help physicians and radiologists in the diagnosis of female genito-urinary malformations, especially of complex cases, the embryology of the female genital tract, the basis for Müllerian development anomalies, the current classifications for such anomalies and the comparison for inclusion and cataloguing of female genital malformations are briefly reviewed. The use of the embryological system to catalogue female genito-urinary malformations may ultimately be more useful in correlations with clinical presentations and in helping with the appropriate diagnosis and treatment...
October 2016: Insights Into Imaging
https://www.readbyqxmd.com/read/27454852/primary-vaginal-dilation-for-vaginal-agenesis-strategies-to-anticipate-challenges-and-optimize-outcomes
#10
Anne-Marie Amies Oelschlager, Katherine Debiec, Heather Appelbaum
PURPOSE OF REVIEW: Primary vaginal dilation is patient controlled, safe, less painful, and much lower cost compared with operative vaginoplasty and is considered first-line treatment for vaginal agenesis for women with Mayer-Rokitansky-Küster-Hauser syndrome and androgen insensitivity syndrome. RECENT FINDINGS: This review will highlight studies that assess the optimal methods of primary vaginal dilation and clarify ideal counseling, frequency of dilation, management of side-effects, and long-term physical and psychological outcomes...
October 2016: Current Opinion in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/27433450/isolated-vaginal-agenesis-associated-with-multiple-gastrointestinal-anomalies-a-case-report
#11
R Angotti, F Molinaro, A L Bulotta, F Ferrara, M Sica, E Bindi, M Messina
More than 50% of infants with esophageal atresia have associated anomalies. We present a case report of a 46XX neonate with long-gap esophageal atresia and tracheoesophageal fistula (EA/TEF), anorectal malformation, bowel duplication and vaginal agenesis. This is an unusual association of abnormalities which had not yet described in literature.
July 2016: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/27424375/severe-hemoperitoneum-during-pregnancy-with-obstructed-hemivagina-and-ipsilateral-renal-anomaly-syndrome-a-case-report
#12
Ayako Muraoka, Hiroyuki Tsuda, Tomomi Kotani, Fumitaka Kikkawa
BACKGROUND: Müllerian tract anomalies have been reported in 2-3% of females. Uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis (OHVIRA) syndrome is a rare condition, with only a few cases of the syndrome occurring during pregnancy having been reported. CASE: A 35-year-old, nulli-gravid woman at 18 weeks of gestation was referred due to cervical incompetence. Her first symptom was genital bleeding. Ultrasonography and MRI led to the diagnosis of OHVIRA syndrome, and pregnancy was confirmed on the affected side with the amniotic sac found to be protruding from the cervix into the vaginal cavity...
May 2016: Journal of Reproductive Medicine
https://www.readbyqxmd.com/read/27403077/evaluation-of-vaginal-agenesis-treated-with-the-modified-mcindoe-technique-a-retrospective-study
#13
Oya Soylu Karapınar, Mustafa Özkan, Ayşe Güler Okyay, Hanifi Şahin, Kenan Serdar Dolapçıoğlu
OBJECTIVE: Retrospective analysis of cases that have undergone neovagina operation because of congenital vaginal agenesis was objected. MATERIAL AND METHODS: Seven cases applying with the complaints of primary amenorrhea or inability to have sexual intercourse were in the study. The cases were diagnosed with congenital vaginal agenesis and operated at Mustafa Kemal University Training and Research Hospital between 2011 and 2014. Vaginoplasty by the modified McIndoe method was performed in all cases...
2016: Journal of the Turkish German Gynecological Association
https://www.readbyqxmd.com/read/27379817/gonadal-agenesis-with-hypoplastic-paramesonephric-ducts-pmnds-derivatives-in-dizygotic-twins
#14
Gianmaria Miolo, Lino Del Pup, Anthony Ash, Massimo Manno, Barbara Pivetta, Giovanni Tessitori, Giuseppe Corona
: The co-occurrence of gonadal agenesis alongside hypoplastic derivatives of the paramesonephric ducts has rarely been observed. PATIENT(S): 16-year-old dizygotic twin sisters were referred to our department because of primary amenorrhea. X-ray, bone densitometry, ultrasonography, pelvic MRI and measurement of pituitary, ovary, and thyroid hormones were performed. Both twins showed hypergonadotropic hypogonadism, bilateral gonadal agenesis, fallopian tube, uterus, and vaginal hypoplasia but normal kidney and urinary tract structures and skeletal system...
July 5, 2016: Gynecological Endocrinology
https://www.readbyqxmd.com/read/27363330/congenital-renal-anomalies-in-cloacal-exstrophy-is-there-a-difference
#15
K D Suson, B Inouye, A Carl, J P Gearhart
INTRODUCTION: Cloacal exstrophy (CE) is the most severe manifestation of the epispadias-exstrophy spectrum. Previous studies have indicated an increased rate of renal anomalies in children with classic bladder exstrophy (CBE). Given the increased severity of the CE defect, it was hypothesized that there would be an even greater incidence among these children. OBJECTIVE: The primary objective was to characterize renal anatomy in CE patients. Two secondary objectives were to compare these renal anatomic findings in male and female patients, and female patients with and without Müllerian anomalies...
August 2016: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/27125227/one-uterus-bridging-three-generations-first-live-birth-after-mother-to-daughter-uterus-transplantation
#16
Mats Brännström, Hans Bokström, Pernilla Dahm-Kähler, Cesar Diaz-Garcia, Jana Ekberg, Anders Enskog, Henrik Hagberg, Liza Johannesson, Niclas Kvarnström, Johan Mölne, Michael Olausson, Jan I Olofsson, Kenny Rodriguez-Wallberg
OBJECTIVE: To determine whether a uterus from the mother of a woman with absolute uterine factor infertility can be transplanted to daughter and carry a pregnancy with delivery of a healthy child. DESIGN: Part of an observational study. SETTING: University teaching hospital. PATIENT(S): Twenty eight-year-old woman with uterine agenesis, her male partner, and her 50-year-old mother. INTERVENTION(S): In vitro fertilization with embryo cryopreservation before live donor uterus transplantation (UTx)...
August 2016: Fertility and Sterility
https://www.readbyqxmd.com/read/26964900/novel-function-of-lhfpl2-in-female-and-male-distal-reproductive-tract-development
#17
Fei Zhao, Jun Zhou, Rong Li, Elizabeth A Dudley, Xiaoqin Ye
Congenital reproductive tract anomalies could impair fertility. Female and male reproductive tracts are developed from Müllerian ducts and Wolffian ducts, respectively, involving initiation, elongation and differentiation. Genetic basis solely for distal reproductive tract development is largely unknown. Lhfpl2 (lipoma HMGIC fusion partner-like 2) encodes a tetra-transmembrane protein with unknown functions. It is expressed in follicle cells of ovary and epithelial cells of reproductive tracts. A spontaneous point mutation of Lhfpl2 (LHFPL2(G102E)) leads to infertility in 100% female mice, which have normal ovarian development, ovulation, uterine development, and uterine response to exogenous estrogen stimulation, but abnormal upper longitudinal vaginal septum and lower vaginal agenesis...
2016: Scientific Reports
https://www.readbyqxmd.com/read/26904393/plastic-neo-vaginal-construction-in-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-an-expert-opinion-paper-on-the-decision-making-treatment-process
#18
Luz Angela Torres-de la Roche, Rajesh Devassy, Sreelatha Gopalakrishnan, Maya Sophie de Wilde, Anja Herrmann, Angelika Larbig, Rudy Leon De Wilde
Vaginal agenesis is a congenital anomaly that affects the life of one of each four thousand women around the world. There is a trend that patients request immediate surgical correction, instead of passive vaginal dilatation. Therefore a differentiated counselling should be provided. We present a comparative chart, based on published evidence, with aspect to the available techniques, which will facilitate the decision-making process in the clinical practice. From our point of view, the best results are achieved with techniques that combine the advantages of the minimal-invasive surgery with those derived of the use of peritoneum as covering tissue of the neovagina...
2016: GMS Interdisciplinary Plastic and Reconstructive Surgery DGPW
https://www.readbyqxmd.com/read/26873917/sigmoid-vaginoplasty-in-testicular-feminising-syndrome-surgical-technique-outcome-and-review-of-the-literature
#19
Rahul Janak Sinha, Ved Bhaskar, Seema Mehrotra, Vishwajeet Singh
Vaginal agenesis occurs in approximately 1:5000 live female births. It results from failure of the sinovaginal bulbs to develop and form the vaginal plate. Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH) is the most common cause of vaginal absence followed by complete or partial androgen insensitivity syndrome. Treatment of these patients encompasses a spectrum from simple non-operative dilation to the more complicated surgical creation of a neovagina. We present a case of a patient with testicular feminising syndrome who was reared as a female and underwent bilateral gonadal excision and sigmoid vaginoplasty...
2016: BMJ Case Reports
https://www.readbyqxmd.com/read/26851190/complexities-of-m%C3%A3-llerian-anatomy-in-46xx-cloacal-exstrophy-patients
#20
Kristina D Suson, Janae Preece, Heather N Di Carlo, Nima Baradaran, John P Gearhart
STUDY OBJECTIVE: To characterize Müllerian anatomy in 46,XX cloacal exstrophy patients. DESIGN: Retrospective review of prospectively maintained, institutionally approved exstrophy-epispadias-cloacal exstrophy database. SETTING: Tertiary care, high-volume exstrophy center (Division of Pediatric Urology, The Johns Hopkins Hospital, Baltimore, Maryland). PARTICIPANTS: We included 31 patients who were genetically female with cloacal exstrophy for whom records included detailed evaluation of Müllerian anatomy...
October 2016: Journal of Pediatric and Adolescent Gynecology
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