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Vaginal agenesis

Ömer Özkan, Özlenen Özkan, Anı Çinpolat, Nasuh Utku Doğan, Gamze Bektaş, Kemal Dolay, Alihan Gürkan, Cumhur Arıcı, Selen Doğan
Although vaginal reconstructions with intestinal segments require particularly complex surgical procedures, this technique has become popular with respect to fairly good functional and esthetic outcomes. This study describes cases of vaginal reconstruction performed using a modified rectosigmoid colon held in an ischemic state in order to reduce secretion and denervated in order to prevent defecation problems. Vaginal reconstructions with rectosigmoid colon were performed on 43 patients. In this retrospective study, 34 patients had Müllerian agenesis, while nine had undergone male to female sex reassignment surgery in which adequate vaginal depth had not been achieved...
March 8, 2018: Journal of Plastic Surgery and Hand Surgery
Tian-Tian Han, Juan Chen, Shu Wang, Lan Zhu
RATIONALE: A combination of Vaginal atresia and septate uterus as a novel genital malformation has been reported the first time. It offers a support for the bidirectional theory. PATIENT CONCERNS: A 23-year-old woman presented with the inability to perform intercourse. The unprecedented variant was misdiagnosed by magnetic resonance imaging and ultrasonography as low vaginal atresia associated with complete septate uterus with functional endometrium in both the cavities...
January 2018: Medicine (Baltimore)
Bao T Nguyen, Katherine L Dengler, Rhiana D Saunders
Background: Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a congenital condition characterized by aplasia of the vagina with or without concurrent uterine and/or cervical aplasia. Type II (MURCS) is a rare form involving MUllerian agenesis, Renal agenesis, and Cervicothoracic Somite anomalies. Case: A 17-yr-old virginal female presented for evaluation of primary amenorrhea and pelvic pain. Her medical history was significant for a bicuspid aortic valve and right radial dysplasia...
February 5, 2018: Military Medicine
Edoardo Errichiello, Cristina Gorgone, Loretta Giuliano, Barbara Iadarola, Emanuela Cosentino, Marzia Rossato, Nehir Edibe Kurtas, Massimo Delledonne, Teresa Mattina, Orsetta Zuffardi
SOX2 variants have been identified in multiple patients with severe ocular anomalies and pituitary dysfunction, in addition to various systemic features. We investigated a 26-year-old female patient suffering from spastic paraparesis, hypoplasia of corpus callosum, hypogonadotropic hypogonadism (HH) and intellectual disability, who was monitored for over 20 years, allowing a detailed genotype-phenotype correlation along time. Whole exome sequencing on the patient and her relatives identified a de novo SOX2 c...
January 22, 2018: European Journal of Medical Genetics
Katrina Roen, Sarah M Creighton, Peter Hegarty, Lih-Mei Liao
OBJECTIVE: To investigate specialist clinicians' experiences of treating vaginal agenesis. DESIGN: Semi-structured interviews. SETTING: 12 hospitals in Sweden and the UK. SAMPLE: 32 health professionals connected to multidisciplinary teams including medical specialists and psychologists. METHODS: Theoretical thematic analysis of recorded verbatim data. RESULTS: The gynecologist and psychologist interviewees share certain observations including the importance of psychological readiness for and appropriate timing of treatment...
January 12, 2018: Journal of Pediatric and Adolescent Gynecology
Michael W Slater, Xochitl Vinaja, Islam Aly, Marios Loukas, Mark Terrell, Justine Schober
Transgender reassignment surgeries have become a major topic of interest within the last decade. Although there are numerous surgical techniques for male-to-female reassignment, a physician must assess each patient's anatomy and past medical history to determine the most suitable technique. Additionally, patient preference is important. The neovaginal constructive technique most commonly used in male-to-female reassignment surgery is penile skin inversion, but various other techniques and tissues can also be used including pelvic peritoneum, buccal mucosa, and bowel...
March 2018: Clinical Anatomy
Oscar J Manrique, M Diya Sabbagh, Pedro Ciudad, Jorys Martinez-Jorge, Kidakorn Kiranantawat, Ngamcherd Sitpahul, Todd B Nippoldt, Ali Charafeddine, Hung-Chi Chen
INTRODUCTION: Vaginal reconstruction and vaginoplasty are indicated in vaginal agenesis, following pelvic tumor resection, trauma and for gender confirmation surgery. Different surgical options have been described when using these techniques. Herein, we present the clinical outcomes and sexual function evaluation using the pedicle transverse colon flap (TCF) for gender confirmation surgery in transgender women. METHODS: This is a retrospective chart review of all transgender women who underwent gender confirmation surgery using the pedicle TCF...
November 11, 2017: Plastic and Reconstructive Surgery
Ali Mahdavi, Hadi Mirfazaelian, Ladan Younesi Asl, Zeynab Hasani, Maryam Bahreini
Müllerian ducts can form upper parts of normal female reproductive system and any failure in ductal fusion may result in to müllerian duct anomalies (MDA). We present a case of MDA and a uterus dysplasia with no evidence of cervical or upper vaginal tissue. This case showes the role of magnetic resonace imaging (MRI) on MDA diagnosis and urges the need for a unified reliable and practical classification more compatible with clinical practice.
January 2018: International Journal of Fertility & Sterility
Karina Kapczuk, Zbigniew Friebe, Kinga Iwaniec, Witold Kędzia
STUDY OBJECTIVE: To assess the clinical course of obstructive Müllerian anomalies found in girls after menarche. DESIGN: A retrospective case series of adolescents who, between 2009 and 2016, were treated for vaginal or uterine obstructive malformations diagnosed after menarche. SETTING: Division of Gynecology, Poznań University of Medical Sciences, Poznań, Poland. PARTICIPANTS AND INTERVENTIONS: Twenty-two patients who, at the age range between 11...
October 10, 2017: Journal of Pediatric and Adolescent Gynecology
Deb Kumar Boruah, Shantiranjan Sanyal, Bidyut Bikash Gogoi, Kangkana Mahanta, Arjun Prakash, Antony Augustine, Sashidhar Achar, Hiranya Baishya
INTRODUCTION: Mayer-Rokitansky-Kuster-Hauser (MRKH) synd-rome is a malformation in female genital tract due to interrupted embryonic development of para-mesonephric ducts leading to uterine and proximal vagina aplasia or hypoplasia. AIM: To analyse the MRI findings in females suspected of MRKH syndrome in a primary amenorrhea workup. MATERIALS AND METHODS: A cross-sectional study comprising of 11 patients of MRKH syndrome presented in a tertiary care centre from March 2016 to February 2017 evaluated in 1...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
Musa Kayondo, Joseph Njagi, Peter Kivuniike Mukasa, Tom Margolis
BACKGROUND: Although vaginal agenesis as may occur in Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare condition, it is associated with not only anatomical problems but also serious psychological and social problems like painful sexual intercourse, primary amenorrhea and infertility. Surgery, which is aimed at reconstruction of a vagina of adequate length and width to serve the function, is the main method of treatment. Many methods for vaginal reconstruction have been described but each has its complications and limitations...
August 29, 2017: BMC Urology
Dubravko Habek, Goran Arbanas, Vlado Jukic
Urethral coitus is a rare type of sexual practice, usually due to vaginal agenesis or hymeneal anomalies. We report a case of urethral coitus in a healthy couple who were evaluated for infertility. The female partner had cribriform hymen and dilated urethral orifice but did not report any problems except infertility and her genital anatomy was normal. The male partner reported concerns over his penile size but was otherwise healthy. After incision of hymen, they were able to have vaginal coitus and successfully conceived...
August 22, 2017: Archives of Sexual Behavior
V Patel, J Hakim, V Gomez-Lobo, A Amies Oelschlager
STUDY OBJECTIVE: To examine providers' experiences with vaginal dilator training for patients with vaginal agenesis. DESIGN AND SETTING: Anonymous electronic survey. PARTICIPANTS: Members of the North American Society for Pediatric and Adolescent Gynecology. INTERVENTIONS AND MAIN OUTCOME MEASURES: How providers learn about vaginal dilator training, common techniques, and methods used for patient training, assessment of patient readiness, common patient complaints, issues leading to early discontinuation...
August 4, 2017: Journal of Pediatric and Adolescent Gynecology
Eun Jung Jung, Moon Hyeong Cho, Da Hyun Kim, Jung Mi Byun, Young Nam Kim, Dae Hoon Jeong, Moon Su Sung, Ki Tae Kim, Kyung Bok Lee
Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly of the urogenital tract, which is characterized by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It usually presents at puberty with pelvic pain, dysmenorrhea, and a vaginal or pelvic mass. Although rare, it may present with purulent vaginal discharge due to secondary infection of the obstructed hemivagina, making diagnosis difficult. A careful pelvic examination to identify the cervix and vagina is the key to the diagnosis of Müllerian duct anomalies and magnetic resonance imaging can provide additional useful information...
July 2017: Obstetrics & Gynecology Science
Ameya Padmawar, Rizwana Syed, Suyash Naval
STUDY OBJECTIVE: To demonstrate possibility of an innovative surgery for management of cervical agenesis. DESIGN: Step wise description of laparoscopic uterovaginal anastomosis. SETTING: Isolated cervical agenesis occurs in 1 in 80000 to 100000 births. According to the American Fertility Society, cervical agenesis should be classified as a type Ib müllerian anomaly. According to ESHRE/ESGE classification, it is classified in class C4 category...
July 24, 2017: Journal of Minimally Invasive Gynecology
Richa Vatsa, Juhi Bharti, Kallol Kumar Roy, Sunesh Kumar, Jai Bhagwan Sharma, Neeta Singh, Seema Singhal, Jyoti Meena
OBJECTIVE: To assess the outcome of amnion vaginoplasty in cases of vaginal agenesis due to Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome managed at the authors' institution. DESIGN: Retrospective study. SETTING: Tertiary care hospital. PATIENT(S): Fifty women with MRKH who underwent neovaginoplasty. INTERVENTION(S): Modified McIndoe's vaginoplasty was done in all the patients, using human amnion graft...
August 2017: Fertility and Sterility
Mariarosaria Campise, Mariano Ferraresso, Evaldo Favi, Claudio Beretta, Caterina Colico, Piergiorgio Messa
Mayer-Rokitansky-Küster-Hauser syndrome is a rare disorder consisting of vaginal aplasia and other müllerian duct abnormalities. Urinary tract malfor-mations possibly leading to renal failure are also common. For these patients, kidney transplant remains the best option. However, aberrant anatomy and scarring from previous operations may actually preclude successful implantation of the graft. In this setting, careful pretransplant evaluation with high-resolution imaging studies and multidisciplinary planning are mandatory...
May 22, 2017: Experimental and Clinical Transplantation
Bethany Skinner, Elisabeth H Quint
Müllerian anomalies include a spectrum of anatomic variants of the genital tract arising from abnormal embryologic development, ranging from incomplete resorption of uterine septa to complete vaginal agenesis. Nonobstructive anomalies are often recognized later than obstructive anomalies, because women do not present with pain. However, nonobstructive anomalies frequently have a negative impact on sexual and reproductive health, and careful evaluation and management of these disorders is warranted. This review focuses on the surgical management of nonobstructive Müllerian anomalies...
May 9, 2017: Journal of Minimally Invasive Gynecology
Morten Herlin, Michael Bjørn Petersen
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital anomaly characterized by uterovaginal agenesis in females with normal secondary sex characteristics and normal karyotype (46,XX). The prevalence of MRKH syndrome is one in 5,000 live female births as recently confirmed by a nationwide population-based study in Denmark. This review kaleidoscopically summarizes the current knowledge of the history, genetics, diagnostics, treatment of vaginal agenesis, psychosexual aspects, and fertility options in MRKH syndrome...
March 27, 2017: Ugeskrift for Laeger
Abdulrahim Gari
OBJECTIVES: To evaluate the surgical feasibility and the long-term anatomical and functional results and complication rates in patients with Mullerian aplasia who underwent vaginal reconstruction. METHODS: A retrospective observational case series study over 8 years was conducted in King Faisal Specialist Hospital & Research Centre - Jeddah, Saudi Arabia. All cases diagnosed as Mullerian aplasia and who underwent surgical correction were included. Painless and satisfactory vaginal intercourse after surgery were the main outcome measured...
January 2017: Pakistan Journal of Medical Sciences Quarterly
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