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https://www.readbyqxmd.com/read/28696998/evaluation-and-management-of-vaginoplasty-complications
#1
Anne-Marie Amies Oelschlager, Anna Kirby, Lesley Breech
PURPOSE OF REVIEW: Indications for vaginoplasty include congenital conditions such as adrenal hyperplasia, cloacal malformations, and Müllerian agenesis, acquired conditions including stenosis from radiation or surgical resection for malignancy, and gender affirmation. All vaginoplasty techniques carry significant risk of both immediate and long-term complications. RECENT FINDINGS: The purpose of this study is to provide a review of the evaluation and management of the neovagina, addressing management of human papilloma virus infections and complications including stenosis, fistula, prolapse, and neovaginal colitis...
July 10, 2017: Current Opinion in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28670423/hysterosalpingography-in-the-assessment-of-congenital-cervical-anomalies
#2
REVIEW
Fatemeh Zafarani, Firoozeh Ahmadi, Gholam Shahrzad
Cervical abnormalities may be congenital or acquired. Congenital anomalies of the cervix are rarely isolated, and more commonly accompany other uterine anomalies. Various imaging tools have been used in the assessment of Müllerian duct anomalies (MDAs). Currently, magnetic resonance imaging (MRI) is the modality of choice for definitive diagnosis and classification of these MDAs. Hysterosalpingography is a basic tool for evaluation of infertility and allows us to detect a spectrum of anatomical malformations of the utero-cervix in the setting of MDAs...
July 2017: International Journal of Fertility & Sterility
https://www.readbyqxmd.com/read/28661551/uterine-malformations-an-update-of-diagnosis-management-and-outcomes
#3
Bala Bhagavath, Greiner Ellie, Kara M Griffiths, Tom Winter, Snigdha Alur-Gupta, Carter Richardson, Steven R Lindheim
Importance: The prevalence of uterine malformations has been reported in up to 7% of the general population and 18% of those with recurrent pregnancy loss. The diagnosis, classification, management, and outcome of the management have been subject to debate for decades. Objective: The aim of this article is to summarize the current knowledge regarding the genetics of müllerian anomalies, the varied classification schemes, the appropriate diagnostic modalities, the surgical methods, and the obstetric outcomes related to these surgical methods...
June 2017: Obstetrical & Gynecological Survey
https://www.readbyqxmd.com/read/28540840/living-donor-kidney-transplant-in-a-patient-with-type-b-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-reconstructed-vagina-and-abnormal-pelvic-vessels-a-case-report
#4
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
https://www.readbyqxmd.com/read/28499629/non-obstructive-reproductive-tract-anomalies-a-review-of-surgical-management
#5
REVIEW
Bethany Skinner, Elisabeth H Quint
Mullerian 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. Non-obstructive anomalies are often recognized later than obstructive anomalies, as women do not present with pain. However, non-obstructive anomalies frequently have a negative impact on sexual and reproductive health, and careful evaluation and management of these disorders is warranted. This review will focus on the surgical management of non-obstructive Mullerian anomalies...
May 9, 2017: Journal of Minimally Invasive Gynecology
https://www.readbyqxmd.com/read/28462155/azoospermia-in-a-male-with-klippel-feil-anomaly
#6
Maria Uloko, Elizabeth Bearrick, Joshua Bodie
Müllerian-duct aplasia, renal agenesis, and cervical somite dysplasia (MURCS) is a rare genetic disorder. Previously thought to be exclusive in females, there have now been a small number of case reports describing a male analogue. We describe a patient with obstructive azoospermia and Klippel-Feil anomaly.
July 2017: Urology Case Reports
https://www.readbyqxmd.com/read/28439388/herlyn-werner-wunderlich-syndrome-with-ureteric-remnant-abscess-managed-laparoscopically-a-case-report
#7
Aurus Dourado Meneses, Walberto Monteiro Neiva Eulálio Filho, Débora Maria Ribeiro Raulino, Eduardo Bruno Lobato Martins, Sabas Carlos Vieira
Herlyn-Werner-Wunderlich (HWW) syndrome is part of a spectrum of Müllerian duct anomalies that occur during embryonic development. The syndrome is characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Only few cases of this disease were reported worldwide. We present a 23-year-old female patient with chronic pelvic pain for years. The patient was diagnosed with HWW syndrome with a history of hematocolpos and vaginoplasty at the age of 12. Five months later, she sought urgent medical care due to intense pain, and a clinical picture suggestive of peritoneal irritation...
March 2017: Oman Medical Journal
https://www.readbyqxmd.com/read/28384960/mullerian-agenesis-associated-with-turner-s-syndrome
#8
Padmanabhan Elamparidhi, Rudrappa Ramesh Kumar, Selvakkalanjiyam Sivaranjinie, Rajakumar Sibhithran
Turner's syndrome is a chromosomal disorder with 45 X0 due to absence of a set of gene from the short arm of X chromosome. The incidence of Turner's syndrome is 1 in 2500 live births. Primary amenorrhea is well known in Turner's syndrome, the reason being streak ovaries; but mullerian agenesis as a cause of primary amenorrhea in Turner's syndrome is a very rare entity. The incidence of Turner's syndrome associated with mullerian agenesis is extremely rare as there is only a hand full of cases reported in literature...
February 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28367207/mclndoe-neovagina-in-patients-with-mullerian-agenesis-a-single-center-experience
#9
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
https://www.readbyqxmd.com/read/28263220/ectopic-pelvic-fibroid-in-a-woman-with-uterine-agenesis-and-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome
#10
Thelina Amaratunga, Iain Kirkpatrick, Yi Yan, Fern Karlicki
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare disorder characterized by aplasia or hypoplasia of the uterus and vagina due to arrest in the development of the müllerian ducts. Women with this syndrome have the normal 46 XX karyotype, normal female secondary sex characteristics, and primary amenorrhea. Only a few cases have been described in the literature where a fibroid develops from a rudimentary, nonfunctioning uterus in patients with MRKH syndrome. In even rarer instances, a fibroid can develop in patients with a congenitally absent uterus...
March 3, 2017: Ultrasound Quarterly
https://www.readbyqxmd.com/read/28216917/uterine-conserving-surgery-in-a-case-of-cervicovaginal-agenesis-with-unicornuate-uterus
#11
Vineet Mishra, Suwa Ram Saini, Sakshi Nanda, Sumesh Choudhary, Priyankur Roy, Tanvir Singh
The presence of cervicovaginal agenesis with unicornuate uterus is a very rare mullerian anomaly. Its true incidence is still unknown. The presence of functioning unicornuate uterus poses a great challenge for a gynecologist because a successful repair could restore normal menses and may preserve a patient's fertility. Hence, we report a case of 16-year-old unmarried female who presented with chief complaints of primary amenorrhea with cyclical lower abdominal pain. On clinical and radiological evaluation, she was diagnosed with complete cervicovaginal agenesis with right unicornuate uterus (hematometra and hematosalpinx)...
October 2016: Journal of Human Reproductive Sciences
https://www.readbyqxmd.com/read/28216916/a-rare-case-of-46-xx-gonadal-dysgenesis-and-mayer-rokitansky-kuster-hauser-syndrome
#12
Sriharibabu Manne, C H Veeraabhinav, Mounica Jetti, Yalamanchali Himabindu, Kiranmai Donthu, Mutyalarayudu Badireddy
46,XX gonadal dysgenesis is a rare genetically heterogeneous disorder characterized by underdeveloped ovaries with consequent, impuberism, primary amenorrhea, and hypergonadotropic hypogonadism. Mullerian agenesis or Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is characterized by congenital aplasia of the uterus and the upper part (2/3) of the vagina in a woman with normal development of secondary sexual characteristics and a normal 46,XX karyotype. The phenotypic manifestations of MRKH syndrome may sometimes overlap with various other syndromes and require accurate delineation...
October 2016: Journal of Human Reproductive Sciences
https://www.readbyqxmd.com/read/28210483/intra-peritoneal-leiomyoma-of-the-round-ligament-in-a-patient-with-mayer-rokitansky-k%C3%A3-ster-hauser-mrkh-syndrome
#13
G Salem Wehbe, R Bitar, T Zreik, M Samaha, C Walter, Z Sleiman
BACKGROUND: The occurrence of an extra-uterine leiomyoma, arising from the intra-peritoneal portion of the round ligament in a lady with Müllerian agenesis diagnosed at the age of forty is extremely rare. We report a case of this rare combination in a Middle Eastern woman. CASE: A 40 years old lady, primarily amenorrheic, presented to our clinic for an infertility consultation. The work- up showed features suggestive of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome with a leiomyoma arising from the intra-peritoneal part of the round ligament...
December 2016: Facts, Views & Vision in ObGyn
https://www.readbyqxmd.com/read/28157642/cervicovaginal-agenesis-treated-with-modified-yang-monti-technique-in-two-steps-case-report-and-literature-review
#14
J Acosta-León, A Pantoja-Rojas, J R Corona-Rivera, E López-Marure, C F Ploneda-Valencia
INTRODUCTION: Müllerian duct anomalies are rare with less than 200 cases published in the literature. Recently, the implementation of the Yang-Monti principle for the creation of the neovagina has been used in 10 previous published cases. Here, we report the first case of cervicovaginal agenesis treated with the modified Yang-Monti technique in two steps in México. PRESENTATION OF CASE: A 14-year-old female presented to the pediatric consult with a history of primary amenorrhea and a chronic-cyclic pelvic pain...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28116887/undescended-or-absent-ovary-without-uterine-anomalies-systematic-review-and-a-single-center-s-experience
#15
Daniela Luvero, Francesco Plotti, Stella Capriglione, Andrea Miranda, Salvatore Lopez, Giuseppe Scaletta, Arianna Dell'anna, Roberto Angioli
INTRODUCTION: Two cases of unilateral adnexal absence and ectopic ovarian hypoplasia with fallopian tube absence are herein reported, along with a systematic review of the literature and a discussion on the main factors causing these malformations. EVIDENCE ACQUISITION: The MEDLINE database was searched for English language medical reports published between 1973 and 2014. The key words used were "ovarian agenesis", "ovarian hypoplasia", "undescended ovary" and "fallopian tube abnormalities"...
February 2017: Minerva Ginecologica
https://www.readbyqxmd.com/read/28003020/vertebral-defect-anal-atresia-cardiac-defect-tracheoesophageal-fistula-esophageal-atresia-renal-defect-and-limb-defect-association-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-in-co-occurrence-two-case-reports-and-a-review-of-the-literature
#16
REVIEW
Thomas Bjørsum-Meyer, Morten Herlin, Niels Qvist, Michael B Petersen
BACKGROUND: The vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome are rare conditions. We aimed to present two cases with the vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser co-occurrence from our local surgical center and through a systematic literature search detect published cases...
December 21, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27920851/zinner-syndrome-a-rare-developmental-anomaly-of-the-mesonephric-duct-diagnosed-on-magnetic-resonance-imaging
#17
Shibani Mehra, Rajeev Ranjan, Umesh Chandra Garga
Developmental anomalies of the urogenital tract are rare but often encountered. Zinner's syndrome is a rare congenital abnormality of mesonephric (Wolffian) duct consisting of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ipsilateral ejaculatory duct obstruction due to developmental arrest in early embryogenesis affecting the caudal end of Mullerian duct and only approximately a 100 cases have been reported so far. Radiologic modalities such as intravenous pyelography, ultrasonography, vasovesiculography, contrast enhanced computed tomography, and magnetic resonance imaging are all helpful in diagnosis of this unusual entity...
December 2016: Radiology case reports
https://www.readbyqxmd.com/read/27920841/wolffian-origin-of-vagina-unfolds-the-embryopathogenesis-of-ohvira-obstructed-hemivagina-and-ipsilateral-renal-anomaly-syndrome-and-places-ohvira-as-a-female-counterpart-of-zinner-syndrome-in-males
#18
Yashant Aswani, Ravi Varma, Pradeep Choudhary, Reeta B Gupta
BACKGROUND: The classical theory of Müllerian origin of upper vagina fails to explain complex urogenital malformations like OHVIRA syndrome; the Acien's hypothesis, however, unravels the hidden embryopathogenesis. As per Acien, Wolffian (mesonephric) ducts instead of Müllerian ducts and sinovaginal bulbs, give rise to the vagina. The new hypothesis, however, retains the concept of origin of the ureters (with ureters inducing renal development) by the former and the uterus by Müllerian ducts...
2016: Polish Journal of Radiology
https://www.readbyqxmd.com/read/27852280/treatment-management-during-the-adolescent-transition-period-of-girls-and-young-women-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-mrkhs-a-systematic-literature-review
#19
REVIEW
Anke Wagner, Sara Yvonne Brucker, Esther Ueding, Dagmar Gröber-Grätz, Elisabeth Simoes, Katharina Rall, Andrea Kronenthaler, Norbert Schäffeler, Monika A Rieger
INTRODUCTION: In health services research, there is a special emphasis on the transition from adolescence into adulthood. During this transition period, adolescents change from pediatric to adult medical care. This process must be carefully structured, particularly when special medical care is required. Challenges and difficulties become apparent particularly in the case of rare diseases. This is increasingly so when the rare disease affects the adolescence-specific development of patients, such as Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS), also known as Müllerian agenesis...
November 16, 2016: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/27846665/menstrual-sexual-and-obstetrical-outcomes-after-vaginal-replacement-for-vaginal-atresia-associated-with-anorectal-malformation
#20
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
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