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mullerian agenesis

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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
#1
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
#2
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/27716927/genetics-of-mayer-rokitansky-k%C3%A3-ster-hauser-mrkh-syndrome
#3
Laura Fontana, Barbara Gentilin, Luigi Fedele, Cristina Gervasini, Monica Miozzo
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, also referred to as Müllerian agenesis, is the second most common cause of primary amenorrhea. It is characterized by congenital absence of the uterus, cervix, and the upper part of the vagina in otherwise phenotypically normal 46,XX females. MRKH syndrome has an incidence of about 1 in 4,500-5,000 newborn females and it is generally divided into two subtypes: MRKH type 1, in which only the upper vagina, cervix and the uterus are affected, and MRKH type 2, which is associated with additional malformations generally affecting the renal and skeletal systems, and also includes MURCS (MÜllerian Renal Cervical Somite) characterized by cervico-thoracic defects...
October 7, 2016: Clinical Genetics
https://www.readbyqxmd.com/read/27693935/distribution-of-female-genital-tract-anomalies-in-two-classifications
#4
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
#5
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/27507534/diagnostic-imaging-and-cataloguing-of-female-genital-malformations
#6
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/27466877/laparoscopic-hemi-hysterectomy-and-trachelectomy-in-a-case-of-herlyn-werner-wunderlich-syndrome
#7
Entidhar Al Sawah, Shayne M Plosker, Emad Mikhail
BACKGROUND: Herlyn-Werner-Wunderlich Syndrome (HWWS) is a rare Müllerian anomaly characterized by uterus didelphys coexisting with an obstructed hemivagina and ipsilateral renal agenesis. CASE: A 13-year-old female presented one-year after menarche with severe dysmenorrhea and a right-sided pelvic mass. Imaging identified a right uterus with hematometra, hematocolpos, absent right kidney, normal left kidney, ureter, left uterus, and vagina compressed to the left by right hematocolpos...
July 29, 2016: Surgical Technology International
https://www.readbyqxmd.com/read/27462606/a-successful-laparoscopic-neovaginoplasty-using-peritoneum-in-m%C3%A3-llerian-agenesis-with-inguinal-ovaries-accompanied-by-primary-ovarian-insufficiency
#8
Seonghye Gweon, Jisun Lee, Suna Hwang, Kyoung Joo Hwang, Miran Kim
The combination of Müllerian agenesis with inguinal ovaries accompanied by primary ovarian insufficiency is extremely rare. A 21-year-old Korean woman was referred to our center with primary amenorrhea. The patient was diagnosed with Müllerian agenesis with inguinal ovaries. Her hormonal profile showed hypergonadotrophic hypogonadism suggesting primary ovarian insufficiency. We performed laparoscopic neovaginoplasty using modified Davydov's procedure and reposition inguinal ovaries in the pelvic cavity. Oral estrogen replacement was applied for the treatment of primary ovarian insufficiency...
July 2016: Obstetrics & Gynecology Science
https://www.readbyqxmd.com/read/27446624/m%C3%A3-llerian-remnant-cyst-as-a-cause-of-acute-abdomen-in-a-female-patient-with-m%C3%A3-llerian-agenesis-radiologic-and-pathologic-findings
#9
Mujtaba Mohammed, Mary Allen-Proctor, Andrij Wojtowycz
We report a case of a 17-year-old female with Müllerian agenesis who presented with right sided abdominal pain clinically suspicious for acute appendicitis. Multimodality imaging workup revealed a heterogeneous cystic right upper quadrant mass with surrounding fluid and inflammatory changes. Surgical resection of this mass was performed and a histopathologic diagnosis of a hemorrhagic Müllerian remnant cyst was made, which to the best of our knowledge has never been described in a patient with Müllerian agenesis...
2016: Case Reports in Radiology
https://www.readbyqxmd.com/read/27424377/mitotically-active-leiomyoma-in-a-woman-with-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-a-case-report
#10
Irene Dimitriadis, Kelly Pagidas, Denis Vaughan, Young B Kim
BACKGROUND: Cases of women with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome developing leiomyomata are rare. A case with mitotically active leiomyomata has not previously been described to our knowledge. CASE: A 43-year-old woman with MRKH syndrome found to have an incidental pelvic mass on imaging studies underwent a diagnostic laparoscopy, followed by resection of leiomyomata and uterine remnant via mini laparotomy. Histopathology revealed focal infarction associated with a mitotically active area in one of the leiomyomata but with no evidence of marked cytologic atypia or hypercellularity...
May 2016: Journal of Reproductive Medicine
https://www.readbyqxmd.com/read/27424375/severe-hemoperitoneum-during-pregnancy-with-obstructed-hemivagina-and-ipsilateral-renal-anomaly-syndrome-a-case-report
#11
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/27363330/congenital-renal-anomalies-in-cloacal-exstrophy-is-there-a-difference
#12
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/27229498/magnetic-resonance-imaging-of-m%C3%A3-llerian-duct-anomalies-in-children
#13
REVIEW
Yi Li, Andrew Phelps, Matthew A Zapala, John D MacKenzie, Tippi C MacKenzie, Jesse Courtier
Müllerian duct anomalies encompass a wide variety of disorders resulting from abnormalities in the embryological development of the Müllerian ducts. In the prepubertal pediatric population, Müllerian duct anomalies are often incidental findings on studies obtained for other reasons. The onset of menses can prompt more clinical symptoms. Proper characterization of Müllerian duct anomalies is important because these anomalies can affect the development of gynecological disorders as well as fertility. Müllerian duct anomalies also carry a high association with other congenital anomalies, particularly renal abnormalities...
May 2016: Pediatric Radiology
https://www.readbyqxmd.com/read/27190929/a-rare-case-of-mayer-rokitansky-kuster-hauser-syndrome-presenting-with-acute-psychosis
#14
Kamal Nath, Bhanita Boro, Subrata Naskar
The psychiatric co-morbidities in female population with mullerian agenesis is an area with limited research. This is probably due to the fact that when those patients are diagnosed not much attention or information is given for long term psychiatric follow-up. Owing to their inability to bear children, these subjects often become socially harassed. Thus these constant stressors may lead to development of psychopathology in future. Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a congenital abnormality with absence of uterus, cervix and vagina, but normal secondary sexual characteristics and external genitalia and occurs in every 1 out of 4000-10,000 females...
April 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27123211/herlyn-werner-wunderlich-syndrome-with-hematocolpos-an-unusual-case-report-of-full-diagnostic-approach-and-treatment
#15
Rohit Bhoil, Ajay Ahluwalia, Narvir Chauhan
Herlyn-Werner-Wunderlich (HWW) syndrome is an uncommon combined müllerian duct anomalies (MDAs) and mesonephric duct malformation of female urogenital tract characterized by uterus didelphys and obstructed hemi-vagina and ipsilateral renal agenesis (OHVIRA) syndrome. We present a rare and unusual case of this syndrome in a 19 year-old female who suffered from hypomenorrhoea and abdominal pain. She had an obstructed hemi-vagina on right side which led to marked distention of ipsilateral cervix, while proximal hemi-vagina compressed the contralateral side causing its partial obstruction resulting in hypomenorrhoea...
April 2016: International Journal of Fertility & Sterility
https://www.readbyqxmd.com/read/27099773/murcs-m%C3%A3-llerian-duct-aplasia-renal-agenesis-cervicothoracic-somite-dysplasia-a-rare-cause-of-primary-amenorrhoea
#16
Sunil Kumar, Shruti Sharma
The agenesis of the Müllerian duct is the second most common cause of primary amenorrhoea after Turner syndrome. The abnormal development of Müllerian duct often associates with the urinary tract and skeletal abnormalities. MURCS (Müllerian duct aplasia-renal agenesis-cervicothoracic somite dysplasia) association is a unique and rare developmental disorder with four common features of uterine hypoplasia or aplasia, renal agenesis or ectopy, vertebral anomalies and short stature. We report a case of young female with primary amenorrhoea...
April 2016: Oxford Medical Case Reports
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/26949305/leiomyoma-arising-from-mullerian-remnant-mimicking-ovarian-tumor-in-a-woman-with-mrkh-syndrome-and-unilateral-renal-agenesis
#18
Wubishet Girma, Wondimagegnehu Woldeyes
BACKGROUND: Leiomyoma with secondary changes arising from Mullerian remnant may mimic ovarian tumor in women with Mayer-Rockitansky-Kuster-Hauser (MRKH) syndrome in patients with pelvic mass and urologic abnormalities. CASE DETAIL: The patient was a 40 years old known case of MRKH, presented with lower abdominal pain and swelling for over one year. On physical examination, large pelvic mass (about 15cm by 10cm) was found and a diagnosis of ovarian tumor was made by imaging which also showed absent right kidney...
October 2015: Ethiopian Journal of Health Sciences
https://www.readbyqxmd.com/read/26851190/complexities-of-m%C3%A3-llerian-anatomy-in-46xx-cloacal-exstrophy-patients
#19
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
https://www.readbyqxmd.com/read/26816677/herlyn-werner-wunderlich-syndrome-a-rare-cause-of-pelvic-pain-and-high-ca-19-9-levels-in-an-adolescent-girl
#20
Emel Unal, Hikmet Gulsah Tanyildiz, Murat Sonmezer, Hatice Gul Erkol, Suat Fitoz
Herlyn-Werner-Wunderlich (HWW) syndrome is a rare developmental anomaly that includes uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. A 13-year-old girl presented with chronic abdominal pain. Magnetic resonance imaging revealed uterus didelphys, hematometrocolpos and renal agenesis on the right side with imperforate hymen. Subsequently the patient was found to have Mullerian duct anomalies. CA 19-9 level was high. At laparoscopy combined with vaginoscopy hematocolpos was drained following which she improved clinically and CA 19-9 level returned to normal...
January 2016: APSP Journal of Case Reports
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