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https://www.readbyqxmd.com/read/29043712/uterus-dysplasia-associated-with-cervico-vaginal-agenesis
#1
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
https://www.readbyqxmd.com/read/29030159/obstructive-m%C3%A3-llerian-anomalies-in-menstruating-adolescent-girls-a-report-of-22-cases
#2
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: Twenty-two patients who, at the age of 11.4-18.2 (median 13.1) years, between 2 and 74 (median 7.5) months after menarche, underwent surgical repair of obstructive genital anomaly MAIN OUTCOME MEASURES: Müllerian defect type, presentation, radiologic findings, pre- and postoperative course RESULTS: Eighteen patients (81...
October 10, 2017: Journal of Pediatric and Adolescent Gynecology
https://www.readbyqxmd.com/read/28921452/the-spectrum-of-imaging-appearances-of-m%C3%A3-llerian-duct-anomalies-focus-on-mr-imaging
#3
REVIEW
Takeru Fukunaga, Shinya Fujii, Chie Inoue, Naoko Mukuda, Atsushi Murakami, Yoshio Tanabe, Tasuku Harada, Toshihide Ogawa
Müllerian duct anomalies (MDAs) are the result of incomplete development, vertical or lateral fusion, or absorption of the müllerian ducts. The range of anomalies includes uterovaginal agenesis or hypoplasia, unicornuate uterus, uterus didelphys, bicornuate uterus, septate uterus, and arcuate uterus. Correct diagnosis and classification of these anomalies are essential because pregnancy outcomes and treatment options vary between the types of anomaly. Furthermore, early identification of MDAs helps to avoid prolonged symptomatic periods and the complications that may subsequently arise, such as infertility, endometriosis, and neoplasm...
September 18, 2017: Japanese Journal of Radiology
https://www.readbyqxmd.com/read/28919146/m%C3%A3-llerian-agenesis-in-cat-eye-syndrome-and-22q11-chromosome-abnormalities-a-case-report-and-literature-review
#4
Abdulmajeed AlSubaihin, John VanderMeulen, Kate Harris, John Duck, Elizabeth McCready
BACKGROUND: Although Müllerian agenesis is the second most common cause of primary amenorrhea the underlying etiology in the majority of cases is unknown. Müllerian agenesis has been reported as a rare finding associated with chromosomal aberrations of the 22q11 chromosomal region including at least one individual with cat eye syndrome (CES) and 10 individuals with deletions or duplications of the 22q11.2 region. However, a potential link between 22q11 abnormalities and uterine malformations has been difficult to adequately ascertain given the limited case reports in the literature...
September 14, 2017: Journal of Pediatric and Adolescent Gynecology
https://www.readbyqxmd.com/read/28807736/uterine-didelphys-with-bilateral-cervical-agenesis-in-a-15-year-old-female
#5
Kate C Arnold, Theresa C Thai, LaTasha B Craig
BACKGROUND: Isolated uterine didelphys requires no treatment in contrast to cervical agenesis, which requires a hysterectomy. Given this, correct diagnosis of Müllerian anomalies is paramount for making recommendations for patient care. CASE: A 15-year-old presented to clinic with pelvic pain and primary amenorrhea. Uterine didelphys with bilateral cervical agenesis was diagnosed by imaging. Hysterectomy was recommended and diagnosis was confirmed at surgery and by anatomic pathology...
August 11, 2017: Journal of Pediatric and Adolescent Gynecology
https://www.readbyqxmd.com/read/28791270/herlyn-werner-wunderlich-syndrome-an-unusual-presentation-with-pyocolpos
#6
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
https://www.readbyqxmd.com/read/28782742/laparoscopic-management-of-m%C3%A3-llerian-duct-remnants-in-the-paediatric-age-evidence-and-outcome-analysis
#7
Maja Raicevic, Amulya Kumar Saxena
BACKGROUND: This study performed a literature analysis to determine outcomes of laparoscopic management in Müllerian duct remnants (MDRs). PATIENTS AND METHODS: Literature was searched for terms 'Müllerian' 'duct' 'remnants' and 'laparoscopy'. Primary end points were age at surgery, laparoscopic technique, intraoperative complications and postoperative morbidity. RESULTS: The search revealed 10 articles (2003-2014) and included 23 patients with mean age of 1...
August 1, 2017: Journal of Minimal Access Surgery
https://www.readbyqxmd.com/read/28751235/laparoscopic-uterovaginal-anastomosis-for-cervical-agenesis-a-case-report
#8
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
https://www.readbyqxmd.com/read/28696998/evaluation-and-management-of-vaginoplasty-complications
#9
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...
October 2017: Current Opinion in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28670423/hysterosalpingography-in-the-assessment-of-congenital-cervical-anomalies
#10
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
#11
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
#12
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/nonobstructive-reproductive-tract-anomalies-a-review-of-surgical-management
#13
REVIEW
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
https://www.readbyqxmd.com/read/28462155/azoospermia-in-a-male-with-klippel-feil-anomaly
#14
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
#15
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
#16
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
#17
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
#18
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...
September 2017: Ultrasound Quarterly
https://www.readbyqxmd.com/read/28216917/uterine-conserving-surgery-in-a-case-of-cervicovaginal-agenesis-with-unicornuate-uterus
#19
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
#20
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
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