Table of Contents
What is the most common Müllerian duct abnormality?
Bicornuate uterus (BU): This is the most common form of müllerian anomaly. Described as a womb with two horns. The womb is not pear-shaped, instead it is shaped like a heart, with a deep indentation at the top.
What is Mullerian duct anomalies?
Müllerian duct anomalies (MDAs) are congenital entities that result from nondevelopment, defective vertical or lateral fusion, or resorption failure of the müllerian (paramesonephric) ducts.
What causes Mullerian duct regression?
Müllerian duct regression is induced by the expression of Amh, encoding anti-Müllerian hormone, from the fetal testes. Subsequently, receptor-mediated signal transduction in mesenchymal cells surrounding the Müllerian duct epithelium leads to duct elimination.
How are Mullerian anomalies diagnosed?
The diagnostic methods are the two-dimensional or three-dimensional ultrasound, MRI, hysterosalpingo-contrast-sonography, X-ray hysterosalpingography, hysteroscopy and laparoscopy. Some müllerian malformations are healed with surgery and/or self-dilatation.
What is herlyn Werner Wunderlich syndrome?
The Herlyn-Werner-Wunderlich syndrome is a rare, congenital disorder characterised by uterus didelphys, unilateral obstructed hemivagina and ipsilateral renal agenesis, all being secondary to mesonephric duct-induced Mullerian anomalies. It is also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA).
What typically happens to the müllerian ducts in females during development?
The müllerian ducts form lateral to the wolffian duct at around 6–8 weeks’ gestation. The müllerian ducts then migrate medially, fuse in the midline, and are incorporated into the urogenital sinus to form the uterovaginal canal by the 10th week of gestation.
How common is Müllerian duct anomalies?
The prevalence of müllerian duct anomalies also varies significantly, with reports ranging from 0.16-10%. When these data are obtained in women with recurrent pregnancy loss who are undergoing hysterosalpingography (HSG), the prevalence of müllerian anomalies is 8-10%.
What mechanism occurs in individuals who have persistent Mullerian duct syndrome?
Most people with persistent Müllerian duct syndrome have mutations in the AMH gene or the AMHR2 gene. The AMH gene provides instructions for making a protein called anti-Müllerian hormone (AMH). The AMHR2 gene provides instructions for making a protein called AMH receptor type 2.
What is the gold standard in diagnosing müllerian abnormalities?
MRI is considered the gold standard in diagnosing congenital or obstructive Mullerian anomalies, such as HWW syndrome, with three-dimensional ultrasonography as a good alternative  .
What typically happens to the Müllerian ducts in females during development?
What is Ohvira syndrome?
The OHVIRA syndrome consists of uterus didelphys, unilateral low vaginal obstruction, and ipsilateral renal agenesis, all 3 components being secondary to mesonephric duct-induced müllerian anomalies. Treatment invariably requires surgical intervention in the form of excision of vaginal septum to relieve obstruction.
What is blind Hemivagina?
Blind hemivagina is a rare malformation which involves Müllerian and Wolffian ducts. Although the condition was first recognized in 1922 (Purslow, 1922) and is represented by the presence of a didelphic uterus and ipsilateral renal agenesis, the pathogenesis remains unclear and its aetiology is still unknown.
What is Müllerian fusion defect?
During typical fetal development, two tube-like structures called the Müllerian ducts fuse together to create the uterus, fallopian tubes and majority of the vagina. When one of these structures does not fully develop, a malformation of the uterus or vagina occurs, and this is referred to as a Müllerian anomaly.
How common is persistent Müllerian duct syndrome?
Persistent Mullerian Duct Syndrome (PMDS) is a very rare condition with less than 300 cases described in the literature (1, 2).
What is the gold standard in diagnosing Mullerian abnormalities?
What typically happens to the Müllerian ducts in males during development?
The Müllerian duct usually breaks down during early development in males, but it is retained in those with persistent Müllerian duct syndrome. Affected individuals have the normal chromosomes of a male (46,XY) and normal external male genitalia.
What is the most common uterine anomaly?
The septate uterus and bicornuate uterus are the most common congenital uterine anomalies. The arcuate uterus is considered a variation of normal uterine development by most obstetricians and gynecologists.
What is müllerian duct?
The Müllerian duct (MD) is the embryonic structure that develops into the female reproductive tract (FRT), including the oviduct, uterus, cervix and upper vagina. The FRT has essential functions in mammals, providing the site of fertilization, embryo implantation and fetal development.
What is Herlyn-Werner-Wunderlich syndrome?
The Herlyn–Werner–Wunderlich syndrome is a rare congenital anomaly characterised by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. It usually presents after menarche with progressive pelvic pain during menses secondary to haematocolpos.
What is Hematometrocolpos?
Hematometrocolpos is a rare congenital abnormality of the female urogenital system that leads to an imperforate hymen and subsequent retrograde menstruation. Complications of hematometrocolpos include abdominal and pelvic pain, hydronephrosis from extrinsic compression of the ureters, and infertility.
Which imaging modalities are used in the evaluation of Müllerian duct anomalies?
Various imaging modalities have been used in the evaluation of MDAs, including hysterosalpingography, ultrasound, and magnetic resonance imaging (MRI). Although hysterosalpingography and ultrasound may suggest a Müllerian duct anomaly, further evaluation by MRI is often required.
What happens when the Müllerian ducts fail?
General Information.— Early developmental failure of the müllerian ducts results in agenesis or hypoplasia of the proximal two-thirds of the vagina, cervix, and uterus.
What are the Müllerian ducts?
The müllerian ducts are paired embryologic structures that undergo fusion and resorption in utero to give rise to the uterus, fallopian tubes, cervix, and upper two-thirds of the vagina.
Does hysterosalpingography and ultrasound suggest Müllerian duct anomaly?
Although hysterosalpingography and ultrasound may suggest a Müllerian duct anomaly, further evaluation by MRI is often required. MRI is the best imaging method available because of its superior ability to reliably visualize complex uterovaginal anatomy. Publication types