Müllerian Duct Anomalies and Mimics in Children and Adolescents: Correlative Intraoperative Assessment with Clinical Imaging

Abstract

Müllerian duct anomalies (MDAs) are congenital entities that result from nondevelopment, defective vertical or lateral fusion, or resorption failure of the müllerian (paramesonephric) ducts. MDAs are common, although the majority are asymptomatic, and have been classified by the American Society of Reproductive Medicine according to clinical manifestations, prognosis, and treatment. Accurate diagnosis of an MDA is essential, since the management approach varies depending on the type of malformation. In females, when a müllerian duct becomes obstructed, the patient may present with an abdominal mass and dysmenorrhea. If the patient is not treated in a timely fashion, the consequences can be severe, extending even to infertility. When an MDA is suspected, ultrasonography (US) should be performed initially to delineate any abnormalities in the genital tract. However, US cannot help identify the type of MDA. In contrast, magnetic resonance imaging is a valuable technique for noninvasive evaluation of the female pelvic anatomy and accurate MDA classification. If obstruction is present, surgical correction of the MDA may be required, and further counseling of the patient with regard to reproductive possibilities becomes important. Supplemental material available at http://radiographics.rsnajnls.org/cgi/content/full/29/4/1085/DC1.

Complex mullarian duct abnormality in a young female: a theraputic dilemma

“…Transverse vaginal septum is classified as class I anomaly by Digwani and Falcone classification system for vaginal anomalies. 1 Embryologically, the upper two-third of the vagina develops from the Mullerian duct and the lower third of the vagina is formed from the ascending sinovaginal bulb which fuses with the mullerian duct. Transverse vaginal septum develops due to failure of resorption of the tissue between the vaginal plate, originating from the urogenital sinus and the caudal aspect of the fused mullerian ducts around the 5 th month of gestation.…”

“…These septa are commonly located in the upper vagina (46% of cases) but can also be located in the mid (40%) or lower (14%) vagina. 1 Cervical agenesis is a congenital disorder of the female genital system that manifests itself in the absence of cervix, the connecting structure between the uterus and vagina. One mild form of condition in which cervix is present but deformed and non-functional are known as cervical atresia or cervical dysgenesis.…”

Magnetic resonance imaging in diagnosis of the mayer-rokitansky-kuster-hauser syndrome

“…However, in approximately 6%-10% of patients with MRKHS, endometrial tissue or variable development of uterus with hematometra may be present, which results in cyclic abdominal pain (16). The rudimentary Müllerian structures can be either functional or nonfunctional (19). If the endometrial layer is functional, the main symptoms will be primary amenorrhea and cyclic abdominal pain due to cryptomenorrhea and hematometra.…”

“…MRKHS is often diagnosed clinically, but in some patients the diagnosis is either radiologically or laparoscopically confirmed. MRI is useful for differentiating between uterine agenesis and hypoplasia, both of which can be best assessed on sagittal images (19). There is no standard MRI protocol for evaluating MRKHS; sequences and acquisition planes vary among centers and investigator preference, and are generally effective in evaluation.…”

Herlyn-Werner-Wunderlich Syndrome: Case Report

“…4 Müllerian kanal anomalilerine %20-25 oranında üriner sistem anomalileri de eşlik etmektedir. Wolf kanalı gelişiminde bir kusur mevcut ise o tarafın böbrek ve toplayıcı sistemi gelişe-meyeceği gibi, uterusta da füzyon anomalileri meydana gelebilmektedir.…”