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Re: DES Info: The teratogenic effects of DES Exposure in DES exposure – Uterus

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This is important education for the millions of Americans exposed and for those who may be unaware of their exposure.  The health is at risk.  Women are being diagnosed too late and dying all to young

Karen M. Fernandes
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Re: DES Info: The teratogenic effects of DES Exposure in DES exposure – Uterus

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Are you posting this on the AARP Online Community as general information for the average reader? It seems like something that might be included in a technical journal, but not here for casual conversation!

Registered on Online Community since 2007!
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DES Info: The teratogenic effects of DES Exposure in DES exposure – Uterus

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Two paired mullerian ducts ultimately develop into the structures of the female reproductive tract. The structures include the fallopian tubes, uterus, cervix, and upper two thirds of the **bleep**. The ovaries and lower one third of the **bleep** have separate embryologic origins not derived from the müllerian system.


Complete formation and differentiation of the müllerian ducts into the segments of the female reproductive tract depend on completion of 3 phases of development as follows:
• Organogenesis: One or both müllerian ducts may not develop fully, resulting in abnormalities such as uterine agenesis or hypoplasia (bilateral) or unicornuate uterus (unilateral).
• Fusion: The process during which the lower segments of the paired müllerian ducts fuse to form the uterus, cervix, and upper **bleep** is termed lateral fusion. Failure of fusion results in anomalies such as bicornuate or didelphys uterus. The term vertical fusion occasionally is used to refer to fusion of the ascending sinovaginal bulb with the descending müllerian system (ie, fusion of the lower one third and upper two thirds of the **bleep**). Complete vertical fusion forms a normal patent **bleep**, while incomplete vertical fusion results in an imperforate hymen.
• Septal resorption: After the lower müllerian ducts fuse, a central septum is present, which subsequently must be resorbed to form a single uterine cavity and cervix. Failure of resorption is the cause of septate uterus.


Ovaries and the lower **bleep** are not derived from the müllerian system. The ovaries are derived from germ cells that migrate from the primitive yolk sac into the mesenchyme of the peritoneal cavity and subsequently develop into ova and supporting cells. The lower **bleep** arises from the sinovaginal bulb, which fuses with the müllerian-derived upper two thirds to form the complete **bleep**.


Mullerian duct anomalies
Suggestion of a mullerian duct anomaly may arise in different clinical situations. In the newborn or infant, the initial presentation may be an obstructed system discovered as a palpable abdominal, pelvic, or vaginal mass (mucocolpos). 
Similarly, an adolescent girl may present to a clinician because of delayed menarche or because of an obstructed system presenting as an intra-abdominal mass (hematocolpos). Many patients also have cyclical pain.

Women of childbearing age often present with various problems of infertility, repeated spontaneous abortions, or premature delivery. As part of an infertility workup, routine imaging often detects the anomaly. Occasionally, the anomaly is discovered incidentally during imaging evaluation for another condition or during surgery such as elective sterilization.


Mullerian duct anomalies are categorized most commonly into 7 classes according to the American Fertility Society (AFS) Classification Scheme as follows:

American Fertility Society (AFS) Classification Scheme
Class I (hypoplasia/agenesis) includes entities such as uterine/cervical agenesis or hypoplasia. The most common form is the Mayer-Rokitansky-Kuster-Hauser syndrome, which is combined agenesis of the uterus, cervix, and upper portion of the **bleep**. Patients have no reproductive potential aside from medical intervention in the form of in vitro fertilization of harvested ova and implantation in a host uterus.


Class II (unicornuate uterus) is the result of complete, or almost complete, arrest of development of 1 mullerian duct (see the image below). If the arrest is incomplete, as in 90% of patients, a rudimentary horn with or without functioning endometrium is present. If the rudimentary horn is obstructed, it may come to surgical attention when presenting as an enlarging pelvic mass. If the contralateral healthy horn is almost fully developed, a full-term pregnancy is believed to be possible (see didelphys uterus).[18]
Uterus, müllerian duct abnormalities. Unicornuate uterus. Note the failure of the development of one half of the uterus. This form may be associated with a rudimentary horn arising from the contralateral müllerian duct.


Class III (didelphys uterus) results from complete nonfusion of both müllerian ducts. The individual horns are fully developed and almost normal in size. Two cervices are inevitably present. A longitudinal or transverse vaginal septum may be noted as well. Didelphys uteri have the highest association with transverse vaginal septa, but septa also may be observed in other anomalies. Consider metroplasty; however, since each horn is almost a fully developed uterus, patients have been known to carry pregnancies to full term. 
Uterus, müllerian duct abnormalities. Didelphys uterus. Note the complete separation but full development of each müllerian duct.


Class IV (bicornuate uterus) results from partial nonfusion of the müllerian ducts (see the image below). The central myometrium may extend to the level of the internal cervical os (bicornuate unicollis) or external cervical os (bicornuate bicollis). The latter is distinguished from didelphys uterus because it demonstrates some degree of fusion between the 2 horns, while in classic didelphys uterus, the 2 horns and cervices are separated completely. In addition, the horns of the bicornuate uteri are not fully developed; typically, they are smaller than those of didelphys uteri. Some patients are surgical candidates for metroplasty.
Uterus, müllerian duct abnormalities. Bicornuate uterus. Note the partial fusion of the lower uterine segment and persistently separated upper uterine segments. Of key importance is the prominent fundal cleft (>1 cm), which distinguishes the anomaly from septate uterus.


Class V (septate uterus) results from failure of resorption of the septum between the 2 uterine horns. The septum can be partial or complete, in which case it extends to the internal cervical os (see the images below). Histologically, the septum may be composed of myometrium or fibrous tissue. The uterine fundus is typically convex but may be flat or slightly concave (< 1 cm fundal cleft). Women with septate uterus have the highest incidence of reproductive complications. Differentiation between a septate and a bicornuate uterus is important because septate uteri are treated by using transvaginal hysteroscopic resection of the septum, whereas if surgery is possible and/or indicated for the bicornuate uterus, an abdominal approach is required to perform metroplasty.


DES Tshaped uterus 2.jpgHidden birth defects - malformations noted in many of the DES exposed. Timing of exposure was a critical component of the deformities



Karen M. Fernandes
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