FEMALE REPRODUCTIVE ORGANS
not visualized by US and is useful in identifying ambiguous genitalia.
Coronal T2-weighted MR images of the uterus are easier to interpret if their oblique axis is placed parallel to the endometrial canal. The degree of obliquity can be estimated from sagittal images.
Prior surgery makes interpretation of imaging findings more difficult; this is especially true with MRI.
Agenesis/Hypoplasia
The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome consists of congenital absence of the vagina and uterus and represents complete cessation of müllerian duct development; this syndrome is believed to be due to a deficiency of estrogen and other receptors. Ovarian neoplasms develop in young girls in association with this syndrome.
Some authors divide this syndrome into a typical or isolated form, consisting of symmetrical nonfunctioning muscular buds (müllerian duct remnants) and normal fallopian tubes, and an atypical, more generalized form consisting of aplasia of one or both buds and with or without fallopian tube dysplasia. Some duct remnants are cystic. Differentiation between the two forms is made on the basis of laparoscopic findings, although MRI can often suggests a diagnosis. The atypical form is associated with skeletal, renal, and ovarian abnormalities. Lack of adequate müllerian duct development leads to vaginal agenesis; with a functioning uterine anlage, MRKH syndrome results in hematometra. With a laparoscopic finding of an atypical MRKH syndrome, appropriate imaging is reasonable, often beginning with MR (Fig. 12.1).
A complex of renal dysgenesis, Gartner’s duct cyst, and ipsilateral müllerian duct obstruction in 10 girls resulted in a dilated Gartner’s duct protruding into the bladder and presenting as a ureterocele in some and extending posterior to the bladder in others (8); all had unilateral müllerian duct obstruction.
Agenesis of a portion of the müllerian ducts and congenital absence of the uterus and vagina is also found in male pseudohermaphrodites. Occasionally genitography is helpful in defining the underlying anatomy.
The appearance of a hypoplastic uterus is that of a normal uterus except for a smaller size. This condition is not common. A small uterus is also seen in such conditions as prior diethylstilbestrol (DES) exposure.
Isolated fallopian tube agenesis is rare and is associated with maldevelopment of mesonephric and paramesonephric ducts, possibly on an ischemic basis. Hysterosalpingography simply reveals fallopian tube nonfilling. Tubal obstruction due to other causes, including prior fallopian tube torsion causing hemorrhage and eventual reabsorption, must be excluded.
Unicornuate Uterus
Abnormal unilateral development of one of the Müllerian ducts results in an unicornuate uterus. These women have a high prevalence of associated urinary tract abnormalities, including an ectopic kidney, renal agenesis, double renal pelvis, horseshoe kidney, and medullary sponge kidney. They also suffer from a high spontaneous abortion rate and a high rate of ovum implantation in a rudimentary horn and subsequent rupture during pregnancy.
Hysterosalpingography reveals a fusiformshaped uterus tapering to its connection with the single fallopian tube. The uterus is displaced toward the side of the functioning tube. When performing this study, one must be careful not to confuse a bicornuate or septate uterus with a unicornuate one. Even if hysterosalpingography does demonstrate what appears to be a unicornuate uterus, a contralateral noncommunicating uterine horn may still be present but simply not communicate with the main uterine cavity, a finding not detected with hysterosalpingography. Such a rudimentary noncommunicating horn is detected by CT, US, or MRI, although US may be nonspecific, defining only a single cavity but without providing sufficient detail. Magnetic resonance imaging is the procedure of choice to provide both uterine and adnexal region anatomic details.
A pregnancy in a noncommunicating rudimentary horn is associated with a high rate of perforation and thus, if detected, resection of this cavity is generally performed. Such a cavity also predisposes to endometriosis, presumably due to retrograde expulsion of menstrual products.