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.
This is radiology images of the uterine agenesis (Mayer-Rokitansky-Küster-Hauser syndrome.] A: Sagittal T2-weighted image shows absence of uterus and vagina. B: Coronal image confirms left renal agenesis.

                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.
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