Pseudounicornuate uterus

Pseudounicornuate uterus is a rare congenital uterine anomaly classified under Müllerian duct malformations. In this condition, the uterus appears to have a single horn (resembling a unicornuate uterus), but a rudimentary or hypoplastic second horn is present, which may or may not communicate with the main uterine cavity. This distinguishes it from a true unicornuate uterus, where the second horn is completely absent. The anomaly results from incomplete or asymmetric development of the paired Müllerian (paramesonephric) ducts during embryonic life, which normally fuse to form the uterus, fallopian tubes, cervix, and upper vagina. The condition primarily affects the female reproductive system. Many individuals may be asymptomatic and the anomaly is discovered incidentally during imaging for other reasons. However, clinical manifestations can include dysmenorrhea (painful periods), recurrent pregnancy loss, preterm delivery, malpresentation of the fetus, and other obstetric complications. If a non-communicating rudimentary horn contains functional endometrium, it may lead to hematometra (accumulation of menstrual blood), pelvic pain, or rarely ectopic pregnancy within the rudimentary horn, which carries a risk of life-threatening rupture. Diagnosis is typically made through imaging modalities such as three-dimensional ultrasound, hysterosalpingography, or magnetic resonance imaging (MRI), which is considered the gold standard for characterizing uterine anomalies. Management depends on the clinical presentation. Asymptomatic patients may require no intervention beyond monitoring during pregnancy. Surgical excision (laparoscopic removal) of a non-communicating rudimentary horn may be recommended to prevent complications such as ectopic pregnancy or hematometra. Reproductive outcomes can be improved with appropriate obstetric surveillance, and assisted reproductive technologies may be considered in cases of infertility.

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