Unicornuate Uterus: Causes, Symptoms, and Diagnosis

A unicornuate uterus is a congenital malformation of the female reproductive system in which one half of the uterus fails to develop properly. This results in an asymmetrical uterus with only one functional horn. Although it is a rare condition, it can have a significant impact on fertility and pregnancy outcomes. This article explores the causes of a unicornuate uterus, its most common symptoms, and the diagnostic methods used to detect this condition.

What is a unicornuate uterus?

The uterus is the organ where pregnancy develops. Typically it has a pear shape with two cavities (horns), known as the right and left uterine horns. These cavities are connected by the uterine body and lead to the cervix. However, in women with a unicornuate uterus, only one uterine horn develops properly, while the other remains underdeveloped or absent.

This malformation originates in the early stages of fetal development when the Müllerian ducts—embryonic structures that eventually form the female reproductive organs—fail to fuse correctly. As a result, the uterus remains incomplete and presents with only one functional horn.

Causes of a unicornuate uterus

A unicornuate uterus is a congenital malformation, meaning it is present from birth. Below are the main factors contributing to this condition.

Genetic Factors

Although the exact cause is not fully understood, genetic factors are believed to play a crucial role in its development. Mutations or alterations in the genes responsible for organ formation during gestation may increase the likelihood of this malformation. In some families, unicornuate uterus occurs more frequently, suggesting a possible genetic predisposition.

Disruptions in embryonic development

A unicornuate uterus results from a failure in the development of the Müllerian ducts during fetal growth. Under normal conditions, these ducts must fuse to form a uterus with two cavities. However, in these cases, one duct does not fully develop or fails to fuse with the other. This issue typically arises during the early weeks of pregnancy, although the exact reasons for this failure remain unclear.

Environmental Factors 

Although less common, certain environmental factors during pregnancy, such as exposure to toxic substances, infections, or hormonal imbalances, may influence the proper development of reproductive organs. However, these factors are not considered primary causes of a unicornuate uterus, as the condition is generally attributed to genetic and embryonic development factors.

Symptoms of a unicornuate uterus

In many cases, it does not present obvious symptoms, and women may be unaware of their condition until they try to conceive or experience pregnancy complications. However, certain signs and associated problems may indicate this malformation. The most common symptoms include:

Difficulties in Conceiving

One of the most frequent complications is difficulty in achieving pregnancy. Although the uterus may be functional, having only one uterine horn means that the space available for embryo implantation is limited. Additionally, if the functional horn is underdeveloped, issues with the endometrial lining may arise, making implantation and pregnancy development more challenging.

Recurrent Miscarriages

A unicornuate uterus is also associated with a higher risk of miscarriage. The asymmetrical shape of the uterus may hinder proper embryo implantation or compromise fetal development during the early weeks of gestation. This will increase the likelihood of early pregnancy loss.

Pelvic Pain or Dysmenorrhea due to unicornuate uterus

Although less common, some women with a unicornuate uterus may experience pelvic pain or dysmenorrhea (severe menstrual cramps). This discomfort may be caused by the abnormal shape of the uterus, which can lead to blood accumulation or endometrial buildup in the non-functional horn, resulting in pain.

Menstrual Irregularities

Some women with a unicornuate uterus may experience irregular menstrual cycles. This can include more painful periods or less regular cycles due to the uterine asymmetry and potential obstruction of menstrual flow in the non-functional horn.

Diagnosis of a Unicornuate Uterus

The diagnosis of a unicornuate uterus typically involves a combination of medical history, imaging studies, and gynecological procedures. Some of the tests used to diagnose this condition include:

Hysterosalpingography (HSG)

Hysterosalpingography is a radiological test that uses a contrast dye to visualize the uterus and fallopian tubes on an X-ray. While it does not always precisely detect a unicornuate uterus, it can sometimes reveal uterine asymmetry or the presence of a single horn.

Gynecological Ultrasound

Ultrasound is a non-invasive and commonly used tool to examine reproductive organs. In women with a unicornuate uterus, ultrasound can detect an abnormal uterine shape. However, this procedure may not always be sufficient for a definitive diagnosis.

Magnetic Resonance Imaging (MRI)

Magnetic resonance imaging (MRI) is one of the most accurate methods for diagnosing uterine malformations. MRI provides detailed images of the uterus and reproductive organs, facilitating the identification of an abormality and the assessment of its severity.

Laparoscopy

In rare cases, laparoscopy may be performed to directly examine the abdomen and reproductive organs. This procedure allows for a clearer visualization of uterine anomalies. It may be useful when other diagnostic methods are inconclusive.

Treatment and Prognosis

The treatment for an asymmetrical uterus generally focuses on addressing reproductive challenges associated with the malformation. If a woman with a unicornuate uterus has difficulty conceiving or experiences recurrent miscarriages, options such as in vitro fertilization (IVF) or, in some cases, surgical intervention may be considered.

However, many women with a unicornuate uterus can carry a pregnancy to term without major complications. Close monitoring during pregnancy is essential to ensure the best possible outcome.

There are many other uterine abnormalities that can impact fertility and pregnancy. Early diagnosis and appropriate management can help women to overcome potential difficulties, offering them the best chances for a healthy pregnancy. If you would like to schedule a consultation at IVI or receive more information, please fill out the contact form below.