This video demonstrate rudimentary uterus with absence of both ovaries and 46 ,XX normal karyotype. Genetic investigation revealed a 46,XX karyotype without any mosaicism. Diagnostic laparoscopy was performed. During laparoscopic pelvic exploration, a rudimentary uterus without ovaries and normal bilateral fallopian tubes were observed. If gonadal agenesis is thought to be the cause of primary amenorrhea in patients with normal secondary sexual characteristics, we believe that laparoscopic evaluation is the gold standard in diagnosis.

A rudimentary uterus is a rare congenital anomaly of the female reproductive system that occurs due to abnormal development of the Müllerian ducts during embryogenesis. It is a form of Müllerian duct anomaly (MDA) and can significantly affect a woman’s reproductive health, menstrual cycles, and fertility.

Anatomy and Types

The uterus develops from two Müllerian ducts that fuse to form a single uterine cavity. In cases of rudimentary uterus, one of the ducts may fail to fully develop, leading to:

• Unicornuate uterus with rudimentary horn: The most common form, where one side of the uterus is underdeveloped or absent.

• Non-communicating rudimentary horn: The rudimentary uterus does not connect with the main uterine cavity.

• Communicating rudimentary horn: Rare, where a small cavity may connect to the functional uterus.

The rudimentary uterus is often smaller than a normal uterus and may contain functional endometrial tissue that responds to hormones.

Causes

Rudimentary uterus is congenital, meaning it is present from birth. It arises due to:

• Abnormal fusion of the Müllerian ducts during fetal development.

• Genetic or environmental factors that disrupt normal uterine formation.

It is not caused by lifestyle or external factors during a woman’s life.

Symptoms

Many women with a rudimentary uterus may not realize it until puberty or later. Common signs include:

• Menstrual irregularities: Painful periods (dysmenorrhea) if the rudimentary horn contains endometrial tissue that has no outlet.

• Pelvic pain: Especially if a non-communicating horn leads to hematometra (accumulation of menstrual blood).

• Infertility: Difficulty conceiving or recurrent pregnancy loss.

• Ectopic pregnancy risk: Rarely, pregnancy can occur in the rudimentary horn, which can be life-threatening if undiagnosed.

Some women remain asymptomatic and discover the condition incidentally during imaging for infertility or other gynecological concerns.

Diagnosis

Early diagnosis is important for symptom management and fertility planning. Common diagnostic methods include:

• Ultrasound (Transvaginal or 3D Ultrasound): Often the first step, can detect abnormal uterine shape.

• MRI (Magnetic Resonance Imaging): Gold standard for detailed anatomical assessment.

• Hysterosalpingography (HSG): Helps evaluate uterine cavity and fallopian tube patency.

• Laparoscopy: Provides a definitive diagnosis and may allow simultaneous surgical management.

Treatment

Treatment depends on symptoms and reproductive goals:

1. Asymptomatic cases: May not require intervention.

2. Pain or hematometra: Surgical removal of the non-communicating rudimentary horn is recommended.

3. Infertility management: Assisted reproductive technologies may be considered for women with unicornuate uterus.

4. Pregnancy monitoring: High-risk pregnancies require close monitoring if the uterus is structurally abnormal.

Prognosis

With proper management, many women with a rudimentary uterus can lead normal lives. Fertility may be preserved in women with a functioning main uterus. Early detection and surgical intervention for symptomatic cases reduce the risk of complications.

Conclusion

A rudimentary uterus is a rare but important congenital anomaly of the female reproductive system. Awareness of symptoms and timely diagnosis can prevent complications such as severe pain, hematometra, or life-threatening pregnancy events. With advances in imaging and minimally invasive surgery, women with this condition have better outcomes in terms of symptom relief and fertility.