Introduction
Mullerian duct anomalies (MDAs) are congenital disorders of the female reproductive system that arise due to abnormalities in the embryological development of the Mullerian ducts. These abnormalities also include failure of development, canalization, fusion, or reabsorption processes that generally occur between six and 22 weeks in the uterus. These defects are estimated to be seen in 0.5 to five percent of the general population. Uterine anomalies are also associated with a risk of spontaneous abortion, cesarean delivery, premature labor, and decreased live births compared to a normal uterus. MDAs make it challenging for most women to conceive or continue the pregnancy to full term, but they are mostly treatable.
What Is Uterine Didelphys?
Didelphys uterus or uterine didelphys, also known as the double uterus, is one of the rare Mullerian duct anomalies. It occurs when the uterus forms abnormally during fetal development. It is characterized by a complete failure of the Mullerian ducts to fuse, resulting in separate uterine cavities and two cervices. A longitudinal vaginal septum is also seen, which may be thin and easily displaced or thick and inelastic. These uterine anomalies can be a reason for delayed natural conception, with mainly secondary infertility (unable to conceive or carry a fetus to term after giving birth previously). Patients with didelphys and unicornuate uterus (only half of the uterus is formed) have term delivery rates of approximately 45 percent, and the pregnancy outcome in most of the untreated cases has poor delivery rates of about 40 percent. However, studies have shown that the fertility of women with untreated uterine didelphys is better than that of those with other MDAs but still less than that of women with normal uterine anatomy.
What Is Uterine Didelphys With Obstructed Hemivagina and Ipsilateral Renal Agenesis?
Uterine didelphys with obstructed hemivagina (distended hemivagina) and ipsilateral renal agenesis (absence of kidney on the same side) is a rare congenital disorder of the female genital system. Obstructed hemivagina and ipsilateral renal agenesis, known as OHVIRA syndrome, is also referred to as Herlyn-Werner-Wunderlich syndrome (HWWS). Uterine didelphys with OHVIRA syndrome occur when the midline fusion of the Mullerian ducts is completely or partially arrested. Ipsilateral renal agenesis includes a partial vaginal septum on the same side and didelphys uterus that occurs due to abnormalities in the embryological development during the eighth week of gestation and affects the Mullerian ducts simultaneously. The exact prevalence of this condition is unknown as it is discovered in the later part of the patient’s life or case of infertility.
What Are the Features of Uterine Didelphys With OHVIRA Syndrome?
Women with uterine didelphys obstructed hemivagina and ipsilateral renal agenesis are mostly asymptomatic and unaware of having a double uterus. They may be diagnosed if they present with complaints of dyspareunia (pain during sexual intercourse) or dysmenorrhea (pain during menstruation). The severity of the symptoms mainly depends on the existence of uterine or vaginal communications. Routine imaging for infertility often helps in the detection of this anomaly. Uterine didelphys with OHVIRA syndrome has been associated with increased chances of infertility, intrauterine growth retardation, preterm labor, postpartum bleeding, or spontaneous abortion. Some women suffering from uterine didelphys with OHVIRA syndrome may present with the following symptoms:
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Frequent menstruation.
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Heavy bleeding.
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Foul-smelling vaginal discharge.
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Severe lower abdominal pain.
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Presence of a paravaginal (palpable) mass.
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Intermenstrual bleeding (vaginal bleeding between periods).
How Is Uterine Didelphys With OHVIRA Syndrome Diagnosed?
The role of imaging tests is to detect, diagnose, and differentiate mullerian malformations from their inoperable forms. An abdominal ultrasound or a transvaginal ultrasound can be inexpensive and noninvasive. A three-dimensional version has a higher sensitivity and specificity to diagnose these malformations. While a computed tomography (CT scan) has a limited role, an MRI can be highly sensitive and can visualize the vaginal septum. In uterine didelphys, a duplicated vagina is observed, and the pelvic ultrasonography demonstrates the individual horns, which are completely developed and normal in size, with two cervices.
Each hemiuteri is associated with a fallopian tube. The ovary may be malpositioned, and a longitudinal or transverse vaginal septum may also be noted. In most cases, pelvic magnetic resonance imaging (MRI) shows uterine didelphys with hemivagina and vaginal wall swelling with ipsilateral renal agenesis. In some cases, an intravenous urogram is recommended, demonstrating an incomplete kidney or any enlargement of the upper or lower pelvicalyceal systems.
How Is Uterine Didelphys With OHVIRA Syndrome Managed?
The rarity and unusual presentation of uterine didelphys with OHVIRA syndrome may contribute to a delay in diagnosis, especially if the patients are asymptomatic. Untreated cases may develop retrograde tubal reflux (backward flow of urine) and endometriosis (tissue similar to the uterine lining grows outside the uterus). Therefore, early diagnosis and treatment can be highly beneficial and prevent future complications such as infertility, chronic pain, and pelvic infections.
Uterine didelphys has the highest association of transverse vaginal septum, and a vertical fusion exists between the Mullerian ducts and the urogenital sinus. Resection (surgical removal) of the vaginal septum with drainage is mostly the treatment of choice for patients with obstructed hemivagina for providing symptomatic relief and preserving reproductive capabilities.
Hysteroscopic septum resection is a safe alternative to vaginoscopic resection and is carried out under transabdominal guidance to preserve the integrity of the hymen in young women. Other surgical procedures include single-stage vaginoplasty (draining the collected blood and resecting the septum) along with suturing the lateral vaginal wall, unilateral hysterectomy (removing one uterus), puncture of the vaginal wall, and mini-laparotomy (combined vaginal and laparoscopic techniques). Two-stage vaginoplasty is performed in some cases, which involves draining the hematocolpos (collected menstrual blood) in the first stage and resecting the septum in another surgery.
Conclusion
A didelphys uterus is a rare anomaly characterized by a failure in the fusion of the Mullerian ducts, which leads to two cervices and separate uterine cavities. Approximately 11 percent of uterine malformations are associated with the didelphic uteri. A high association exists between Mullerian duct abnormalities and renal anomalies such as unilateral agenesis. Most of the women with this condition are asymptomatic but complain of dysmenorrhea or dyspareunia, or patients primarily present with complaints of infertility and repeated pregnancy loss. An early diagnosis of the condition with appropriate surgical intervention can help reduce long-term morbidity or prevent infertility and other obstetric complications.
