Summary

Duplicated or double uterus associated with double cervices and double vaginae and orifices is a very rare anomaly. We report a case of an adult female with incidental finding of double vaginal openings, double cervices and a septate uterus. The pathophysiology and associated complications of this rare condition was discussed. Doctors are reminded to stay vigilant and to pay careful attention to details even when doing the most routine and unsuspecting task.

摘要

雙子宮兼雙子宮頸和雙陰道是非常罕見的缺陷。本文記述一位意外被發現同時擁有雙陰道口、雙子宮頸和子宮間隔的成年女姓病例，討論有關病理生理和併發症。醫生即使在處理最常規和無可疑的工作時，都應當注意細節並且保持警覺性。

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Introduction

Maldevelopment of the female reproductive ducts occur in up to 2% to 3% of women.¹ The clinical picture can range from completely asymptomatic to presentation with acute and severe symptoms. This paper describes a rare case of double uteri, double cervices and double vaginae picked up in adulthood, after numerous previous gynaecological examinations of apparently normal findings, and the implications to the patient and doctors at large.

Case report

The patient is a 28-year-old woman with good past health. After menarche at the age of 13, she had regular monthly menstruation with normal flow. There was no menstrual pain. She has been sexually active and there was no sexual dysfunction. She had regular gynaecological examinations and cervical smears done by various gynaecologists since 1999 and was told to have no abnormality. In 2001, after a routine gynaecological exam she was told that she had slightly enlarged ovaries. She was referred for abdominal ultrasonography, which reported normal findings. Patient sought a second opinion from another gynaecologist later with gynaecological examination and ultrasonography repeated. She was told that she had polycystic ovaries and a bicornuate uterus. In 2003, she took out a routine body-check programme in a private hospital, which included gynaecological examination and cervical smear, and was told that the results were normal. She had another gynaecological examination a year later, which again was said to be normal. In 2005 she again booked for a routine gynaecological check-up. This time the examining doctor told her that two cervices were found. She presented to our clinic for a second opinion and advice on subsequent actions needed.

Physical examination revealed a generally healthy female. On inspection of the vulva and perineum there were two vaginal openings lying parallel to each other. Digital examinations revealed two vaginae separated by a thick septum, and two cervices were felt. Speculum examinations revealed two normal looking cervices. The uterus was anteverted, normal sized and smooth in contour. No pelvic mass was felt and no tenderness was elicited. The physical findings were explained to the patient. Ultrasonography was arranged in order to assess the structure of the uterus and to rule out other associated malformations in the urogenital tract.

Abdominal and transvaginal ultrasonography was performed which showed a completely septate uterus with no definite fundal cleft. The ovaries were unremarkable. The kidneys were normal.

During consultation the patient revealed a desire to get pregnant and that she had tried unprotected sex for several years without any conception. In view of the structural abnormality of her reproductive tract, which may adversely affect her fertility, she was referred to a gynaecologist for expert opinion. Hysteroscopy was performed, which revealed a double uterus and vagina and double cervices. She was given clomid for ovulation induction for her infertility.

Literature review

Under the influence of maternal oestrogens, the mullerian or paramesonephric ducts of the genetically female embryo form the fallopian tubes and subsequently fuse together on the median septum to create the uterus, cervix and the upper part of the vagina. The lower part of the vagina is formed by a protrusion of the urogenital sinus, the vaginal sinus, which develops progressively to form the vaginal fornices. This process begins from the tenth to 17^th week of pregnancy and is completed just prior to term¹. In case of non-development or non-fusion (partial or complete) of the mullerian ducts, a variety of anomalies can result, ranging from complete agenesis to doubling of the female reproductive organs. These anomalies have been classified into: agenesis/hypoplasia, unicornuate uterus, didelphic uterus, bicornuate uterus, septate or arcuate uterus.² (Figure 1) Double cervices and vaginae with a septate uterus is an uncommon mullerian malformation. An advanced Medline search showed that this is an uncommonly reported anomaly.³ Mullerian duct anomalies may be accompanied by anomalies in other systems in the body, particularly in the urinary tract, such as renal agenesis and a double collecting systems (renal basin and ureters). Muscular and skeletal system, collagen-vascular diseases, digestive tract (chiefly imperforate anus), cardiac anomalies and anomalies of the eyes and ears are other rarer associations. The direct cause of the congenital anomalies of the female genital tract is not known, but it is believed that multifactorial, polygenic, and familial factors could play a part. Some of these factors include the exposure of the pregnant woman to doses of ionizing radiation, intrauterine infections, and drugs such as thalidomide and diethylstilbestrol.⁴

Women with congenital anomalies of the genital tract may be asymptomatic, but generally they can suffer a wide range of symptoms, which can manifest themselves at any of the various stages of life, from childhood to senescence; before pregnancy, during pregnancy, and after. Primary amenorrhoea and chronic pelvic pain are common. Longitudinal vaginal septa can be found alone or in combination with other anomalies, chiefly uterus didelphys, in up to 57% of cases. In such cases, pain during sexual intercourse is common.

Approximately 25% of women with mullerian anomalies have fertility and obstetric problems as compared with 10% of the normal healthy population⁵. There is generally no problem in conceiving but rather in maintaining a normal pregnancy to its completion. Still, the incidence of unexplained infertility among these cases could be as high as 35%. Spontaneous abortion in the first and second trimester of pregnancy, abnormal foetal presentation, premature birth, dystocia, pathologic conclusions of pregnancy, stillbirth, and ectopic pregnancy are among the many problems that appeared during pregnancy and labour. There is the possibility of simultaneous pregnancy in both uteri or in one uterus even after the excision of the other one for whatever medical reason.

The means of diagnosis encompass a wide variety of possibilities, including simple vaginal palpation, hysterosalpingography (HSG), laparoscopy, hysteroscopy, and ultrasound.¹ Recent studies also show that MRI is a valuable tool in diagnosing mullerian duct anomaly subtypes, so as to spare patients mutilating surgery and to prevent pregnancy-associated complications.⁶

The therapeutic approach in mullerian duct anomalies is specific to the type of anomaly, and includes non-treatment, medical therapy, and surgical treatment. It is important to carry out comprehensive examinations and to be certain that the fertility and obstetric problems stem purely from uterine causes before taking surgical steps to correct those problems.

Discussion

Congenital anomaly of the female genital tract is rare. Indeed, from the Medline search that we conducted, there were less than a handful of cases reported in the medical literature. Therefore, it is worth reporting when we come across one such case. Moreover, our case serves as a reminder to all doctors of the importance of a basic physical examination skill - careful inspection. Gynaecological examination is part and partial of most doctors' everyday work. As with all other clinical examinations, a careful and focused approach is needed when we examine the female genital tract. Our case is a good illustration of how a simple but careful inspection of the female genital tract could lead to discovery of a rare anomaly, which was missed repeatedly even by experienced gynaecologists. Although congenital duct anomalies are rare, the failure to recognize one can have potential consequences. Take this case for example. Since the patient has two cervices and she is sexually active, if we fail to notice the anomaly and just perform cervical smear on one cervix only, which was what happened in the past few years, we could miss early pathological changes in the other cervix. And the patient might be falsely reassured. The patient might return years later with some really serious pathology, such as carcinoma of the cervix. Subfertility is a problem faced by many such patients, an early diagnosis of the congenital anomaly can alert the doctor and patient to this associated possibility and to take action accordingly.

Conclusion

We describe a patient with the rare condition of uterus didelphys, double cervices and double vaginae and review the knowledge we have thus far of this condition. Our case report highlights the importance of careful physical examination in the clinician's everyday practice: not just with patients presenting with symptoms, but also with patients who come in for a "mere routine check".

Key messages

1. Uterus didelphys is a rare congenital mullerian duct anomaly in females.
2. Uterus didelphys is associated with fertility and obstetric problems, and anomalies in other systems of the body.
3. Doctors are reminded that careful inspection is a basic but important examination skill.

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References