Uterus didelphys
| Uterus didelphys | |
|---|---|
 | |
| Ultrasound showing didelphys | |
| Classification and external resources | |
| Specialty | medical genetics |
| ICD-10 | Q51.1 |
| ICD-9-CM | 752.2 |
Uterus didelphys (sometimes also uterus didelphis) represents a uterine malformation where the uterus is present as a paired organ when the embryogenetic fusion of the Müllerian ducts fails to occur. As a result, there is a double uterus with two separate cervices, and rarely a double vagina as well. Each uterus has a single horn linked to the ipsilateral fallopian tube that faces its ovary.
In non human species (e.g. nematodes), a didelphic genital tract may be normal rather than a malformation. Such species are described as didelphic, as opposed to monodelphic, with a single tract.
Etiology
The uterus is formed during embryogenesis by the fusion of the two paramesonephric ducts (also called Müllerian ducts). This process usually fuses the two Müllerian ducts into a single uterine body but fails to take place in these affected women who maintain their double Müllerian systems. A didelphic uterus will have a double cervix and is usually associated with a double vagina. The cause of the fusion failure is not known. Associated defects may affect the vagina, the renal system and, less commonly, the skeleton.
The condition is less common than these other uterine malformations: arcuate uterus, septate uterus, and bicornuate uterus. It has been estimated to occur in 1/3,000 women.[1]
Presentation
Women with the condition may be asymptomatic and unaware of having a double uterus. However, a study by Heinonen showed that certain conditions are more common. In his study of 26 women with a double uterus gynecological complaints included dysmenorrhea and dyspareunia. All patients displayed a double vagina. The fetal survival rate in 18 patients who delivered was 67.5%. Breech presentation was present in 43% and premature delivery common (21%).[2]
Diagnosis
A pelvic examination will typically reveal a double vagina and a double cervix. Investigations are usually prompted on the basis of such findings as well as when reproductive problems are encountered. Not all cases of uterus didelphys involve duplication of the cervix and vagina.
Helpful techniques to investigate the uterine structure are transvaginal ultrasonography and sonohysterography, hysterosalpingography, MRI, and hysteroscopy. More recently 3-D ultrasonography has been advocated as an excellent non-invasive method to evaluate uterine malformations.[3]
Uterus didelphys is often confused with a complete uterine septum. Often more than one method of investigation is necessary to accurately diagnose the condition. Correct diagnosis is crucial as treatment for these two conditions is very different.[4] Whereas most doctors recommend removal of a uterine septum, they generally concur that it is better not to operate on a uterus didelphys. In either case, a highly qualified reproductive endocrinologist should be consulted.
Frequency
In the United States, uterus didelphys is reported to occur in 0.1–0.5% of women. It is difficult to know the exact occurrence of this anomaly, as it may go undetected in the absence of medical and reproductive complications.
Management
Patients with a double uterus may need special attention during pregnancy as premature birth and malpresentation are common. Cesarean section was performed in 82% of patients reported by Heinonen.[2]
Uterus didelphys, in certain studies, has also been found associated with higher rate of infertility, miscarriage, intrauterine growth retardation, and postpartum bleed.[4]
Syndrome
A specific association of uterus didelphys (double uterus), unilateral hematocolpos (inadequate draining of menstrual blood) and ipsilateral renal agenesis (having only one kidney) has been described.[5]
Multiple pregnancy
A number of twin gestations have occurred where each uterus carried its pregnancy separately. A recent example occurred on February 26, 2009, when Sarah Reinfelder of Sault Ste. Marie, Michigan delivered two healthy, although seven weeks premature, infants by cesarean section at Marquette General Hospital.[6] It is possible that the deliveries occur at different times, thus the delivery interval could be days or even weeks.
Triplets
A UK woman with a double uterus gave birth to triplets in 2006. Hannah Kersey, of Northam in Devon, gave birth to a pair of identical twins from an egg that implanted into one womb and then divided, and to an infant from a single egg that implanted into the other womb. This was the first known birth of viable triplets in a woman with a double uterus.[7] It is estimated that the possibility of such a birth is about 1 in 25 million.[8] A triplet pregnancy in a woman with uterus didelphys was reported from Israel in 1981; one baby died in utero, and of the remaining babies, one was delivered at 27 weeks gestation and the other 72 days later.[9]
Popular culture
The film Dead Ringers depicts a woman with a trifurcate uterus.
See also
References
- ↑ Grimbizis, G. F.; Camus, M; Tarlatzis, BC; Bontis, JN; Devroey, P (2001). "Clinical implications of uterine malformations and hysteroscopic treatment results". Human Reproduction Update. 7 (2): 161–74. doi:10.1093/humupd/7.2.161. PMID 11284660.
- 1 2 Heinonen, P.K. (1984). "Uterus didelphys: a report of 26 cases". European Journal of Obstetrics & Gynecology and Reproductive Biology. 17 (5): 345–50. doi:10.1016/0028-2243(84)90113-8. PMID 6479426.
- ↑ Woelfer, B; Salim, R; Banerjee, S; Elson, J; Regan, L; Jurkovic, D (2001). "Reproductive outcomes in women with congenital uterine anomalies detected by three-dimensional ultrasound screening". Obstetrics & Gynecology. 98 (6): 1099–103. doi:10.1016/S0029-7844(01)01599-X. PMID 11755560.
- 1 2 Pui, M (2004). "Imaging diagnosis of congenital uterine malformation". Computerized Medical Imaging and Graphics. 28 (7): 425–33. doi:10.1016/j.compmedimag.2004.05.008. PMID 15464882.
- ↑ Madureira, A. J.; Mariz, C. M.; Bernardes, J. C.; Ramos, I. M. (2006). "Case 94: Uterus Didelphys with Obstructing Hemivaginal Septum and Ipsilateral Renal Agenesis". Radiology. 239 (2): 602–6. doi:10.1148/radiol.2392031187. PMID 16641359.
- ↑ "Associated Press "Mich. woman with 2 wombs delivers twin daughters"".
- ↑ "Triplets for woman with two wombs". BBC News. December 21, 2006. Retrieved 2007-06-27.
- ↑ "Howstuffworks "13 People with Extra Body Parts"".
- ↑ Mashiach, S; Ben-Rafael, Z; Dor, J; Serr, DM (1981). "Triplet pregnancy in uterus didelphys with delivery interval of 72 days". Obstetrics and gynecology. 58 (4): 519–21. PMID 7279347.