Answer for CoW 09 October 2016
Uterus Didelphys
Findings
Complete duplication of uterine horns and duplication of cervix with widely divergent apices and intercornual distance – 6.9cm, with vaginal septum noted. Features suggestive of uterus didelphys.
Discussion
Uterus didelphys is a type of Müllerian duct anomaly (class III) where there is complete duplication of uterine horns as well as duplication of the cervix, with no communication between them. Clinical presentation Many patients are asymptomatic although some may occasionally experience dyspareunia as a result of the vaginal septum. Pathology It results from failed ductal fusion that occurs between the 12th and 16th week of pregnancy and is characterised by two symmetric, widely divergent uterine horns and two cervices. Associations renal agenesis vaginal septum which can include a transverse vaginal septum there is a vaginal septum in 75% of cases, and obstruction to one horn is possible from occasional transverse septae. Radiographic features Classically shows two widely spaced uterine corpora, each with a single Fallopian tube. Separate divergent uterine horns with large fundal cleft (as distinct from aseptate uterus) Uterus didelphys should be differentiated from a bicornuate uterus (separation of horns only) and a septate uterus (midline uterine septum). Hysterosalpingogram (HSG) HSG demonstrates two separate endocervical canals that open into separate fusiform endometrial cavities, with no communication between the two horns. Each endometrial cavity ends in a solitary fallopian tube. If the anomaly is associated with an obstructed longitudinal vaginal septum, only one cervical os may be depicted, and it may be cannulated with the endometrial configuration mimicking a unicornuate uterus. Pelvic ultrasound Separate divergent uterine horns are identified with a large fundal cleft. Endometrial cavities are uniformly separate, with no evidence of communication. Two separate cervices need to be documented. MRI MR imaging is considered the ideal imaging modality for evaluation of MDAs. MR imaging provides clear anatomic detail of both the internal uterine cavity and the external contour. Standard pelvic MR imaging protocols include axial T1-weighted and T2-weighted images (T2-weighted imaging is essential for evaluation of uterine anatomy). Contrast material is generally reserved for assessment of incidentally discovered additional disease. MR imaging demonstrates two separate uteri with widely divergent apices, two separate cervices, and usually an upper vaginal longitudinal septum. In each uterus, normal uterine zonal anatomy is preserved. For the purpose of MDA classification, oblique coronal T2-weighted images of the uterus are the most critical, since these are necessary for proper assessment of the uterine fundal contour. Newer 3D T2-weighted sequences provide submillimeter section thickness along with multiplanar reformatting capability. The advantage of multiplanar reformatting is that it significantly reduces imaging time (particularly important in pediatric and sedated or anesthetized patients) and avoids the need for exact prescription of the imaging plane, since this can be performed retrospectively at the workstation Complications infertility unilateral hydrocolpos/haematocolpos (if a vaginal septum is present) endometriosis.
Findings
Complete duplication of uterine horns and duplication of cervix with widely divergent apices and intercornual distance – 6.9cm, with vaginal septum noted. Features suggestive of uterus didelphys.
Discussion
Uterus didelphys is a type of Müllerian duct anomaly (class III) where there is complete duplication of uterine horns as well as duplication of the cervix, with no communication between them. Clinical presentation Many patients are asymptomatic although some may occasionally experience dyspareunia as a result of the vaginal septum. Pathology It results from failed ductal fusion that occurs between the 12th and 16th week of pregnancy and is characterised by two symmetric, widely divergent uterine horns and two cervices. Associations renal agenesis vaginal septum which can include a transverse vaginal septum there is a vaginal septum in 75% of cases, and obstruction to one horn is possible from occasional transverse septae. Radiographic features Classically shows two widely spaced uterine corpora, each with a single Fallopian tube. Separate divergent uterine horns with large fundal cleft (as distinct from aseptate uterus) Uterus didelphys should be differentiated from a bicornuate uterus (separation of horns only) and a septate uterus (midline uterine septum). Hysterosalpingogram (HSG) HSG demonstrates two separate endocervical canals that open into separate fusiform endometrial cavities, with no communication between the two horns. Each endometrial cavity ends in a solitary fallopian tube. If the anomaly is associated with an obstructed longitudinal vaginal septum, only one cervical os may be depicted, and it may be cannulated with the endometrial configuration mimicking a unicornuate uterus. Pelvic ultrasound Separate divergent uterine horns are identified with a large fundal cleft. Endometrial cavities are uniformly separate, with no evidence of communication. Two separate cervices need to be documented. MRI MR imaging is considered the ideal imaging modality for evaluation of MDAs. MR imaging provides clear anatomic detail of both the internal uterine cavity and the external contour. Standard pelvic MR imaging protocols include axial T1-weighted and T2-weighted images (T2-weighted imaging is essential for evaluation of uterine anatomy). Contrast material is generally reserved for assessment of incidentally discovered additional disease. MR imaging demonstrates two separate uteri with widely divergent apices, two separate cervices, and usually an upper vaginal longitudinal septum. In each uterus, normal uterine zonal anatomy is preserved. For the purpose of MDA classification, oblique coronal T2-weighted images of the uterus are the most critical, since these are necessary for proper assessment of the uterine fundal contour. Newer 3D T2-weighted sequences provide submillimeter section thickness along with multiplanar reformatting capability. The advantage of multiplanar reformatting is that it significantly reduces imaging time (particularly important in pediatric and sedated or anesthetized patients) and avoids the need for exact prescription of the imaging plane, since this can be performed retrospectively at the workstation Complications infertility unilateral hydrocolpos/haematocolpos (if a vaginal septum is present) endometriosis.
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!