Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2011;5(1):1-8.
doi: 10.3941/jrcr.v5i1.572. Epub 2011 Jan 1.

Uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: common presentation of an unusual variation

Luther Adair 2nd  1 , Maria Georgiades, Rhonda Osborne, Tsz Ng
Affiliations
Case Reports

Uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: common presentation of an unusual variation

Luther Adair 2nd et al. J Radiol Case Rep. 2011.

Abstract

Mullerian duct abnormalities are congenital malformations that are easily missed and can lead to incorrect diagnosis and unnecessary operative procedures. In this case, a young female presented with cyclic pelvic pain that continued after previous surgical resection of an ovarian cyst. Further investigation with clinical examinations and multimodality imaging demonstrated ipsilateral renal agenesis and a Class III Mullerian duct anomaly (MDA) requiring a second operative procedure. It is believed that this case is a variant of the described obstructed hemi-vagina with ipsilateral renal agenesis (OVIRA) anomaly as pathologically there was ipsilateral renal agenesis and complete vaginal agenesis in our case. It is imperative to have a high clinical suspicion of mullerian duct abnormalities when encountering a patient with other urogenital anomalies. This will decrease the amount of misdiagnoses, guide appropriate surgical intervention, and decrease the risk of future reproductive complications.

Keywords: didelphys; ipsilateral renal agenesis; unilateral; uterine; vaginal agenesis.

PubMed Disclaimer

Figures

Figure 1
Figure 1
13 year old female with uterus didelphys, unilateral distal vaginal agenesis, and ipsilateral renal agenesis. Transabdominal ultrasound. Transverse sections demonstrated widely divergent uterine corpi, raising the index of suspicion for uterine duplication anomaly. Normal appearing uterus on the right (filled arrow). Distended endometrial cavity identified on the left (open arrow). Complex cyst superior the urinary bladder was initially presumed to be a complex left ovarian cyst (arrow head). (Protocol: 3.5 MHz curvilinear transducer (Phillips).
Figure 2
Figure 2
13 year old female with uterus didelphys, unilateral distal vaginal agenesis, and ipsilateral renal agenesis. Axial T2 noncontrast demonstrates a normal right uterine horn (long arrow). Distended lower uterine segment with blood products (short arrow). Distended tubular structure noted extending to the expected left adnexa superior to the distended left uterus, compatible with hematosalpinx (arrow head). (Protocol: 1.5 Tesla MRI (GE Signa Excite), TR/TE: 2800/80, 5mm slice thickness, non-contrast).
Figure 3
Figure 3
13 year old female with uterus didelphys, unilateral distal vaginal agenesis, and ipsilateral renal agenesis. Coronal long axis non-fat saturated FSE T2. After administration of sterile saline plus sterile ultrasound gel into the vaginal fornix via a small sterile catheter (arrowhead) high T2 signal extended from the vaginal fornix into a normal appearing cervix and uterine horn confirming patency on the right (filled arrow) and corresponding with a prior physical exam performed by the surgeon. Note blind-ending uterus (long arrow). Please note the lower uterine segment (open arrow). (Protocol: 1.5 Tesla MRI (GE Signa Excite), TR/TE: 4000/110, 5mm slice thickness, non-contrast).
Figure 4
Figure 4
13 year old female with uterus didelphys, unilateral distal vaginal agenesis, and ipsilateral renal agenesis. Coronal long axis non-fat saturated FSE T2. Left uterine horn and distended, blind-ending left lower uterine segment ended in intermediate signal tissue at the superior aspect of what appeared to be the vagina as noted on prior MRI (open arrow). Normal right uterine horn (filled arrow) and blind-ending uterus (long arrow). (Protocol: 1.5 Tesla MRI (GE Signa Excite), TR/TE: 4000/110, 5mm slice thickness, non-contrast).
Figure 5
Figure 5
13 year old female with uterus didelphys, unilateral distal vaginal agenesis, and ipsilateral renal agenesis. Pathological specimen. Left uterine horn (arrowhead) and distended, blind-ending left lower uterine segment (filled arrow). Hematosalpinx (open arrow).
Figure 6
Figure 6
13 year old female with uterus didelphys, unilateral distal vaginal agenesis, and ipsilateral renal agenesis. Uterine didelphys diagram. Normal right ovary, fallopian tube, uterus, cervix, and vagina. Left uerine horn with normal left ovary, left hematosalpinx, hematometra, and blind-ending left uterine segment (open arrow). Note there is no left vagina or exocervix.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Troiano RN, McCarthy SM. Müllerian duct anomalies: imaging and clinical issues. Radiology. 2004;233(1):19–34. - PubMed
    1. Beatriz LP, Junqueira BP, Allen LM, Spitzer RF, Lucco KL, Babyn PS, Doria AS. Müllerian Duct Anomalies and Mimics in Children and Adolescents: Correlative Intraoperative Assessment with Clinical Imaging. Radiographics. 2009;29:1085–1103. - PubMed
    1. Rock JA, Breech LL. Surgery for anomalies of mullerian ducts. In: Rock JA, Jones HW III, editors. TeLinde’s operative gynecology. 9th ed. New York, NY: Lippincott Williams & Wilkins; 2003. pp. 732–736.
    1. Gray SW, Skandalakis JE, Broecker BH. Female reproductive system. In: Skandalakis JE, Gray SW, editors. Embryology for surgeons. 2nd ed. Baltimore, Md: Lippincott Williams & Wilkins; 1994. pp. 816–847.
    1. Li S, Qayyum A, Coakley FV, Hricak H. Association of renal agenesis and mullerian duct anomalies. Journal of Computed Assisted Tomography. 2000;24( 6):829–834. - PubMed

Publication types

  NODES
admin 1
Association 1
Note 5
twitter 2