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Case Reports
. 2015 May;9(5):TD06-8.
doi: 10.7860/JCDR/2015/11123.5891. Epub 2015 May 1.

Imaging Diagnosis of Herlyn-Werner-Wunderlich Syndrome- An Extremely Rare Urogenital Anomaly

Shibani Mehra  1 , Komal Chamaria  2 , U C Garga  3 , Ankur Kataria  2 , Ashim Ahuja  2
Affiliations
Case Reports

Imaging Diagnosis of Herlyn-Werner-Wunderlich Syndrome- An Extremely Rare Urogenital Anomaly

Shibani Mehra et al. J Clin Diagn Res. 2015 May.

Abstract

Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital anomaly of the urogenital tract resulting from maldevelopment of both Mullerian and Wolffian ducts. It is characterized by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. It generally presents at puberty shortly following menarche with the symptom of acute pelvic pain. Management of these cases is surgical and consists mainly of vaginoplasty with excision of the vaginal septum in order to release the obstruction and prevent the long term complication of recurrent pyocolpos and infertility. We report here a case of Herlyn-Werner-Wunderlich syndrome in a 13-year-old adolescent girl, emphasizing the role of imaging in the accurate and prompt diagnosis of this rare developmental urogenital anomaly. Only a few hundred such cases have been reported in literature till date.

Keywords: Hematometra; MRI; Obstructed hemivagina; USG; Uterus Didelphys; Vaginal atresia; Vaginal septum.

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Figures

[Table/Fig-1A,B]:
[Table/Fig-1A,B]:
Ultrasound of pelvis showing two uterine cavities in the pelvis. Right sided uterus with its cervix and vagina (white arrow in A) is normal in size, shape and echo texture with normal endometrial cavity. Left sided uterine cavity (yellow arrow in B) is grossly distended and filled with fluid containing fine internal echoes within
[Table/Fig-2]:
[Table/Fig-2]:
T2W TRUFI coronal MR image is showing absence of left kidney
[Table/Fig-3A-D]:
[Table/Fig-3A-D]:
T2W sagittal MR images of the pelvis [A and B] show two uteri with two separate cervices each with its hemi vagina; there is no communication between the duplicated genital structures at any level. Left sided hematometrocolpos is demonstrated (arrow in image A) indicating presence of vaginal septum. The axial MR images [C and D] show two uteri (Arrow in image c) and two hemivagina with left hematometrocolpos(Arrow in image d) due to presence of a septum in the left hemivagina
[Table/Fig-4A-C]:
[Table/Fig-4A-C]:
T2W Coronal, Sagittal MR images of pelvis demonstrating the septum as a hypo intense band in the mid vagina (arrow) causing obstruction and hematometrocolpos
See this image and copyright information in PMC

References

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