Herlyn-Werner-Wunderlich Syndrome: a pictorial review.
Number:27ESURABS0017
Type:Educational Electronic Presentation
Authors: Lorenzo Vassallo, Mirella Fasciano
Keywords:Herlyn-Werner-Wunderlich - MRI - Urogenital congenital anomalies - Mullerian ducts
SECTIONS
Educational objective

To understand the epidemiology and pathology of Herlyn-Werner-Wunderlich (HWW) syndrome and the contribution of different imaging methods to the diagnosis.


Topic Review

The HWW syndrome is a rare congenital anomaly of the female urogenital tract that associates Mullerian and Wolffian duct anomalies (MDA). The syndrome is characterized by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis.
With normal external genitalia, diagnosis is often delayed until after menarche with most affected patients presenting with dysmenorrhea, increasing pelvic pain and a palpable mass secondary to the associated hematocolpos or hematometra.
The diagnosis and treatment at an early stage can relieve symptoms, prevent acute and long-term complications and preserve fertility.

An early detection and treatment of HWW syndrome shortly after menarche would be desirable in order to relieve acute symptoms, prevent the occurrence of several medical complications and preserve normal fertility. However, the correct diagnosis can be...

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Figure 1. Sovrapubic US demonstrates a pelvic dilated round cavity with homogeneous, hypoechogenic content compatible with hematocolpos.
Figure 2. Axial T2-weighted image shows the enormously dilated left hemivagina.


Conclusions

The syndrome of HWW is a rare cause of abdominal pain in adolescents characterized by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis.

Early detection and treatment is crucial to relieve acute symptoms, preserve normal fertility and prevent acute and long-term complications.

Despite the correct diagnosis can be difficult due to the absence of specific findings upon physical examination and to non-specific symptoms it should be considered in the differential diagnosis in pediatric female patients presenting with progressive lower abdominal pain and dysmenorrhea after menarche.

MRI is the most powerful tool when compared to other imaging modalities, in establishing the diagnosis of MDA and associated conditions and must be performed in young females suspected to have HWW syndrome in order to make a correct and detailed...

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