obstruierte Hemivagina und ipsilaterale renale Anomalie (OHVIRA) Syndrom

Herlyn-Werner-Wunderlich
syndrome - OHVIRA syndrome: a rare congenital anomaly. MRI, T2WI coronal image: didelphys uterus as two separate uterus and cervices (blue arrow) hematocolpos in one of the cervical cavities (yellow arrow) non visulaization of right kidney (red arrow)
www.eurorad.org • CC by-nc-sa-4.0
Herlyn-Werner-Wunderlich
syndrome • Herlyn-Werner-Wunderlich syndrome - Ganzer Fall bei Radiopaedia
Ibrahim H, Herlyn-Werner-Wunderlich syndrome. Case study, Radiopaedia.org (Accessed on 04 Jan 2023) https://doi.org/10.53347/rID-53476 • CC by-nc-sa 3.0 (modified)
Herlyn-Werner-Wunderlich
syndrome • Herlyn-Werner-Wunderlich syndrome - Ganzer Fall bei Radiopaedia
Hammad M, Herlyn-Werner-Wunderlich syndrome. Case study, Radiopaedia.org (Accessed on 04 Jan 2023) https://doi.org/10.53347/rID-47168 • CC by-nc-sa 3.0 (modified)
Herlyn-Werner-Wunderlich
syndrome • Uterus didelphys with hematometrocolpos - Ganzer Fall bei Radiopaedia
Abdrabou A, Uterus didelphys with hematometrocolpos. Case study, Radiopaedia.org (Accessed on 04 Jan 2023) https://doi.org/10.53347/rID-35795 • CC by-nc-sa 3.0 (modified)
Herlyn-Werner-Wunderlich
syndrome • Herlyn-Werner-Wunderlich syndrome - Ganzer Fall bei Radiopaedia
Albakheet S, Herlyn-Werner-Wunderlich syndrome. Case study, Radiopaedia.org (Accessed on 04 Jan 2023) https://doi.org/10.53347/rID-33378 • CC by-nc-sa 3.0 (modified)
Herlyn-Werner-Wunderlich
syndrome • Uterus didelphys with Herlyn-Werner-Wunderlich syndrome - Ganzer Fall bei Radiopaedia
Ibrahim D, Uterus didelphys with Herlyn-Werner-Wunderlich syndrome. Case study, Radiopaedia.org (Accessed on 04 Jan 2023) https://doi.org/10.53347/rID-27203 • CC by-nc-sa 3.0 (modified)
Herlyn-Werner-Wunderlich
syndrome. Absence of the right kidney.
www.eurorad.org • CC by-nc-sa-4.0
Herlyn-Werner-Wunderlich
syndrome - OHVIRA syndrome: a rare congenital anomaly. MRI, T2WI sagital image: hematocolpos in one of the cervical cavities (yellow arrow), urinary bladder (asterisk).
www.eurorad.org • CC by-nc-sa-4.0
Herlyn-Werner-Wunderlich
syndrome. Two separate uterine cavities with two separate cervices (uterus didelphys) and a voluminous haematometrocolpos. A large cystic mass adjacent to the right uterus is identified that corresponds to a haematosalpinx (arrow).
www.eurorad.org • CC by-nc-sa-4.0
Herlyn-Werner-Wunderlich
syndrome. Axial MRI images showed T1 hyperintense fluid collection (a) intermediate signal intensity on T2-weighted images (b): haematocolpos. The image corresponds to the one shown by the ultrasound examination (figure 1).
www.eurorad.org • CC by-nc-sa-4.0
Herlyn-Werner-Wunderlich
syndrome. Axial MRI images showed T1 hyperintense fluid collection (a) intermediate signal intensity on T2-weighted images (b): haematocolpos. The image corresponds to the one shown by the ultrasound examination (figure 1).
www.eurorad.org • CC by-nc-sa-4.0
Herlyn-Werner-Wunderlich
syndrome. Cystic pelvic lesion with fine internal echoes (arrow) measuring 60x85mm located posterior to the bladder.
www.eurorad.org • CC by-nc-sa-4.0
Herlyn-Werner-Wunderlich
syndrome - OHVIRA syndrome: a rare congenital anomaly. MRI, T1WI axial image: Bicornuate uterus (red arrow). Blood showing relatively heterogenously high signal in one of the cervical cavities (asterisk) in comparison to low fluid signal in urinary bladder (white arrow).
www.eurorad.org • CC by-nc-sa-4.0
Herlyn-Werner-Wunderlich
syndrome - OHVIRA syndrome: a rare congenital anomaly. MRI T2-FS coronal: non-fatty high T2 signal within the obstructed horns (blue arrow), haemtocolpos (yellow arrow), non-visualization of right kidney (red arrow).
www.eurorad.org • CC by-nc-sa-4.0
Herlyn-Werner-Wunderlich
syndrome • Herlyn-Werner-Wunderlich syndrome - Ganzer Fall bei Radiopaedia
Boddapati S, Herlyn-Werner-Wunderlich syndrome. Case study, Radiopaedia.org (Accessed on 04 Jan 2023) https://doi.org/10.53347/rID-80160 • CC by-nc-sa 3.0 (modified)
nicht verwechseln mit: Wunderlich-Syndrom
Radiopaedia • CC-by-nc-sa 3.0 • de

Herlyn-Werner-Wunderlich (HWW) syndrome, is a rare anomaly characterized by Müllerian duct anomalies (MDA) associated with mesonephric duct anomalies . This entity is also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA). It is not to be confused with the Wunderlich syndrome.

Clinical presentation

Presentation is typically after menarche with pelvic pain due to the obstructed hemivagina. There may be a palpable pelvic mass.

Pathology

Herlyn-Werner-Wunderlich (HWW) syndrome is characterized by a triad of type III Müllerian duct anomaly (uterus didelphys), obstructed hemivagina and mesonephric duct anomalies. Most often the latter manifests as renal agenesis  but duplicated kidneys, dysplastic kidneys, rectovesical bands or crossed fused ectopia may also be seen.

Radiographic features

Irrespective of imaging modality, the typical findings are:

Treatment and prognosis

Treatment is surgical, with resection of the septum dividing the two hemivaginas in order to relieve the obstruction. Subsequent successful pregnancy in the obstructed uterus has been reported .

Differential diagnosis

When uterovaginal obstruction results in significant dilatation, the imaging differential may include a large adnexal mass such as a cystadenoma or endometrioma.

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu obstruierte Hemivagina und ipsilaterale renale Anomalie (OHVIRA) Syndrom:
Uterus
didelphys
Wikipedia • CC BY-SA 4.0
Endometriose
 
Wikipedia • CC0
Fehlbildungen
der Gebärmutter
Wikipedia • CC BY-SA 4.0
einseitige
Nierenagenesie
Wikipedia • CC BY-SA 4.0
Anomalien des
urogenitalen Systems
Wikipedia • CC BY-SA 4.0
blinde
(verschlossene) Hemivagina
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