Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), also known as Herlyn-Werner-Wunderlich syndrome, is a rare syndrome with. Herlyn-Werner-Wunderlich (HWW) syndrome, is a rare anomaly characterised by Müllerian duct anomalies (MDA) associated with mesonephric duct anomalies. Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital anomaly of the urogenital tract involving Müllerian ducts and Wolffian.
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Hence, it should be obtained before any surgical intervention. The pelvic mass is the collection of blood products within the obstructed hemivagina. Clinical characteristics of 70 patients with Herlyn-Werner-Wunderlich syndrome.
The Herlyn-Werner-Wunderlich Syndrome – A Case Report with Radiological Review
Open in a separate window. A pelvic ultrasound was performed revealing a 7 cm fluid collection with diffuse low level syndorme echoes, which appeared contiguous with the endocervix figure 2.
A high index of suspicion in patients with renal anomalies and endometriosis is needed to detect the Herlyn-Werner-Wunderlich syndrome for timely diagnosis to avoid complications from the syndrome. The ureteric bud wynderlich dorsocranially into the metanephric blastema inducing differentiation of the metanephric nephrons.
Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys: Consulting a specialist in this case a gynecologist is recommended. Copyright Journal of Radiology Case Reports. Classification 1, a completely obstructed hemivagina and Classification 2, an incompletely obstructed hemivagina. Fluid density pelvic mass, kidneys are present. Author information Copyright and License information Disclaimer.
However, she reported an ultrasound performed in a small military medical clinic in Samoa, prior to her arrival, revealed absence of her left kidney. J Pediatr Adolesc Gynecol ; HWWS patients should be differentiated according to these two classifications.
Expectant mothers are often manages with cervical sutures and C-sections to wunderpich fetal distress during labour. Systematic analysis of the literature suggests management of these cases to consist mainly of vaginoplasty with excision of the vaginal septum in order to prevent the long term complication of recurrent wunderich and infertility from chronic cryptomenorrhoea.
Imaging of the abdomen reveals agenesis of the ipsilateral kidney. Patients often have ascending genital system infection [ Figure 3 ].
Rare disease: Herlyn–Werner–Wunderlich syndrome: a rare cause of pelvic pain in adolescent girls
The right side is affected twice more frequently than the left side [ 56 ]. Yuan-yuan Ji Source of Support: The left uterine cavity and cervical canal showed a hypoechoic collection with multiple internal echoes suggestive heelyn haematometra and haematotrachelos Figure 1C, 1D. Fluid density mass in the pelvis with absent ipsilateral kidney. This new classification allows for earlier diagnosis and treatment.
Symptoms begin to develop in menarche, and complications related to a retrograde menstrual flow arise, along with pelvic adhesions and endometriosis. Embryological development, classification, and MRI assessment.
Herlyn–Werner–Wunderlich syndrome: a rare cause of pelvic pain in adolescent girls
J Pediatr Surg ; In conclusion, the prognosis of HWWS is good with early diagnosis and early treatment, except for patients with Classification 1. Tubular low T1, high T2 signal pelvic mass. Prognosis Excellent with generally no effect on reproduction after treatment. It could be argued that surgical excision might be better as there have been reported cases of malignant transformation occurring in the ectopic ureter [ 3 ].
Support Radiopaedia and see fewer ads. Didelphic uterus with left hemivaginal obstruction LVhydrometrocolpos of the left uterine horn LU and ipsilateral renal agensis. The authors report a case of a girl with this condition who had a prenatal diagnose of right renal agenesis and presented at 13 years old with pelvic pain caused by haematocolpos.
The right uterine horn cavity is seen to communicate with the upper end of the fluid collection in wunderlihc hemivagina small black wunverlich.
The clinical details associated with these two types are distinctly different. Furthermore, MRI is superior to ultrasound by allowing for better characterization of anatomic relationships given its multiplanar capabilities and wider field of view, important for surgical planning. Turk Ger Gynecol Assoc. However, the distal part of the vagina, originating from the urogenital sinus is not affected [ 4 ]. MRI findings of the pelvis reveal a didelphic uterus. Unusual form of genital malformation with aplasia of the right kidney.
Herlyn-Werner-Wunderlich syndrome: a rare presentation with pyocolpos
These treatments inadvertently cause a delay in diagnosis as they diminish or eliminate menses. Full resection of the vaginal septum resulted in good outcomes and fertility. The Herlyn—Werner—Wunderlich HWW syndrome is a rare variant of mullerian duct anomalies characterised by the combination of uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis.
A well-defined elongated retort-shaped tubular anechoic cystic lesion measuring approx. If not treated in time, the condition wuneerlich progress to secondary endometriosis, pelvic adhesion, pyosalpinx, and even pyocolpos [ Figure 1 ]. How to cite this URL: Abstract Obstructed hemivagina and ipsilateral synvrome anomaly OHVIRAalso known as Herlyn-Werner-Wunderlich syndrome, is a rare syndrome with only a few hundred reported cases described since Most patients with Classification 2 have normal menstrual cycle, but longer menstrual periods, illness attacks years wundrlich menarche.
The left uterus was enlarged due to fluid collection in the uterine cavity and cervical canal measuring 8. Clinical suspicion and awareness of the syndrome are therefore imperative to making a timely diagnosis and preventing these complications.
In patients with uterine and vaginal abnormalities, a work-up for associated renal anomalies should be performed.