Abstract Obstructed hemivagina and ipsilateral renal anomaly OHVIRA , also known as Herlyn-Werner-Wunderlich syndrome, is a rare syndrome with only a few hundred reported cases described since Only a handful of these cases have been associated with pyocolpos. Symptoms usually present shortly after menarche when hematocolpos develops during menstruation resulting in dysmenorrhea and a pelvic mass. The pelvic mass is the collection of blood products within the obstructed hemivagina. The first study in the diagnostic work-up is usually ultrasonography, which typically demonstrates a pelvic fluid collection which can simulate other disease processes thus confounding the diagnosis.
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It generally occurs at puberty and exhibits non-specific and variable symptoms with acute or pelvic pain shortly following menarche, causing a delay in the diagnosis. Cases presentation two cases of HWW syndrome in adolescents and a differential diagnosis for one case of a different MDA, and the impact of magnetic resonance MR imaging technology to achieve the correct diagnosis.
Conclusions MR imaging is a very suitable diagnostic tool in order to perform the correct diagnosis of HWW syndrome. Peer Review reports Background The association of renal agenesis with ipsilateral blind hemivagina and didelphys uterus is reported as Herlyn-Werner-Wunderlich HWW syndrome; this triad was initially disclosed in an English report published in [ 1 ].
The exact cause, pathogenesis and embryologic origin of HWW syndrome are unclear and remain a subject of discussion [ 6 ]. HWW syndrome is usually discovered at puberty with non-specific symptoms, like increasing pelvic pain, dysmenorrhea and palpable mass due to the associated haematocolpos or hematometra, which result from retained, longstanding menstrual flow in the obstrucucted vagina.
It rarely occurs with primary infertility in early adulthood when the vaginal septum is incomplete [ 7 ]. It is really difficult to achieve to an accurate diagnosis because menstruation is often regular and when patient complains symptoms of cyclic dysmenorrhea, they are usually given anti-inflammatory drugs and oral-contraceptives, thus causing a delay in the diagnosis because they reduce or eliminate menses; ultimately, HWW is an uncommon syndrome, not often thought of as a diagnostic possibility [ 8 ].
The potential complications of this syndrome are distinct in acute complications, such as pyohematocolpos, pyosalpinx, or pelviperitonitis, and long-term complications, such as endometriosis, pelvic adhesions and increased risk of abortion or infertility [ 9 , 10 ].
Table 2 Hww syndrome: mr findings Case presentation Patient 1 A years-old female presented with a history of severe abdomino-pelvic pain, which increased lasting 2 to 7 days with each of her menstrual cycles, hindering her daily activities.
Gynecologic history indicated menarche at 13 years of age, followed by 3 years of irregular menses. The patient denied any recent abdominal trauma, abnormal vaginal bleeding, nausea, vomiting or diarrhea. A bimanual physical examination indicated a right-sided cystic and tender pelvic mass, movable, mildly tender to palpation. The medical staff recommended further evaluation such as US, which showed the absence of right kidney and the possibility of uterine anomaly; so Magnetic Resonance Imaging MRI was performed in order to evaluate the possible genito-urinary anomaly.
The MRI examination was performed on a 1. A contrast agent was not used as it was not considered necessary. Figure 1 Herlyn-Werner-Wunderlich syndrome C Drawing illustrates the triad renal agenesis, didelphys uterus, and obstructed hemivagina and in this case the presence of an endometriosic cyst.
A Axial turbo spin-echo T2 weighted B Axial turbo spin-echo T1 fat-saturated weighted MR image showing centrally a hematocolpos asterisk , a finding corresponding to the obstructed right hemivagina. Mild dilation of the right endometrial cavity curved arrow with fluid exhibiting a signal intensity similar to methaemoglobin due to haematometra. Full size image In the right ovary an expanding mass of 31 mm was hyperintense on T1-weighted images and mildly hypointense or hyperintense on T2-weighted images Figure 2A.
The mass was characterized by a typical "shading" sign Figure 2B and exhibited features compatible with an endometriosic cyst; there was mild fluid in the pouch of Douglas. B Sagittal T1 TSE fat-suppressed weighted image showing distention of the right endometrial cavity long straight arrow with high-signal-intensity material distending the right endometrial cavity and cervix asterisk , a finding indicative of right hematometrocolpos; on the right ovary presence of an endometriosic cysts appearing hyperintense on T1 fat-saturated image short straight arrow.
There was no evidence of an expansive process in the left ovary. The extension of examination to the upper abdomen revealed right renal agenesis and apparent compensatory hypertrophy of the left kidney Figure 3.
B Coronal T2 Trufi MR image showing the omolateral renal aplasia with respect to the pelvic abnormalities and a solitary left kidney with a mild hypertrophy. Full size image Patient 2 A years-old woman presented with severe abdominal pain that occurred since the patient achieved menarche at 14 years of age; the patient also had irregular menstruation with severe dysmenorrhea and she reported that the pain worsened immediately prior to menstruation and improved with menstrual flow.
Upon physical examination, the findings were non-specific, with the exception of lower abdominal tenderness, arising from the pelvis, mildly tender to palpation.
A gynecologic examination did not reveal any anomalies of her external genitalia or hymen. So, MRI was then performed, using the same sequences protocol employed for Patient 1. MRI indicated a uterine-vaginal malformation consisting of didelphys uterus communicating with a double vagina, of which the right vagina was obstructed Figure 4. A Axial turbo spin-echo T2 weighted MR image B Axial turbo spin-echo T1 fat-saturated weighted MR image showing centrally a wide hematocolpos asterisk , corresponding to the obstructed right hemivagina.
On the left side the uterus normally communicating with non-obstructed hemivagina. Full size image Figure 5 A Sagittal T2-weighted MR image demonstrates an enormously dilated hemivagina asterisk due to the presence of the transverse septum causing the obstruction and low T2-signal-intensity contents corresponding to a high T1-signal intensity; B consistent with blood product due to the presence of haemoglobin. There was no evidence of an expansive process in the ovaries, but the extension of the examination to the upper abdomen showed right renal agenesis and apparent compensatory hypertrophy of the left kidney Figure 6.
Figure 6 A Coronal T2 weighted images show the wide hematocolpos asterisk. B Trufi coronal image demonstrates the absence of right kidney, omolateral to the pelvic alteration, and a mild compensatory hypertrophy of the left kidney. Full size image Patient 3: an example of differential diagnosis A years-old female presented with severe abdominal pain. She reported that the pain had begun a few months earlier and had subsequently worsened over the past several weeks.
The pain worsened immediately prior to menstruation and then improved once menstrual flow began. The patient also reported worsening constipation and denied problems with urination, nausea, vomiting or diarrhea. The patient underwent menarche 2 months prior with menses exhibiting 3 to 4 days of very light flow.
Physical examination did not reveal any alteration of her external genitalia. A previous sonography revealed a solitary left kidney as well as probable unicornuate uterus.
MRI examination confirmed an unicornuate uterus with a left single horn open into a normal vagina and a controlateral non-communicating rudimentary horn that contained material with a signal intensity similar to methaemoglobin Figure 7. B Axial fat-saturated T1-weighted images. C Drawing illustrates a year-old girl with an unicornuate uterus and rudimentary right horn with an endometrial cavity that does not communicate with the uterus class II MDA type and shows an obstructed functional right uterine horn long straight arrow containing blood degradation products and a corresponding high T1fs-signal intensity, exhibiting no communication with the cavity of the normal left uterine horn short straight arrow.
Full size image The ipsilateral Fallopian tube was characterized by fluid sero-mucinous distension Figure 8. In the right paramedian side, at the level of the Douglas, posterior to the ideal point of continuity between uterus and rudimentary horn, there were some adjacent nodules most likely of endometrial etiology 14 mm, 38 mm in total Figure 9.
In addition, a moderate rate of pelvic fluid was present. Figure 8 A Sagittal T2-weighted MR image shows a normal left uterine cavity communicating with a normal cervix long straight arrow , which in turn communicates with a normal vagina. B Right hematosalpinx asterisk with a distended non-communicating horn filled with fluid moderately hyperintense on T2 sequences due to haemoglobin degradation products.
Herlyn–Werner–Wunderlich syndrome: a rare cause of pelvic pain in adolescent girls
Gartner duct cysts 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 5. Differential diagnosis When uterovaginal obstruction results in significant dilatation, the imaging differential may include a large adnexal mass such as a cystadenoma or endometrioma. References 1. Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatr Radiol.
Herlyn-Werner-Wunderlich syndrome: a rare presentation with pyocolpos
It generally occurs at puberty and exhibits non-specific and variable symptoms with acute or pelvic pain shortly following menarche, causing a delay in the diagnosis. Cases presentation two cases of HWW syndrome in adolescents and a differential diagnosis for one case of a different MDA, and the impact of magnetic resonance MR imaging technology to achieve the correct diagnosis. Conclusions MR imaging is a very suitable diagnostic tool in order to perform the correct diagnosis of HWW syndrome. Peer Review reports Background The association of renal agenesis with ipsilateral blind hemivagina and didelphys uterus is reported as Herlyn-Werner-Wunderlich HWW syndrome; this triad was initially disclosed in an English report published in [ 1 ]. The exact cause, pathogenesis and embryologic origin of HWW syndrome are unclear and remain a subject of discussion [ 6 ]. HWW syndrome is usually discovered at puberty with non-specific symptoms, like increasing pelvic pain, dysmenorrhea and palpable mass due to the associated haematocolpos or hematometra, which result from retained, longstanding menstrual flow in the obstrucucted vagina.