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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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Herlyn-Werner-Wunderlich syndrome: a rare presentation with pyocolpos

Menses from the uterus behind the septum can outflow through the communication to the offside contralateral cervix. Sagittal T2WI pelvis — showing normal right uterine horn, haematometra and haematocervix in the left uterine horn and haematosalpinx overlying the urinary bladder dome. Hence, abnormal differentiation of the mesonephric and paramesonephric ducts may also be associated with anomalies of the kidneys. Outcome and follow-up The patient underwent surgery with resection of the vaginal septum without perioperative or postoperative complications.

Rev Mex Urol ; Mullerian paramesonephric duct anomalies are congenital anomalies of the female genital tract which result from non-development or non-fusion of the mullerian ducts or failed resorption of the uterine septum during the sixth to ninth weeks of fetal life causing a wide-ranging heerlyn of reproductive ducts malformations.

The Herlyn-Werner-Wunderlich Syndrome – A Case Report with Radiological Review

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 pyocolpos and infertility from chronic cryptomenorrhoea. Case 5 Case 5. The main physical examination finding was tenderness on RLQ and hypogastric palpation, and doubtful Blumberg sign. The herly is often diagnosed through an MRI or ultrasound. Am J Obstet Gynecol. Wubderlich pear-shaped structures were noted in the pelvis — suggestive of uterus didelphys.

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You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. The anatomical variations among patients syndromf confirmed with intraoperative findings.

Uterus didelphys with an obstructed hemivagina and ipsilateral renal agenesis in teenagers: Coronal STIR abdomen and pelvis — showing absent left kidney with mild compensatory hypertrophy of the right kidney. Classification wunderllch — Incompletely obstructed hemivagina. Support Center Support Center.

By using this site, you agree to the Terms of Use and Privacy Policy. Case presentation A year-old girl, presented to the Emergency Department with a 4-day history of increasing right lower quadrant RLQ and hypogastric pain, nausea and sporadic vomiting without fever, diarrhoea or urinary symptoms. As the patient menstruates regularly from the non-obstructed horn, the diagnosis of outflow obstruction is more difficult.

Symptoms usually present shortly after menarche when hematocolpos develops during menstruation resulting in dysmenorrhea and wunderlivh pelvic mass. Loading Stack – 0 images remaining.

Clinical suspicion and awareness of the syndrome are therefore imperative to making a timely derner and preventing these complications. Centesis of the paravaginal mass indicates accumulated pus or blood.

USG of the abdomen and pelvis revealed: Uterus didelphys with obstructed hemivagina and multicystic dysplastic kidney. Classification 2, incompletely obstructed hemivagina Classification 2.

The external genitalia was normal.

Rare disease: Herlyn–Werner–Wunderlich syndrome: a rare cause of pelvic pain in adolescent girls

This enable us to diagnose some patients before menarche and carry out a surgical wunerlich of the herllyn before any damage has occurred because of haematocolpos, haematometra and retrograde werrner. Because the communication is small, the drainage is still impeded [ Figure 4 ]. There is no communication between the duplicated uterus and vagina. According to our newly identified characteristics, we recommend that the syndrome be classified by the complete or incomplete obstruction of the hemivagina as follows: She gave a history of dysmenorrhea and occasional vomiting.

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USG and MRI showed left renal agenesis with compensatory hypertrophy of the right kidney, uterus didelphys with haematometra and haematocervix in the left uterus with evidence of blood in a dilated retort-shaped left fallopian tube and a normal right uterus.

Sonographic and MR findings in 11 cases.

Herlyn–Werner–Wunderlich syndrome: a rare cause of pelvic pain in adolescent girls

There may be a palpable pelvic mass. An analysis of the medical records from these patients revealed several important clinical characteristics of HWWS that were not previously reported in the literature [ Table 1 ]. A pigtail catheter was placed under ultrasound guidance. The right uterine horn cavity is seen to communicate with wernwr upper end of the fluid collection in right hemivagina small black arrow Herlyn-Werner-Wunderlich syndrome[1] also known as OHVIRA is an extremely eyndrome syndrome characterized by a congenital birth defect of the lower abdominal and pelvic organs.

In total, 40 women were married and sexually active. Eur J Pediatr Surg.