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

Because the communication is small, the drainage is still impeded [ Figure 4 ]. A small communication exists between two vaginas with resultant incomplete obstruction. Hence, abnormal differentiation of the mesonephric and paramesonephric ducts may also be associated with anomalies of the kidneys. Correspondence to Dr Ana Cristina Aveiro, moc. Conclusions HWW syndrome has variable onset of presentation.

Resection of the vaginal septum is the treatment of choice of obstructed hemivagina. The pelvic mass is the collection of blood products within the obstructed hemivagina. According to syndro,e newly identified characteristics, we recommend that HWWS be classified according to the complete or incomplete obstruction of the hemivagina as follows: She denied fevers, chills, nausea, vomiting or diarrhea.

Eur J Pediatr Surg ; Views Read Edit View history. Khaladkar, Department of Radiology, Dr. Eur J Pediatr Surg. US and MR findings. Only a handful of these cases have been associated with pyocolpos. In patients with uterine and vaginal abnormalities, work up for associated renal anomalies should be performed.


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

However, she reported an ultrasound performed in a small military medical clinic in Samoa, prior to her arrival, revealed absence of her left kidney. Note the right uterine horn small arrow.

This article has been cited by other articles in PMC. Those cases where an ectopic ureter inserts into the obstructed hemivagina, laproscopic surgical ligation vs surgical removal is advocated to prevent recurrent fluid accumulation in the obstructed hemivagina [ 3 ]. The menses can outflow through the small communication, but the drainage is impeded.

OHVIRA – Wikipedia

OHVIRA obstructed hemivagina with ipsilateral renal agenesis with pyocolpos is a rare clinical presentation. National Center for Biotechnology InformationU. Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis.

Edit article Share article View revision history. Renal agenesis is ipsilateral to the dilated wunderlicu cavity.

Herlyn-Werner-Wunderlich syndrome | Radiology Reference Article |

Department of Radiology, Dr. 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.

Menses from the uterus behind the septum can flow through communication to the contralateral cervix. CT Fluid density mass in the pelvis with absent ipsilateral kidney. Vaginal septotomy is usually done by hysteroscopic approach rather than laparoscopic technique [ 12 ]. Embryological development, classification, and MRI assessment. Support Center Support Center. Tubular fluid density pelvic mass, kidneys present.


USG of the abdomen and pelvis — showing an absent left kidney, uterus didelphys, haematometra in the left uterine horn. Resection of as much of the obstructing vaginal septum as possible is the optimal surgery for patients with Classifications 1.

derner Wunderlich in described association of right renal agenesis with a bicornuate uterus and simple vagina in the presence of an isolated haematocervix [ 3 ].

The pathogenesis is not well understood, but is believed to be related to Mullerian duct fusion anomaly which results in failed development wermer the Wolffian duct on the side of the obstructed hemivagina causing ipsilateral renal agenesis. A pigtail catheter was placed under ultrasound guidance. The development of the genital system.

The unique feature of our case is haematometra and haematocervix with cervical and vaginal atresia found on the left side classification 1. Unusual form of genital malformation with aplasia of the right kidney. If neglected, the condition can progress to secondary endometriosis, pelvic adhesions, pyosalpinx and pyocolpos [ 1011 ]. Wunrerlich information Article notes Copyright and License information Disclaimer.