HERLYN WERNER WUNDERLICH SYNDROME PDF

August 25, 2019 posted by

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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An abdominal ultrasonography complemented by pelvic CT was performed on the day of syndromf, confirmed the absence of the right kidney and showed an image suggestive of haematocolpos on the right side and two uterine bodies. In total, 40 women were married and sexually active.

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

It presents soon after menarche or shows delayed presentation depending on the type. MRI with multiplanar image acquisition provides more detailed information. Department of Radiology, Dr. Discussion 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 series of reproductive ducts malformations.

Uterus didelphys, blind hemivagina and ipsilateral renal agenesis: Correspondence to Dr Ana Cristina Aveiro, moc. J Pediatr Adolesc Gynecol ; She also denied any gynecological problems except for the recent cyclic abdominal pain and enlarging abdominal mass. The right uterine horn cavity is seen to communicate with the upper shndrome of the fluid collection in right hemivagina small black arrow.

Two pear-shaped structures were noted in the pelvis — suggestive of uterus didelphys. The new classification allows for earlier diagnosis and treatment. The onset of clinical manifestations was much earlier and more serious in patients with wundfrlich obstructed hemivaginal septa compared with those with incomplete obstructions. Fertil Steril ; European Journal of Radiology.

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Classification 2 — Incompletely obstructed hemivagina. Journal of Pediatric and Adolescent Gynecology.

Uterus didelphys with an obstructed hemivagina and ipsilateral renal agenesis syndeome teenagers: Articles Cases Courses Quiz. Hence, clinical features are similar to as in classification 1. Journal List Pol J Radiol v. Abdominal magnetic resonance imaging.

Consulting a specialist in this case a gynecologist is recommended. With a population of 1. Risk factors No known risk factors Treatment Hemihysterectomy with excision of the vaginal septum in order to prevent the long term complication of recurrent pyocolpos wfrner hematometrocolpos. Most patients werneg recover completely after resection of the vaginal septum. MRI is considered as a gold standard for diagnosis and pre-operative planning of the Herlyn-Werner-Wunderlich syndrome.

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

New Classification of Herlyn-Werner-Wunderlich Syndrome

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 series of reproductive ducts malformations. Her medical history revealed a right-sided renal agenesis diagnosed prenatally. Menses from the uterus behind the septum can flow through communication to the contralateral cervix.

Endometriosis in association with Herlyn-Werner-Wunderlich syndrome. A year-old Caucasian female presented to our institution with progressive painful distention of the lower abdomen.

A case of Herlyn—Werner—Wunderlich syndrome with recurrent hematopyometra. They are inductor elements for hedlyn fusion of the Mullerian ducts. No obvious dilatation of the right fallopian tube was noted. An interesting case report on Herlyn-Werner-Wunderlich syndrome with wernfr and communicating blind duplex ureters. In this classification, the hemivagina is completely obstructed; the cervix behind the septum is maldeveloped or atresic, and menses from the uterus behind the septum cannot outflow through the atresic cervix.

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Eur J Pediatr Surg.

Wolffian ducts play an important role in the development of internal genital organs and give rise to kidneys [ 8 ]. Copyright Journal of Radiology Case Reports. As a result, two separate symmetrical uterine cavities develop with two cervices and no communication between these cavities; this is often associated with a vaginal septum, which can have a transverse wall that blocks one of the hemivaginas.

New Classification of Herlyn-Werner-Wunderlich Syndrome

Case Report Presentation of a clinical case of a year-old patient with HWW syndrome presenting with regular menses, dysmenorrhea and painful lump in hypogastric region on the left side of midline. MRI is considered gold standard for diagnosis. Treatment for patients with classification 1. Endometriosis is common complications, if not treated in time; the condition can progress to secondary endometriosis, pelvic adhesion, pyosalpinx, and even pyocolpos.

The external genitalia was normal. According to our analysis, 24 patients were categorized as Classification 1 and 55 were categorized as Classification 2. Abstract The Herlyn—Werner—Wunderlich syndrome is a rare congenital anomaly characterised by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. Sitemap What’s New Feedback Disclaimer. We present an unusual case of a didelphic uterus with pyocolpos in an obstructed hemivagina and ipsilateral renal anomaly figure 1.

Conclusions HWW syndrome can present early or late, depending on the type. Uterus is also visualized in this image arrowhead.