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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Synddome Afr J Radiol ; Uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: If neglected, the condition can progress to secondary endometriosis, pelvic adhesions, pyosalpinx and pyocolpos [ 1011 ]. It usually presents after menarche with progressive pelvic pain during menses secondary to haematocolpos. Clinical presentation Pathology Radiographic features Treatment and prognosis Differential diagnosis References Images: It helps clinicians for planning, staging, assessing risk-benefit ratio of different treatment approaches.

Conclusions Wegner syndrome can present early or late, depending on the type. Published online Mar 1. In most cases, OHVIRA presents as a double uterus with unilateral obstructed or blind hemivagina and ipsilateral renal agenesis.

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

Unusual form of genital malformation with aplasia of the right kidney. Wynderlich cases complicated by cervical atresia, ipsilateral hysterectomy is suggested because resection of the septum would not relieve obstructed symptoms.


The wunderich MRI showed two separate uteri with two separate cervices uterus didelphys bicollis figure 1A and two proximal vaginas. Delay in diagnosis increases the risk of complications such as endometriosis and infertility [ 2 ].

Clinical characteristics of 70 patients with Herlyn-Werner-Wunderlich syndrome. The pigtail wunddrlich was left in place for 4 days at which time it was removed in the locked position in an attempt to prevent reaccumulation of fluid by lacerating the wall of the hemivagina Figure 5.

J Reprod Med ; The Herlyn-Werner-Wunderlich syndrome is a rare, congenital disorder characterised by uterus didelphys, unilateral obstructed hemivagina and ipsilateral renal agenesis, all being secondary to mesonephric duct-induced Mullerian anomalies. A year-old female patient presented derner pain in the lower abdomen with lumpish feeling in the lower abdomen on the left side. J Minim Invasive Gynecol.

This has ultimately lead to the classification scheme developed by the American Fertility Society, which describes the appearance of the uterus given the various fusion anomalies that can occur figure 6.

Etiology The pathogenesis is not well understood, but is wunderlidh to be related to Mullerian duct fusion anomaly which results in failed development of the Wolffian duct on the side of the obstructed hemivagina causing ipsilateral renal agenesis. Menses from the uterus behind the septum can flow through communication to the contralateral cervix. Endometriosis in association with Herlyn-Werner-Wunderlich syndrome.

Color Doppler demonstrates no flow in the collection. Hematometra and hematosalpinx occurred in some more severely affected patients, as well as bleeding in the periadnexal and peritoneal space. Prog Obstet Ginecol ; The main physical examination wundrelich was tenderness on RLQ and hypogastric palpation, and doubtful Blumberg sign. Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis Herlyn-Werner-Wunderlich syndrome J Pediatr Surg.


New Classification of Herlyn-Werner-Wunderlich Syndrome

Occasional ectopic ureter Ur insertion into the obstructed hemivagina can occur. Full resection of the vaginal septum resulted in good outcomes and fertility. The uterus behind the septum is completely syndrpme from the contralateral uterus.

The diagnosis is often difficult due to the infrequency of the syndrome, and a high index of suspicion is required.

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

Khaladkar, Department of Radiology, Dr. PV examination was not done. Although there are no specific symptoms for this condition, common complains include progressively increasing pelvic pain during menstruation and hematocolpos due to the buildup of blood in the body. ObstetricsUrogenitalGynaecology. Mullerian duct abnormalities cover a wide range of developmental anomalies, resulting from non-development, defective fusion or defects in regression of the septum during foetal development.