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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Chin Med J, wubderlich J Pediatr Surg ; Fluid density mass in the pelvis with absent ipsilateral kidney. Some kidney abnormalities may occur.

New Classification of Herlyn-Werner-Wunderlich Syndrome

Acute onset of abdominal pain, fever, and vomiting are common symptoms seveal months after menarche. However, the distal part of the vagina, originating from the urogenital sinus is not affected [ 4 ]. Occasional ectopic ureter Ur insertion into the obstructed hemivagina can occur. She was released 2 days after surgery, showing an uneventful recovery. Endometriosis in association with Herlyn-Werner-Wunderlich syndrome. Resection of as much of syyndrome obstructing vaginal septum as possible is the optimal surgery for patients with Classifications 1.

Khaladkar, Department of Radiology, Dr. In patients with uterine and vaginal abnormalities, work up for associated renal anomalies should be performed. The surgical approach depends on the imaging findings. The left uterus was enlarged due to fluid collection in the uterine cavity and cervical canal measuring 8.


Although US can be used to diagnose this condition, MRI plays an important role in further characterizing the didelphic uterus, obstructed hemivagina, and ipsilateral renal agenesis. Patients with this classification have similar clinical features as patients with Classification 1. The patient was sent to radiology for further characterization.

A year-old Caucasian female syndromd to our institution with progressive painful distention of the lower abdomen. The follow-up period ranged from 1 to months.

Herlyn-Werner-Wunderlich syndrome | Radiology Reference Article |

Abstract The Herlyn—Werner—Wunderlich syndrome is a rare congenital anomaly characterised by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. Although a longitudinal vaginal septum was not directly visualised, its presence was admitted.

OHVIRA obstructed hemivagina with ipsilateral renal agenesis with pyocolpos is a rare clinical presentation. All patients underwent resection of the vaginal septum and drainage of hematocolpos. Below, we describe the wwunderlich of each classification in detail. 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.

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

Uterus didelphys with unilateral obstructed hemivagina with hematometrocolpos and hematosalpinx with wunderlichh renal agenesis. The median time between menarche and the onset of cyclic pelvic pain was 0. Journal of Clinical and Diagnostic Research: Color Doppler demonstrates no flow in the collection.


The patient had reported overall good health. Published online Jul Evaluation of the genital tract by means of MRI scanning is recommended in all girls with known renal abnormalities detected antenatally or thereafter, before the onset of menstruation.

Support Center Support Center. If one of the Wolffian ducts is absent, the ipsilateral kidney and ureter will fail to fuse in the midline. It was coursing superiorly over the uterine fundus and extending in the right adnexa — suggestive of left haematosalpinx. J Reprod Med ; CT Fluid density mass in the pelvis with absent ipsilateral kidney. She denied any prior surgeries, use of medications or allergies. Department of Radiology, Dr. OHVIRA syndrome obstructed hemivagina and ipsilateral renal anomaly with uterus didelphys, an unusual presentation.

However, if one of the Wolffian ducts is absent, the kidney and ureter on the ipsilateral side will fail to fuse at midline.