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At a glance

It is a rare genetic disorder characterized by the presence of a uterus didelphys, a blind hemivagina and ipsilateral renal agenesis. Individuals affected may have either completely or incompletely obstructed vaginal septum. The most common presentation is acute or progressive lower abdominal pain, dysmenorrhea (especially in Type I), and abdominal mass in the lower abdomen secondary to hematocolpos and/or hematometra.

Synonyms

HWWS; Obstructed Hemivagina Ipsilateral Renal Anomaly Syndrome; OHVIRA; Renal Agenesis Ipsilateral Blind Hemivagina Syndrome.

History

The Herlyn-Werner Syndrome was initially described by Herlyn and Werner in 1971. Wunderlich identified in 1976 an association of ipsilateral renal agenesis with a bicornuate uterus and simple vagina in the presence of an isolated hematocervix.

Classification

There is a new classification based on the vaginal anatomical diagnosis.

  • Type I: Defined by the presence of a completely obstructed hemivagina.

  • Type II: Associated with an incompletely obstructed hemivagina. This variation helps determine the type of surgical treatment.

Incidence

This is a medical condition that requires high suspicion for the diagnosis. It is usually seen in adolescents or young adult. There are only six cases reported in literature with an early onset before 5 years. The exact incidence remains unknown. It should be suspected in an adolescent with lower abdominal pain, endometriosis and ipsilateral renal agenesis. The estimated overall prevalence of Mullerian Duct Anomalies (MDA) is 2 to 3% of women in the general population. The presence of a uterus didelphys constitutes 11% of all MDAs. Hypoplasia or agenesis of the uterus and proximal vagina constitute 5 to 10% of MDAs. Associated renal anomalies are present in approximately 43%. This medical condition is classified as Type III MDA and is associated by mesonephric duct anomaly. It accounts for approximately 5% of all MDAs.

Genetic inheritance

Studies of uterine organogenesis have implicated the Hox and Wnt genes as regulators of uterine morphology. However, there are no known specific gene mutations that can be associated with this particular type of MDA.

Pathophysiology

It is a very rare congenital anomaly of the urogenital tract that affects the Mullerian and mesonephric ducts. Mullerian duct abnormalities cover a wide range of developmental anomalies, resulting from nondevelopment, defective fusion or defects in regression of the septum during fetal development. About 75% of patients with didelphys uterus parent a partial or complete vaginal septum anomaly that is commonly longitudinal in the Herlyn-Werner-Wunderlich Syndrome. This suggests a disorder of the lateral fusion between the inferior portions of two Mullerian ducts.

Diagnosis

The diagnosis is often difficult due to the infrequency of the syndrome. A high index of suspicion is required when a young adolescent consult for lower abdominal pain and menarches. Early ...

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