Herlyn-Werner-Wunderlich Syndrome

Reference work entry

Herlyn-Werner-Wunderlich syndrome is a rare Müllerian anomaly consisting of a didelphic uterus, a hemivaginal septum, and ipsilateral renal agenesis. The diagnosis should be suspected in adolescent females with a pelvic mass and ipsilateral renal agenesis.

Synonyms and Related Disorders

Blind hemivagina with ispsilateral renal agenesis

Genetics/Basic Defects

  1. 1.

    Anatomical defect is caused by a failure of lateral fusion of Müllerian ducts (didelphic/septate uterus and double vagina) combined with a failure of vertical fusion between the Müllerian duct and the urogenital sinus (hemivaginal septum) ipsilateral to the side of the renal agenesis.

  2. 2.
    Various types of uterine anomalies secondary to nondevelopment or failure of fusion of the distal segments of the Müllerian ducts
    1. a.

      Hypoplasia/agenesis of the uterus

    2. b.

      Unicornuate uterus

    3. c.

      Didelphic uterus

    4. d.

      Bicornuate uterus

    5. e.

      Septate uterus

    6. f.

      Arcuate uterus

  3. 3.
    Various types of vaginal anomalies secondary to the different...


Esophageal Atresia Renal Agenesis Imperforate Anus Urogenital Sinus Vaginal Septum 
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© Springer Science+Business Media, LLC 2012

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