Perinatology.  2019 Dec;30(4):236-239. 10.14734/PN.2019.30.4.236.

Two Cases of Herlyn-Werner-Wunderlich Syndrome in Neonates and Adolescents with Hydrocolpos and Hematocolpometra

• Park M
• Jeon GH

• KMID: 2468545
• DOI: http://doi.org/10.14734/PN.2019.30.4.236

Abstract

The Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly that is characterized by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. It is often diagnosed in patient with pelvic or abdominal pain during puberty. Treatment is basically resection of obstructed vaginal septum by surgical approach. We describe two case of HWW syndrome, one is a neonate who was diagnosed as hydronephrosis and pelvic mass in postnatal ultrasonography, and the other is a 13-year-old girl with abdominal pain.

Keyword

Congenital abnormalities; Uterine diseases; Hydronephrosis; Hematometra

MeSH Terms

Abdominal Pain
Adolescent*
Congenital Abnormalities
Female
Hematometra
Humans
Hydrocolpos*
Hydronephrosis
Infant, Newborn*
Puberty
Ultrasonography
Uterine Diseases
Uterus

Figure

• 

  Fig. 1 A cystic mass that bulges between labia minora (case 1).

• 

  Fig. 2 Trans-abdominal sonography shows hydrocolpos, large cystic mass due to imperforate hymen (case 1).

• 

  Fig. 3 Selected MRI images of case 2. (A) The axial MR image shows two uteri (arrows). (B) The coronal MR image shows hematocolpos (marked as H) which is slightly hypointense on T2-weighted image. MRI, magnetic resonance imaging; MR, magnetic resonance.

• 

  Fig. 4 Two-dimensional ultrasonography image of double uterus (arrow) one year after surgery in case 2.

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