Korean J Obstet Gynecol.  2012 Dec;55(12):966-969. 10.5468/KJOG.2012.55.12.966.

A case of Herlyn-Werner-Wunderlich syndrome diagnosed in asymptomatic newborn

• Kang M
• Kim SW
• Park JY
• Lim HJ
• Park CW
• Park JS
• Jun JK

• KMID: 2274206
• DOI: http://doi.org/10.5468/KJOG.2012.55.12.966

Abstract

Herlyn-Werner-Wunderlich (HWW) syndrome is characterized by the triad of uterine didelphys, obstructed hemivagina, ipsilateral renal agenesis. Symptoms generally occur just after menarche about 12 to 13 years old and it is usually diagnosed by pelvis magnetic resonance imaging. It can be treated by marsupialization of obstructed hemivagina or vaginal septum resection. It can be complicated by infertility, endometriosis, pelvic cavity adhesion, pyosalpinx, pyocolpos, and recurrent miscarriage. Together with a brief review of literature, we report a case of HWW syndrome diagnosed in asymptomatic newborn who was suspected to have a genitourinary anomaly on prenatal ultrasonography.

Keyword

Herlyn-Werner-Wunderlich syndrome; Newborn; Asymptomatic

MeSH Terms

Abortion, Habitual
Congenital Abnormalities
Endometriosis
Female
Humans
Infant, Newborn
Infertility
Kidney
Kidney Diseases
Magnetic Resonance Imaging
Menarche
Pelvis
Pregnancy
Ultrasonography, Prenatal
Congenital Abnormalities
Kidney
Kidney Diseases

Figure

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  Fig. 1 Prenatal obstetric ultrasonographic view: suspicious for hydronephrosis grade III with right ectopic kidney with pelvocalyceal dilatation, dilated ureter, suspicious for ureterovesical junction obstruction or ureteropelvic junction obstruction.

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  Fig. 2 Magnetic resonance imaging pelvis (non-contrast): there is no normal right kidney in renal fossa and uterine didelphys is detected.

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  Fig. 3 Magnetic resonance imaging pelvis (non-contrast): there is an ectopic insertion of right ureter to urethra with marked dilatation of dysplastic pelvocalyceal system and ureter.

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