Journal List > Perinatology > v.30(4) > 1144133

Perinatology. 2019 Dec;30(4):236-239. Korean.
Published online Dec 31, 2019.  https://doi.org/10.14734/PN.2019.30.4.236
Copyright © 2019 The Korean Society of Perinatology
Two Cases of Herlyn-Werner-Wunderlich Syndrome in Neonates and Adolescents with Hydrocolpos and Hematocolpometra
Minjeong Park, MD and Gyun-Ho Jeon, MD
Department of Obstetrics and Gynecology, Haeundae Paik Hospital, Inje University College of Medicine, Busan, Korea.

Correspondence to Gyun-Ho Jeon, MD. Department of Obstetrics and Gynecology, Haeundae Paik Hospital, Inje University College of Medicine, 875 Haeun-daero, Haeundae-gu, Busan 48108, Korea. Tel: +82-51-797-2020, Fax: +82-51-797-2030, Email: jeon285@hotmail.com
Received Nov 14, 2018; Revised Mar 03, 2019; Accepted Jun 10, 2019.

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.


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.

Keywords: Congenital abnormalities; Uterine diseases; Hydronephrosis; Hematometra

Figures


Fig. 1
A cystic mass that bulges between labia minora (case 1).
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Fig. 2
Trans-abdominal sonography shows hydrocolpos, large cystic mass due to imperforate hymen (case 1).
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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.
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Fig. 4
Two-dimensional ultrasonography image of double uterus (arrow) one year after surgery in case 2.
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