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. 2015:11:59-63.
doi: 10.1016/j.ijscr.2015.04.027. Epub 2015 Apr 24.

Herlyn-Werner-Wunderlich syndrome: An "early" onset case report and review of Literature

R Angotti  1 , F Molinaro  2 , A L Bulotta  2 , E Bindi  2 , E Cerchia  2 , M Sica  2 , M Messina  2
Affiliations

Herlyn-Werner-Wunderlich syndrome: An "early" onset case report and review of Literature

R Angotti et al. Int J Surg Case Rep. 2015.

Abstract

Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital mullerian anomaly consisting of uterus didelphys, hemivaginal septum, and unilateral renal agenesis [1,2]. Most authors reported cases of Herlyn-Werner-Wunderlich syndrome with prepuberal or postpuberal onset with cyclical abdominal pain and a vaginal mass (3-8). Only six cases are reported in Literature with early onset of this syndrome under 5 years (9-14). Our case is about 3 years old girl, with all the features of this syndrome who came to our attention for lower abdominal mass. The aim of this article is to share our experience and focus the attention on the importance of high level of suspicion of HWWS in neonatal period to early diagnosis and treatment. The possible early presentation of this syndrome should be suspected in all neonates (females) with renal agenesia confirmed postnatally or with prenatal diagnosis. It is common, in fact, an error of evaluation with planning of removal of mass, that can damage patients in term of chance for a successful reproductive outcome. For all these reasons, our team consider HWWS as differential diagnosis in newborn with prenatal ultrasonography of a cystic mass behind the urinary bladder in the absence of a kidney and plan a pelvic ultrasound (with aim to identify an uterus, normal or dydhelfus, and presence or absence of pelvic mass), an examination under anesthesia and cystoscopy and vaginoscopy, if it is necessary. A high level of suspicion, indeed, is the key to early diagnosis.

Keywords: Congenital mullerian anomaly; Herlyn–Werner–Wunderlich syndrome.

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Figures

Fig.1
Fig.1
MRI showing two cysts. Upper (U) 6 × 2 cm and lower (L) 1.5 × 2.5 cm.
Fig. 2
Fig. 2
Physical examination of genitalia. U (urethra), I (hymen), VS (vaginal septum).
Fig. 3
Fig. 3
(A) Identification of transverse vaginal septum; (B) section by bipolar of vaginal septum; (C and D) evidence of spill of mucus (hydrocolpus); (E and F) evidence of left hemivagina (E) and right hemivagina (F).
Fig. 4
Fig. 4
(A and B) Removal of vaginal septum.
See this image and copyright information in PMC

References

    1. Karaca L., Pirimoglu B., Bayraktutan U., Ogul H., Oral A., Kantarci M. Herlyn–Werner–Wunderlich syndrome a very rare urogenital anomaly in a teenage girl. J. Emerg. Med. 2014;(December (8)) pii: S0736-4679(14) 1108-1. - PubMed
    1. Van Leerdam L.E., Beerendonk C.C., Klein W.M., Sir O. Herlyn–Werner–Wunderlich syndrome. Nederlands Tijdschrift Voor Geneeskunde. 2014;158(December):A7886. Dutch. - PubMed
    1. Yavuz A., Bora A., Kurdoğlu M., Goya C., Kurdoğlu Z., Beyazal M., Akdemir Z.J. Herlyn–Werner–Wunderlich syndrome merits of sonographic and magnetic resonance imaging for accurate diagnosis and patient management in 13 cases. Pediatr. Adolesc. Gynecol. 2014 - PubMed
    1. Wang J., Zhu L., Lang J., Liu Z., Sun D., Leng J., Fan Q. Clinical characteristics and treatment of Herlyn–Werner–Wunderlich syndrome. Arch. Gynecol. Obstet. 2014;290(November (5)):947–950. - PubMed
    1. Fedele L., Motta F., Frontino G., Restelli E., Bianchi S. Double uterus with obstructed hemivagina and ipsilateral renal agenesis: pelvic anatomic variants in 87 cases. Hum. Reprod. 2013;28(June (6)):1580–1583. - PubMed

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