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. 2019:63:129-134.
doi: 10.1016/j.ijscr.2019.08.035. Epub 2019 Sep 13.

Herlyn-Werner-Wunderlich syndrome: Diagnosis and treatment of an atypical case and review of literature

Camila Girardi Fachin  1 , João Lucas Aleixes Sampaio Rocha  2 , Amanda Atuati Maltoni  2 , Raquel Lins das Chagas Lima  2 , Vitória Arias Zendim  2 , Miguel Angelo Agulham  3 , Alina Tsouristakis  4 , André Ivan Bradley Dos Santos Dias  3
Affiliations

Herlyn-Werner-Wunderlich syndrome: Diagnosis and treatment of an atypical case and review of literature

Camila Girardi Fachin et al. Int J Surg Case Rep. 2019.

Abstract

Introduction: Herlyn-Werner-Wunderlich syndrome (HWWS), also known as OHVIRA syndrome, is a rare congenital abnormality of Müllerian duct development characterized by uterus didelphys, unilateral blind hemivagina, and ipsilateral renal agenesis.

Presentation of case: In this report, the authors present the case of a 12-year-old patient presenting with progressive and disabling abdominal pain after the onset of menarche diagnosed with HWWS, describe the various diagnostic modalities and treatment options available, along with a current review of the literature.

Discussion: With normal external genitalia, HWWS is usually asymptomatic until menarche when patients present with worsening abdominal pain during menses and a palpable pelvic or abdominal mass. Untreated, HWWS may lead to a number of complications including endometriosis, infertility, and spontaneous abortion.

Conclusion: Greater awareness of HWWS will lead to earlier detection and is the key to alleviating patient suffering and avoiding potentially severe complications.

Keywords: Case report; Hemi-hysterectomy; Herlyn-Werner-Wunderlich syndrome; Müllerian ducts abnormalities; OHVIRA syndrome; Renal agenesis.

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Conflict of interest statement

The authors have none conflicts of interests to declare.

Figures

Fig. 1
Fig. 1
CT demonstrating absent left kidney, uterine duplication, suggestive of cervical duplication, and distension of the left cavity by hypodense material.
Fig. 2
Fig. 2
Abdominal MRI, coronal view, showing the absence of the left kidney.
Fig. 3
Fig. 3
Pelvic MRI, transverse T2-weighted image, depicting a duplicated uterus and the presence of two endometrial cavities: right-sided cavity with 3.8 mm thickness and distended left cavity filled with hyperintense material.
Fig. 4
Fig. 4
Images obtained via hysteroscopy. A) Single and normal cervix on the right. B) Absence of bulging on the left lateral wall of the vagina (dashed line) or its recess. C) Ostium of the right uterine tube (dashed circle), without alterations. D) Absence of ostium of the left uterine tube.
Fig. 5
Fig. 5
Laparoscopic hysteroscopy. Visualization of the larger left hemiuterus (left side of image) and right hemiuterus of smaller volume. Note the myometrial bridge connecting the two structures.
Fig. 6
Fig. 6
Left hemiuterus and uterine tube after laparoscopic resection.
Fig. 7
Fig. 7
Schematic representation of the reproductive tract of the patient reported in this study. It is possible to note the existence of the two hemiuteri, connected to each other by a bridge of uterine tissue. The right hemiuterus is the only one containing a cervix, which communicates with a non-septate vagina.
See this image and copyright information in PMC

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