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CASE REPORT
Year : 2017  |  Volume : 10  |  Issue : 2  |  Page : 172-174  

Magnetic resonance imaging diagnosis of Herlyn-Werner-Wunderlich syndrome


1 Department of Radiodiagnosis, SMS, Jaipur, Rajasthan, India
2 Department of Pathology, PT BDS PGIMS, Rohtak, Haryana, India

Date of Web Publication14-Mar-2017

Correspondence Address:
Padam Parmar
Department of Pathology, PT BDS PGIMS, Rohtak - 124 001, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.202117

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  Abstract 

Herlyn-Werner-Wunderlich syndrome (HWW) is a triad of didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis. It is a combined anomaly of Mullerian and mesonephric ducts. It usually presents in adolescent females after menarche with nonspecific symptoms of pelvic pain, dysmenorrhea, and rarely a palpable pelvic mass. We report here, a case of an 18-year-old female presenting with complaints of lower abdominal pain and dysmenorrhea where magnetic resonance imaging (MRI) confirmed the diagnosis of HWW syndrome. MRI is the imaging modality of choice for diagnosis of HWW syndrome and associated complications such as endometriosis.

Keywords: Herlyn-Werner-Wunderlich syndrome, didelphys uterus, obstructed hemivagina, ipsilateral renal agenesis, magnetic resonance imaging


How to cite this article:
Yadav T, Parmar P. Magnetic resonance imaging diagnosis of Herlyn-Werner-Wunderlich syndrome. Med J DY Patil Univ 2017;10:172-4

How to cite this URL:
Yadav T, Parmar P. Magnetic resonance imaging diagnosis of Herlyn-Werner-Wunderlich syndrome. Med J DY Patil Univ [serial online] 2017 [cited 2017 Jun 22];10:172-4. Available from: http://www.mjdrdypu.org/text.asp?2017/10/2/172/202117


  Introduction Top


The diagnosis of Herlyn-Werner-Wunderlich syndrome (HWW) syndrome is often delayed because these patients present after menarche with nonspecific symptoms of pelvic pain and dysmenorrhea and are managed symptomatically. For the diagnosis of this syndrome, a high index of suspicion is required. When untreated, this may result in acute complications such as pyohematocolpos, pyosalpinx, or pelviperitonitis, urinary obstruction, and long-term complications, such as endometriosis, pelvic adhesions, and increased risk of abortion, or infertility. It usually presents at puberty, but rarely presents in adulthood.[1] Magnetic resonance imaging (MRI) is considered the noninvasive modality of choice for the diagnosis of Mullerian anomalies with the advantages of no radiation exposure, multiplanar imaging with better soft tissue resolution, better anatomic delineation of pelvic structures, and higher sensitivity for diagnosing complications such as endometriosis.[1],[2]


  Case Report Top


An 18-year-old unmarried female presented to the Obstetrics and Gynaecology Outpatient Department with complaints of lower abdominal pain and dysmenorrhea. She attained menarche at the age of 14 years and had an otherwise normal menstrual history except dysmenorrhea. Basic laboratory tests were within normal limits. An abdominal ultrasound was performed, which revealed absent left kidney, didelphic uterus and dilated left hemivagina with low-level internal echoes. A suspicion of HWW syndrome was made, and MRI pelvis was performed to rule out complications such as endometriosis.

MRI confirmed uterus didelphys with two separate uterine cavities, cervices, and vaginas [Figure 1]a, [Figure 1]b and [Figure 2]. The right uterine cavity, cervix, and vagina were normal. The left uterine cavity was seen in continuity with dilated left hemivagina [Figure 3], whose contents were hyperintense on both T1-weighted and T2-weighted MRI with dependent T2 hypointensity [Figure 2], [Figure 3], [Figure 4] consistent with hematocolpos with an obstructing left vaginal septum. Bilateral ovaries were normal. There was no evidence of peritoneal endometriosis. MRI of the upper abdomen confirmed the absence of left kidney with compensatory hypertrophy of the right kidney [Figure 5]. Hence, a diagnosis of HWW syndrome was confirmed with the presence of uterus didelphys, left hematocolpos, left hemivaginal obstruction, and left renal agenesis.
Figure 1: Axial fat-suppressed T2-weighted image (a) Two separate uterine cavities (black arrows) with two separate cervices (arrowhead) (b) Dilated left hemivagina (thin arrow) and compressed right hemivagina (thick arrow)

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Figure 2: Coronal fat-suppressed T2-weighted image depicting two separate uterine cavities (black arrows), dilated left hemivagina (red arrow) and normal right ovary (arrowhead)

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Figure 3: Sagittal T2-weighted image showing left uterine cavity in continuity with dilated hemivagina (thick arrow) with dependent T2 hypointense signal (T2 shading sign) consistent with hematocolpos

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Figure 4: Axial T1-weighted image showing dilated left hemivagina with hyperintense contents (arrow) suggestive of hemorrhagic contents

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Figure 5: Coronal fat-suppressed T2-weighted image of the upper abdomen showing absent left kidney with compensatory hypertrophy of right kidney

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After baseline laboratory investigations, transvaginal excision of the left-sided septum was done in this patient. Her postoperative course was uneventful; she was discharged 4 days after the surgery.


  Discussion Top


HWW syndrome has been considered to arise from an anomalous Müllerian (paramesonephric) as well as Wolffian (mesonephric) duct development. The Wolffian ducts are important in the origin of the kidneys and are also needed for adequate Müllerian ducts fusion. A congenital anomaly of the caudal portion of one of the Wolffian ducts may result in ipsilateral renal agenesis and obstructed hemivagina. On the side where the Wolffian duct is absent the Müllerian duct cannot fuse with the opposite side duct which results in the didelphic uterus. The longitudinal vaginal septum is due to the incomplete disappearance of the partition between the fused Müllerian ducts.[2],[3],[4]

As a hemivagina is obstructed due to the septum, outflow of menstruation from that side is not possible which results in hematocolpos, rarely hematometra, and hematosalpinx. Furthermore, blood reflux into the abdominal cavity may result in endometriosis and may lead to pelvic adhesions and increased risk of abortion or infertility. Patients with HWW syndrome usually present postmenarche with dysmenorrhea, pelvic pain, or pelvic mass.[5]

Pelvic ultrasound and MRI are the imaging modalities useful in the diagnosis and classification of Müllerian duct anomalies. On ultrasound, hematocolpos appears as a fluid collection posterior to the urinary bladder with low-level internal echoes. The transverse scan is helpful in delineating the uterine anomaly (didelphic/bicornuate bicollis uterus). Renal agenesis may be diagnosed in prenatal or neonatal period, and female neonates with renal malformations need to be screened for genital malformations. If HWW syndrome or other obstructive Mullerian anomalies are suspected, then follow-up with ultrasound examination of these asymptomatic patients should be done until after puberty so as to detect these genitourinary malformations early and prevention of future complications. Differentiation of a bicornuate bicollis uterus from uterus didelphys is sometimes difficult.[2],[5]

MRI is considered the noninvasive modality of choice for the exact diagnosis of Mullerian anomalies with the advantages of no radiation exposure, multiplanar imaging with better soft tissue resolution, better anatomic delineation of pelvic structures, and higher sensitivity for diagnosing complications such as endometriosis. HWW syndrome has a good prognosis after surgical excision of the septum and drainage of the obstructed vagina. Early detection with prompt drainage is necessary to prevent secondary endometriosis.[6]

The treatment of choice in these cases is surgical excision of the vaginal septum to relieve the obstruction and prevent the development of further complications.

 
  References Top

1.
Del Vescovo R, Battisti S, Di Paola V, Piccolo CL, Cazzato RL, Sansoni I, et al. Herlyn-Werner-Wunderlich syndrome: MRI findings, radiological guide (two cases and literature review), and differential diagnosis. BMC Med Imaging 2012;12:4.  Back to cited text no. 1
    
2.
Orazi C, Lucchetti MC, Schingo PM, Marchetti P, Ferro F. Herlyn-Werner-Wunderlich syndrome: Uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatr Radiol 2007;37:657-65.  Back to cited text no. 2
    
3.
Pinsonneault O, Goldstein DP. Obstructing malformations of the uterus and vagina. Fertil Steril 1985;44:241-7.  Back to cited text no. 3
    
4.
Burgis J. Obstructive müllerian anomalies: Case report, diagnosis, and management. Am J Obstet Gynecol 2001;185:338-44.  Back to cited text no. 4
    
5.
Arikan II, Harma M, Harma MI, Bayar U, Barut A. Herlyn-Werner-Wunderlich syndrome (uterus didelphys, blind hemivagina and ipsilateral renal agenesis) — A case report. J Turk Ger Gynecol Assoc 2010;11:107-9.  Back to cited text no. 5
    
6.
García González P, Meana Morís AR, Gracía Chapullé A, Matesanz Pérez JL. The role of MRI in congenital cystic lesions in the pelvis: A case of uterus didelphys with double vagina, hematocolpos, and ipsilateral renal agenesis. Radiologia 2009;51:194-7.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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