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CASE REPORT
Year : 2017  |  Volume : 10  |  Issue : 2  |  Page : 168-171  

Herlyn–Werner–Wunderlich syndrome


1 Department of Obstetrics and Gynaecology, Dr. D.Y. Patil Medical College, Hospital and Research Centre, Pune, Maharashtra, India
2 Department of Radiology, Dr. D.Y. Patil Medical College, Hospital and Research Centre, Pune, Maharashtra, India

Date of Web Publication14-Mar-2017

Correspondence Address:
Nikita Gonnade
Department of Obstetrics and Gynaecology, Dr. D.Y. Patil Medical College, Pune, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.202109

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  Abstract 

Herlyn–Werner–Wunderlich syndrome is a rare developmental anomaly, in which there is uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. The most common presenting complaint is abdominal pain with or without abdominal mass, secondary to hematocolpos. Since the obstruction is unilateral, monthly periods continue and diagnosis can be delayed leading to complications with far-reaching effects. In this report, we describe the evaluation and successful management of one such case in a 13-year-old girl who reported with chronic pelvic pain and abdomino-pelvic mass.

Keywords: Herlyn–Werner–Wunderlich, mullerian duct, renal agenesis, uterus didelphys


How to cite this article:
Bal H, Duggal B S, Gonnade N, Khaladkar S. Herlyn–Werner–Wunderlich syndrome. Med J DY Patil Univ 2017;10:168-71

How to cite this URL:
Bal H, Duggal B S, Gonnade N, Khaladkar S. Herlyn–Werner–Wunderlich syndrome. Med J DY Patil Univ [serial online] 2017 [cited 2017 Nov 18];10:168-71. Available from: http://www.mjdrdypu.org/text.asp?2017/10/2/168/202109


  Introduction Top


Developmental anomalies of the mullerian duct system are some of the most fascinating disorders encountered by gynecologists. The mullerian ducts are the precursors of the female reproductive tract. A wide range of malformations can occur when this system is disrupted leading to varied clinical presentations. Thus, a planned and detailed diagnostic protocol should be in place for determining the type of defect and its classification. Once an accurate diagnosis is made, many treatment options exist, and they are tailored to the specific mullerian anomaly. The patients may present with abdominal pain, cyclical dysmenorrhea, primary amenorrhea, pelvic mass/abdomino-pelvic mass, or rarely retention of urine and infertility.

Here, we report one such case of a young girl who came to us with pain abdomen.


  Case Report Top


A 13-year-old girl presented with the complaint of pain in the lower abdomen on and off for 4 months. She had attained menarche 1 year back and thereafter was menstruating regularly every month with normal menstrual flow but significant dysmenorrhea. In addition to dysmenorrhea, she frequently suffered from pain on the left lower abdomen which was moderate to severe in intensity.

On clinical examination, her vital parameters were normal. On abdominal examination, a nontender mass with restricted mobility arising from the pelvis was felt just above the symphysis pubis. On bimanual abdomino-rectal examination, this mass was found to be 6–7 cm in size, tense cystic, and located to the left of the uterus.

Provisional clinical diagnosis was left ovarian cyst with a probability of intermittent torsion.

Ultrasonography revealed uterus of normal size. There were two well-defined anechoic cystic lesions measuring 9.5 cm × 4.8 cm × 4 cm and 5.2 cm × 4.6 cm × 4.2 cm with multiple internal echoes in the left adnexa suggestive of left par-ovarian cyst/left cornua of a didelphyic uterus with hematometra. The left kidney was not visualized, and the right kidney hypertrophied.

Magnetic resonance imaging (MRI) was done for further confirmation. MRI reported didelphyic uterus with normal right uterine horn and fallopian tube. The left uterine horn with fallopian tube was over distended due to hematometra, hematotrachelos, and hematosalpinx. Bilateral ovaries were normal, and the left kidney was not visualized [Figure 1] and [Figure 2].
Figure 1: Sagittal view of both halves of the uterus

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Figure 2: Coronal view of magnetic resonance imaging depicting left hematosalpinx and absent kidney

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Impression

The impression was Herlyn–Werner–Wunderlich (HWW) syndrome.

The plan then was to do a vaginoscopy and find the level of obstruction. Vaginoscopy was done under anesthesia with a 4 mm hysteroscope. On vaginoscopy, no obvious bulge in lateral vaginal wall suggesting obstructing septum could be seen. A single cervix was visualized. Hysteroscope could be easily negotiated through cervical canal probably because of the associated cervical incompetence and the canal was normal. The right uterine cavity with the right ostium was seen. The left uterine cavity could not be visualized. Obstruction at the level of the left hemivagina was unlikely and probably sited at a higher level. The patient was thereafter taken up for laparotomy.

At laparotomy, we found uterus didelphys with left-sided uterus and cervix enlarged and appeared to end blindly on the lateral vaginal wall. The left tube was dilated more on the lateral half. The right-sided uterus, tube, and both ovaries were healthy. There was no evidence of endometriosis. The left kidney could not be palpated in renal fossa or lower down [Figure 3],[Figure 4],[Figure 5]. Hence, our peroperative diagnosis was uterus didelphys (2 uteri and 2 cervices) with unilateral obstruction at the level of cervix on the left side leading to left-sided hematotrachelos, hematometra, and hematosalpinx.
Figure 3: Operative view of the right and left horn of uterus

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Figure 4: View of the left-sided hematosalpinx with normal ovaries

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Figure 5: Draining hematometra through hysterotomy incision

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Unilateral left-sided subtotal hysterectomy with salpingectomy was performed. The patient had an uneventful recovery. Histopathological examination of the left fallopian tube revealed hydrosalpinx with chronic xanthogranulomatous salpingitis. Brown pigment-laden macrophages seen in the wall were consistent with retrograde menstruation. The left uterine horn was unremarkable. The patient was asymptomatic on her follow-up visit 2 weeks later.


  Discussion Top


Mullerian anomalies are congenital defects of the female genital tract resulting from nondevelopment or nonfusion of the paramesonephric ducts or failed uterine septum resorption.[1] The mullerian ducts fuse together in the midline and form uterus, cervix, and upper four-fifth of the vagina. The lower one-fifth of the vagina is formed from the sinovaginal bulb which is the protrusion of the urogenital sinus.[2] A wide range of malformation occurs when this system is disrupted and accordingly clinical presentation varies widely. This particular case is a rare form of mullerian anomaly called HWW syndrome.[3]

HWW syndrome is a mullerian anomaly characterized by the combination of uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. Described first in 1922, the exact cause is not known. Incidence reported is 0.1%–3.8%.[4] As per the American Fertility Society Classification of uterovaginal anomalies, this is Class IIIA-asymmetric obstructed disorder of uterus or vagina usually associated with ipsilateral renal agenesis.[5]

Ours was a variant of HWW syndrome characterized by a combination of uterus didelphys with obstruction at the level of cervix, not hemivagina, and ipsilateral renal agenesis [Figure 6].
Figure 6: Schematic representation of Herlyn–Werner–Wunderlich syndrome

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HWW syndrome is usually discovered at puberty with nonspecific symptoms. These may be acute or chronic pelvic pain, dysmenorrhea, and palpable mass due to the associated hematometra or hematocolpos, which results from retention of menstrual blood behind the obstructed vagina.[6]

It is difficult to achieve an accurate diagnosis because menstruation is often regular, and when patient complains of dysmenorrhea, they are usually given anti-inflammatory drugs or oral contraceptives. The potential complications of this syndrome are pyohematocolpos or pyosalpinx, which may lead to pelvic peritonitis. Long-term complications are endometriosis, pelvic adhesions leading to chronic pelvic pain, and infertility.

Diagnosis is usually confirmed by ultrasonography and MRI. MRI is the most sensitive investigation.[7] Laparoscopy is not mandatory but could be helpful in confirming the diagnosis when radiological imaging is inconclusive.

Laparo-hysteroscopy can also be therapeutic in some selected cases with drainage of hematocolpos or hematometrocolpos by vaginal septum resection.

Variants of classical HWW syndrome have been reported in literature. Kwon has reported a case of late detection of HWW syndrome in pregnancy where there was a septate uterus with obstructed hemivagina. The case was diagnosed in labor when cesarean section had to be done for obstructed labor due to the unilateral vaginal bulge resulting from hematocolpos.[8] Another report of 87 cases of HWW syndrome revealed 72.4% cases of classic variety and 27.6% were variants with uterine septum or cervical agenesis.[9]


  Conclusion Top


HWW syndrome is a relatively rare obstructive malformation of mullerian ducts. The aim of reporting this case is to highlight the entity and the importance of early management. Timely detection and appropriate treatment will prevent future complications and preserve fertility of the girl when she matures into a woman.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Jindal G, Kachhawa S, Meena GL, Dhakar G. Uterus didelphys with unilateral obstructed hemivagina with hematometrocolpos and hematosalpinx with ipsilateral renal agenesis. J Hum Reprod Sci 2009;2:87-9.  Back to cited text no. 1
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2.
Aveiro AC, Miranda V, Cabral AJ, Nunes S, Paulo F, Freitas C. Herlyn-Werner-Wunderlich syndrome: A rare cause of pelvic pain in adolescent girls. BMJ Case Rep 2011;2011:41-7.  Back to cited text no. 2
    
3.
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. 3
    
4.
Zurawin RK, Dietrich JE, Heard MJ, Edwards CL. Didelphic uterus and obstructed hemivagina with renal agenesis: Case report and review of the literature. J Pediatr Adolesc Gynecol 2004;17:137-41.  Back to cited text no. 4
    
5.
Buttram VC Jr., Gibbons WE. Müllerian anomalies: A proposed classification. (An analysis of 144 cases). Fertil Steril 1979;32:40-6.  Back to cited text no. 5
    
6.
Purslow CE. A case of unilateral haematocolpos, haematometra and haematosalpinx. J Obstet Gyaecol Br Emp 1922;29:643.  Back to cited text no. 6
    
7.
Troiano RN. Magnetic resonance imaging of mullerian duct anomalies of the uterus. Top Magn Reson Imaging 2003;14:269-79.  Back to cited text no. 7
    
8.
Kwon D. A variant of the HWW syndrome in pregnancy. Ultrasound Obstet Gynecol 2014;44 Suppl 1:181-369.  Back to cited text no. 8
    
9.
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;28:1580-3.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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