About an Uncommon Case Report
Mariam Mahtate*, Khaoula Lakhdar, Soukaina Cherradi, Aziz Slaoui, Najia Zeraidi, Amina Lakhdar and Aziz Baydada
Department of Gynecology-Obstetrics and Endoscopy, University Mohammed V, Morocco
Submission: March 20, 2023; Published:April 19, 2023
*Corresponding author: Mariam Mahtate, Department of Gynecology-Obstetrics and Endoscopy, University Mohammed V, Rabat, Morocco
How to cite this article: Mariam M, Khaoula L, Soukaina C, Aziz S, Najia Z, et al. Herlyn-Werner-Wunderlich Syndrome: About an Uncommon Case Report. Glob J Reprod Med. 2023; 9(5): 555774. DOI: 10.19080/GJORM.2023.09.555774.
Herlyn-Werner-Wunderlich syndrome (HWWS), defined by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis, is a rare Mullerian duct malformation, usually diagnosed after menarche, when symptoms related to haematocolpos arise. HWW generally occurs at puberty and exhibits variable symptoms including pelvic pain shortly following menarche, dysmenorrhoea, palpable mass due to the associated haematocolpos or haematometra or haemoperitoneum due to retrograde menstruation.
Ultrasonography and MRI are extremely useful in the diagnosis and classification of Müllerian duct anomalies. 3D ultrasound is more effective in the diagnosis of uterine malformation. Early detection of this relatively rare syndrome can lead to the provision of immediate treatment to preserve future fertility. We present a rare case report on Herlyn-Werner-Wunderlich syndrome from Morocco.
HWW syndrome is an extremely rare congenital anomaly of the urogenital system due to a congenital developmental defect of the Mullerian ducts characterized by a triad, didelph uterus, obstructed hemi-vagina and ipsilateral renal agenesis . The most common symptomatology of this syndrome in postmenarche is pelvic pain, pelvic mass, hematocolpos, primary amenorrhea, menstrual cycle disorder, abortion and prematurity . The diagnosis of this syndrome is essentially radiological based on ultrasound combined with MRI for a better evaluation of associated malformations .
A proper diagnosis and prompt treatment can prevent the possible complications associated with this syndrome; pyocolpos, endometriosis and infertility . We hereby present an uncommon case of a 13-year-old female patient referred to our department for an episode of acute urine retention associated with a pelvic pain after the onset of puberty in whom the diagnosis of HWW syndrome was made on pelvic MRI.
We hereby report the uncommon case of A 13-year-old patient was referred to our structure for an episode of acute retention of urine associated with pelvic pain that increases in intensity
at the same period of the menstrual cycle, during menstruation since her menarche. The patient reported urinary disorders such as dysuria and mictional imperiosis of progressive aggravation without associated digestive disorders before this episode of retention. The gynecological history indicates menarche at the age of 12 years with a regular cycle with minimal periods. The clinical examination found a pelvic curvature reaching the umbilicus associated with abdominal sensitivity, the gynecological examination found no external genital anomaly. In front of this atypical clinical presentation, a tumoral origin was suspected after bladder drainage.
The pelvic ultrasound showed a hypoechogenic median retro vesical liquid formation (Figure 1), associated with a uterine anomaly that was difficult to distinguish with the absence of the right kidney; a pelvic MRI was requested for a better evaluation of the urinary tract. Parental consent was given for the MRI with injection of the contrast product. Pelvic MRI performed in three planes in T2, T1, diffusion and gadolinium injection sequence in favor of a didelphic bicervical uterus with hematocolps at the level of the right hemivagina which extends to the homolateral hemi uterus, the left hemi uterus, ovaries of normal size and morphologies with right renal agenesis, without signs of deep endometriosis (Figure 2).
On the basis of the MRI radiological images in front of this
bicornuate bi-cervical uterus associated with a hematocolpos
of the right hemovagina in favor of an obstructed hemivagina
and a right ipsilateral agenesis the diagnosis of HWW syndrome
was made. The surgical management was done by Identification
and resection of the vaginal septum and reached up to the right
cervix for the drainage of tarry blood (Figure 3). Thus, vaginal
canal was reconstructed (Figure 4). There were no perioperative
or postoperative complications. She was discharged 5 days after
surgery. The patient had regular clinical and echocardiographic
check-ups. The patient has been symptom free for 6 months after
Purslow in 1922 was the pioneer in presenting a case of
didelphic uterus with obstructed hemivagina, Miller in the same
year presented the same case with ipsilateral renal anomaly .
Known as Herlyn-Werner-Wünderlich syndrome or with the
acronym OHVIRA (Uterus didelphys associated with Obstructed
Hemi-Vagina and Ipsilateral Renal Anomaly), is a rare malformation
of the Müllerian ducts. The association with renal agenesis was
reported in 1971 . Recently, many cases of morphological
diversity such as other uterine abnormalities, varying degrees
of vaginal obstruction, and various renal/ureteral abnormalities
have been noted . Didelphic uterus accounts for 5% of Müllerian malformations, with an incidence of renal agenesis of 1/1,000. 25- 50% of her affected, get genital abnormalities .
The common embryological development of the urinary and
genital systems explains the associated malformations of both
systems. The female internal organs derive from the Müllerian
ducts, which give their origin to the tubes, uterus and upper 2/3
of the vagina, the Wolffian ducts give the origin to the kidneys and
induce proper fusion of the Müllerian ducts .
The aetiology and the pathogenesis of SHWW is unknown. It
can be caused by exposure to environmental factors, teratogens,
radiation, drugs, but most have a multifactorial polygenic basis. It is
not associated with chromosomal abnormalities, so the Karyotype
is normal, other genital, urological, rectal malformations or
skeletal dysplasia may be associated, exceptionally, it is acquired
. our patient presented the congenital malformation generating
a didelphic bi cervical uterus with associated hematocolpos with
right renal agenesis classified as U3b C2 V2 according to the
American society of reproductive medicine (ASMR) .
The diagnosis is rarely made antenatally or in childhood
and coincides with the menarche around the age of 14 .
The symptoms are essentially attributed to menstruation by
dysmenorrhea, hematocolpos, oligomenorrhea as is the case in
our patient or primary amenorrhea. As well as symptoms related
to hematocolpos by compression of the surrounding organs
causing acute retention of urine, or pyelocaliceal dilatation.
several symptoms can confuse the clinician nonspecific. As we can
see the patients at the stage of complications, pyohematocolpos,
pyosalpinx, pelviperitonitis, endometriosis, pelvic adhesions thus
affecting the fertile prognosis of the patient hence all the interest
to think about the diagnosis in any young patient presenting with
gynecological symptoms. our patient was diagnosed at the stage
of complication by an acute retention of urine thus discovering the
syndrome via ultrasound and more exactly by MRI.
Diagnosis is based on clinical history and imaging exams,
specifically ultrasound and MRI . Ultrasound is accessible
and assesses the renal system, 3D transvaginal ultrasound more
accurately evaluates the uterus and adnexa and MRI is more
precise . The differential diagnosis is made with abnormalities
of uterine development, unicornuate uterus with contralateral
corne, bicornuate uterus, imperforate hymen, and hypoplasia
or agenesis of the cervix. The management relieves symptoms,
avoids complications and conserves fertility. Surgery is usually
conservative, resection of the vaginal septum, marsupialization
of the obturated hemivagina as in our case, and drainage of
collections if present.
Efforts should be made to preserve the affected hemiuterus
because pregnancy can be achieved there. Some studies show
that 87% have successful pregnancies, 23% abortions, 15%
preterm deliveries and intrauterine growth retardation [13-15].
It’s recommended a therapeutic abstinence in renal agenesis; it is
important to prevent urinary tract infections and to monitor renal
HWWS is a rare condition relatively unknown to medical
professionals, and associated dysmenorrhea is often misdiagnosed
as another, more common cause of dysmenorrhoea in juvenility.
The correct and accurate diagnosis of female reproductive system
disorders, including HWW syndrome, is necessary to avoid
complications. Early detection of this relatively rare syndrome can
lead to the provision of immediate treatment to preserve future
Written informed consent was obtained from the patient for
publication of this case report and any accompanying images. A
copy of the written consent is available for review by the Editorin-
Chief of this journal.