Pediatr Radiol (2007) 37:657–665 DOI 10.1007/s00247-007-0497-y
ORIGINAL ARTICLE
Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases Cinzia Orazi & M. Chiara Lucchetti & Paolo M. S. Schingo & Paola Marchetti & Fabio Ferro
Received: 2 January 2007 / Revised: 5 March 2007 / Accepted: 4 April 2007 / Published online: 15 May 2007 # Springer-Verlag 2007
Abstract Background Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is a rare entity, sometimes referred to as Herlyn-Werner-Wunderlich syndrome (HWW). It usually presents after menarche with progressive pelvic pain, sometimes with regular menses, and a palpable mass due to hemihaematocolpos. The diagnosis is generally made only if the suspicion of this genitourinary syndrome is raised. Objective To highlight the imaging diagnostic clues in this rare condition. Materials and methods We report on 11 adolescents with this condition. Results Sonography mostly allowed the correct diagnosis by showing uterovaginal duplication, haematocolpos or haematometrocolpos, and the absence of the ipsilateral kidney. MRI provided more detailed information regarding
C. Orazi : P. M. S. Schingo Department of Diagnostic Imaging, I.R.C.C.S. Bambino Gesù Pediatric Hospital, Rome, Italy P. M. S. Schingo (*) Via Luigi Ronzoni 65, 00165 Rome, Italy e-mail:
[email protected] M. C. Lucchetti : F. Ferro Operative Unit of Andrological and Gynecological Surgery, I.R.C.C.S. Bambino Gesù Pediatric Hospital, Rome, Italy P. Marchetti Department of General Surgery, I.R.C.C.S. Bambino Gesù Pediatric Hospital, Rome, Italy
uterine morphology, the continuity with each vaginal channel (obstructed and nonobstructed), and the bloody nature of the contents. Conclusion Early and accurate diagnosis of this syndrome is important so that adequate and prompt surgical therapy (excision of the vaginal septum) can provide relief of pain and prevent further complications. It is also advisable to look for an obstructed Müllerian system whenever a multicystic dysplastic kidney or the absence of a kidney is discovered in a fetus, or girl postnatally. Keywords Müllerian duct anomaly . Renal agenesis . Haematocolpos . Herlyn-Werner-Wunderlich syndrome . MRI . Ultrasonography
Introduction The internal genital organs and lower urinary tract derive from two paired urogenital structures that develop in both sexes—the Wolffian (mesonephric) ducts and the Müllerian (paramesonephric) ducts [1–3]. In the female, the Müllerian ducts, located just lateral to the Wolffian ducts, which act as inductor elements, grow downwards and towards the midline, crossing the Wolffian ducts, and come into contact with each other. They then fuse to form the uterovaginal canal, from which the fallopian tubes, uterus and the upper two-thirds of the vagina develop [1, 3, 4]. As a consequence of nondevelopment or failure of fusion of the distal segments of the Müllerian ducts, various types of uterine anomalies can ensue, such as hypoplasia/ agenesis, unicornuate, didelphys, bicornuate, septate and arcuate uterus, in addition to a peculiar anomaly of uterine morphology that is related to diethylstilbestrol. These malformations have been subdivided into seven classes by
11 years 6 months
12 years 2 months
4
5
12 years 5 months
12 years 4 months
12 years
14 years
Acute abdominal pain, hyperpyrexia, vomiting Dysmenorrhoea
Regular menstruation, foul-smelling clots in vaginal discharge
Recurrent pelvic pain, irregular menstruation
Didelphic uterus, right hemihaematometrocolpos, periadnexal fluid collection, absent right kidney, hypertrophic left kidney
Bicornuate bicollis uterus, haematocolpos, absent right kidney, hypertrophic left kidney Didelphic uterus, slightly dilated right hemivagina, absent right kidney, hypertrophic left kidney
Didelphic uterus, right hemihaematometrocolpos, divergent horns, periadnexal fluid collection, absent right kidney, hypertrophic left kidney
Bicornuate bicollis uterus, divergent horns, right hemihaematocolpos, absent right kidney, hypertrophic left kidney Didelphic uterus, divergent horns, slightly dilated right hemivagina, absent right kidney, hypertrophic left kidney, right paravaginal Gartner’s duct and blind ectopic ureter
Thick bulging vaginal septum
Resection of the septum, drainage of the obstructed right hemivagina containing congealed blood, dilated right cervix
Resection of the Incomplete septum vaginal septum, congealed blood coming from cranial communication Haemoperitoneum, blood from right fallopian tube
Removal of the septum
Scoliosis
13 years 6 months
Recurrent pelvic Bicornuate uterus, pain, regular haematocolpos, resected menstruation, ureterocele, absent right palpable pelvic mass kidney, hypertrophic left on rectal kidney exploration
Resection of the septum
3
US at 11 years, right renal agenesis and ureterocele
Bicornuate bicollis uterus, divergent horns, right hemihaematocolpos (displaced to the left), absent right kidney, hypertrophic left kidney
14 years
2 14 years 6 months
Rib anomalies, scoliosis, pulmonary artery atresia
Excision of the septum
Foul-smelling vaginal discharge, incomplete vaginal septum, cranially displaced cervices Only one vaginal ostium pervious, appreciable thick vaginal septum Vaginal obstructing septum
Didelphic uterus, divergent horns, left hemihaematocolpos, absent left kidney, hypertrophic right kidney
Bicornuate uterus, left-sided haematocolpos, absent left kidney, hypertrophic right kidney
Polymenorrhoea, dysmenorrhoea, acute abdominal pain, fever
15 years
14 years
1
Prenatal US, left renal agenesis
Associated anomalies
Surgery
Colposcopy
MRI
US
Age at Clinical presentation presentation
Patient Menarche Previous history
Table 1 Clinical histories and imaging findings
658 Pediatr Radiol (2007) 37:657–665
12 years 1 month
13 years
Menorrhagia (one episode) after 3 months amenorrhoea, no pelvic pain
Pelvic recurrent pain
13 years 8 months
11
Regular menstruation, acute abdominal pain, hyperpyrexia, vomiting Bicornuate bicollis uterus, Bicornuate bicollis uterus, right hemihaematocolpos, right hemihaematocolpos, absent right kidney and right absent right kidney and ovary, hypertrophic left kidney right ovary, hypertrophic left kidney Didelphic uterus, diverging Didelphic uterus, diverging horns, left horns, left hemihaematocolpos, hemihaematocolpos, absent left absent left kidney, hypertrophic kidney, hypertrophic right kidney right kidney
Didelphic uterus, left hemihaematometrocolpos, haematosalpinx, haemoperitoneum, absent left kidney, hypertrophic right kidney, left paravaginal Gartner’s cyst and blind ectopic ureter
Didelphic uterus, left hemihaematometrocolpos, haematosalpinx, absent left kidney, hypertrophic right kidney
Regular menstruation, acute dysmenorrhoea
Only one vaginal ostium pervious, vaginal septum
Healed up obstructing vaginal septum
Only one vaginal ostium pervious, thick, obstructing vaginal septum
Bicornuate bicollis uterus, slightly diverging horns, left hemihaematocolpos, absent left kidney, hypertrophic right kidney
Bicornuate bicollis uterus, haematocolpos, absent left kidney, hypertrophic right kidney
Irregular menstruation, progressive pelvic pain
13 years 4 months
12 years 9 months
10
9
11 years 10 years Neonatal 8 months US, multicystic left kidney, nephrectomy 12 years 12 years Neonatal 4 months 1 month US, multicystic left kidney, blind dilated left ureter
8
Excision of the septum, drainage of the obstructed left hemivagina
Haemoperitoneum, “ruptured haemorrhagic ovarian cyst”, right oophorectomy, simple drainage of haematocolpos Resection of the vaginal septum
Resection of the septum, drainage of the obstructed left hemivagina, containing blood clots
Excision of the septum
Resection of the septum, drainage of the obstructed left hemivagina, containing blood clots
Bicornuate bicollis uterus, slightly diverging horns, left hemihaematocolpos, absent left kidney, hypertrophic right kidney
Bicornuate bicollis uterus, left hemihaematocolpos and slightly dilated left cervix, absent left kidney, hypertrophic right kidney
Regular menstruation, cyclic pelvic pain
12 years 7 months
12 years US at 2 months 6 years, left renal agenesis
7
Excision of the septum, drainage of the obstructed right hemivagina, containing congealed blood
Only one vaginal ostium pervious, appreciable thick vaginal septum Only one vaginal ostium pervious, thick, obstructing vaginal septum Obstructing vaginal septum
Didelphic uterus, divergent horns, right hemihaematocolpos, absent right kidney, hypertrophic left kidney
Didelphic uterus, right hemihaematocolpos, absent right kidney, hypertrophic left kidney
Regular menstruation, acute dysmenorrhoea
Surgery
Colposcopy
MRI
US
Clinical presentation
12 years
Age at presentation
11 years 7 months
Previous history
6
Patient Menarche
Table 1 (continued) Associated anomalies
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Fig. 1 Patient 10. a–c Longitudinal (a) and transverse (b, c) US images show dilated vagina containing low-level echoes, bicornuate bicollis uterus, diverging uterine horns, and duplicated endometrial echoes. d–f Sagittal (d) and coronal (e, f) T2-W MR images show
bicornuate bicollis uterus, right hemihaematocolpos, and inhomogeneous high-signal contents. g T2-W coronal MR image shows absence of the right kidney and hypertrophied left kidney. h Diagrammatic representation
the American Fertility Society (now the American Society of Reproductive Medicine) [5]. After completing their fusion, the Müllerian ducts join the dorsal wall of the urogenital sinus, from which the lower third of the vagina develops, while the Wolffian ducts open laterally [1]. Due to the different embryological origin of the upper two-thirds of the vagina, which originates from the Müllerian ducts of mesodermal origin, and the lower third of the vagina, which originates from the urogenital sinus of
endodermal origin, vaginal anomalies, such as hypoplasia/ aplasia, duplication or septa, may exist alone or in association with Müllerian anomalies. A complete longitudinal vaginal septum is present in the majority (75%) of women with uterus didelphys [6–8], and in 25% of women with bicornuate bicollis uterus [6]. The close interrelationship between the urinary and reproductive systems during embryogenesis may explain the coexistence of urinary and reproductive tract abnormalities.
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Fig. 2 Patient 5. a Longitudinal US scan shows dilated right hemivagina and endometrial cavity with low-echogenicity contents, and a periadnexal effusion. b, c Sagittal (b) and axial (c) T2-W MR images show a didelphic uterus and right hemihaematometrocolpos. d Diagrammatic representation
The ureteric bud sprouts dorsally from the mesonephric duct at its opening in the urogenital sinus, evolves into the ureter and grows into the metanephrogenic mass, forming the renal pelvis and intrarenal collecting system [1, 3, 4]. The mesonephric ducts regress in the female, except for some possible remnants: the epoophoron, the paraoophoron, and Gartner’s duct cyst [1]. Disruption of mesoderm development may account for the possible association of axial skeletal abnormalities and Müllerian malformations [9, 10]. Uterovaginal duplication with obstructed hemivagina is most often accompanied by ipsilateral renal agenesis in a rare entity [11–17]. It was recognized as early as 1922 [18] and is sometimes referred to as Herlyn-Werner-Wunderlich (HWW) syndrome [9, 19, 20]. The association of renal agenesis and ipsilateral blind hemivagina was reported as Herlyn-Werner syndrome in 1971 [21], while the association of renal aplasia and bicornuate uterus with isolated haematocervix and simple vagina was reported by Wunderlich in 1976 [22]. Early and accurate diagnosis of this entity is important, so that resection of the obstructing vaginal septum can provide relief of pain and prevent further complications.
Materials and methods Our series includes 11 adolescents, ranging in age from 11 to 15 years, who were admitted to our hospital during the
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period 2000–2006 with symptoms occurring shortly after menarche, such as lower abdominal pain, progressive dysmenorrhoea, irregular menses, discharge of longstanding, partially clotted menstrual blood, menorrhagia, and acute abdominal pain. Two patients (patients 5 and 10) had undergone surgery for acute abdominal pain in other hospitals and were then referred to our children’s hospital. On admission, all the patients underwent US examination (Acuson Sequoia 512 with multifrequency convex probes ranging from 8 to 4 MHz) of the pelvis and urinary system. Subsequently they underwent MRI (Siemens Magnetom Vision 1.5-T with body phased-array coil). Multiplanar T1-weighted (T1-W), T1-W with fat saturation, and T2-weighted (T2-W) sequences were used to assess the vagina, cervix, uterus and ovaries. The presence and position of the kidneys were evaluated both by US and by MRI. After parental informed consent had been obtained, surgical treatment of the haematocolpos/haematometrocolpos was performed as soon as possible. Hormonal suppression of menses was not undertaken preoperatively. All patients were followed postoperatively at 3 months and then annually.
Results The clinical histories and imaging findings are summarized in Table 1. The syndrome was detected between 2 months to 1 year after menarche. Sonography mostly allowed the correct diagnosis by showing uterovaginal duplication (didelphic/bicornuate bicollis uterus) and haematocolpos. Lack of visualization of one kidney confirmed the hypothesis of HWW syndrome. MRI allowed a more detailed assessment of uterine morphology and of the continuity with each vaginal channel. The bloody contents could be assessed on the basis of the signal behaviour. The absence of one kidney was also confirmed (Fig. 1). A dilated endometrial cavity (haematometros) and fallopian tube (haematosalpinx) occurred in some more severely affected patients, as well as bleeding in the periadnexal and peritoneal space (haemoperitoneum) (Fig. 2). In patients 4 and 9 (Fig. 3), mesonephric remnants communicating with the blind hemivagina were observed. They appeared as small tubular structures along the anterolateral aspect of the hemivagina with the same contents, and represented blind ectopic ureters and Gartner’s duct cysts. Müllerian anomalies were not suspected before menarche, even in the girls in whom renal agenesis or multicystic kidney had been diagnosed earlier in childhood, at birth, or prenatally. The previous history, including prenatal and neonatal data, was only retrieved after the appearance of
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Fig. 3 Patient 9. a, b Longitudinal (a) and transverse (b) US images show a dilated left hemivagina and endometrial cavity containing lowlevel echoes, and a dilated left fallopian tube. c–f Coronal T2-W (c), coronal T1-W fat-saturated (d, e) and axial T1-W fat-saturated (f) MR
images show didelphic uterus, left hemihaematometrocolpos and haematosalpinx, small left paravaginal Gartner’s cyst and blind ectopic ureter with bloody contents, communicating with the obstructed vagina. g Diagrammatic representation
symptoms. We can hypothesize that, in newborns, the examination was not accurately extended to the genital system or, in infants, these anomalies were no more evident because of the infantile size and character of the uterus. The right side was affected in 6 patients out of 11. There were no affected siblings or relatives in our series. US was extended to the siblings of two of the patients (patients 4 and 11), and no anomalies were shown.
Discussion The exact aetiology and pathogenesis of HWW syndrome is still unknown [17, 23]. It has been considered to represent anomalous Müllerian (paramesonephric) as well as Wolffian (mesonephric) duct development [17, 24]. The Wolffian ducts, besides giving origin to the kidneys, are also inductor elements for adequate Müllerian ducts fusion.
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Therefore a developmental anomaly of the caudal portion of one of the Wolffian ducts may be the cause of unilateral renal agenesis associated with imperforate hemivagina [4, 11, 16, 19]. On the side where the Wolffian duct is absent the Müllerian duct is displaced laterally so it cannot fuse with the contralateral duct, resulting in didelphic uterus, and cannot come into contact with the urogenital sinus centrally. The contralateral Müllerian duct gives rise to a vagina while the displaced component forms a blind sac— an imperforate or obstructed hemivagina [24, 25]. Mesonephric remnants may remain in the vaginal wall, and occasionally may become cystic (Gartner’s duct and/or cyst) [4]. The vaginal introitus is not involved because of its origin from the urogenital sinus [24, 26]. This rare condition is usually discovered at puberty, shortly after menarche. Recurrent and progressive pelvic pain is the clinical complaint in most patients. Haematocolpos resulting from retained longstanding, partially clotted menstrual blood in the obstructed hemivagina is often clinically detected as a pelvic mass. The syndrome remains unrecognized at first, because the menstrual flow from the unobstructed system may be regular, in spite of a dilated obstructed hemivagina. Haematocolpos is suspected only months after menarche and the diagnosis is generally made only if the suspicion of the existence of this syndrome is raised [9, 19, 24]. A right-sided prevalence of the obstructed system has been described [9, 17, 25]. Clinical presentation may be further delayed, with vaginal discharge of smelly longstanding partially clotted blood, if a communication between the two vaginas exists. Small communications at the low isthmic or cervical level, as well as at the vaginal level, representing either congenital or acquired conditions, can be present [16, 27, 28]. Not only a dilated hemivagina (haematocolpos), but also a dilated uterine cavity (haematometros) and fallopian tube (haematosalpinx) may occur, as well as bleeding into the peritoneal space (haemoperitoneum) as a consequence of blood stasis and retrograde menstruation in the obstructed system. Acute onset of abdominal pain, fever and vomiting can be the presenting symptoms, and haemoperitoneum, due to bleeding from the fallopian tube, can be found at surgery [16, 17, 24]. Endometriosis can result from blood reflux into the abdominal cavity and may have dire consequences [6, 8, 9, 16, 17, 25, 29]. Sonography and MRI are extremely useful in the diagnosis and classification of Müllerian duct anomalies [24]. The evidence of haematocolpos on US, appearing as a fluid collection with low-level echoes, can make the detection of the uterine anomaly (didelphic/bicornuate bicollis uterus) easier [12, 24, 30]. Renal agenesis or multicystic dysplasia is now usually a prenatal or neonatal diagnosis, and female neonates with renal malformations need to be screened for genital
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malformations [3]. It would be advisable to look for an obstructed Müllerian system, whenever a multicystic dysplastic kidney or the absence of a kidney in a fetus or newborn, or the absence of a kidney in a girl is discovered [24]. Early high-grade obstruction of the pelvi-ureteric junction, resulting in multicystic dysplasia and eventually in disappearance of the affected kidney, occurs more frequently in the presence of obstructed Müllerian duct anomalies, and any defect in the Wolffian system (mesonephros) can induce defects in the Müllerian duct at the same level; so the absence of one kidney in a female is almost always associated with genital anomalies [2, 3, 19, 24, 31, 32]. The uterine anomaly can be better detected in the neonatal period, because the uterus, still under maternal hormonal stimulation, is characterized by a prominent myometrium and an echogenic endometrium, and can readily be evaluated [2, 3]. Muco- or hydrocolpos have only rarely been reported in newborns and infants [26, 33]. However, if a cystic mass is detected behind the urinary bladder in a newborn in the absence of a kidney, uterus didelphys with ipsilateral imperforate vagina and hydrocolpos should be considered [24, 26]. The small size and tubular shape of the uterus in infancy makes it almost impossible to evaluate the uterine anomaly in this age group [3, 31]. We believe that follow-up US examination of these asymptomatic patients should be performed until the end of puberty in order to detect genitourinary malformations [31]. It is generally reported that even when follow-up US examinations of the urinary system have been regularly carried out, the uterovaginal anomaly is generally disclosed only when it is looked for after menarche because of dysmenorrhoea and recurrent pelvic pain secondary to increasing haematocolpos [24, 31]. MRI with multiplanar image acquisition provides more detailed information regarding uterine morphology (the disposition of the uterine horns), the cervices, the continuity with the vaginal channels (obstructed and nonobstructed), and the nature of the low-echogenicity contents (T1-W fatsaturated hyperintensity, indicative of blood). The accuracy of MRI for diagnosing uterine malformations is well established [6, 11, 30, 34] and even 100% accuracy has been reported [35, 36]. Differentiation of a bicornuate bicollis uterus (with a certain degree of communication maintained between the two horns) from uterus didelphys, resulting from complete failure of fusion of Müllerian ducts (both entities belonging, however, to class III of the AFS classification), may not be easily achievable, even by means of MRI, which should demonstrate in the former a large fundal cleft and variable caudad fusion and in the latter widely divergent apices, two separate cervices, and in each uterus normal endometrium-tomyometrium width and ratio [5, 6]. However, a longitudinal vaginal septum is present in 75% of didelphic uteri and in
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25% of bicornuate bicollis uteri, and a complication with defects in vertical fusion resulting in oblique or associated transverse vaginal septum is the underlying anomaly of haematocolpos/haematometrocolpos in obstructive forms [6]. Moreover, MRI is helpful in confirming the absence of one kidney [11, 30]. This is the least common anomaly of the Müllerian ducts, but it carries the best prognosis [15]. Early recognition prompts surgical removal of the obstructing vaginal septum, with rapid relief of symptoms and prevention of complications related to chronic cryptomenorrhoea, such as endometriosis, pelvic adhesions, and infectious collections (pyocolpos, pyometros and pyosalpinx) [9, 12, 23]. Surgical excision of the septum and drainage of the obstructed vagina is the definitive treatment [9, 12, 16, 23, 24]. Resolution of the obstruction is also considered mandatory in order to prevent secondary endometriosis [37, 38]. Moreover, fertility, which is not significantly decreased in women with didelphic uterus, can be preserved [8, 19, 20, 24].
Conclusion Uterovaginal duplication with obstructed hemivagina is most often accompanied by ipsilateral renal agenesis as a rare entity, and is sometimes referred to as HWW syndrome; the exact aetiology and pathogenesis are still unknown. It is usually discovered at puberty, shortly after menarche, because of cyclic, increasing lower abdominal pain, secondary to haematocolpos. A pelvic mass is generally appreciable in the adolescent, while only rarely is a dilated hemivagina (muco-/hydrocolpos) discovered in newborns and infants. If a cystic mass is detected behind the urinary bladder in a newborn, in association with the absence of a kidney, the diagnosis of uterus didelphys with imperforate vagina and hydrocolpos should be considered. Whenever a multicystic dysplastic kidney or the absence of a kidney is detected in a fetus or in a newborn, it would be advisable to look for genital anomalies. In the neonatal period the uterus is still rather prominent, under maternal hormonal influence, and anomalies can be better appreciated. When the absence of a kidney is discovered in an asymptomatic infant, the small size and the tubular shape of the uterus make it almost impossible to evaluate uterine anomalies, so follow-up should be performed until the end of puberty.
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