Figure - available from: Journal of Medical Case Reports
This content is subject to copyright. Terms and conditions apply.
Sagittal T2-weighted image showing dilation of cervical canal (short arrow) with abrupt termination at vaginal fornix (long arrow), resulting in obstructed hemivagina. Endometrial cavity was mildly dilated and contained hemorrhagic collection
Source publication
![Fig. 8 Herlyn-Werner-Wunderlich syndrome based on vaginal morphology [9]](publication/336918648/figure/fig4/AS:820046972592128@1572525842322/Herlyn-Werner-Wunderlich-syndrome-based-on-vaginal-morphology-9_Q320.jpg)
Background:
Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The most common presentation is abdominal pain, dysmenorrhea, and abdominal mass secondary to hematocolpos. We present the first case report on Herlyn-Werner-Wunderlich syndrome from B...
Similar publications
Background: Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital urogenital defect. It is detected by unilateral low vaginal obstruction, uterus didelphys, and ipsilateral kidney agenesis. It usually becomes apparent with pain, dysmenorrhea, and presence of a vaginal or pelvic mass. Purulent vaginal discharge may also happen rarely because...
Citations
... 2,3 This is a rare congenital syndrome with the reported incidence of 0.1% to 3% in the general population. 4 Common presenting symptoms include increasing pelvic pain and dysmenorrhea following menarche. 5 In our case the symptoms appeared four years after menarche instead of following the typical pattern of symptom presentation soon after achieving menarche. ...
... 6 Although the aetiology is not well established, it is thought to be a result of environmental and genetic insults to the development of paramesonephric ducts and metanephros. 4 The overall incidence of uterine malformation among females is estimated to be 7 to 10%. Genital developmental malformations usually arise as a result of non-fusion Free Full Text Articles are Available at www.jnma.com.np or failed resorption of the uterine septum. 1 Normally, the fallopian tubes, uterus, and upper two-thirds of the vagina develop from paramesonephric, and the lower one-third vagina from the urogenital sinus. ...
... According to vaginal morphology, HWW syndrome is classified as class 1 (completely obstructed hemivagina) and class 2 (incompletely obstructed hemivagina), both of which are divided into subclasses. 1,3,4 Our patient presented with the classification class 1 and subclass 1.1 (with blind hemivagina). ...
Herlyn-Werner-Wunderlich syndrome is a rare congenital malformation of the Mullerian ducts characterized by uterine didelphys with obstructed hemivagina and ipsilateral renal agenesis. Commonly, such patients present with pelvic pain, dysmenorrhea following menarche, and an abdominal mass secondary to hematometrocolpos. In this report, a case of a 14-year-old female presented with abdominal pain, back pain and acute urinary retention. She attained menarche at the age of 10 years; however, symptoms of dysmenorrhea only appeared 4 years later. She was eventually diagnosed with the help of ultrasound and computed tomography urogram. She was managed conservatively with an incision and drainage procedure and was also advised for resection of vaginal septum. The nonspecific nature of symptoms such as regular menstruation with cyclical abdominal pain impedes the diagnosis which can lead to an array of complications. Awareness of this syndrome can help avoid misdiagnosis and allow for early surgical intervention.
... Therefore, the obstructed side develops hematocolpos, which may evolve with hematometra and hematosalpinx. In these cases, the age of onset of symptoms is earlier with a short time from menarche (Girardi Fachin et al., 2019;Zhu et al., 2015;Nishu et al., 2019). ...
... Considering all the possible anatomical variations, it is understandable that most patients remain asymptomatic until puberty (Nishu et al., 2019). Commonly, after menarche, pelvic pain, dysmenorrhea and menstrual alterations appear, worsening at each menstrual cycle, associated with an increase in abdominal volume and a palpable abdominal mass (Gutiérrez-Montufar et al., 2021;Jomaa et al., 2021;Vo Nhu et al., 2021;Nishu et al., 2019). ...
... Considering all the possible anatomical variations, it is understandable that most patients remain asymptomatic until puberty (Nishu et al., 2019). Commonly, after menarche, pelvic pain, dysmenorrhea and menstrual alterations appear, worsening at each menstrual cycle, associated with an increase in abdominal volume and a palpable abdominal mass (Gutiérrez-Montufar et al., 2021;Jomaa et al., 2021;Vo Nhu et al., 2021;Nishu et al., 2019). It is common to seek emergency services in the context of pain exacerbation, simulating acute abdominal conditions. ...
The Herlyn-Werner-Wunderlich syndrome (HWWS) is characterized by the triad of uterus didelphys, obstructed hemivagina, and renal agenesis. The typical clinical presentation involves chronic pelvic pain, dysmenorrhea, and palpable abdominal mass, related to hematocolpos/hematometra. It is a rare disease, with a challenging clinical and radiological diagnosis. Surgery is the definitive treatment. Complications such as endometriosis, infertility and chronic pelvic pain occur more frequently and severely when diagnosis and treatment are delayed. This is a case report of a twelve-year-old patient admitted to the Gynecology Department of the Federal University of Rio de Janeiro's General Hospital (HUCFF/UFRJ), in March 2021, with progressive symptoms of dysmenorrhea and abdominal distention due to palpable abdominal mass. She had a previous history of congenital solitary kidney. Magnetic Resonance Imaging (MRI) showed a double uterus with hematometra and hematocolpos on the left side, pelvic endometriosis and left renal agenesis. Conservative clinical treatment with inhibition of the hypothalamic-pituitary-ovarian (H-P-O) axis was initiated while a definitive surgical approach was being defined. In June 2022, the patient underwent left hemi-hysterectomy and salpingectomy, achieving full remission of symptoms. Given the rarity of this syndrome and its potential complications, our report aims to familiarize clinicians with it, mostly those who work with children and adolescents, so that more patients have access to early diagnosis and adequate treatment. Consequently, future fertility can be effectively preserved.
... 8 In 30% cases associated renal anomalies are found with MDA like renal agenesis 9 . In HWW Syndrome, there is typically absence of ipsilateral renal agenesis, uterus didelphus and ipsilateral vaginal atresia on the same side 10,11,12 . In our case it was on the left side that both renal and vaginal atresia were noted.The other anomaly found was urethra vaginal fistula which is also very rare 5 . ...
Herlyn Werner Wunderlich Syndrome (HWW) is a rare congenital malformation of female urogenital tracts. We report our first case of HWWS with urethrovaginal fistula in Lady Aitchison Hospital Lahore. A 27 years old lady presented with hematuria and urinary retention having single opening at vulva with menstrual blood and urine draining through it. Examination under anesthesia showed uretheral opening in anterior wall of lower 1/3 of vagina.MRI showed uterus didelphus with two cervices and vagina (atresia of lower 2/3 on left side) and absent left kidney.Surginal treatment by creating a separate opening of neourethra was made by Urology Department of Mayo Hospital. Keywords: Herlyn Werner Wunderlich syndrome, urethrovaginal fistula, urogenital tract
... La población incluida presenta una edad media al diagnóstico de 16,8 años (0 a 53 años). Un reporte de caso de diagnóstico ecográfico antenatal (54), 4 de diagnóstico neonatal (22,26,38,50), 12 entre 1 y 12 años (28,37,48,51 ,52,59,61,66,73,78,80,82), 26 entre 13 y 19 años (27,29,30,32,39,41,42,(44)(45)(46)(47)49,55,56,58,60,63,(68)(69)(70)(71)(72)74,76,77,79) y 15 reportes de mayores de 20 años (31,(33)(34)(35)(36)40,43,53,57,62,64,65,67,75,81). ...
... Los síntomas más frecuentes entre las pacientes adolescentes son dismenorrea y dolor pélvico (21,(27)(28)(29)(30)(31)32,51,55,56,64,67,68,69,71,72,74,75,78,79), solo un estudio reportó asociación a retención urinaria (19). En un estudio se realizó el diagnóstico durante el embarazo (62) asociado a sangrado vaginal. ...
... A continuación, se exponen las características clínicas presentadas por las pacientes adultas con SHWW. El síntoma más frecuente es dismenorrea, con en el 64 % de los casos (12)(13)(14)(15)(17)(18)(19)(20)24,28,29,36,39,(41)(42)(43)49,51,53,60,(63)(64)(65), seguida de dolor pélvico con el 35,2 % de los casos (9,11,13,16,20,27,28,32,34,40,44,46,47,52,58,(60)(61)(62)66,(70)(71)(72)(73)(74)(76)(77)(78)(79)(80). Se identificó endometriosis en un 10,3 % de casos (14,20,24,26,63). ...
Objective:
To report a case of Herlyn-Werner-Wunderlich syndrome (HWWS) and conduct a review of the literature to determine clinical and imaging findings in these patients.
Material and methods:
A 16-year-old female patient who presented to a military hospital in Bogotá complaining of recurrent pelvic pain, receiving a final diagnosis of HWWS. A systematic search was conducted in the different databases of systematic reviews, cohort studies, case series and case reports of patients of any age diagnosed with HWWS. Clinical presentation characteristics and the most frequent diagnostic technologies used were obtained. A narrative summary of the findings is presented.
Results:
Overall, 77 publications with a total of 676 patients were included. The most frequent symptom was dysmenorrhea (63.9%), followed by pelvic pain (35.2%). The most frequently used diagnostic technologies were pelvic ultrasound in 92.1% of cases and nuclear magnetic resonance in 74.6%. Hysteroscopy and laparoscopy are seldom used for diagnosing this condition.
Conclusion:
HWWS is an infrequent disease condition. It must be part of the work-up for renal agenesis in pediatric patients and of the differential diagnosis in primary dysmenorrhea in adolescence. The role of hysteroscopy in this condition must be further assessed in larger cohort studies.
... Absence of one kidney by ultrasound in small hospitals should alert clinicians about this syndrome. Radiological review by Khaladkar et.al 12 showed an absence of left kidney and a study by Nishu et.al 13 found non-visualization of ipsilateral kidney by ultrasound similar to the ultrasound findings in our patient. Similar finding of an absent right kidney and possible uterine anomaly on ultrasound which was later confirmed by MRI was described by Del Vescovo et.al 11 . ...
Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital syndrome with features of uterus didelphys, ipsilateral absent kidney and obstructed hemivagina. Ultrasound findings of absent kidney or abnormalities in the kidney should alert the clinicians about the syndrome. Complications like endometriosis, infertility and pelvic inflammation occurs with late diagnosis.A 42-year-old nulliparious married woman who presented with right sided abdominal pain to the National Referral Hospital was diagnosed to have HWWS. The diagnosis was supported by ultrasound, CT and MRI findings. Patient was treated for endometriosis and had symptomatic improvement. The diagnosis of the syndrome is challenging as it is rare but clinicians should suspect the syndrome in women who present with infertility. Ultrasound scan is the basic investigation to screen the syndrome which is available in most hospitals.
... It is first reported in a 16-year-old patient in 1922 [2]. The etiology is unknown, but it implies a failure of the müllerian ducts to fuse during embryogenesis [1,3]. Furthermore, genetic and environmental factors may affect embryogenesis. ...
... Furthermore, genetic and environmental factors may affect embryogenesis. Its incidence varies between 0.1 and 3% [3].Herlyn Werner Wunderlich syndrome is defined by the classic triad: a didelphic uterus, hemivaginal septum, and unilateral renal agenesis [1]. However, cases have been reported without renal agenesis [4]. ...
... Herlyn Werner Wunderlich syndrome represents a failure of lateral and vertical fusion [9]. The etiology is unclear, but environmental and genetic factors may influence embryogenesis [3]. ...
Introduction
Herlyn-Werner-Wunderlich syndrome is a rare congenital disorder that consists of a didelphic uterus, hemivaginal septum, and unilateral renal agenesis. Both diagnosis and management can be challenging in low-income countries.
Case presentation
a 13-year-old female who experienced recurrent pelvic pain, dysmenorrhea, and abdominal mass for one month. We could not obtain the MRI as it was unavailable in our hospital, and the patient cannot afford its expensive cost in a private center. Instead, US showed an absent right kidney and doubled uterus with a heterogeneous cystic formation. A pelvic CT scan revealed an absent right kidney, an enlarged left kidney, a double uterus, and a left hematocolpos. We established the diagnosis of Herlyn-Werner-Wunderlich syndrome and started planning for correcting the anomalies via laparotomy.
Discussion
ultrasound detects uterus didelphys, hematocolpos, hematometrocolpos, and renal agenesis but cannot identify the vaginal septum and the type of müllerian defect. The MRI is more convenient for a thorough assessment, but it is a high-priced diagnostic method.
Conclusion
although MRI is the gold standard for establishing the diagnosis and preoperative planning, ultrasound and CT scan can be an alternative, less expensive, and reliable approach. In addition, Laparoscopy or vaginoscopy may not be available in these countries. Therefore, correcting the anomalies can be achieved with laparotomy, which results in acceptable outcomes without any complications.
... Now an analysis of HWWs clinical characteristics in a large sample of patients (more than 80 cases) has been reported only one [6]. Most of the studies are in the form of case reports, and there are different manifestations of diseases [7][8][9][10][11]. Early accurate diagnosis and resection of the oblique vaginal septum are the objective. ...
Background: To analyze the clinical characteristics of patients with Herlyn–Werner–Wunderlich syndrome (HWWs) in two classification methods.
Methods: 87 HWWs patients syndrome admitted to the Shengjing hospital of China Medical University between April 2003 to June 2020 were retrospectively reviewed.
Results: Type II of average age at time of diagnosis (25.11±8.96) was significantly longer than the other two types(13.06±2.55; 13.06±2.55). The menarche and the interval between menarche and time of diagnosis was significant difference between types I and II. The average age at time of diagnosis, the menarche and the interval between menarche and time of diagnosis was significant difference between classification I and II. Cystic mass in vaginal wall was a significant difference between type I and II. Endometriosis of type I and II was a more common than type III. Sex life was a significant difference between type I and II. Mediastinum of uterus of type II was a more common than type I and III. The time of diagnosis, menarche, interval between menarche and time of diagnosis, cystic mass in vaginal wall, abnormal vaginal discharge, sex life, changes of urination and defecation and mediastinum of uterus were significant difference between classification 1 and classification 2. The site of olique septum vaginalis was significant difference in two classification methods. The absence of kidney in HWWs type was significant difference in two classification methods.
Conclusion: Type I HWWs should be suspected in cases involving adolescent girls presenting with cyclical pelvic pain, change of urination and defecation. Two classification can be easy to treat in clinical work.
... A typical presentation of this syndrome is dysmenorrhea in adolescent age group, a few months to years after attaining menarche. 9 Patients can also present with pelvic pain, mass, abnormal vaginal discharge, infertility, endometriosis, complicated pregnancy, and labor. 10 The initial clinical diagnosis is incorrect in the majority of the cases because of its rare incidence and misleading presenting signs and symptoms. ...
Herlyn–Werner–Wunderlich syndrome is a rare complex congenital anomaly of the urogenital tract involving Mullerian ducts and mesonephric ducts. It is also called OHVIRA syndrome (Obstructed Hemivagina and Ipsilateral Renal agenesis). It is characterized by a triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. Patients usually present after menarche with pelvic pain, dysmenorrhea, mass, and rarely with primary infertility in later years. We report a case of a multiparous female who presented to the hospital with pain in lower abdomen for the past 2 months and acute retention of urine for 1 day. This is an atypical presentation in a multiparous female that has been described in a single case report so far. ¹ Intravenous pyelogram and MRI of the patient revealed uterine didelphys, obstructed right hemivagina causing hematohemicolpos and right renal agenesis. Thorough knowledge of imaging features can enable a radiologist to make a correct diagnosis even in an atypical presentation.
... Herlyn-Werner-Wunderlich Syndrome (HWWS) is a rare congenital malformation syndrome characterized by a triad of uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis [1], also known as OHVIRA [2]. OHVIRA stands for obstructed hemivagina and ipsilateral renal anomaly [3]. The prevalence of obstructive Müllerian malformations is unknown, but is reported to be 0.1% to 3.8% in women [4,5]. ...
... Tuna et al. state that 6% of patients with uterine duplication have an obstructed hemivagina, and that renal agenesis is found in 63-81% of uterine duplications and in 92-100% of those with obstructed hemivagina [6]. According to Nishu et al., the frequency is 0.1-3% [3], while Fachin et al. state that it is 5% [7]. The lack of unambiguous information may be due to the fact that there is a lack of common terminology, and that could be the reason that it is reported as rare. 2 of 9 Moreover, it should be remembered that in some cases the disease is asymptomatic during childhood and puberty. ...
Background:
Herlyn-Werner-Wunderlich Syndrome is a rare malformation syndrome characterized by uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. Symptoms appear most often after menarche and are secondary to hematocolpos. We compare clinical symptoms, diagnosis and treatment of two patients, a 13-year-old and a 17-year-old.
Case report:
Despite the non-uniform clinical symptoms, it should be noted that in both patients, the 13-year-old and the 17-year-old, hematocolpos, which was probably the cause of lower abdominal pain, was diagnosed with ultrasound. The diagnosis was complemented by laparoscopy, which determined the diagnosis of malformation of uterus didelphys with obstructed hemivagina. The patients had a history of kidney agenesis, which, after gynecological diagnosis, turned out to be ipsilateral. In the 13-year-old, agenesis was diagnosed by uroscintigraphy, while in the 17-year-old it was diagnosed by urography. Incision and drainage of the residual vagina was performed in the course of therapeutic management. In both cases, the clinical situation required a repeated widening of the orifice.
Conclusions:
Lower abdominal pain accompanying hematocolpos suggested Herlyn-Werner-Wunderlich Syndrome (HWWS) as the cause of symptoms. 3D transvaginal ultrasound enabled the determination of a congenital uterine defect with high probability, although inconclusive cases required confirmation by laparoscopy. Incision of the blocked vagina and drainage of hematocolpos were the key components of treatment. The treatment of HWWS is a multi-step process.
... Etiologi pasti dari sindrom Herlyn-Werner-Wunderlich masih tidak diketahui dan kejadiannya kira-kira 0,1% hingga 3,8% (Ekici, Oyardi, Dagistanli, Albayrak, & Cetin, 2019). Magnetic Resonance Imaging (MRI) abdomen merupakan salah satu alat penunjang diagnostik yang efektif untuk mengetahui kelainan anatomis abdomen dengan pilihan sekuen protokol yang tepat sehingga mendapatkan gambaran atau citra MRI yang optimal untuk menegakkan diagnosa sindrom Herlyn-Werner-Wunderlich (Nishu et al., 2019). ...
Background: Herlyn-Werner-Wunderlich syndrome (HWW) is a very rare genetic syndrome, a combination of anomalies between the mullerian and mesonephric ducts characterized by uterine didelphys, hemivaginal obstruction, and ipsilateral renal agenesis, also known as Obstructed Hemivaginal syndrome and Ipsilateral Renal Anomaly (OHVIRA). Magnetic Resonance Imaging (MRI) is an effective diagnostic support tool for finding out anatomical abdominal abnormalities with the right choice of protocol sequences to obtain optimal MRI images. Method: This study used a qualitative descriptive approach by presenting a case of a 20 year old female patient who had complaints of lower abdominal pain, no previous medical history and irregular menstruation. MRI Abdomen without contrast was performed using an MRI 1.5 Tesla signa explorer GE Healthcare at EMC Tangerang Hospital. Using the Coronal T2 SSFSE sequence, T1 fat sat LAVA, Axial T2 SSFSE, T1 fat sat LAVA breath hold method and Coronal 3D IFIR (MRA renal without contrast). Specifically for the pelvic area with the Axial T2 Propeller, Coronal T2 fat sat, and Sagittal T2 sequences. Result: In the Axial T2 propeller sequence, pelvic area retrieval revealed two separate uterine corpuses (uterus didelphys) accompanied by a heterogeneous oval lesion most likely originating from the left side of the vagina (hemivagina). Whereas in the Coronal T2 sequence the whole abdomen was taken and confirmed by the Coronal 3D IFIR, the right kidney was anatomically visible while the left kidney was not visible (agenesis). Conclusion: Abdominal MRI is a very accurate diagnostic tool to perform Herlyn-Werner-Wunderlich (HWW) syndrome without contrast agent with SSFSE T2 sequences, T2 propeller and 3D IFIR which have an important role in visualizing the quality of MRI images.
