Herlyn-Werner-Wunderlich Research Articles

A Rare Cause of Abdominal Pain: Herlyn-Werner- Wunderlich Syndrome

Herlyn-Werner-Wunderlich (HWW) syndrome is a congenital urogenital syndrome. Typical features are unilateral blind-ending hemivagina with uterine didelphy and ipsilateral renal agenesis. Patients usually progress asymptomatically until menarche, and they are diagnosed with radiological imaging by applying to the hospital with these conditions such as abdominal pain, abdominal bloating, primary amenorrhea along with menarche. In this case report, it is aimed to present a patient who applied to our emergency department with abdominal pain and is diagnosed with HWW syndrome. In this case, it is emphasized that emergency physicians should regard to HWW syndrome in the differential diagnosis of abdominal pain.

Diagnosis approach, treatment, evaluation and fertility preservation in patient with Herlyn-Werner-Wunderlich syndrome: a case report

Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare female congenital anomaly of urogenital tract. The definite etiology of HWW syndrome is still unknown. It may be caused by the abnormal development of Mullerian and Wolffian ducts. The characteristic triad of this syndrome includes didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis, recently known as Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome. A 17 y.o. presented with intermittent lower abdominal pain. Physical examination revealed in normal limit. From the ultrasonography examination, it was found uterus didelphys with left hemiuterus hematomethra, hematocolpos, with normal anatomy and function of the right kidney, but there was no left kidney. From pelvic magnetic resonance imaging (MRI) and laparoscopy showing a complete duplication of the uterus from the horn to the cervix with no connection between the two uterine cavities. Both ovaries were normal. The right fallopian tube was normal. The left tube was enlarged, attached to the uterus and the left ovary. No left kidney was found. From the previous surgery, there was a misdiagnosed with brown cyst. Physical examination such as rectal toucher often missed, regardless it can be used to determine some differential diagnosis. MRI is most accurate for providing details regarding the altered anatomy and for identifying associated hematocolpos, hematosalping or hematometra for HWW syndrome cases. The laparoscopy guiding diagnostic and operative management of the vaginal septectomy procedure was performed. In conclusion, misdiagnosis of HWW syndrome can be occurred on ultrasound examination. MRI is an examination that is considered appropriate to help confirm the diagnosis. Laparoscopy can be used with the aim of establishing a diagnosis or in surgical management, in this case, a septectomy is performed.

Herlyn-Werner-Wunderlich: A Rare Disease

Herlyn-Werner-Wunderlich (HWW) is a rare Mullerian disorder characterized by uterine didelphys, hemivaginal obstruction, and ipsilateral renal agenesis. This paper aimed to describe a rare case of HWW in order to give better understanding of the disease, as well as diagnosis and treatment. Then urgent action is taken in the form of incision and drainage of pus. Methods: We searched medical record of a 17-year-old girl complaining of vaginal pain since menarche. Ultrasound examination found hematocolpos and vaginal septum, pelvic MRI found uterus didelphys and left renal agenesis, then the septal incision was performed to facilitate pus drainage. Conclusion: Herlyn-Werner-Wunderlich is a rare condition with varying age of onset. Anamnesis, physical examination, ultrasonography, and MRI are needed to confirm diagnosis. As an urgent measure, a septal incision and pus drainage are performed to reduce symptoms and complications that could occur in the future. In addition, the patient needs to undergo septal resection as a follow-up treatment which can only be done in a hospital where urogenital and reconstruction divisions are available.

Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome–A “no-touch-approach”: A case report

<b>Introduction:</b> Herlyn-Werner-Wunderlich (HWW)/obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare Müllerian duct anomaly (MDA). Usually presenting after menarche classically with dysmenorrhea. Here we present a challenging case, where the diagnosis was missed initially.<br /> <b>Case presentation:</b> A 10-year-old girl presenting with dysmenorrhea and constipation, treated repeatedly with analgesics, laxatives and enema. A pelvic ultrasound and magnetic resonance imaging (MRI) confirmed the diagnosis of OHVIRA syndrome, which requires surgical intervention. We describe a “no -touch- technique” for resecting the septum. The patient had immediate relief of symptoms and a smooth post-operative recovery.<br /> <b>Conclusions:</b> Early diagnosis and surgical management are crucial not only to relieve symptoms but to prevent long term complications that may affect the patients’ reproductive potential. Despite pelvic ultrasound being an affordable and a useful diagnostic tool, the diagnosis can be missed by inexperienced operators. Therefore, MRI is recommended to confirm and classify MDAs. The hysteroscopic “no-touch-technique” to resect the septum is an effective, safe and a convenient approach to treat the condition while preserving hymen integrity.

Herlyn-Werner-Wunderlich (HWW) syndrome: a case report

Herlyn-Werner-Wunderlich (HWW) syndrome OHVIRA syndrome (Obstructed HemiVagina Ipsilateral Renal Agenesis) is rare type of Mullerian duct anomaly, with uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Usually presenting in post-pubertal adolescent girls presenting as pain abdomen or pelvic masses on the side of the obstructed Hemivagina. Here we represent a case report of a 14-year-old girl who presented to our outpatient department complaining lower abdominal pain for 2 months with previous normal menstrual cycles. On General Physical examination well-developed breasts and normal axillary and pubic hair distribution were noted. The Baseline laboratory analysis and hormonal profile were reported normal. Magnetic Resonance Imaging (MRI) provided a fruitful help in correct diagnosis of this rare anomaly. The treatment modality being the surgery to relieve obstruction and its associated complications.

Two Cases of Herlyn-Werner-Wunderlich Syndrome with Severe Dysmenorrhea, One Misdiagnosis and One Missed Diagnosis

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare variant of mullerian duct anomalies characterized by the triad of uterine didelphys, obstructed hemivagina and ipsilateral renal agenesis. We report two cases of HWW syndrome in young women with abdominal pain. This diagnosis can easily be missed if a clinician is not aware of the syndrome. In one case, the computed tomography image of obstructed hemivagina with hematocolpos was initially mistaken for a cystic tumor of adnexa by the radiologist; in the other case, correct diagnosis was missed for years despite numerous visits to different hospitals due to lack of awareness of the syndrome.

MRI presentations of Müllerian duct anomalies in association with unilateral renal agenesis

To evaluate the presentations of Müllerian duct anomalies (MDAs) associated with unilateral renal agenesis (URA) on magnetic resonance imaging (MRI). From 2014 to 2021, 92 patients with MDAs coexisting with URA who had undergone MRI were reviewed, and imaging patterns were analysed. Based on the different imaging patterns, there were 74 cases of Herlyn-Werner-Wunderlich (HWW) syndrome (80.4%), nine cases of unicornuate uterus (10.9%), and nine cases of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome (8.7%). In HWW syndrome, URA was ipsilateral to the vaginal or cervical obstruction. All the nine cases of unicornuate uterus were obstructive, and the most common subtype was unicornuate uterus with a non-communicating functioning rudimentary horn. The other two cases of unicornuate uterus with no rudimentary horn were obstructed due to cervical os obliteration, one of which was complicated with contralateral absent ovary. URA was contralateral to the unicornuate uterus in all cases. In MRKH syndrome, seven patients had bilateral rudiments and ovaries, all of which were unilaterally or bilaterally located above the pelvic brim, and URA was ipsilateral to the ectopic ovary in all cases. The other two patients had unilateral absent rudiment, ovary, and ipsilateral URA. MRI presentations of URA-associated MDAs are diverse, with HWW syndrome being the most common form, followed by different patterns of unicornuate uterus and MRKH syndrome. An ectopic or absent ovary might be associated with URA in MRKH syndrome and unicornuate uterus.

Malformasi Genital Perempuan: Kasus di RSUP Dr. Sardjito Yogyakarta Tahun 2019

BackgroundReproductive tract malformations are rare in general population but are commonly encountered in women with infertility and recurrent pregnancy loss. Their true prevalence in the general population is not absolutely known mainly owing to methodological bias. Common uterine anomalies are important owing to their impact on fertility, and certain Mullerian malformations are particularly important because they cause serious clinical symptoms and affect woman’s quality of life. Identification of symptoms and timely diagnosis are an important key to the management of these defects. Although MRI being gold standard in delineating uterine anatomy, recent advances in imaging technology, specifically 3-dimensional ultrasound, achieve accurate diagnosis. Surgical management depend on the type of anomaly and its complexity and also involves multiple specialties; thus, patients should be referred to centres with experience in the treatment of complex genital malformationsObjectiveDetermined the description of cases of female genital malformation at Dr. Sardjito Hospital in 2019.MethodThis is descriptive study. The subject in this study were all new patient with female genital malformation at Obgyn Polyclinic Dr. Sardjito Hospital January-December 2019. Obtained 30 cases of female genital malformation.ResultThere is 30 cases of genital malformation in 2019. They were 4-44 years with mode at group 16-20 years. They consist of vaginal and cervicovaginal agenesis 14 cases, Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrom 6 cases, hematometra of hemiuterine 4 cases, Herlyn-Werner-Wunderlich (HWW) syndrome 3 cases, imperforate hymen, didelphys uterus and genital ambiguous each 1 case. Five cases of cervicovaginal agenesis have been done sigmoid vaginoplasty, all of them have good sexual function after procedure. One case MRKH syndrome has been done sigmoid vaginoplasty with good sexual function after that. Two cases HWW syndrome have been surgical treatment that are vaginal septectomy and vaginal septectomy with laparascopic cystectomy.ConclusionThe correct knowledge of embryology of the genitourinary is essential for the understanding, study, diagnosis and management of genital malformations, especially complex ones and those that lead to reproductive problems. Surgical techniques for correcting genital malformations depend on the type of anomaly and complexity.

Preoperative MRI presentations of Herlyn-Werner-Wunderlich syndrome.

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare complex female urogenital anomaly, with diverse anatomical presentations. Due to obstruction, most patients with HWW syndrome need to be addressed surgically. The treatment strategy should be tailored to the different anatomical variants of each patient. Therefore, a detailed and comprehensive preoperative evaluation is needed. In this review, we describe the embryology and clinical manifestations of HWW syndrome and discuss and illustrate its diverse preoperative magnetic resonance imaging presentations to guide clinical treatment.

Successful pregnancies in an adolescent with Herlyn-Werner-Wunderlich (HWW) syndrome: a case report and literature review

BackgroundMüllerian anomaly is a congenital defect in the development of the female reproductive system that varies according to the stage of developmental failure resulting in agenesis, hypoplasia, or fusion defect in one or both Mullerian ducts; in such patients, it is expected to have higher pregnancy and delivery complications.Case presentationThis case presentation of a 14-year-old adolescent girl with uterine didelphys, obstructed right hemi-vagina, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome, HWW) that was corrected surgically, then got married at the age of 16 with successful two consecutive term uncomplicated pregnancies of favorable maternal and fetal outcomes without a history of any miscarriage or recurrent collections.ConclusionsHerlyn-Werner-Wunderlich syndrome is a rare case and represents a challenge to diagnose and treat especially in pediatrics and adolescents. Early recognition and treatment will help to improve outcome and reduce possible complications resulting from obstruction with retrograde menstruation and its consequences. Successful early management of our patient improved her quality of life by relieving her cyclical obstructive pain, preserve fertility, and normal sexual life.

Surgical therapy for herlyn-werner-wunderlich (HWW) syndrome cases

Introduction: One of the rare forms of Mullerian congenital ducts is Herlyn-Werner-Wunderlich (HWW) syndrome. HWW syndrome is usually found at puberty with nonspecific symptoms. Pelvic pain can be found both acute and chronic, dysmenorrhoea, as well as hematometra and hematocolpos. Early detection and treatment results in a good prognosis with fertility preservation. Case presentation: Two cases of Herlyn-Werner-Wunderlich (HWW) syndrome surgically performed were reported at Dr. Sardjito Yogyakarta Indonesia Central General Hospital in 2017-2018. Both cases are still children and at the age of puberty. The chief complaint in both cases it was reported to have the same main complaint, abdominal pain, with the condition having never experienced menstruation yet. On further examination it is known that pain is caused due to hematometra and hematosalping that occur in both cases. The first case with hematometra in the right hemiuterus and right hematosalpinx, while second case with hematometra in the uterus didelphys and bilateral hematosalpinx. One case with a normal vagina, and the other case of vaginal agenesis. There was previous history of surgery in both of cases. There are abnormalities in the urinary tract that occur together with these genital abnormalities. Surgical therapy is carried out with the main goal being to drain menstrual blood from hematometra and hematosalpinx. In first case which has a vagina within the normal range (9 cm long) and the inferior area of ​​the right hemiuterus which is bulging toward the vagina, an area can be identified for the drainage incision to drain the entire hematometra and hematosalpinx. In the second case with vaginal agenesis, neovagina which is made by sigmoid colon vaginoplasty surgery can be a way of menstrual blood flow for a lifetime. Conclusion: Clinical manifestations of Herlyn-Werner-Wunderlich (HWW) syndrome cases can be classified into 2 groups, complete and incomplete. Surgical treatment is based on the condition of the uterus and vagina. In the condition of cervical agenesis but with a normal vagina, surgery can be done by making an incision in the drainage of the uterine cervical tissue. If vaginal agenesis is obtained, vaginoplasty surgery can be performed, and one of the options is sigmoid colon vaginoplasty.

Gynatresia in Herlyn-Werner-Wunderlich Syndrome: A Case Report

Background: Female genital malformations are a consequence of abnormalities in the differentiation of the Müllerian ducts at different phases during embryogenesis, resulting in a variety of clinical outcomes. HerlynWerner-Wunderlich (HWW) syndrome is a rare congenital anomaly of the female urogenital tract involving the paramesonephric ducts (Müllerian ducts). The syndrome is characterized by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. Case: This paper describes the case of a 14-year-old girl presenting with pelvic pain, abdominal swelling and the triad that is characteristic of HWW syndrome. Diagnosis was based on the patient’s clinical history and imaging findings. The patient underwent surgery involving complete drainage of a hematocolpos retained in the obstructed vaginal cavity and resection of the vaginal septum. The procedure was successful in providing symptomatic relief. Twelve months following surgery, the patient’s menstrual cycles were regular, with no dysmenorrhea. Discussion: Greater understanding of this syndrome is crucial in ensuring that diagnosis is reached at an early stage, thus preventing complications such as retrograde menstruation, endometriosis, infections, adhesions, infertility, and acute or chronic pelvic pain.

Herlyn-Werner-Wunderlich syndrome: Case report

Introduction: Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital malformation of the urogenital tract characterized by the triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis, which is usually diagnosed after menarche. It is treated by resecting the vaginal septum and draining the obstructed hemivagina and has a favorable postoperative prognosis. Case presentation: This is the case of 22-year-old woman with a 9-year history of dysmenorrhea, pelvic pain, and irregular menstrual cycles that started after she had her first menstruation, who visited the gynecology service of a secondary care hospital in Sogamoso, Boyacá (Colombia). The patient reported having visited multiple specialists and being treated with vitamin E, metformin, and contraceptives, without experiencing an improvement of symptoms and signs. After being assessed, and taking into account ultrasound and MRI findings, she was diagnosed with HWW syndrome. She underwent a colpotomy plus resection of right paracervical mass of approximately 60x60mm and vaginal septum, achieving complete resolution of symptoms. Finally, 10 months after the surgery, she reported being pregnant without complications. Conclusion: HWW syndrome is a rare malformation that should be considered as a differential diagnosis in women of any age with paramesonephric duct anomalies, pelvic pain, dysmenorrhea, and presence of masses in the genital tract, since its early diagnosis and timely treatment considerably improve the quality of life of these patients by reducing the severity of symptoms, decreasing the incidence of complications, and improving obstetric prognosis.

Herlyn Werner Wunderlich Syndrome with Vagino-umbillical Fistula: A Most Unique Presentation

The Herlyn–Werner–Wunderlich (HWW) syndrome is an uncommon Mullerian duct anomaly composed of a triad of obstructed hemivagina, renal agenesis, uterine didelphys with a prevalence of [Formula: see text] females. Aside from its usual presentation of cyclic hypogastric pain and abdominal mass, there has been no other reported case in literature of the syndrome presenting with a vagino-umbilical fistula. Presented here is a unique case of a 35-year-old woman presenting with purulent, foul-smelling discharge extruding from a fistula on her umbilicus and her vagina associated with a tender hypogastric mass. Three-dimensional transvaginal ultrasonography aided the diagnosis of HWW syndrome with a concomitant vagino-umbilical fistula. Excision of the vaginal septum together with antibiotic coverage resolved her symptoms. The syndrome’s embryopathogenesis, classification, diagnostics, definitive surgical management and other issues during follow up are discussed.

Herlyn-Werner-Wunderlich Syndrome Presenting with Abdominal Pain: A Case Report

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly characterized by triad of uterus didelphys, obstructed hemi vagina, and ipsilateral renal agenesis. The most common presentation is abdominal pain, dysmenorrhea, and abdominal mass secondary to hemi-hematometrocolpos. We report an emergency presentation of this syndrome during the pandemic of COVID-19 in Qatif Central Hospital, Saudi Arabia, April 2020. An 11-year-old Saudi girl presented to the emergency room with three months history of cyclical lower abdominal pain. The pain was progressive, continuous and not relieved by analgesics. Abdominal examination revealed a tender abdominal mass mainly in the left iliac fossa, Ultrasound evaluation showed two uterine bodies. The left uterus was distended with complex fluid. Pelvic MRI findings consistent with Herlyn-Werner-Wunderlich (HWW) syndrome were found with uterine didelphys and left sided hematometra resulting from obstructed hemi-vagina and ipsilateral agenesis of the left kidney. Resection of the vaginal septum and drainage of hematometra was done. The patient recovered with normal cyclical menstruation. Cyclical or continuous lower abdominal pain with or without amenorrhea is the usual presentation of HWW syndrome during adolescence. Diagnosis is made by ultrasonography and MRI. Early diagnosis and accurate management can provide pain relief, prevent future complications, and preserve fertility.

Diagnosis and treatment of Herlyn-Werner-Wunderlich syndrome: a case report

IntroductionHerlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly characterized by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. It is one of the rare anomalies of the genitourinary system and is usually identified in the first few years after menarche.Case reportAn 18-year-old patient presented with painful menses for 1 year and was suspected for vaginal haematoma. Symptoms appeared 5 years after menarche.DiscussionMagnetic resonance imaging of the pelvis revealed a congenital anomaly of the genital tract. Two steps of surgical management were performed. First, a hysteroscopic incision of the vaginal septa and evacuation of vaginal haematoma were performed. Subsequently, the vaginal septum was partially excised during laparohysteroscopy.ConclusionsIpsilateral renal agenesis was confirmed by ultrasound during hospitalization, and the diagnoses of obstructed hemivagina and ipsilateral renal anomaly syndrome were established.The aim of this study was to present the diagnostic and therapeutic approaches in HWW syndrome, and to draw attention to the challenges in its diagnosis, which can coexist with multiple complications.

PERANAN MRI ABDOMEN PADA KASUS HERLYN WERNER WUNDERLICH

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.

Herlyn–Werner–Wunderlich syndrome: diverse presentations and diagnosis on MRI

To evaluate the diverse presentations of Herlyn-Werner-Wunderlich (HWW) syndrome on magnetic resonance imaging (MRI) and its surgical correlations. From 2014 to 2019, a total of 40 patients with HWW syndrome underwent pelvic MRI. All MRI images were reviewed by two experienced radiologists in consensus. Uterine anomalies, obstruction sites, laterality and communications, urinary anomalies, and other abnormal findings were recorded. MRI findings were correlated with ultrasonography and surgical results. On MRI, the uterus was didelphic in 25 (62.5%), septate in nine (22.5%) and bicornuate in six cases (15%); an ipsilateral vaginal septum was found in 30 cases (75%), and ipsilateral cervical obstruction (obliterated cervical os) was found in 10 cases (25%). Twenty-seven patients underwent surgical treatment. MRI diagnoses of obstruction sites correlated completely with the surgical results. Although obstruction communications could not be found with ultrasonography, MRI identified nine cases of cervical communication, but failed to detect the five cases of surgically confirmed fistula on the vaginal septum. Moreover, MRI identified nine cases of ipsilateral ureteral remnants and/or paravaginal cystic structures, all with insertion into the obstructed vagina or cervix, and one case of ipsilateral ureterocele. HWW syndrome can present as different combinations of uterine anomalies, unilateral cervico-vaginal obstruction, and ipsilateral renal anomalies; ipsilateral paravaginal cystic structures and/or ureter remnants inserting into the obstructed vagina or cervix can occasionally be seen. MRI can provide a comprehensive preoperative evaluation and better identification of obstruction sites, but seems insensitive for detecting vaginal communications in HWW syndrome.

Intraoperative Endoscopic Ultrasound Guided Surgical Treatment of Herlyn-Werner-Wunderlich Syndrome. Case Report and a Systematic Literature Review

Aim: Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal-ureteral agenesis, due to an embryogenesis defect. Clinically, HWW results in hematometrocolpos on the obstructed hemivagina side, which produces a mass effect with pain. The diagnosis is difficult because this syndrome is infrequent, and its clinical presentation is variable. Early detection and treatment are important to prevent further complications that could affect the reproductive performance. Ultrasound (US) is the first step when a Mullerian anomaly is suspected but MRI has a higher accuracy to manage surgery. Surgical treatment consists in vaginal septum excision. This review provides information about HWW syndrome and about its diagnosis and treatment. The main objective of this report is to illustrate the clinical presentation, the ultrasound features of a rare syndrome and the use of a laparoscopic ultrasound guidance to allow a fertility sparing treatment without complications. Material and Methods: A search of PubMed Database identified articles published from the inception to February 2019. Results: 186 articles were identified: 125 articles were excluded for any reason. Overall, 63 articles were incorporated for further assessment. Conclusions: The case reported was treated successfully by minimally invasive surgical drainage procedure supported by full time intraoperative ultrasound guidance. Only few researches described transvaginal and transabdominal probe use during surgery; this is the first case in the literature in which a laparoscopic ultrasound probe was used to guide surgeons in the excision of the vaginal septum to prevent complications and to preserve future fertility.

Herlyn-Werner-Wunderlich Syndrome with Central Precocious Puberty: A Case Report

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly of the genitourinary tract comprising uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Patients with HWW syndrome usually present symptoms such as dysmenorrhea, abdominal pain, pelvic mass, and purulent vaginal discharge. If not treated at an appropriate time, complications such as infertility, endometriosis, pyosalpinx, and subsequent pelvic adhesions may occur. Here, we report a case of HWW syndrome in a 7-year-old-girl who was also diagnosed as having central precocious puberty. She was brought to the pediatric department with chief complaints of lump in her breast and vaginal discharge. When she was around 2 months old, she was confirmed to have a single kidney on ultrasonography. We checked her past medical history and diagnosed her as having HWW syndrome based on the results of imaging studies, including abdominal ultrasonography and pelvic magnetic resonance imaging. She underwent treatment with gonadotropin-releasing hormone analogue for 2 years. During 24 months of follow-up, she showed no serious problems or complications. If renal anomalies are identified immediately after birth or in infancy, further screening tests should be conducted prior to menstruation for determining congenital abnormalities of the reproductive tract and vice versa. Key words: HWW syndrome, Central precocious puberty, Renal agenesis, Hemivagina, Uterus didelphys