Herlyn-Werner-Wunderlich: A Rare Disease
Sumber: Medicinus Vol. 36 ISSUE 3, DECEMBER 2023https://medicinus.co/journal/article/view/medicinus-vol36-no3-30-35
Yoki Citra Perwira, Jeffy Winarya WahyudiSiloam Hospitals, Jember
Abstract
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.Keywords: Herlyn-Werner-Wunderlich, rare disease, uterus didelphys
Introduction
Reproductive system duplication and hemivaginal obstruction were first identified by Purslow in 1922. Since then, many cases have been found, especially the Herlyn-Werner-Wunderlich syndrome or longitudinal vaginal septal obstruction with related uterine anomaly. This anomaly is thought as a result from a developmental disorder of the Wolffian duct caudal, causing ipsilateral agenesis, and an anomaly of the two ducts, including Müllerian and Wolffian ducts.1-3
Uterine triad didelphys, hemivaginal obstruction, and ipsilateral renal agenesis are three main characteristic of Herlyn- Werner-Wunderlich (HWW) syndrome. Epidemiological studies showed that HWW is a rare condition, but two cases have been reported in Indonesia, each of them was found in Surabaya and Yogyakarta. This syndrome can also lead to hemivaginal obstruction and ipsilateral renal agenesis (OHVIRA), which is estimated to have an incidence rate of 0.1- 3.8%.1,2,4,5
Mullerian disorders are not detected in early childhood and can lead to delay in diagnosis. This condition usually not found until the child enters adulthood or when complications arise in the reproductive organs.6,7 We found a 17-year-old woman complaining of acute vaginal pain since menarche. Physical examination found signs of inflammation on the vaginal surface. Translabial ultrasonography was carried out and found vaginal septum as well as hematocolpos. Therefore, urgent action was taken by incising the septum and draining pus to control the symptoms.
Case presentation
We presented a case report by tracing medical records and found a 17-year-old Javanese girl who came to the emergency department; complaining of pain in the genital area, that had been felt for one year and had been getting worse in the past week. The patient had menarche when she was 16 years old and the pain persisted in the area genital area since then. The patient has a history of irregular menstruation; menstruation occurs every two or three months and the duration of menstruation was 20-25 days.
The patient had already consulted another obstetrician and was diagnosed with endometriosis, then was treated with birth control pills. However, the symptoms persisted and the vaginal pain worsened. From the parents’ information, the patient had an excellent antenatal history, the patient was born vaginally without intervention, with normal weight and at full term with the help of a midwife. Vital signs on physical examination were within normal range and on vaginal inspection, it was found that the genital region appeared hyperemic with white spots. Patient was diagnosed provisionally with suspected vaginitis with abnormal uterine bleeding and suspected endometriosis. In laboratory examination, there was an increase in leukocytes of 16,910 µl; 80% neutrophil segments; and 10% lymphocytes. Furthermore, the patient received intravenous fluid drip (IVFD) of ringer lactate 500 ml/8 hours, IV ketorolac 30 mg/8 hours; IV ranitidine 50 mg/12 hours; ceftriaxone 1 g/12 hours, and oral codeine 10 mg/12 hours.
Translabial ultrasonography revealed vaginal septum and hematocolpos (Figure 1). Diagnosis of Herlyn-Werner- Wunderlich was made by comparing several types based on Chinese classification, namely type II or III. Furthermore, due to the urgency of the patient’s condition with pain VAS of 8, septal incision was performed immediately along with pus draining which obtained pus mixed with blood (Figure 2).
Figure 1. Translabial ultrasonography imaging found vaginal septum (left) and hematocolpos (right)
Figure 2. Drainage of vaginal septal incision and drainage of pus mixed with blood (left) and septal incision (right)
After the patient had undergone surgical procedure, he received IV antibiotic with metronidazole 500 mg/18 hours followed by a pelvic MRI to evaluate anatomic morphology of the urogenital tract. Result of MRI showed that there was agenesis of the left kidney, visible duplication of the uterus, cervix, and vagina, with a deep fundal cleft (Figures 3). The patient was discharge three days posttreatment with good symptom control.
Figure 3. Coronal T2 showed an agenesis kidney on the left (left) and uterus didelphys (right)
Discussion
Congenital uterine abnormalities occur in 1-5% of the general population. Meanwhile, Herlyn-Werner-Wunderlich (HWW) syndrome has a very rare incidence in the world. Studies show an age of onset between 10-40 years based on complete and incomplete vaginal obstruction.7,8
Herlyn-Werner-Wunderlich is also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) and has demonstrated uterine duplication with hemivaginal obstruction. In this disorder, renal anomaly will be found to occur in 90- 95% of the cases. This obstruction is characterized as a unilateral vaginal septum that blocks the channel on the right or left of the hemivagina. This will explain the finding of hematocolpos in radiological imaging.5
Abnormalities that were found in Herlyn-Werner-Wunderlich syndrome occur in embryo around eighth week of gestational age, which can result in Mullerian anomaly. After that, there is a failure of reabsorption of the uterovaginal septum, so it predicted the emergence of an isolated vaginal septum. This embryonic mechanism is also thought to be due to impaired development of the caudal part of one mesonephric duct with secondary involvement of the ipsilateral Mullerian duct.5
On the affected side, an anomaly of the mesonephric duct leads to failure of regular ureteric bud formation and renal differentiation. This disturbance has the resulting consequences of renal agenesis and the abnormal location of the ipsilateral Müllerian ducts. This results in the failure of the abnormal union of the Mullerian ducts with their opposite partners and with the urogenital sinus, thus creating a double uterus and cervical-vaginal obstruction.2
Several classifications of HWW have been found, and the goal is to find out the anatomical anomaly. In a study conducted in China in 2014, HWW was classified into three types (Figure 4): type I imperforate oblique vaginal septum, type II perforate oblique vaginal septum, and type III imperforate oblique vaginal septum and cervical fistula. Type I has symptoms of dysmenorrhea, types II and III have symptoms of vaginal discharge.7
Another HWW classification in a study conducted in China in 2015 divided HWW into two types: complete obstruction and incomplete hemivaginal obstruction. In complete obstruction, hemivagina is divided into blind hemivagina dan cervicovaginal atresia without communicating uteri. Finally, incomplete hemivaginal obstruction is divided into partial reabsorption of the vaginal septum and with communicating uteri.7,9
Clinical symptoms in Herlyn-Werner-Wunderlich depend on complete or incomplete vaginal oblique septal obstruction and have no specific signs and symptoms. Patients often experience dysmenorrhea that worsens on one side, vaginal discharge, unilateral abdominal pain, chronic pelvic pain, and urinary incontinence. Spontaneous abortion or preterm birth have been reported in pregnant patients.2,5,6
On physical examination, a bulge will be found on the lateral side of the vaginal wall. In some cases, prolonged obstruction feels like a soft lump on abdominal examination. Other symptoms include intermenstrual spotting, purulent discharge from the vagina and sepsis are rare.1,5
Diagnostic examinations such as ultrasonography can be performed initially to observe abnormalities of the urinary tract and the presence of hematocolpos. Meanwhile, noncontrast-abdominal magnetic resonance imaging (MRI) can also be used as a diagnostic test because it provides accurate results in HWW syndrome diagnosis. However, comparing ultrasonography with MRI results provides detailed information about uterus morphology and help to detect communication between uterine and vaginal lumen.2,10,11
Once diagnosis is made, Herlyn-Werner-Wunderlich syndrome could be treated surgically, which is the most optimal procedure in reducing obstruction, resecting the vaginal septum by incising, dilating, and draining to reduce the symptoms of obstruction. In other literature, hysteroscopic septal resection can also be performed in symptomatic cases and can be a prophylactic measure for asymptomatic patients. Apart from that, a laparoscopic procedure can also be performed as a treatment option for hematocolpos.5,12-14
The prognosis of the HWW syndrome is good after surgical management. Clinicians must understand the pathophysiology, clinical symptoms, and time management as the key to reducing patient symptoms and reducing potentially aggravating complications. Pregnancy rate appears reasonable, the occurrence of infertility is noted in 5-15% of these patients and is consistent with the rest of the general population.1,15
Conclusion
Herlyn-Werner-Wunderlich has a rare epidemiology with varying ages of onset. Diagnosis is made by history taking, physical examination, ultrasonography, and MRI. In urgent situation, a septal incision and pus drainage can be performed to reduce symptoms and complications that could develop 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 with a urogenital and reconstruction division.
Acknowledgements
The author would like to thank the Siloam Jember Hospitals and all staff who participated to assist the preparation and writing of this case report.
DAFTAR PUSTAKA
Sanfilippo JS, Lara-Torre E, Gomez-Lobo V. Sanfilippo’s Textbook of Pediatric And Adolescent Gynecology. CRC Press; 2019.p.90.
Pfeifer SM (Eds). Congenital Müllerian Anomalies. Springer. Switzerland: Springer Nature; 2016.p.133–8.
Amesse LS. Mullerian Duct Anomalies. Available from: https://emedicine.medscape.com/article/273534-overview#a1?form=fpf cited Oct 22, 2022.
Fulghesu AM (Eds). Good practice in pediatric and adolescent gynecology. Springer. Cagliari: Springer Nature; 2018.p.45-9.
Dietrich J, Ponsky TA, Sanfilippo J (Eds). Endometriosis in Adolescents. Springer. Switzerland: Springer Nature Switzerland AG; 2020.p.263-78.
Wang J, Zhu L, Lang J, Liu Z, Sun D, Leng J, et al. Clinical characteristics and treatment of Herlyn-Werner-Wunderlich syndrome, Arch Gynecol Obstet. 2014;290(5):947-50.
Tong J, Zhu L, and Lang J. Clinical characteristics of 70 patients with Herlyn–Werner–Wunderlich syndrome. International Journal of Gynecology & Obstetrics 2013;121(2):173-5.
Chai JL and Burk KS. Congenital uterine anomalies. Available from: https://rad.bwh.harvard.edu/congenital-uterine-anomalies cited Oct 22, 2022.
Zhu L, Chen N, Tong J, Zhang L, Lang J. New classification of Herlyn-Werner-Wunderlich syndrome Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4837842/ cited Nov 15, 2022.
Witrianti RD, Iman N, Fajarini ES. Peranan MRI abdomen pada kasus Herlyn-Werner-Wunderlich. Jurnal Radiografer Indonesia 2020;3(1):42-5.
Sidhu HS, Madaan PK. Herlyn-Werner-Wunderlich syndrome in a multiparous female [Internet]. 2020 [cited 2022 Oct 22]. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7869122/
Wang J, Zhu L, and Lang J. Clinical characteristics and treatment of Herlyn–werner–wunderlich syndrome. BJR Case Rep. 2020;7(1):20200132.
Grimbizis GF, Camus M, Tarlatzis BC, Bontis JN, Devroey P. Clinical implications of uterine malformations and hysteroscopic treatment results. Hum Reprod Update. 2001;7(2):161-74.
Boyraz G, Karalok A, Turan T, Özgül N. Herlyn-Werner-Wunderlich syndrome; laparoscopic treatment of obstructing longitudinal vaginal septum in patients with hematocolpos - a different technique for virgin patients. J Turk Ger Gynecol Assoc. 2020;21(4):303–4.
Fachin CG, Rocha JLAS, Maltoni AA, Lima RLC, Zendim VA, Agulham MA, et al. Herlyn-Werner-Wunderlich syndrome: Diagnosis and treatment of an atypical case and review of literature. Int J Surg Case Rep. 2019:63:129-34.