IOSR Journal of Dental and Medical Sciences (IOSR-JDMS) e-ISSN: 2279-0853, p-ISSN: 2279-0861.Volume 20, Issue 5 Ser.10 (May. 2021), PP 01-04 www.iosrjournals.org DOI: 10.9790/0853-2005100104 www.iosrjournal.org 1 | Page The Herlyn-Werner-Wunderlich Syndrome: A Rare Cause of Urinary Retention, Pelvic Pain and Dysmenorrhea in an Adolescent Girl Dr. Priyanka 1 , Dr. Shikha Sachan 2 , Dr. Divya Dwivedi 3 , Prof. Nisha Rani Agrawal 4 Abstract: The Herlyn-Werner-Wunderlich Syndrome also referred as OHVIRA syndrome is a rare congenital mullerian anomaly characterised by uterus didelphys (or sometimes septate uterus), hemi-obstructed vagina with ipsilateral renal agenesis. Although usual presentation is after menarche but few reports of pre-pubertal presentation in infants and a delayed presentation until during pregnancy have also been reported. The key to diagnosis in such cases is high index of suspicion and detailed work up in order to avoid late diagnosis of such rare presentations leading to complications that might compromise the fertility and affect the quality of life of an individual and add to morbidity. We are describing here a case of an adolescent girl who presented with history of urinary retention, spasmodic dysmenorrhea and lower abdominal pain after an year of menarche who was diagnosed in time and managed appropriately with an uncomplicated post-operative period. Keywords: Herlyn Werner Wunderlich Syndrome, OHVIRA Syndrome, Hemi- obstructed vagina, uterus didelphys, mullerian duct anomalies, urinary retention, pelvic pain --------------------------------------------------------------------------------------------------------------------------------------- Date of Submission: 15-05-2021 Date of Acceptance: 31-05-2021 --------------------------------------------------------------------------------------------------------------------------------------- I. Introduction Herlyn-Werner-Wunderlich syndrome (HWWS), first described [1,2] between 1971–1976, is most likely the result to an insult in early pregnancy leading to embryonic arrest at around 7- 8 weeks of gestation, at which point the adjacent müllerian and mesonephric ducts are simultaneously affected. HWWS is a combination of type III mullerian anomaly according to the American Fertility Society with mesonephric duct anomaly with vaginal septum. The classic renal manifestation of OHVIRA syndrome is ipsilateral renal agenesis, but reports of duplicated kidneys, dysplastic kidneys, rectovesical bands, or crossed fused ectopia have also been described [3]. HWW syndrome is usually discovered at puberty with non-specific symptoms, like increasing pelvic pain, muco-purulent discharge dysmenorrhea and palpable mass due to the associated haematocolpos or hematometra, which result from retained, longstanding menstrual flow in the obstructed hemi-vagina. It rarely occurs with primary infertility in early adulthood when the vaginal septum is incomplete [4]. The possible early presentation of this syndrome should be suspected in all neonates (females) with renal agenesis confirmed postnatally or with prenatal diagnosis [5]. It is common, in fact, an error of evaluation with planning of removal of mass, that can damage patients in term of chance for a successful reproductive outcome. MRI is considered as a gold standard for diagnosis and pre-operative planning of the Herlyn-Werner- Wunderlich syndrome. It evaluates uterine morphology, detects communication between vaginal and uterine lumen, characterises fluid contents, has the capability of multiplanar imaging but with no radiation, can detect associated renal agenesis and diagnose complications like endometriosis [6]. The rarity of this condition may contribute to diagnostic delay. Early diagnosis with appropriate surgical intervention with vaginoplasty and septostomy decreases the long-term morbidity of these women. The surgical outcome is excellent, and it is associated with a successful reproductive performance in the future. II. Case Report 16-year-old unmarried female, referred to our out-patient Department from periphery with complaints of retention of urine for 15 days, spasmodic dysmenorrhoea since menarche and lower abdominal pain for past 4 months which has been aggravated for the past 3 days. She had attained menarche 1 year back and her cycles were regular with average flow. She had associated dysmenorrhoea, which was persistent throughout the cycle and later persisted even beyond it. Later it culminated to persistent lower abdominal pain for the last 4 months that was gradually progressive and had increased in intensity for past 3 days. There was no history of altered bowel habits, haematuria, per vaginal discharge, or similar family history. For these complaints’ patient consulted a local practitioner, who advised an USG that reported “A large cystic mass in right adnexa of about