DLSUMC’s Department of Radiology team was named champion in two categories during the 2018 Battle of the Brains Contest, Case Presentations, and Scientific Lectures of the Philippine College of Radiology Southern Luzon Chapter, Inc. held in Ginazel Hotel, Batangas City on September 22, 2018. Academic Radiology publishes original reports of clinical and laboratory investigations in diagnostic imaging, the diagnostic use of radioactive isotopes, computed tomography, positron emission tomography, magnetic resonance imaging, ultrasound, digital subtraction angiography, image-guided interventions, and related techniques. Case presentation: A 21-year-old virgin woman was admitted to the emergency department (ED) with severe abdominal pain, without fever and vaginal discharge. Dr. Mitchell Rosen is a reproductive endocrinology and fertility expert at the UCSF Center for Reproductive Health. It is often associated with renal agenesis ipsilateral to the obstruction, termed obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome [6,8,9]. This syndrome is a combination of Müllerian duct anomalies and mesonephric duct anomalies. An early correct diagnosis is a goal This entity is also known as obstructed hemivagina and ipsilateral renal anomaly ( OHVIRA ). Müllerian duct anomalies are frequently associated with congenital anomalies of other organ systems, and in particular, the urinary system. The hemivagina obstruction can be complete or partial with a fenestration in the vaginal septum. He also is director of the UCSF Fertillity Preservation Center, which helps patients with cancer preserve their ability to have children, despite aggressive cancer treatment. However, an initial screening ultrasound can sometimes diagnose it as in our case. 2 Department of Radiology, Nizwa Hospital, Sultanate of Oman. OHVIRA is a rare congenital anomaly constituting 10% of all Mullerian duct abnormalities. Patients usually present in adolescence, soon after menarche, with pain and pelvic mass and rarely with infertility in adulthood. This entity is also known as obstructed hemivagina and ipsilateral renal anomaly ( OHVIRA ). It is not to be confused with the Wunderlich syndrome. Presentation is typically after menarche with pelvic pain due to the obstructed hemivagina. There may be a palpable pelvic mass. Find Dr. Sheyn's phone number, address, hospital affiliations and more. Clinical Radiology prizes - Volume 74 (2019) Congratulations to our winners: Editor's Medal. Donald School Journal of Ultrasound in Obstetrics and Gynecology (2019): 10.5005/jp-journals-10009-1589 1Weill Cornell Medicine-Qatar, Education City, Doha, Qatar The main treatment modality is the resection of the vaginal septum through vaginoscopy without hymenotomy. It is a triad of obstructed hemi-vagina, uterine didelphys and ipsilateral renal anomaly. It also addresses what you may experience and how to prepare for the exams. She subsequently became pregnant and was first seen at 21 weeks gestation. The Herlyn-Werner-Wunderlich syndrome (HWWS) is a complex congenital malformation, originally described as a triad of symptoms: didelphys uterus, low genital obstruction and unilateral renal anomaly. Syndrome 4. Following several earlier studies reporting cases of patients with a double uterus associated with an obstructed hemivagina and ipsilateral renal agenesis (1-3), the association of ‘unilateral renal agenesis (URA) and ipsilateral blind hemivagina’ was first presented as a syndrome in 1987 after the description of 12 cases with diagrams of the anomaly presented for each case (). Schutt AK, Barrett MR, Trotta BM, et al. (1) OHVIRA syndrome, previously known as Herlyn-Werner-Wunderlich syndrome, was first reported in 1922. Herlyn-Werner-Wunderlich syndrome, also known as OHVIRA (Obstructed hemivagina and ipsilateral renal anomaly) is an extremely rare syndrome characterized by a congenital birth defect of the lower abdominal and pelvic organs. We demonstrate the utility of three dimensional intraoperative ultrasound to better delineate anatomy and aid in optimal resection of the vaginal septum. MR imaging revealed the typical features of didelphys uterus, obstructed … Obstructed hemivagina with uterus didelphys and ipsilateral renal anomaly (OHVIRA) syndrome, formerly known as Herlyn–Werner–Wunderlich syndrome, is a rare congenital malformation that involves abnormal development of the Müllerian and Wolffian ducts during the development of genitalia in female embryos. Most of the women suffering from OHVIRA syndrome are diagnosed late due to the rare incidence Case presentation: A 21-year-old virgin woman was admitted to the emergency department (ED) with severe abdominal pain, without fever and vaginal discharge. (2009) reported that OHVIRA syndrome mostly presents with severe dysmenorrhea and a palpable mass due to unilateral hematocolpos in early postmenarcheal age. CASE REPORT OHVIRA Syndrome Priya Pillai1, Rana Abualsaud2, Zainab Awni3, Arash Rafii4, Badreldeen Ahmed5 Keywords: Ipsilateral renal agenesis, Obstructed hemivagina, Right hematocolpos, Uterine didelphys. Usually they are treated symptomatically until they develop an abdominal mass and pressure symptoms. A rare syndrome that can be seen in the setting of uterus didelphys is obstructed hemivagina and ipsilateral renal anomaly (OHVIRA); some cases do not fit the classic definition. Pakistan Journal of Radiology. Agwany ASE. The condition was missed on ultrasoundand diagnosed on plain CT scan abdomen and pelvis. Bangladesh Specialized Hospital Ltd. (BSHL) is established with a vision to achieve excellence in the field of Health Care and Research. (OHVIRA) also known as Herlyn Werner Wunderlich syndrome, constitutes 0.16–10% of these Mullerian duct anomalies (11). Leyla Karaca. Herlyn-Werner-Wunderlich syndrome is a very rare cause of abdominal pain in young women. J Pediatr Adolesc Gynecol 2002;15:175-7 7. Right-sided dominance of OHVIRA has also been reported [47,48,49,50]. The hallmarks of OHVIRA syndrome are uterus didelphys, hemivaginal obstruction, and ipsilateral renal agenesis. 3 The incidence rate of this syndrome is 0.1 - 3 %. Suspected Mullerian duct anomalies are already frequently evaluated by MR 1. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is another name for it [ 2 ]. Kamio M, Nagata C, Sameshima H, et al. It classically occurs in the setting of uterine didelphys or, rarely, a septate uterus. RadiologyInfo.org tells you how various x-ray, CT, MRI, ultrasound, radiation therapy and other procedures are performed. It is characterized by the triad of didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis. Mitchell.Rosen@ucsf.edu. Sunil K Bajaj, Ritu Misra, ET AL, OHVIRA: Uterus didelphys, blind hemivagina and ipsilateral renal agenesis: Advantage MRI, J Hum Reprod Sci. 15. Abstract. Mitchell.Rosen@ucsf.edu. 2012;120(4):948-51. Ultrasound indicated a 7.1 x 7.0 x 7.2 cm thick-walled right adnexal structure containing debris and fluids, which represented uterine didelphys with hematometra of the right-sided uterine horn (Figure 1 & … PMCID: PMC6176674 PMID: 30319221 OHVIRA syndrome presenting with acute abdomen: a case report and review of the literature. A warm and friendly ambience, high quality medical services and personalized nursing care have been the hallmarks of our hospital OHVIRA syndrome was pointed out and the patient un-derwent a vaginoscopic vaginal septectomy (fig 1c). Magnetic resonance imaging (MRI) may be indicated if there is a clinical suspicion of other gynecological abnormalities. Radiological investigations led us to the diagnosis of OHVIRA syndrome. The HWW or OHVIRA syndrome includes didelphic uterus which is characterized by complete or partial duplication of the vagina, cervix, and uterus and is included in class III of American Fertility Society (AFS) classification. Fertility and Sterility 2007; 87: 918-922 6. College of Medicine In terms of diagnosis, the MRKH syndrome was the most common anomaly (75 cases, 33.3%). Herlyn-Werner-Wunderlinch Syndrome: A Case of Obstructed Hemivagina with Ipsilateral Renal Agenesis (OHVIRA Syndrome) and Didelphys Uterus. It is usually regarded as a disease of adolescence; however, due to a number of possible problems, the management of patients before puberty should not be overlooked. Uterus didelphys occurs when the midline fusion of the mullerian ducts is arrested, either completely or incompletely. The classical presentation is that of a young girl presenting with severe cyclical dysmenorrhea, after attaining menarche. While some of these conditions are noticed as soon as the baby is born, others aren’t typically discovered until later in her life. Although it is probable that the left vaginal walls had several perforations in this case, the patient did not have menstrual molimina syndrome. Obstructed Hemi-Vagina with Ipsilateral Renal Agenesis - OHVIRA. Ohvira Syndrome: Imaging In Diagnosis In An Asymptomatic Newborn Case Report,IJSR - International Journal of Scientific Research(IJSR), IJSR is a double reviewed monthly print journal that accepts research works. Schutt AK, Barrett MR, Trotta BM, et al. In this case, a joint approach allowing for successful placement of a TOA drain placed laparoscopically and a transvaginal catheter placed under image-guidance allowed for the correct incision site into the vaginal septum. The Herlyn-Werner-Wunderlich syndrome (HWWS) is a complex congenital malformation, originally described as a triad of symptoms: didelphys uterus, low genital obstruction and unilateral renal anomaly. The girl was discharged home on the next day. Clinical presentation is nonspecific and includes voiding symptoms such as dysuria, ejaculatory disorders, and hypogastric or perineal pain. In most cases, OHVIRA presents as a double uterus with unilateral obstructed (or blind) hemivagina and ipsilateral renal agenesis. It can also affect the urethra, urethral sphincter, ureters, bladder and spleen. Although the true incidence is unknown, it has been reported to be between 0.1% and 3%. OHVIRA most commonly presents with symptoms of menarche pelvic pain, dysmenorrhea and an abdominal mass. It is about Fourteen adolescents with OHVIRA syndrome managed by vaginoscopic incision of the oblique vaginal septum using a “No-touch” technique over an 8-year period. We present this case of uterus didelphys with right sided haematometrocolpos due to obstructed hemivaginaand ipsilateral renal agenesis-Herlyn Werner Wunderlich syndrome. 4 This syndrome is a triad of obstructed hemivagina with kidney’s agenesis on the same side and uterine didelphys. Women with didelphic uterus may ... Radiology 2006 May;239(2):602-606. Wunderlich syndrome is clinically characterized by Lenk's triad: 1. Welcome! Department of Radiology. October 7, 2019 admin. AJR Am J Roentgenol 1993;160(2):331–334. Background: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare congenital condition of the female urogenital tract, presenting intraoperative challenges. Only a handful of these cases have been associated with pyocolpos. Obstet Gynecol. J Pediatr Adolesc Gynecol. OHVIRA syndrome: Rare cause of chronic vaginal discharge in an unmarried female: Nigam, A. and Raghunandan, C. and Yadav, R. and Tomer, S. and Anand, R. Congenital Anomalies. The OHVIRA syndrome generally occurs shortly after menarche, and patients usually present with pelvic pain or dysmenorrhea, with or without palpable mass due to hematocolpos or hematometra (1). syndromes, such as Klippel-Feil syndrome [4,7]. An early correct diagnosis is a goal Purpose Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare syndrome characterized by Müllerian duct and renal anomalies. 3 … JIMA article OHVIRA syndrome. A total of eight additional anomalies (vertebral and renal) were detected, six in MRKH and two in OHVIRA syndrome cases. Agenesis and Ipsilateral Renal Agenesis. Surgical intervention is often required … The syndrome of obstructed hemivagina and ipsilateral renal anomaly was initially reported in 1922 and is known as Herlyn-Werner-Wunderlich syndrome, or more recently, by the acronym OHVIRA (obstructed hemivagina and ipsilateral renal anomaly).1 The OHVIRA syndrome usually occurs in the setting of uterine didelphys.
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