This entity is also known as obstructed hemivagina and ipsilateral renal anomaly ( OHVIRA ). Radiology Case. Herlyn-Werner-Wunderlich (HWW) syndrome, is a rare anomaly characterized by Müllerian duct anomalies (MDA) associated with mesonephric duct anomalies 3. Triad of obstructed hemivagina, ipsilateral renal anomaly, and uterine didelphys The hemivagina obstruction can be complete or partial with a fenestration in the vaginal septum Renal agenesis is the most common renal anomaly. OHVIRA (Obstructed Hemi-Vagina Ipsilateral Renal Anomaly) syndrome also known as Herlyn-Werner-Wunderlich syndrome. Author information: (1)Department of Radiology, Srinakharinwirot University, Nakhon Nayok, Thailand. Herlyn-Werner-Wunderlich syndrome (HWWS), is a rare congenital anomaly of the female urogenital tract caused by abnormalities in the development of the Mullerian and Wolffian ducts resulting in uterus didelphys, obstructed hemivagina, and associated ipsilateral renal anomaly .Renal anomalies range from ectopic ureteric insertion and renal dysplasia to complete … Zhang H(1), Ning G(1), Fu C(1), Bao L(1), Guo Y(2). Background: Herlyn-Werner-Wunderlich (HWW) syndrome is an uncommon variant of Müllerian duct anomalies, consisting of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Furthermore, as in this instance, the patient may have no previous medical history prior to the acute presentation with shock. Lenk's triad of acute onset flank pain, flank … Clinical manifestations usually include flank pain, a palpable mass, gross hematuria and hypovolemic shock. A classic case of OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly), also known as Herlyn-Werner-Wunderlich (HWW) syndrome. 1 article features images from this case 15 public playlist includes this case Wunderlich syndrome is classically characterized by the Lenk's triad: acute flank pain, flank mass, and hypovolemic shock. It is also called OHVIRA syndrome (Obstructed Hemivagina and Ipsilateral Renal agenesis). Herlyn-Werner-Wunderlich syndrome is commonly associated with early pelvic endometriosis and can be complicated by infections (pyocolpos, pyometra, or pyosalpinx). Herlyn–Werner–Wunderlich syndrome is a rare complex congenital anomaly of the urogenital tract involving Mullerian ducts and mesonephric ducts. It is characterized by a triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. Embryogenesis of vagina and embryopathogenesis of Herlyn-Werner-Wunderlich syndrome. The Lenk’s classic triad consisted of acute flank or abdominal pain, palpable abdominal mass and hypovolemic shock [2]. Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital anomaly of the urogenital tract involving Müllerian ducts and Wolffian structures, and it is characterised by the triad of didelphys uterus, obstructed hemivagina and ipsilateral renal agenesis. Herlyn–Werner–Wunderlich syndrome is a rare complex congenital anomaly of the urogenital tract involving Mullerian ducts and mesonephric ducts. Albi, G. et al. chatthara@yahoo.com Herlyn-Werner-Wunderlich (HWW) syndrome is a rare developmental anomaly that is consists of uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. One condition called Wunderlich syndrome is spontaneous, nontraumatic kidney bleeding confined to the subcapsular and perirenal space. Renal arteriography with embolization is an important therapeutic method to control the bleeding and to avoid surgery. Wunderlich syndrome is a rare condition characterized by shock due to severe spontaneous renal hemorrhage into the subcapsular and perirenal space, its commonest cause is AML 1 symptoms and the risk of bleeding increase with the size of the lesion (82-90% of angiomyolipoma >40 mm produce symptoms) 2,3,4,5 OHVIRA syndrome is a rare congenital anomaly characterized by uterine didelphys, obstructed hemivagina and ipsilateral renal anomaly. Herlyn–Werner–Wunderlich syndrome is a rare complex congenital anomaly of the urogenital tract involving Mullerian ducts and mesonephric ducts. Nishu DS(1), Uddin MM(2), Akter K(3), Akter S(3), Sarmin M(4), Begum S(3). Only a handful of these cases have been associated with pyocolpos. neoplasms are the most common causes, with renal cell carcinoma and angiomyolipoma being the most common malignant and benign neoplasms respectively. Herlyn-Werner-Wunderlich (HWW) syndrome is a rare müllerian duct anomaly with uterus didelphys, … Although uncommon, WS … Herlyn-Werner-Wunderlich syndrome is a complex Müllerian anomaly necessitating magnetic resonance imaging or two-dimensional ultrasound imaging for diagnosis and surgical planning. CT plays an important role in the man- Participants: Thirteen patients with Herlyn-Werner-Wunderlich syndrome referred to our department … The most common presentation is abdominal pain, dysmenorrhea, and abdominal mass secondary to hematocolpos. Herlyn-Werner-Wunderlich syndrome is a rare complex congenital anomaly of the urogenital tract involving Mullerian ducts and mesonephric ducts. Herlyn-Werner-Wunderlich syndrome is a rare Mullerian ductal anomaly that is characterized by the presence of a hemivaginal septum, a didelphic uterus and ipsilateral renal agenesis. Study objective: To review the main sonographic and magnetic resonance imaging manifestations of Herlyn-Werner-Wunderlich syndrome, characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis, and to discuss the value of techniques for its diagnosis and treatment. The triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis characterizes this syndrome. Perirenal hemorrhage without a history of trauma complicating an angiomyolipoma is known as Wunderlich syndrome. If perirenal hemorrhage is encountered on ultrasound, a thorough workup should be performed to exclude a bleeding renal cell carcinoma. The WS is characterized by Lenk’s triad: acute abdominal pain, mainly in the flank, palpable mass and hypovolemic shock. AB - Background: Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is a rare entity, sometimes referred to as Herlyn-Werner-Wunderlich syndrome (HWW). WS is much less common than hemorrhage occurring after iatrogenic or traumatic conditions. Wunderlich syndrome (WS) refers to spontaneous renal or perinephric hemorrhage occurring in the absence of known trauma. named after the German physician Carl Reinhold August Wunderlich (1815-1877), who published the first case description in 1856, Background: Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Herlyn-Werner-Wunderlich syndrome presenting with dysmenorrhea: a case report. Author information: (1)From the Departments of *Radiology, and †Obstetric & Gynecology, Faculty of Medicine, Ondokuz Mayis University, Samsun, Turkey. Herlyn-Werner-Wunderlich syndrome is a very rare cause of abdominal pain in young women. Wunderlich syndrome (WS) is a rare condition characterized by a non-traumatic spontaneous acute renal hemorrhage into the subcapsular and perirenal spaces, found and described in this case. WS is much less common than hemorrhage occurring after iatrogenic or traumatic conditions. Dating back from initial description in 1856, the eponym Wunderlich syndrome (WS) refers to the occurrence of spontaneous renal haemorrhage into the subcapsular and/or perinephric spaces, in absence of trauma and anticoagulation [1-4]. MRI is the most reliable diagnostic imaging method available for the diagnosis of this pathology. It is also called OHVIRA syndrome (Obstructed Hemivagina and Ipsilateral Renal agen-esis). Wunderlich syndrome (WS) is a rare condition characterized by acute onset of spontaneous, nontraumatic renal hemorrhage into the subcapsular and perirenal spaces. It is also called OHVIRA syndrome (Obstructed Hemivagina and Ipsilateral Renal agenesis). This syndrome is a combination of Müllerian duct anomalies and mesonephric duct anomalies. It usually presents in a post-pubertal adolescent or adult woman in whom hematometrocolpos produces a pronounced mass effect and pain on the side of the obstructed hemivagina. Wunderlich syndrome (WS) refers to spontaneous renal or perinephric hemorrhage occurring in the absence of known trauma. WS is much less common than hemorrhage occurring after iatrogenic or traumatic conditions. Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital anomaly of the female urogenital tract that associates Müllerian duct anomalies with mesonephric duct anomalies. Wunderlich syndrome can refer to one of several conditions. Jul-Sep 2016;26 (3):417-418. doi: 10.4103/0971-3026.190411. Wunderlich syndrome (WS) is a rare condition characterized by a non-traumatic spontaneous acute renal hemorrhage into the subcapsular and perirenal spaces, found and described in this case. Due to the retroperitoneal extravasation of blood, the FAST scan can be negative and falsely reassuring. (2002). We present the case of a 24-year-old hypertensive who presented with acute flank pain and was found to have perinephric hematoma. It is characterized by a triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. The WS is characterized by Lenk s triad: acute abdominal pain, mainly in … Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital anomaly of the female urogenital tract that associates Müllerian duct anomalies with mesonephric duct anomalies. We report a case of a 14-year-old patient who presented to the emergency department complaining of proctalgia and pelvic … Wunderlich syndrome is a rare clinical syndrome characterized by the sudden onset of spontaneous, nontraumatic hemorrhage into renal subcapsular and retroperitoneal region. Author information: (1)Department of Gynaecology and Obstetrics, Cumilla Medical College and Hospital (CuMCH), Cumilla, Bangladesh. Clinical Radiology57, 840–845. It is not to be confused with the Wunderlich syndrome. We present the first case report on Herlyn-Werner-Wunderlich syndrome from Bangladesh. Herlyn–Werner–Wunderlich syndrome (HWWS), also known as obstructed hemivagina and ipsilateral renal agenesis syndrome, is a rare, combined anomaly of malformation of the Müllerian duct and mesonephric duct of the female urogenital tract. Herlyn-Werner-Wunderlich syndrome (HWWS), defined by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis, is a rare Mullerian duct malformation, usually diagnosed after menarche, when symptoms related to haematocolpos arise. Herlyn-Werner-Wunderlich (HWW) syndrome is a congenital anomaly of the Müllerian and Wolffian ducts (1) characterized by a triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis (2, 3, 4, 5). Indian J Radiol Imaging. Abstract Wunderlich syndrome (WS) refers to spontaneous renal or perinephric hemorrhage occurring in the absence of known trauma. CONCLUSIONS: CT is the best imaging method to establish the diagnosis and in some cases the aetiology of Wünderlich's syndrome. The triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis characterizes this syndrome. 2 article feature images from this case 2012 Mar; 6(3):9-15 :: DOI: 10.3941/jrcr.v6i3.877 ABSTRACT Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), also known as Herlyn-Werner-Wunderlich syndrome, is a rare syndrome with only a few hundred reported cases described since 1922. Introduction. A triad of uterus didelphys, unilateral hemivagina obstruction due to a longitudinal septum and ipsilateral renal agenesis is diagnostic [1]. Wunderlich syndrome is classically characterized by the Lenk's triad: acute flank pain, flank mass, and hypovolemic shock. It is also called OHVIRA syndrome (Obstructed Hemivagina and Ipsilateral Renal agenesis). Wunderlich syndrome is a rare cause of intra-abdominal hemorrhagic shock. It is characterized by a triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. Wunderlich syndrome is defined as a spontaneous renal bleeding confined in subcapsular, perinephric and retroperitoneal spaces. It may be the first manifestation of a renal angiomyolipoma (AML), or the rupture of a renal artery or intraparenchymal aneurysm. The renal condition should not be confused with other conditions … Abstract. Wunderlich’s syndrome (spontaneous renal hemorrhage) is a rare but potentially life-threatening complication of renal angiomyolipoma in the setting of tumors >4 cm. sharmennishu29@gmail.com. Wunderlich syndrome is a urological emergency characterized by spontaneous renal bleeding into the subcapsular and perirenal spaces or resulting in retroperitoneal hemorrhage.Renal . Wunderlich syndrome (WS) is a rare condition characterized by acute onset of spontaneous, nontraumatic renal hemorrhage into the subcapsular and perirenal spaces. It is generally difficult to diagnose the uterine malformation before menarche owing to its small size. of Wunderlich syndrome, Cubillana et al also found conservative management to be the most acceptable option, unless a malignant pathology could be dem-onstrated.16 In conclusion, we have presented a case of Wunderlich syndrome due to rupture of a renal AML, which is the commonest cause reported in most series. It is characterized by a triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. It usually presents after menarche with progressive pelvic pain, sometimes with regular menses, and a palpable mass due to hemihaematocolpos. Herlyn-Werner-Wunderlich syndrome: diverse presentations and diagnosis on MRI.
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