Wunderlich Syndrome Research Articles

Wunderlich syndrome. Clinical and therapeutic aspects of a long-term experience

Wunderlich's syndrome is defined as a clinical manifestation secondary to sudden spontaneous rupture of renal parenchyma in the absence of injury, resulting in hemoretroperitoneum. This syndrome may occur in patients with benign or malignant neoplasm of the kidney, arterial venous fistulae, immunovasculitis and other phlogosis of the kidney. We present 19 cases of acute spontaneous hemoretroperitoneum or Wunderlich's syndrome diagnosed from 1996 to 2009, related to the following conditions: 7 renal carcinomas, 4 angiomyolipomas, 2 adrenal hemorrhages, 1 bleeding polycystic kidney, 2 hemorrhagic renal cysts and three cases of immunovasculitis. Overall, 6 patients were treated with acetylsalicylic acid and 4 with dicumarolics for cardiovascular disease, while 13 patients were suffering from hypertension? In 19 cases there was no mortality and the following treatment was performed: 11 nephrectomy, 4 partial nephrectomy, 2 adrenalectomy, 2 selective embolization of intrarenal branches as single treatment, while in three other cases, the embolization was carried out from a surgical procedure, conservative (partial nephrectomy) in one case, ablative (nephrectomy) in two other cases. The spontaneous hemoretroperitoneum is a rare syndrome associated with acute kidney disease often unknown to the patient and only in selected cases with small hematoma; it is possible to perform arteriography with selective embolization of branches avoiding renal access surgery. So far, it is unknown to which extent anti-platelet and anticoagulant drug treatment contributes to this syndrome but, in our experience, they have increased the morbidity of the clinical condition.

Simultaneous presentation of Wunderlich's syndrome and thyroid nodule in a patient with Tuberous sclerosis: Are thyroid lesions a part of Tuberous Sclerosis Complex?

A multisystem involvement is seen in tuberous sclerosis with variety of manifestations, but thyroid lesions are rarely reported in this syndrome. Angiomyolipomas of the kidney are common in tuberous sclerosis, and when present are a significant cause of morbidity. Wunderlich’s syndrome is the most dreaded complication in such patients. We present the case of a 25-year-old male patient who had tuberous sclerosis and presented to us with flank pain and haematuria. On evaluation he was found to have bilateral angiomyolipomas along with a thyroid nodule and had Wunderlich’s syndrome.

Kidney Cancer Revealed by Wunderlich's Syndrome: One Case at the Teaching Clinic of Urology CNHU-HKM of Cotonou

Kidney Cancer Revealed by Wunderlich's Syndrome: One Case at the Teaching Clinic of Urology CNHU-HKM of Cotonou

Wunderlich Syndrome in a patient with dengue infection – An entity to remember

Dengue infection has become a serious public health concern. Dengue management protocols and the management of common complications arebeing updated often based on understanding the Pathophysiology of dengue infection. This understanding has definitely improved dengue management resulting in reduction in dengue deaths. At times, uncommon complications may be overlooked in busy and crowded clinical settings. Spontaneous localized retro-peritoneal bleeding including bleeding into psoas muscle or Wunderlich Syndrome has been reported in few instances associated with dengue fever in this country and abroad. Thislife-threatening possibility must be born in mind when a clinician notices sudden deterioration of a dengue patient withoutsignificant thrombocytopenia, generalized bleeding manifestation or other evidence of extra vascular fluid loss. The management of this entity requires urgent restoration of the circulatory volume including urgent blood transfusion though, in general, clinicians are moreconcerned about fluid overload in the management of dengue patients. As awareness of this entity seems poor, further research and reporting of similar cases in medical literature will shed more light on this obscure entity and increase the awareness among clinicians with favorable results.

Chromophobe Renal Cell Carcinoma Presented with Wunderlich Syndrome

Introduction: Chromophobe renal cell carcinoma (RCC) like other types of RCCs maybe asymptomatic but few of them existing with rare presentations. Case Presentation: A male patient attended the emergency department of our center with sudden right flank pain. According to investigations performed, he had a tumor mass measuring 75 mm with extracapsular extension and heterogeneous enhancement in lower pole of the right kidney and a perinephric hematoma secondary to rupture of capsular vessels. Conclusions: Wunderlich syndrome can be the first presentation of chromophobe RCC.

VE21 - Robotic-assisted Partial nephrectomy after a retroperitoneal tumor hemorrhage in a patient with wunderlich syndrome

VE21 - Robotic-assisted Partial nephrectomy after a retroperitoneal tumor hemorrhage in a patient with wunderlich syndrome

Herlyn–Werner–Wunderlich syndrome with a protruding hymen over the vaginal introitus in a neonate

Herlyn–Werner–Wunderlich syndrome with a protruding hymen over the vaginal introitus in a neonate

EP12.08: Herlyn–Werner–Wunderlich syndrome: performance of MRI findings compared to ultrasound imaging and intraoperative assessment during Caesarean section

EP12.08: Herlyn–Werner–Wunderlich syndrome: performance of MRI findings compared to ultrasound imaging and intraoperative assessment during Caesarean section

A Rare Cause of Acute Abdomen Due to Hemorrhage of Renal Mass: Wunderlich Syndrome

Introduction: Wunderlich syndrome is an emergency medical condition that refers to spontaneous nontraumatic bleeding confined to the perinephric space. Renal angiomyolipomas are the most frequent etiology. Herein, we present a case of Wunderlich syndrome whose vital signs abruptly worsened and went into hypovolemic shock and required an urgent surgical approach.Case Report: A 55 year old woman applied to our emergency room with flank pain for 4 days, which worsened severely that morning. In her physical examination, her abdomen was tight and tender and she felt pain with palpation on the left flank. Her abdomen sonography revealed a mass in the upper pole of her left kidney consistent with angiomyolipoma. Also there was subcapsular fluid collection. Intravenous (IV) contrast enhanced computerized tomography (CT) was done immediately, which showed a 9.5 cm diameter mass with arterial contrast extravasation into the mass and subcapsular area. There was a large perinephric hematoma. After 2 hours in the emergency room the patient became haemodynamically unstable and entered the operation room with hypovolemic shock. Radical nephrectomy was performed.Conclusion: Wunderlich syndrome is a rare cause of acute abdomen. Therefore, urgent and specific management to diagnose and treat it as soon as possible is needed in cooperation with emergency doctors, radiologists, and urologists

Acute Flank Pain in the Emergency Room: Rare Case and Review of the Literature

Wunderlich syndrome is defined as spontaneous renal hemorrhage. Generally, the kidney mass may be due to secondary causes such as vascular pathologies.Wunderlich syndrome is rarely idiopathic.Wunderlich syndrome is a classical clinical symptom of Lenk's triad, characterized by flank pain, mass, and hypotension. Wunderlich syndrome can be life threatening due to gross hemorrhage. Our patient applied with the complaint of abdominal pain.

Clinical and radiological findings for early diagnosis of Herlyn–Werner–Wunderlich syndrome in pediatric age: experience of a single center

Objective: The authors present their experience in the management of pediatric patients with Herlyn–Werner–Wunderlich syndrome (HWWS) considering clinical classification and anatomical characteristics of the malformation.Methods: All the data of the patient presented at our Pediatric Surgery Unit from February 2010 to August 2015 were collected. According to the type of malformations, patients were divided in 3 groups: A (completely obstructed hemivagina), B (incompletely obstructed hemivagina), and C (communication between the duplicated cervices).Results: Six patients were treated in the study period. The mean age was 9 years (2 months–15 years). According to the characteristics of the HWWS, we had 5 patients in group A, 1 in group B, and none in group C. One-stage surgical treatment was performed in all cases of complete obstruction, but in one case a second look was necessary for a better resection of the septum. At a mean follow-up of 18 months all patients were symptoms free.Conclusions: Prognosis of this malformation is good in case of early diagnosis and treatment. We suggest that when a renal agenesia is diagnosed, the patient needs an ultrasonographic follow-up of the contralateral kidney but also of the genital tract to find each minimal abnormalities, furthermore, a MRI scanning before the onset of menstruation can be necessary.

Diagnostic Evaluation of Wunderlich Syndrome in Chronic Kidney Disease by Point-of-Care Ultrasonography

Wunderlich syndrome is a rare condition, which means spontaneous non-traumatic renal hemorrhage confined to the subcapsular or perirenal space. It usually presents as acute flank or abdominal pain. Computed tomography (CT) is currently the best method for diagnosis and further survey of possible etiology. We report a bed-ridden patient with right urothelial carcinoma who had unexpectedly rapid deterioration of renal function after admission. Bedside point-of-care ultrasonography provides excellent diagnostic performance. Case report and literature review. A 84-year-old female who had right urothelial carcinoma complicated with right hydronephrosis status post right double J catheter implantation was admitted due to urinary tract infection. During hospitalization, acute deterioration of renal function in the presence of chronic kidney disease developed. Point-of-care ultrasonography showed a hypoechoic lesion surrounding the cortical surface of right kidney. Further abdominal CT disclosed right perirenal hematoma, consistent with Wunderlich syndrome. Ultrasound is a non-invasive, convenient and reliable tool for expeditious evaluation of perirenal hematoma/hemorrhage. In terms of CT scan-related contrast-induced nephropathy, this approach provides comparative effectiveness and safety in early diagnosis of Wunderlich syndrome.

Herlyn–Werner–Wunderlich syndrome

Herlyn–Werner–Wunderlich syndrome is a rare developmental anomaly, in which there is uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. The most common presenting complaint is abdominal pain with or without abdominal mass, secondary to hematocolpos. Since the obstruction is unilateral, monthly periods continue and diagnosis can be delayed leading to complications with far-reaching effects. In this report, we describe the evaluation and successful management of one such case in a 13-year-old girl who reported with chronic pelvic pain and abdomino-pelvic mass.

Herlyn—Werner—Wunderlich Syndrome: Premenarche

ABSTRACT Aim To increase awareness about this rare condition in order to aid early diagnosis and effective treatment which can eventually prevent complications and infertility issues in the future. Materials and methods A case of a 10 year old pre-menarchal girl, who presented with this syndrome, was managed surgically. After this, a literature search was done and similar case reports were reviewed. Results The Herlyn-Werner-Wunderlich syndrome (HWW) is a rare Müllerian anomaly with an incidence of 0.1-3.8%. It is characterised by uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. This condition can be treated with surgery. Since delay in diagnosis may lead to complications, early diagnosis and treatment is beneficial. When Müllerian anomalies are encountered, a screening should also be made for congenital renal abnormalities and vice versa. This condition usually presents postmenarche with abdominal pain due to hematocolpos or dysmenorrhoea. However, there are rare occasions when this condition can present even before menarche. Conclusion The clinical presentation of HWW syndrome requires a high index of clinical suspicion. Though computed tomography or ultrasound can be used to confirm the diagnosis, magnetic resonance imaging is considered to be the preferred imaging modality. Early diagnosis and surgical management is important in order to prevent complications like retrograde tubal reflux, leading to endometriosis and infertility. How to cite this article David A, Gudi SN, Shankar R. Herlyn— Werner—Wunderlich Syndrome: Premenarche. J South Asian Feder Obst Gynae 2017;9(2):207-210.

Acute Flank Pain, Flank Mass and Hypovolemic Shock due to Kidney Cysts Rupture: An Emblematic Case of Wunderlich Syndrome

Spontaneous perinephric hemorrhage (SPH) due to kidney cysts rupture is uncommon compli- cation but when it occurs can cause an emergency situation that may endanger the patient's life. Wunderlich first described the condition of spontaneous, non-traumatic renal bleeding confined to the subcapsular and perirenal spaces. Herein, we report a case of Wunderlich syndrome (WS) due to spontaneous rupture of a simple kidney cyst. In our case, the spontaneous retroperito- neal hemorrage (SRH) was associated to a probable giant haemorragic cyst that is uncommon since cyst rupture occurs into caliceal system rather than the perinephric space. Wunderlich syndrome after spontaneous, non-traumatic kidney cyst rupture is a rare entity. A multimodal- ity approach is necessary to a have a definitive diagnosis in order to choose the best treatment possible for the patient.

Gossypiboma Following Emergency Nephrectomy for Wunderlich Syndrome

We describe the imaging findings of a case of an intra-abdominal gossypiboma (retained surgical sponge) in an asymptomatic 61-year-old man who underwent an emergency nephrectomy because of a Wunderlich syndrome secondary to a renal cell carcinoma. A follow-up computed tomography was performed 4 months after the emergency surgery and showed an extraperitoneal lesion with gas bubbles and radiopaque markings in the left hemiabdomen, consistent with a retained surgical sponge ("gossypiboma" or "textiloma") in the anterior pararenal space. The patient underwent scheduled surgery for extraction of the textiloma.

Herlyn-Werner-Wunderlich (HWW) Syndrome

Abstract Background Herlyn–Werner–Wunderlich (HWW) syndrome is a rare congenital anomaly of the urogenital tract, characterised by triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. We report the rare presentation, radiographic findings and outcome of a young girl with HWW syndrome who underwent surgical management at our hospital. Case A young girl who attained menarche presented with dysmenorrhoea and frequent loose stools for a month. She underwent successful hysterolaparoscopic resection of septum with evidence of retrograde menstruation. Conclusion HWW is a rare condition. The most urgent concern is retrograde menstruation due to the obstructed hemivagina. It is parallel to the patent vaginal canal, less pliable and closer to cervix. These anatomical differences contribute to diagnostic delay and surgical challenges.

Embryogenesis of vagina and embryopathogenesis of Herlyn-Werner-Wunderlich syndrome.

Embryogenesis of vagina and embryopathogenesis of Herlyn-Werner-Wunderlich syndrome.

Radiological Manifestations of Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: A Rare Complex Müllerian and Wolffian Duct Anomaly

AbstractObstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare complex Müllerian and Wolffian duct anomaly. It is also known as Herlyn–Werner–Wunderlich syndrome (HWWS). It includes unilateral renal anomalies and uterine didelphys. It generally occurs at puberty and exhibits non-specific and variable symptoms with acute or pelvic pain shortly following menarche, causing a delay in the diagnosis. We report here a 16-year-old female presenting with progressive cyclical pelvic pain, where magnetic resonance imaging (MRI) suggested the diagnosis of the OHVIRA syndrome. She was managed by surgical resection of the septum and draining of the obstructed vagina.

Herlyn–Werner–Wunderlich Syndrome: Clinical Presentation and Outcome After Repeat Surgery

Abstract Background: Herlyn–Werner–Wunderlich syndrome (HWW syndrome), is a rare congenital anomaly consisting of the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The diagnosis of HWW syndrome is often delayed because of less understanding of Mullerian anomalies by practitioners. Case: A 13-year-old female presented with severe dysmenorrhea. A diagnosis of HWW syndrome was confirmed by magnetic resonance imaging. Drainage of her hematocolpos was performed by resection of the vaginal septum. Laparoscopic fulguration of endometriosis lesions was performed; the lesions had been formed as a result of retrograde menstrual blood flow. Results: The patient's postoperative period was uneventful, and she was relieved of dysmenorrhea completely. After 2 years, she developed dysmenorrhea again as a result of restenosis of the septum and development of hematocolpos. Her vaginal septum was excised with suturing of the edges, and she was advised to retain her vaginal stent for 1 mon...