Wunderlich Syndrome Research Articles

Possibilities of Early Ultrasound Diagnostics of the Herlin — Werner — Wunderlich syndrome (Case Report)

Herlin — Werner — Wunderlich syndrome or OHVIRA (Obstructed hemivagina and ipsilateral renal anomaly) is a rare type of uterine and vaginal doubling defect (0,1 – 10,0 % of all uterine abnormalities) which is characterized by a triad of symptoms: uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal dysgenesis. The manifestation of the disease usually present after menarche, is accompanied by severe pain syndrome, leads to redoubted complications and organ-carrying operations with decreasing the fertility. At an earlier age, it can manifest itself as an infection of the genitourinary system. The diagnosis is based on clinical and anamnestic data using instrumental methods, the most accessible and safe of which is ultrasound. This article presents three clinical cases of patients 5 – 6 years old with a description of the ultrasound picture of the OHVIRA syndrome.

284 Wunderlich Syndrome - Case Report of a Surgical Emergency Due to Spontaneous Non-Traumatic Retroperitoneal Haemorrhage

Abstract A 42-year-old female with no other significant co-morbidities presented with complaints of sudden onset abdominal pain, she was in state of shock- her haemoglobin dropped drastically to 6.8g/dl from 11.8g/dl. She was resuscitated and was given blood transfusion. CT Scan was suggestive of retroperitoneal haemorrhage due to rupture of Renal angiomyolipoma (RAML). Patient underwent selective embolization of renal artery the next day and was discharged after 3 days of post-operative stay. Wunderlich Syndrome-is a rare condition in which spontaneous nontraumatic renal haemorrhage occurs into the subcapsular and perirenal spaces is most commonly caused due to spontaneous rupture of RAML.It is characterized by Lenk’s triad – Acute flank pain, flank mass, hypovolemic shock. Size of AML (>4 cm), prothrombotic states such as pregnancy are main causes of AML rupture. Patients are managed conservatively or with help of selective arterial embolization. Nephrectomy is last resort in a hemodynamically unstable patient or in cases of embolization failure. When dealing with acute abdomen with haemodynamic instability in non-traumatic cases it is essential to consider possibility of Wunderlich Syndrome. Early diagnosis, availability of Interventional radiology is key to successful management and potentially avoiding a Nephrectomy.

A CASE OF HEMMORRHAGIC SHOCK CAUSED BY WÜNDERLICH SYNDROME

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Wünderlich syndrome (WS) is a rare condition caused by acute renal hemorrhage and characterized by flank pain, flank mass and hypovolemic shock. We report a complex case of WS in an elderly gentleman with left flank pain and nausea. CASE PRESENTATION: An 81-year-old man with diabetes mellitus type 2, end-stage renal disease on hemodialysis, chronic atrial fibrillation on apixaban and coronary artery disease with history of percutaneous coronary intervention on clopidogrel who presented to a tertiary care center with acute onset left flank pain and nausea for six hours. Upon arrival, the patient was found to have a blood pressure of 78/54 mmHg and a ventricular paced rhythm at 70 bpm. His physical examination was significant for tenderness to palpation over the left flank, as well as a palpable flank mass. His hemoglobin at presentation was 12.1 gm/dL but a repeat value resulted 6.6 gm/dL. Computed tomography (CT) of the abdomen and pelvis revealed multiple renal cysts and a 25 x 11 cm hemorrhagic left kidney concerning for a ruptured cyst with extravasation of blood extending to the retroperitoneum along the psoas muscle. The patient was emergently transfused packed red blood cells, four-factor prothrombin complex concentrate and fresh frozen plasma to reverse the effect of his chronic anticoagulation and antiplatelet therapy. A multidisciplinary decision was reached that an urgent nephrectomy would be associated with a prohibitively high perioperative risk, as releasing the hematoma into the retroperitoneum may result in a complex, uncontrollable hemorrhage. The patient subsequently underwent a renal arteriogram and interventional radiology-guided selective embolization of left inferior pole artery. Repeat CT with contrast showed a stable left renal, perinephric and retroperitoneal hematoma. The patient recovered from hypovolemic shock and was successfully weaned off vasopressor support. DISCUSSION: Wünderlich syndrome is a potentially life-threatening condition that requires prompt recognition and management. The most common etiologies are malignant neoplasms (61%) followed by angiomyolipomas (29.1%). Hemorrhagic ruptured cysts are a rare cause of WS, and rupture into the perinephric space is among the rarest presentations. Emergent angioembolization may be effective. Follow-up often includes exploratory laparotomy or laparoscopy. However, if malignancy is not clearly identified on CT imaging, conservative management with repeat imaging is a reasonable alternative. CONCLUSIONS: Although rare, Wünderlich syndrome is a rapidly progressing condition with high mortality that requires time-sensitive diagnosis and emergent management. REFERENCE #1: 1. G Albi, L del Campo, D Tagarro, Wünderlich's Syndrome: Causes, Diagnosis and Radiological Management, Clinical Radiology, Volume 57, Issue 9, 2002, Pages 840-845, ISSN 0009-9260, https://doi.org/10.1053/crad.2002.0981 REFERENCE #2: Zhang JQ, Fielding JR, Zou KH. Etiology of spontaneous perirenal hemorrhage: a meta-analysis. J Urol. 2002;167(4):1593-1596. doi:10.1097/00005392-200204000-00006 REFERENCE #3: Medda M, Picozzi SC, Bozzini G, Carmignani L. Wunderlich's syndrome and hemorrhagic shock. J Emerg Trauma Shock. 2009;2(3):203-205. doi:10.4103/0974-2700.55346 DISCLOSURES: No relevant relationships by Ana de Diego Diaz, source=Web Response No relevant relationships by Jeany Villamizar, source=Web Response No relevant relationships by Ann Vu, source=Web Response

Urgent Transcatheter Arterial Embolization for Wunderlich Syndrome With Hypovolemic Shock Secondary to Ruptured Renal Angiomyolipoma.

Purpose: Wunderlich syndrome (WS) with hypovolemic shock secondary to ruptured renal angiomyolipoma (rAML) represents an urgent condition. Hence, we reported our experience with transcatheter arterial embolization (TAE) using different embolic materials under this condition.Methods: This retrospective study consisted of 22 patients. Embolic materials including particles, microcoils, and liquid embolic agents were selectively used based on the decisions of interventional radiologists. Technical success was defined as the complete occlusion of bleeding vessels on the final renal angiogram. Clinical success was defined as the absence of re-hemorrhage needed for repeat endovascular or surgery treatment after TAE.Results: The articulated materials were used when WS presented without aneurysms; a combination of particulate materials and microcoils or Glubran 2 alone were used for WS with aneurysms. The technical success based on 24 episodes of TAEs in 22 patients was 100% (24 of 24). Repeat TAE was achieved in two patients with hemorrhages re-occurring two days after the initial embolization with microcoils alone. The clinical success was 90.9% (20 of 22). No nontarget embolization or embolization-related complications occurred during the TAE procedure. Of the patients, 27.3% (6 of 22) experienced minor complications of post-embolization syndrome (PES). During a median follow-up time of 34 months, no recurrent hemorrhage that required repeat endovascular or surgical treatment for hemostasis occurred.Conclusion: Urgent TAE with the selective use of different embolic materials is an effective alternative to control WS with hypovolemic shock secondary to ruptured rAML. The condition of presenting with or without aneurysms may determine the embolic materials employed.

СИНДРОМ ХЕРЛИНА–ВЕРНЕРА–ВУНДЕРЛИХА: КЛИНИЧЕСКОЕ НАБЛЮДЕНИЕ ПАЦИЕНТКИ 17 ЛЕТ

Herlyn–Werner–Wunderlich syndrome (HWWS) is a rare syndrome in clinical practice in which there is a combination of complete uterus didelphys and OHVIRA syndrome (obstructed hemivagina and ipsilateral renal agenesis). HWWS refers to associative defects of the reproductive and urinary systems and occurs due to anomalies in the development of the Müllerian ducts. The review part of the article presents questions of its epidemiology, and provides historical information. The clinical picture, methods of examination and treatment are considered. Particular attention is paid to the difficulty of diagnosing HWWS and the need for its early diagnosis in order to preserve the reproductive health of girls. A clinical case of a 17-year-old patient with HWWS diagnosed during urgent laparoscopy, the complication of which was hematopiocolpos, is presented. The patient was referred to a level 3 hospital for specialized medical care, where she was operated on.

POS-429 A RARE VARIANT OF GENETICALLY CONFIRMED TUBEROUS SCLEROSIS COMPLEX PRESENTING WITH BILATERAL RENAL ANGIOMYOLIPOMA IN WUNDERLICH SYNDROME

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder with a prevalence of approximately 1 to 6,000-12,000 worldwide. Renal angiomyolipoma (AML) associated with TSC is often life-threatening due to the greater propensity to develop hemorrhage. A rare condition associated with it is the Wünderlich syndrome (WS), atraumatic renal hemorrhage into the subcapsular and perirenal spaces. We present a case of a patient who presented with the cutaneous and renal features of TSC with bilateral AML in Wünderlich syndrome. Case Report A 28-year-old female was admitted for non-traumatic right flank pain. She presented with the classic cutaneous features of TSC: hyperpigmented skin papules located on the center area of her face, periungual angiomas, and angiofibroma (Figure 1). She was also unable to finish high school. She previously consulted another hospital where a whole abdominal ultrasound was done, revealing a complex retroperitoneal mass on the right measuring 14.9 x 9.3 x 14 cm. She was then advised urologic evaluation and transferred to our institution. She had no dysuria, hypogastric pain, nor hematuria. Physical examination revealed her abdomen was enlarged and a right flank mass (doughy, round, non-tender, measuring 3 cm x 3 cm) was palpated. She was hypotensive on admission and required aggressive resuscitation. Abdominal computed tomography (CT) with contrast showed bilaterally enlarged kidneys and fluid collection (611 mL) in the right perirenal space (Figure 2). The Urology service at the time opted to observe the patient (no surgical plans) at the time since there was no evidence of active hemorrhage. The consideration was an angiomyolipoma in Wünderlich syndrome (shock, flank mass, flank pain). Genetic testing of TSC related genes revealed mutations in the TSC2 gene (loss-of-function variant), consistent with tuberous sclerosis complex. Genetic counseling was rendered to the patient and her immediate family. Furthermore, echocardiography with doppler studies, cranial and chest CT with contrast done were all unremarkable. She opted to be discharged against medical advice and was scheduled for a close outpatient follow-up. She was sent home on sirolimus 2 mg once daily. On her follow-up two weeks post-discharge, she reported improved overall well-being and a subjective decrease in the right flank mass. In summary, the diagnosis of TSC should be considered in young females of Southeast Asian descent presenting with multiple dermatologic lesions and multiple renal masses and development delay. Particularly, in those presenting with renal angiomyolipoma are at high risk for developing life-threatening hemorrhage. Hence, timely and appropriate management is needed (i.e. embolization, nephrectomy, or chemotherapy). Also, a thorough workup must be done to rule out the involvement of vital organs (i.e. cardiac rhabdomyoma, and intracranial space-occupying lesions. Lastly, it should be emphasized that early recognition of the disease and its manifestations is the keystone of the prevention of these fatal complications.

Diagnostics and treatment of Herlyn–Werner–Wunderlich syndrome in a newborn girl

Herlin-Werner-Vanderlich syndrome refers to the Müllerian duct anomalies, including uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. At the first stage of treatment, a reasonable intervention is opening the hemivaginal septum by extra-vaginal access under visual control or using vaginoscopy. We identified a rare variant of the defect identified, since the longitudinal septum of the hemivagina did not prolapse through the introinus, which required a complex diagnosis and surgical aid in the newborn. A significant volume of hydrometrocolpos was associated with the accumulation of urine from the right hypoplasmic kidney with an abnormal flow of the ectopic ureter into the cavity of the atresized vagina. It determined the need for surgical treatment for urgent indications. In this regard, a biochemical analysis of the fluid obtained after drainage of the hydrometrocolpos with the detection of the concentration of urea, creatinine, total protein, and cellular composition is mandatory.

Herlyn Werner Wunderlich Syndrome with Vagino-umbillical Fistula: A Most Unique Presentation

The Herlyn–Werner–Wunderlich (HWW) syndrome is an uncommon Mullerian duct anomaly composed of a triad of obstructed hemivagina, renal agenesis, uterine didelphys with a prevalence of [Formula: see text] females. Aside from its usual presentation of cyclic hypogastric pain and abdominal mass, there has been no other reported case in literature of the syndrome presenting with a vagino-umbilical fistula. Presented here is a unique case of a 35-year-old woman presenting with purulent, foul-smelling discharge extruding from a fistula on her umbilicus and her vagina associated with a tender hypogastric mass. Three-dimensional transvaginal ultrasonography aided the diagnosis of HWW syndrome with a concomitant vagino-umbilical fistula. Excision of the vaginal septum together with antibiotic coverage resolved her symptoms. The syndrome’s embryopathogenesis, classification, diagnostics, definitive surgical management and other issues during follow up are discussed.

Herlyn–Werner–Wunderlich syndrome in the prepubescent period (literature review and clinical observations)

Herlyn–Werner–Wunderlich syndrome (OHVIRA syndrome) is a combined malformation of the genitourinary systeme, characterized by various combinations of uterus dydelphys with unilateral obstructed (or blind) hemivagina and ipsilateral renal agenesis. The causes of mistakes in diagnosis and treatment are common because of relative rarity of anomaly, insufficient awareness of practitioners about the syndrome and the lack of multidisciplinary approach. Untimely and tactically chaotic diagnosis of Herlyn–Werner–Wunderlich syndrome leads to a misconception about the clinical situation, wrong choice in treatment, and, as a consequence, to complications such as strictures, widespread adhesions and inflammation, as well as irreversible changes in the topography of organs of the small pelvis with a subsequent deterioration in reproductive status of patient. This article provides a review of the literature on the problem, considers the clinical cases of diagnosing this defect in prepubertal patients.

A rare case of spontaneous perirenal hemorrhage - Wunderlich syndrome

Introduction. Spontaneous perirenal hemorrhage or Wunderlich syndrome represents a rare entity in urological settings. The vast majority of the causes are represented by angiomyolipoma and renal cell carcinoma. In other cases, vascular abnormalities, polycystic kidneys, polyarthritis nodosa, or pyelonephritis could represent the cause of perirenal bleeding. The treatment depends on the clinical parameters at the presentation as well as on the possible presence of renal malignancies. Our goal was to present a rare case of a healthy men who presented the idiopathic Wunderlich syndrome. Case outline. We present a case of a 50-year-old patient with spontaneous perirenal hemorrhage that was not due to any defined cause even after a six-months follow-up. Conclusion. In some rare cases of perirenal bleeding, the cause cannot be defined at the presentation, even with today?s advanced radiological imaging methods. Thus, it is important to be aware of the fact that in those cases, a longer follow-up is needed, knowing that the presence of perirenal hematoma can always obscure the real diagnosis. Sometimes, even in cases where the proper follow-up has been done, the real cause of the bleeding remains unknown.

Wunderlich syndrome pada penderita clear cell renal cell carcinoma

Wunderlich's syndromes is a collection of symptoms consisting of pain, palpable mass in the flank region, hematuria associated with spontaneous renal bleeding. Renal cell carcinoma (RCC) is defined as a group of malignancies originating from the epithelium of the renal tubules. Reportedly a man, aged 47 years, with complaints of pain in the waist and upper left abdomen, accompanied by reddish urine since 3 days before being admitted to the hospital, but also experienced one month ago. On physical examination, there was a solid mass in the abdomen accompanied by tenderness. Ultrasound examination of the upper-lower abdomen showed a solid lobulated mass of impression originating from the left kidney. MSCT examination found a mass of the left kidney measuring about 11.2 cm x 16.7 cm x 20.2 cm which caused enhancement of the left renal artery. The patient then underwent a radical nephrectomy, found a left kidney tumor measuring 20 cm which was attached to the diaphragm. The result of the PA histo examination, obtained tumor cells with an increased N / C ratio, polygonal to round oval shape, moderate pleimorphic core, hyperchromatic, prominent core children, wide clear vacuole cytoplasm and partly eosinophilic with the impression of Clear Cell Renal Cell Carcinoma Grade. III. Patient diagnosed as CCRCC with wunderlich syndrome manifestation. .Further management was planned for systemic chemotherapy or radiation treatment, but the patient refused. The patient was discharged on the 20th day of treatment in good condition.

Angioembolization prior to nephrectomy in Wunderlich syndrome: a case report of collision angiomyolipoma and clear cell renal cell carcinoma

A 29-year-old man with previously undiagnosed tuberous sclerosis complex arrived at the emergency department due to anemia-related gross hematuria. A computed tomography scan revealed a bilateral renal mass consistent with angiomyolipoma and a right retroperitoneal hematoma. Angioembolization of the right renal artery was performed prior to nephrectomy. The histopathologic results were collision angiomyolipoma and clear cell renal cell carcinoma.
 Relevance: The multisystemic presentation of tuberous sclerosis complex combined with the histologic findings of collision angiomyolipoma and clear cell renal cell carcinoma is a rare entity in the medical literature.
 Clinical implications: Angioembolization results in fewer postoperative complications of ileus, pain, or infection, and its utility in the present case was important for facilitating the approach during nephrectomy and reducing the risk for intraoperative bleeding.
 Conclusions: The present case report provides a description of Wunderlich syndrome in a patient with tuberous sclerosis complex and the histopathologic diagnosis of a collision tumor, consisting of angiomyolipoma and clear cell renal cell carcinoma. Angioembolization of the right renal artery was performed before nephrectomy, reducing the risk for intraoperative bleeding.

Angioembolización previa a nefrectomía en el síndrome de Wünderlich: reporte de caso de un angiomiolipoma mixto y carcinoma renal de células claras

Un paciente masculino de 29 años con complejo de esclerosis tuberosa no diagnosticado previamente se presenta al departamento de urgencias por hematuria macroscópica anemizante. La tomografía computarizada reveló una masa renal bilateral compatible con angiomiolipoma y un hematoma retroperitoneal derecho. Se realizó una angioembolización de la arteria renal derecha previo a la nefrectomía. Los resultados de histopatología revelaron un tumor de colisión de angiomiolipoma y carcinoma renal de células claras.
 Relevancia: La presentación multisistémica del complejo de esclerosis tuberosa en combinación con los hallazgos histológicos de un tumor mixto de angiomiolipoma y carcinoma de células claras es una entidad poco frecuente en la bibliografía existente.
 Implicaciones clínicas: La angioembolización presenta menos complicaciones posoperatorias como íleo, dolor postoperatorio o infecciones, además en este caso, es de utilidad importante para facilitar el abordaje durante la nefrectomía y disminuir así el riesgo de sangrado transoperatorio.
 Conclusiones: Se describe un reporte de caso de Síndrome de Wünderlich en un paciente con complejo de esclerosis tuberosa, con un diagnóstico histopatológico de tumor mixto consistente en angiomiolipoma y carcinoma de células claras donde la angioembolización de la arteria renal antes de la nefrectomía se usó para reducir el riesgo de hemorragia intraoperatoria durante la nefrectomía.

WÜNDERLICH SYNDROME: A CASE OF HYPOVOLEMIC HEMORRHAGIC SHOCK

SESSION TITLE: Medical Student/Resident Critical Care Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Wünderlich syndrome (WS) is a rare presentation of hypovolemic hemorrhagic shock. It is a fatal disease described as a syndrome of spontaneous non-traumatic subcapsular and retroperitoneal hemorrhage. The etiology of WS is varied, with renal causes including malignancy being the most common, followed by renal vasculature abnormalities, vasculitis, cystic rupture, pyelonephritis, and idiopathic. The appropriate treatment depends on the accuracy of diagnosis and precise determination of its cause. To our knowledge, this is the first case report of WS due to acute pyelonephritis in a patient with congenital solitary kidney and Jehovah's Witness religious beliefs which impacted his care. CASE PRESENTATION: A 77 year old male with a two day history of flank pain accentuated on the right side, fevers, and confusion presented as critically ill and requiring vasopressor support. His medical history comprises prostate cancer in remission and atrial fibrillation on aspirin. He was diagnosed clinically with pyelonephritis, and culture positive urinary tract infection and sepsis with Escherichia coli. The initial hemoglobin was of 10.3 g/dL. Computed tomography (CT) of the abdomen showed nonspecific stranding and a solitary right kidney. He clinically deteriorated prompting repeat CT imaging that showed a large right perirenal hematoma with retroperitoneal blood. He underwent emergent coil angioembolization of a hemorrhagic branch of the inferior pole renal artery. His hemoglobin of 4 g/dL recovered with erythropoietin and intravenous iron. He briefly required hemodialysis for his acute kidney injury. The patient survived and the function of his congenital solitary kidney was preserved. DISCUSSION: The classic signs of WS include Lenk’s triad of flank pain, abdominal mass, and hypovolemic shock. However, in only 20% of cases, all three symptoms present. Acute onset of flank pain is the most common symptom. Although pyelonephritis is a rare cause of WS, in our case we believe that bleeding diathesis and etiology of WS was driven by cytokine induced endothelial damage from pyelonephritis along with aspirin induced platelet dysfunction. CT is preferred for the initial imaging workup. Angioembolization is a minimally invasive treatment modality for acute hemorrhage. Exploratory laparotomy or nephrectomy is typically recommended if malignancy is suspected. CONCLUSIONS: WS requires prompt recognition to prevent death from exsanguination. Shock is relatively common with WS, however, surgical exploration is not absolutely necessary with the advent of angioembolization. The unconventional treatment method with erythropoietin instead of blood may be considered in patients with Jehovah’s Witness religious beliefs. Reference #1: Katabathina VS, Katre R, Prasad SR, Surabhi VR, Shanbhogue AK, Sunnapwar A. Wunderlich syndrome: cross-sectional imaging review. J Comput Assist Tomogr. 2011;35(4):425-33. Reference #2: Chen YC, Lin YC. Wunderlich syndrome. QJM. 2013;106(2):187-8. Reference #3: Chamarthi G, Koratala A. Wunderlich syndrome. Clin Case Rep. 2018;6(9):1901-1902. DISCLOSURES: No relevant relationships by Francois Kaleta, source=Web Response No relevant relationships by Mitra Sahebazamani, source=Web Response No relevant relationships by Pratyaksha Sankhyan, source=Web Response

VP62.38: Chronic pelvic pain caused by Herlyn–Werner–Wunderlich syndrome: a case report

VP62.38: Chronic pelvic pain caused by Herlyn–Werner–Wunderlich syndrome: a case report

Rapid development of recurrences after partial nephrectomy for papillary renal cell carcinoma initially presented as Wunderlich syndrome

The journal is the official publication of the College of Surgeons of Sri Lanka. Its mission is to reach the highest standard of scientific surgical practice by dissemination of high quality scientific information and to foster and promote the growth of scientific surgery in Sri Lanka and in the region. The Sri Lanka Journal of Surgery publishes peer-reviewed leading articles, review articles, scientific articles, perspectives and case reports in the field of surgery and broadly related surgical sciences The journal adheres to the publication ethics as portrayed by the Committee on Publication Ethics (COPE) and is a member of this organisation. It is listed as an ICMJE journal which follow its recommendations (ICMJE recommendations) on Conduct, Reporting, Editing and Publication of Scholarly Work in Medical Journals.

Angiomiolipoma hemorrágico espontáneo asociado a esclerosis tuberosa que conduce a nefrectomía urgente – Reporte de un caso y revisión de la literatura

Descripción: Acudió una mujer de 34 años a urgencias con dolor abdominal moderado en hipocondrio derecho (duración 3h), acompañado de hematuria y distensión abdominal progresiva y dolorosa. La tomografía reveló una tumoración sólida heterogénea de 14 cm, en riñón derecho, con hemorragia en su interior. Por datos de bajo gasto hemodinámico se transfundieron hemoderivados y mantuvo en vigilancia, persistiendo con descenso de hemoglobina hasta 7.2 gr/dl, por lo que se sometió a angioembolización selectiva urgente, tras lo cual remitió temporalmente el sangrado, recurriendo con hematuria y choque hipovolémico, que derivó en tratamiento quirúrgico definitivo mediante nefrectomía total. Su recuperación transcurrió sin incidentes. El examen histopatológico reveló un angiomiolipoma renal con hemorragia extensa.
 Relevancia: El síndrome de Wünderlich (Angiomiolipoma hemorrágico espontáneo) es una entidad rara cuya complicación más grave e indeseable es la hemorragia refractaria, que puede ser mortal.
 Implicaciones clínicas: Es importante la intervención temprana en pacientes con angiomiolipoma hemorrágico para prevenir potenciales complicaciones como hemorragia masiva y poner en riesgo la vida del paciente.
 Conclusiones: En caso de angiomiolipoma con hemorragia intratumoral y síntomas del síndrome de Wünderlich, la nefrectomía total resulta buena opción para preservar la vida del paciente, después del manejo conservador y/o angioembolización selectiva.

Renal Angiomyolipoma: What the Radiologist Needs to Know

Renal angiomyolipoma (AML) is the most common benign solid renal neoplasm seen in daily clinical practice, with an estimated prevalence of 0.2% to 0.6%.1 It is a triphasic mesenchymal neoplasm composed of varying amounts of dysmorphic vasculature, smooth muscle, and mature adipocytes. Pathologically, AML is now considered among the family of perivascular epithelioid cell tumors. Approximately 80% of cases are sporadic, with mean age at presentation being 43 years and most cases identified at fourth to sixth decades of life. There is also a strong female predilection, with female-to-male ratio of 4:1 in sporadic cases.1 Twenty percent of cases are associated with tuberous sclerosis (TS). Of note, AMLs may also be found in patients with lymphangioleiomyomatosis. AMLs are often found incidentally in imaging done for other purposes, but if symptomatic, they present most frequently with retroperitoneal hemorrhage, which can be fatal. The shock due to severe hemorrhage from rupture has been described in the literature as Wunderlich syndrome. Patients may present with multiple other symptoms and signs, including palpable mass, flank pain, hematuria, or renal failure. Although AML is almost always benign, a rare potentially malignant variant named epithelioid angiomyolipoma is described, which may mimic renal cell carcinoma (RCC).2

Wunderlich syndrome and regression of angiomyolipoma

Wunderlich syndrome and regression of angiomyolipoma

Herlyn–Werner–Wunderlich syndrome: diverse presentations and diagnosis on MRI

To evaluate the diverse presentations of Herlyn-Werner-Wunderlich (HWW) syndrome on magnetic resonance imaging (MRI) and its surgical correlations. From 2014 to 2019, a total of 40 patients with HWW syndrome underwent pelvic MRI. All MRI images were reviewed by two experienced radiologists in consensus. Uterine anomalies, obstruction sites, laterality and communications, urinary anomalies, and other abnormal findings were recorded. MRI findings were correlated with ultrasonography and surgical results. On MRI, the uterus was didelphic in 25 (62.5%), septate in nine (22.5%) and bicornuate in six cases (15%); an ipsilateral vaginal septum was found in 30 cases (75%), and ipsilateral cervical obstruction (obliterated cervical os) was found in 10 cases (25%). Twenty-seven patients underwent surgical treatment. MRI diagnoses of obstruction sites correlated completely with the surgical results. Although obstruction communications could not be found with ultrasonography, MRI identified nine cases of cervical communication, but failed to detect the five cases of surgically confirmed fistula on the vaginal septum. Moreover, MRI identified nine cases of ipsilateral ureteral remnants and/or paravaginal cystic structures, all with insertion into the obstructed vagina or cervix, and one case of ipsilateral ureterocele. HWW syndrome can present as different combinations of uterine anomalies, unilateral cervico-vaginal obstruction, and ipsilateral renal anomalies; ipsilateral paravaginal cystic structures and/or ureter remnants inserting into the obstructed vagina or cervix can occasionally be seen. MRI can provide a comprehensive preoperative evaluation and better identification of obstruction sites, but seems insensitive for detecting vaginal communications in HWW syndrome.