Management Outcomes of Large Renal Angiomyolipoma Presenting with Wunderlich Syndrome-Experience from a Tertiary Center.

Renal angiomyolipoma is an uncommon, benign-mixed mesenchymal tumor consisting of thick-walled blood vessels, smooth muscles, and mature adipose tissues. Twenty percent of these tumors are associated with tuberous sclerosis. Wunderlich syndrome (WS), an acute nontraumatic spontaneous perirenal hemorrhage, can be a presentation of large angiomyolipoma. This study evaluated the presentation, management, and complications of renal angiomyolipoma with WS in eight patients who presented to the emergency department between January 2019 and December 2021. The presenting symptoms included flank pain, palpable mass, hematuria, and bleeding in the perinephric space on computerized tomography. Demographic data, symptoms at presentation, comorbidities, hemodynamic parameters, the association with tuberous sclerosis, transfusion requirements, need for angioembolization, surgical management, Clavien-Dindo complication, duration of hospital stay, and 30-day readmission rates were evaluated. The mean age of presentation was 38 years. Of the eight patients, five (62.5%) were females and 3(37.5%) were males. Two (25%) patients had tuberous sclerosis with angiomyolipoma, and three (37.5%) patients presented with hypotension. The mean packed cell transfusion was three units, and the mean tumor size was 7.85 cm (3.5-25 cm). Three of them (37.5%) required emergency angioembolization to prevent exsanguination. Embolization was unsuccessful in one patient (33%) who underwent emergency open partial nephrectomy, and one (33%) patient developed post-embolization syndrome. A total of six patients underwent elective surgery-four underwent partial nephrectomy (laparoscopic - 1, robotic - 1, open - 2) and two underwent open nephrectomy. Three patients encountered Clavien-Dindo complications (Grade 1, n = 2 and IIIA, n = 2). WS is a rare, life-threatening complication in patients with large angiomyolipoma. Judicious optimization, angioembolization, and prompt surgical intervention will help deliver better outcomes.

Spontaneous perirenal hemorrhage (Wunderlich syndrome) in the fetus: a case report.

BACKGROUND: Spontaneous perirenal hemorrhage (Wunderlich syndrome) in the fetus is a rare urinary system disease. Prenatal ultrasound diagnosis presents challenges due to the lack of specific clinical features. CASE PRESENTATION: A 27-year-old Chinese woman gravida 2 para 0 found her fetus with the left Wunderlich syndrome accompanying bilateral hydronephroses and bladder dysfunction with an early diagnosis through prenatal ultrasound and postnatal magnetic resonance imaging. After a timely emergency cesarean section, the infant was administrated antimicrobial prophylaxis and an indwelling catheter treatment. Ultrasound follow-up showed his urinary system gradually developed normally. CONCLUSION: A fetus with bilateral hydronephroses accompanying bladder dysfunction should be observed because of the risk of spontaneous renal rupture with hemorrhage formation. Ultrasound and magnetic resonance imaging play a vital role in the diagnosis and follow-up of Wunderlich syndrome. Early diagnosis facilitates better pregnancy planning and appropriate care of newborns.

Arterial malformations leading to bilateral spontaneous renal hemorrhage in a dog.

OBJECTIVE: To describe the clinical presentation, diagnostic imaging, management, and follow-up of a dog that presented with sequential, bilateral, spontaneous, subcapsular and perirenal hemorrhage (Wunderlich syndrome) due to bilateral renal arterial malformations. CASE SUMMARY: A 9-year-old intact male Field Spaniel presented for acute onset abdominal pain following a possible syncopal episode. Abdominal ultrasonography, contrast-enhanced ultrasound, and computed tomography (CT) revealed right-sided perirenal hemorrhage that extended into the peritoneum. The dog was discharged following stabilization with analgesia, fluid therapy, and tranexamic acid. One month later, the patient presented with identical clinical signs. A CT scan at this stage revealed bilateral kidney infarcts with new left-sided perirenal hemorrhage. Abdominal ultrasound and contrast-enhanced ultrasound were repeated and showed similar findings. Follow up with four-dimensional CT angiography revealed bilateral renal arterial malformations, likely causing spontaneous renal hemorrhage and secondary subcapsular hematomas , retroperitoneal and peritoneal hemorrhage. UNIQUE INFORMATION PROVIDED: This is the first reported case of both bilateral renal arterial malformations and bilateral spontaneous subcapsular and perirenal hemorrhage in the absence of neoplasia, coagulopathy, or trauma in a dog. Advanced imaging modalities, including selective angiography, were required to diagnose this condition successfully. Arterial malformations should be considered as a differential diagnosis in cases of hemoretroperitoneum. Nephrectomy due to perirenal hemorrhage should be cautiously considered, especially in the absence of angiography, because of the sequential bilateral nature of this case. Conservative management in this case resulted in a good long-term outcome 10 months later.

Spontaneous perirenal hemorrhage due to ruptured renal arteriovenous malformation: A case report.

Spontaneous perirenal hemorrhage is bleeding in perirenal space with no associated trauma or iatrogenic manipulation. It is a rare condition, prevalence of renal arteriovenous malformation (AVMs) was <0,04%. A 51-year-old man presented with a dull pain at right flank for 4 days before admission. Physical examination revealed tenderness in the right flank area. An abdominal CT showed large right perirenal hematoma. The patient underwent surgical exploration, and an active source of bleeding was found at the posterior aspect of the right kidney. Pathology showed evidence of ruptured arteriovenous malformation. Open surgery was performed due to the large hematoma with pain.

Spontaneous perirenal hemorrhage (Wunderlich syndrome): An analysis of 28 cases.

INTRODUCTION: This study aimed to analyze the characteristics, etiology, and treatment of a series of patients with spontaneous perirenal hemorrhage (Wunderlich syndrome [WS]). METHODS: We retrospectively reviewed the records of 26 patients hospitalized for WS in a tertiary urological center between 2011 and 2018. All patients were evaluated for perirenal hemorrhage observed on computed tomography (CT) in the emergency department. Clinical variables (age, underlying diseases, symptoms, shock, and hospitalization period), laboratory test results, and radiological and pathological results were reviewed. RESULTS: The series included 28 events from 26 patients with a mean follow-up period of 20.2 ±â€¯18.0 months. Flank pain was most common symptoms (92%). Twelve patients (46%) had visible renal lesions and associated hematoma and 14 only showed perirenal hematoma. In six patients with shock (systolic blood pressure < 90 mm Hg), 2 underwent emergency angioembolization. Twelve patients (46%) underwent exploration and total nephrectomy. In the final diagnosis, 4 cases of renal cell carcinoma, 3 of angiomyolipoma, 4 of simple renal cyst, 2 of acquired cystic kidney disease, 4 of sarcoma or other malignancy, 4 of chronic pyelonephritis, and 5 of idiopathic WS were observed. Patient age was associated with prediction of renal cell carcinoma in the patients with WS. CONCLUSION: Renal masses are the main cause of WS, and CT is the diagnostic procedure of choice. Old age is a possible risk factor for renal cell carcinoma in etiology of WS. Surgical treatment is preferred in patients diagnosed with renal malignancy and in cases of hemodynamic instability.

A preliminary study on classification and therapeutic strategies for spontaneous perirenal hemorrhage.

BACKGROUND: The aim of our study was to report our experience in the classification and therapeutic management strategies for spontaneous perirenal hemorrhage (SPH). METHODS: From September 2005 to April 2015, 20 patients with SPH were newly diagnosed in our hospital. Their clinical features, image findings, identification of underlying causes, and therapeutic management were retrospectively analyzed, and relevant literature was reviewed. In this study, patients were classified according to the degree of severity of the disease or emergency imaging diagnosis of underlying causes. On the basis of the former, patients were classified as critical and noncritical, and on the basis of the latter, patients were classified as renal cell carcinoma (RCC), undefined solid neoplasm, angioleiomyolipoma (AML), and unknown cause. RESULTS: In the acute stage, contrast-enhanced computed tomography (CT) was superior to ultrasonography for both diagnostic accuracy of SPH (p = 0.02) and etiology discovery power (p = 0.004). The results of contrast-enhanced magnetic resonance imaging (MRI) were identical to those of contrast-enhanced CT. We summarized a flowchart in the whole classification and therapeutic strategies of SPH. According to the imaging diagnosis of underlying causes, all the patients with undefined solid neoplasm or RCC underwent emergency operation. Patients with AML or unknown cause underwent selective arterial embolization (SAE) or conservative management according to the critical degree. Acute hemorrhage was controlled in 19 cases, of which 14 were cured by the operation and only one critical patient with severe shock died shortly despite rescue efforts. CONCLUSIONS: Contrast-enhanced CT or MRI is the first choice of imaging examination, which could not only accurately diagnose SPH but also detect the underlying causes. Choice of therapeutic strategies for SPH should vary according to the identification of critical patients and imaging diagnosis of underlying cause.

[The 461th case: fever, hematuria, and right lumbar pain].

A 56-year-old female was admitted to the Department of Rheumatology, Peking Union Medical College Hospital with complaint of recurrent fever and acute lumbar pain. Fever was complicated with malaise, cough and occasional blood-streaked sputum. Lab tests showed elevated white blood cell count, increased serum creatinine, erythrocyte sedimentation rate and C-reactive protein. Other lab findings included severe anemia, hematuria, and proteinuria. Immunological examinations were positive for antinuclear antibodies, antineutrophil cytoplasmic antibodies and antiglomerular basement membrane antibody. Ultrasonography and CT scan detected a huge spontaneous perirenal hematoma at right side. Angiography revealed multiple microaneurysms on bilateral renal arteries and branches. A diagnosis of systemic vasculitis was suggested. Under the combination therapy of corticosteroids and cyclophosphamide, the patient presented sustained remission for one year. This case indicates that prompt and sufficient treatment of primary disease is essential to a promising outcome.

Spontaneous perirenal hemorrhage in hemodialysis patient treated with selective embolization: A case series and review of the literature.

INTRODUCTION: Spontaneous perirenal hemorrhage (SPH) or Wunderlich syndrome, is a rare but potentially life-threatening condition. It is characterized by an unexpected bleeding in the kidneys and usually presents as an abdominal pain. Angiography and more recently selective renal arterial embolization are emerging as effective modalities for the diagnosis and treatment of SPH. In this article, we report a total of three cases of SPH in hemodialysis (HD) patients. METHODS: This is the experience of diagnosis and treatment of SPH in HD patients. FINDINGS: All three were female, between 37 and 54 years of age and were undergoing HD for end stage renal disease (ESRD). Two of patients presented with left flank or abdominal pain after termination of HD therapy, while the third patient presented with left abdominal pain during the dialysis session. All patients received anti-coagulation therapy for HD, but no abnormal levels of coagulation index were found. These patients were diagnosed using CT and two of them were diagnosed with acquired cystic kidney disease (ACKD). Selective renal arterial embolization was performed in the case of active bleeding. DISCUSSION: We are aware that HD patients have elevated risk of bleeding related complications, additionally the presence of an acute abdominal pain increases the suspicion of SPH as a possible cause. ACKD can be considered one of the possible risk factors for SPH in long-term HD patients. Interventional treatment for kidney injury is useful and safe for active bleeding in most cases.

The 461th case: fever, hematuria, and right lumbar pain / 中华内科杂志

A 56-year-old female was admitted to the Department of Rheumatology,Peking Union Medical College Hospital with complaint of recurrent fever and acute lumbar pain.Fever was complicated with malaise,cough and occasional blood-streaked sputum.Lab tests showed elevated white blood cell count,increased serum creatinine,erythrocyte sedimentation rate and C-reactive protein.Other lab findings included severe anemia,hematuria,and proteinuria.Immunological examinations were positive for antinuclear antibodies,antineutrophil cytoplasmic antibodies and antiglomerular basement membrane antibody.Ultrasonography and CT scan detected a huge spontaneous perirenal hematoma at right side.Angiography revealed multiple microaneurysms on bilateral renal arteries and branches.A diagnosis of systemic vasculitis was suggested.Under the combination therapy of corticosteroids and cyclophosphamide,the patient presented sustained remission for one year.This case indicates that prompt and sufficient treatment of primary disease is essential to a promising outcome.

Spontaneous perirenal hemorrhage in cauda equina syndrome: a case report.

Neurogenic bladder is a common cause of acute pyelonephritis (APN) in cauda equina syndrome (CES). Perirenal hemorrhage, a rare complication of APN, can be a life-threatening condition. To our knowledge, there is no previous report of perirenal hemorrhage as a complication of APN in CES. A 57-year-old male, diagnosed with CES, due to a L3 burst fracture 3 months earlier, was presented with fever and chills. His diagnosis was APN due to neurogenic bladder. After treatment for APN, he was transferred to the department of rehabilitation medicine for management of his CES. Because of large post-voiding residual urine volumes, he performed self-catheterization after voiding. However, he presented again with fever and chills, and recurrent APN was diagnosed. On the third day of antibiotic treatment, he had acute abdominal pains and hypovolemic shock. Abdominal computed tomography and angiography showed left APN and a perirenal hematoma with left renal capsular artery bleeding. After embolization of the left renal capsular artery, no further active bleeding occurred. Because APN due to neurogenic bladder can lead to critical complications, such as perirenal hemorrhage, the physician should pay attention to the early diagnosis and treatment of urinary tract infection and the management of neurogenic bladder after CES.

Spontaneous Perirenal Hemorrhage in Cauda Equina Syndrome: A Case Report

Neurogenic bladder is a common cause of acute pyelonephritis (APN) in cauda equina syndrome (CES). Perirenal hemorrhage, a rare complication of APN, can be a life-threatening condition. To our knowledge, there is no previous report of perirenal hemorrhage as a complication of APN in CES. A 57-year-old male, diagnosed with CES, due to a L3 burst fracture 3 months earlier, was presented with fever and chills. His diagnosis was APN due to neurogenic bladder. After treatment for APN, he was transferred to the department of rehabilitation medicine for management of his CES. Because of large post-voiding residual urine volumes, he performed self-catheterization after voiding. However, he presented again with fever and chills, and recurrent APN was diagnosed. On the third day of antibiotic treatment, he had acute abdominal pains and hypovolemic shock. Abdominal computed tomography and angiography showed left APN and a perirenal hematoma with left renal capsular artery bleeding. After embolization of the left renal capsular artery, no further active bleeding occurred. Because APN due to neurogenic bladder can lead to critical complications, such as perirenal hemorrhage, the physician should pay attention to the early diagnosis and treatment of urinary tract infection and the management of neurogenic bladder after CES.

Spontaneous Perirenal Hemorrhage in Cauda Equina Syndrome: A Case Report

Neurogenic bladder is a common cause of acute pyelonephritis (APN) in cauda equina syndrome (CES). Perirenal hemorrhage, a rare complication of APN, can be a life-threatening condition. To our knowledge, there is no previous report of perirenal hemorrhage as a complication of APN in CES. A 57-year-old male, diagnosed with CES, due to a L3 burst fracture 3 months earlier, was presented with fever and chills. His diagnosis was APN due to neurogenic bladder. After treatment for APN, he was transferred to the department of rehabilitation medicine for management of his CES. Because of large post-voiding residual urine volumes, he performed self-catheterization after voiding. However, he presented again with fever and chills, and recurrent APN was diagnosed. On the third day of antibiotic treatment, he had acute abdominal pains and hypovolemic shock. Abdominal computed tomography and angiography showed left APN and a perirenal hematoma with left renal capsular artery bleeding. After embolization of the left renal capsular artery, no further active bleeding occurred. Because APN due to neurogenic bladder can lead to critical complications, such as perirenal hemorrhage, the physician should pay attention to the early diagnosis and treatment of urinary tract infection and the management of neurogenic bladder after CES.

Perirenal hematoma and involvement of the temporal artery in a patient with polyarteritis nodosa (PAN) / Hematoma perirrenal e envolvimento da artéria temporal em paciente com poliarterite nodosa (PAN)

Descrevemos o caso de um paciente masculino, 48 anos, com poliartralgias e mialgias migratórias, associadas a febre e emagrecimento com dois meses de evolução; um episódio de dor testicular bilateral; e cefaleia unilateral pulsátil com espessamento de artéria temporal esquerda sugerindo arterite temporal. Na evolução, o paciente apresentou hematoma perirrenal esquerdo espontâneo, infartos esplênicos e insuficiência renal aguda oligúrica. Foi tratado com prednisona e ciclofosfamida. Foram realizadas biópsias de artéria temporal esquerda e músculo quadríceps. Os achados clínicos, laboratoriais, radiológicos e intercorrências levaram ao diagnóstico de poliarterite nodosa (PAN).

We report the case of a 48-year-old male with a 2-month history of migratory polyarthralgia, and myalgia associated with fever and weight loss; one episode of bilateral testicular pain; and unilateral pulsatile headache with thickening of the left temporal artery suggestive of temporal arteritis. The patient evolved with spontaneous left perirenal hematoma, splenic infarcts, and oliguric acute renal failure. Treatment included prednisone and cyclophosphamide. The left temporal artery and the quadriceps muscle were biopsied. Clinical, laboratorial, and radiological findings, as well as the intercurrences, led to the diagnosis of polyarteritis nodosa.