Wunderlich Syndrome A Case Report and Literature Review

Introduction

Wunderlich Syndrome, also known as Spontaneous Renal Hematoma is a rare condition in which there is hemorrhage into the subscapular or perirenal space in the absence of trauma. ^id Its rarity and nonspecific presentation pose a challenge when arriving at a diagnosis. Knowledge of the underlying causes and treatment are vital to managing these patients.

Case Report

A 43 year old African American male presented to the emergency room with sudden onset of left flank pain associated with nausea and vomiting. The patient has a history of uncontrolled hypertension for 6 months and chronic kidney disease. He is non compliant with medications secondary to financial constraints.  On physical examination, the patient’s pulse was 74 bpm, temperature 98.4F and a respiratory rate of 16 breaths/minute. Blood pressure ranged from 217/131 mm/Hg to 240/131 mm/Hg. The patient was awake and alert, heart had regular rate and rhythm, lungs were clear to auscultation. There was no costo-vertebral angle tenderness. His abdomen was non-tender and non-distended. The remainder of the physical exam was unremarkable. Abnormal laboratory findings are included in Table 1. ^{[fig. id]} The patient had a drop in Hemoglobin/Hematocrit from 15.4 (g/dl)/44.1% to 9.9 (g/dl)/29.1% over the course of 2 days. Urine toxicology was positive for benzodiazepines, opiates and cannabinoids.

An electrocardiogram in the emergency room had ST elevation in leads V1-V3, T wave abnormalities in leads 1, AVL, V5-V6 and prolonged QT of 513 msec. The patient had an echocardiogram which revealed severe concentric left ventricular hypertrophy with preserved left ventricular systolic function. The left ventricular ejection fraction was 65% with Grade I diastolic dysfunction. Non-contrast CT scan of the abdomen showed acute left-sided perinephric fluid collection, most likely hemorrhagic in nature. He then had a contrast-enhanced CT scan which showed a gradually expanding hematoma compressing the left kidney with no evidence of extravasation (see figures 1 and 2). It also showed a 5.2 cm renal cyst in the midpole and 1.4 cm renal cyst in the lower pole of the left kidney which were most likely hemorrhagic. Nephrology and interventional radiology were consulted for a possible embolization. Renal angiogram showed a patent left renal artery and no evidence of active bleeding. The next day, renal ultrasound showed no increase in the size of the hematoma. Repeat CT showed a stable left subcapsular hematoma. The patient was discharged with a WBC of 13,200 mm3 on Carvedilol, Hydralazine and Isosorbide Mononitrate. Follow up MRI three months after initial diagnosis showed no malignancies and three hemorrhagic foci with improvement in the hematoma. In the outpatient setting, the patient’s blood pressure has been moderately controlled on multiple anti-hypertensive agents including Amlodipine 10mg daily, Carvedilol 25mg twice a day, Hydralazine 100mg three times a day, Isosorbide Mononitrate 120mg daily and Spironolactone 25mg daily.

Discussion

Spontaneous Renal Hematoma (SRH) was originally described by Bonet in 1679 and further explored by Wunderlich in 1856.  It is a rare condition in which there is hemorrhage into the subscapular or perirenal space in the absence of trauma. ^id
There are a number of important causes of SRH. The largest available meta-analysis states 61.5% of cases are due to tumors (31.5% malignant and 29.7% benign—the most common tumor being angiomyolipoma3,4), 17% of cases are due to vascular disease, 2.4% of cases are due to infection and 6.7% are caused idiopathicallyy. ^id  However, several reports in literature exist of hypertension as a rare underlying cause. ^id
Spontaneous renal hemorrhage classically presents with acute flank pain, symptoms of internal bleeding (such as bruising, anemia, tachycardia) and tenderness to palpation. This classic set of symptoms is termed Lenk’s triad. However, nausea, vomiting, and low-grade fever with decreasing hemoglobin are more commonly seen with SRH. ^{id id id id} SRH may also be associated with hypotension from a hypovolemic state or a paradoxic hypertension from a condition known as Page kidney. In Page kidney, compression of the kidney by hemorrhage or a mass may result in ischemia, renin release, and renin-dependent hypertension. ^id
The rarity and nonspecific presentation of Wunderlich poses a challenge when arriving at a diagnosis. Wunderlich is often detected by imaging when searching for another cause of flank pain.

Ultrasound is generally the first choice for SRH because it is quick and inexpensive. However CT scans are often needed to confirm sonogram findings and to rule out an underlying mass. MRI can differentiate blood from tumor accurately as well as picking up small tumors that a CT scan may not identify. Angiography is mandatory when CT scan fails to show an underlying cause. It can demonstrate renal artery aneurysms and other vascular pathologies which can be the source of the patient’s bleed. ^{id id id} In previous years, nephrectomy was considered a treatment when there was no discernible cause for SRH. Embolizing the blood vessel through renal angiography is now widely performed to avoid surgery, although SRH can be considered a surgical emergency when there is hypovolemic shock. Most patients present with stable vital signs and can be managed conservatively. CT scans should be performed every 3 months until the hematoma resolves or until a definitive diagnosis is made. ^{id id id}

With a history of longstanding, uncontrolled blood pressure and by excluding neoplasm, infection, and vascular causes, it is reasonable to ascertain that our patient’s spontaneous hematoma was attributed to his hypertension.

Conclusion

Wunderlich syndrome is a spontaneous renal hematoma that may be caused by a small number of reported etiologies, including neoplasm, infection, vascular, hypertension, and idiopathic. Knowledge of these etiologies and the work-up of Wunderlich are vital to exclude an underlying malignancy, vascular disorder or life-threatening infection.  As this case illustrates, a high index of suspicion is necessary in patients presenting with acute flank pain and elevated blood pressures.