Wunderlich syndrome, also known as spontaneous retroperitoneal hemorrhage (SRH) and spontaneous subcapsular hematoma, was first described in 1700 by Bonet and was more completely explained by Wunderlich in 1856.[rx] Although SRH is commonly associated with Lenk’s triad (acute flank pain, symptoms of internal bleeding, and tenderness to palpation), the most common signs and symptoms described are abdominal pain (67%), hematuria (40%), and shock (26.5%).[rx–rx]
Retroperitoneal Hemorrhage is a rare clinical entity; signs and symptoms include pain, hematuria, and shock. Spontaneous retroperitoneal hemorrhage can be caused by tumors, such as renal cell carcinoma and angiomyolipoma; polyarteritis nodosa; and nephritis. The least common cause is segmental arterial mediolysis. Although computed tomography is used for the diagnosis of spontaneous retroperitoneal hemorrhage, it can miss segmental arterial mediolysis as the cause of the hemorrhage. The diagnosis of segmental arterial mediolysis as a cause of spontaneous retroperitoneal hemorrhage requires angiography, with pathologic confirmation for a definitive diagnosis.
Causes of Wunderlich Syndrome
Complicating the diagnosis of retroperitoneal bleeding is a myriad of etiologies. The most commonly described initial delineation of causes is whether a patient’s bleeding is associated with trauma or not.[rx][rx][rx]
Traumatic causes include penetrating and blunt trauma, typically of the abdomen and pelvis, whereas non-traumatic causes are divided into those occurring spontaneously and those not occurring spontaneously. Spontaneous etiologies are more varied and include patients undergoing anticoagulation or those on fibrinolytic therapy. Patients with hemorrhagic pancreatitis, renal or adrenal malignancies, gynecological complications, and aneurysmal dilations of the aortic and renal arteries may also experience spontaneous hemorrhage into this potential space.[rx]
Abdominal aortic aneurysm rupture
Renal artery rupture
Cystic medial necrosis
Segmental arterial mediolysis
Renal cell carcinoma
Transitional cell carcinoma
Non-neoplastic Renal Pathology
Coagulopathy Infectious Disease
Renal cortical abscess
Symptoms of Wunderlich Syndrome
Symptoms depend on the anatomic location of the retroperitoneal hemorrhage
- Nonlocalizing abdominal, flank, or low back discomfort
- Subtle increasing abdominal girth with more cephalad located hemorrhage
- Pelvic hematomas may compress the bladder causing urinary symptoms
- Pancreaticoduodenal hematomas may cause gastric outlet obstruction
- Perinephric hematomas may manifest in hematuria
- Femoral nerve palsy
- Flank and groin ecchymosis are a late sign of retroperitoneal hemorrhage
- Pain swelling
- Urination problems and incontinence
Diagnosis of Wunderlich Syndrome
Laboratory tests confirmed that the patient was sickle screen positive, but her erythrocyte sedimentation rate, coagulation studies, platelet function, complement studies, and rheumatoid factor were all within normal limits. The patient remained hemodynamically stable but continued to require blood products and diltiazem hydrochloride (Cardizem; Biovail Corporation, Mississauga, Ontario, Canada) to control her blood pressure. A repeat angiography on hospital day 3 revealed resolution of the microaneurysms and replacement with several blind-ending vessels in the left kidney. The patient’s right kidney and celiac axis were normal. By hospital day 5, the patient had received 10 units of PRBCs, 4 units of fresh frozen plasma, and 5 units of platelets. A repeat CT scan showed an increase in the size of the left perinephric hematoma from 6 to 10.25 cm with extension into the pelvis, free blood in the peritoneum, a lateral infarct of the left kidney, and large, bilateral pleural effusions. The patient was taken to the operating room, where a partial nephrectomy was attempted. Despite control of the renal vasculature, uncontrolled renal hemorrhage occurred, and a left nephrectomy with evacuation of the hematoma was performed. Intraoperatively she received 7 units of PRBCs, 4 units of fresh frozen plasma, 25 units of cryoprecipitate, and 15 units of platelets. The patient’s recovery was uneventful and she was discharged on postoperative day 3.
History and Physical
Retroperitoneal bleeding is a life-threatening condition. Diagnosis is made difficult even for the astute clinician as the presentation can be variable and nonspecific. Accurate diagnosis requires a high clinical index of suspicion as well as an accurate and focused history and physical examination. Rapid elucidation of recent trauma, known malignancy, recent surgical procedures, history of aneurysmal disease, current use of anticoagulation, and ongoing pancreatitis are keys to narrowing a wide differential diagnosis.[rx]
The most common presentation of bleeding into the retroperitoneal space is abdominal or flank pain.[rx][rx] This may be accompanied by femoral nerve palsies in 20 to 30% of cases or radiation of pain into the groin or pelvis. Presentation exists on the spectrum of hemorrhagic shock with patients demonstrating anxiety and tachycardia early on in the disease process and hypotension, confusion, and hypothermia later. Physical examination in some cases may demonstrate the “Wunderlich syndrome” described as abdominal, back, flank, or groin pain, a palpable abdominal mass, and shock.[rx][rx]
Eponyms abound in the description of abdominal bleeding and are of use in a minority of cases. Grey-Turner’s sign occurs with the development of a hematoma along the lateral abdominal wall secondary to perirenal space bleeding tracking along with the quadratus lumborum. Cullen’s sign is a periumbilical ecchymosis that develops when retroperitoneal bleeding dissects along the falciform ligament anteriorly. Fox’s sign is ecchymosis of the anteromedial thigh from blood tracking along the psoas and iliacus muscles’ fascial planes. Bryant’s sign occurs when the scrotum fills with blood and becomes ecchymotic from abdominal bleeding tracking along the spermatic cord. Finally, Stabler’s sign is the development of a hematoma and bruising overlying the inguinal ligament.
In some cases, symptoms may be absent or overshadowed by other distracting injuries, particularly in trauma. Here, careful attention to the mechanism of injury and commonly associated injuries must be paid. Genitourinary involvement of bleeding may manifest as hematuria, while oliguria may develop from adjacent extrinsic compression by a developing hematomal mass. Additionally, pancreaticoduodenal involvement can present with gastric outlet obstruction.
The single most useful laboratory measurement to narrow the differential diagnosis towards retroperitoneal bleeding is falling hemoglobin; however, it must be emphasized that patients bleed whole blood, so it may take some time with or without crystalloid volume resuscitation to recognize this acute hemorrhagic anemia. Other useful studies include prothrombin time, partial thromboplastin time, international normalized ratio, and thromboelastography to identify a patient’s anticoagulation status. Urine analysis can evaluate for urinary tract involvement. If bleeding is suspected or ongoing, a blood type and crossmatch is essential.
The focused assessment with sonography for trauma (FAST) has profoundly altered the approach to diagnosis and management of abdominal trauma. Unfortunately, one well documented profound shortcoming of abdominal ultrasonography is the inability to effectively evaluate the retroperitoneal space. Because of this, ultrasound remains an incredibly useful tool in evaluating for concomitant free fluid in the peritoneal space, but cannot be reliably used to rule in or out retroperitoneal bleeding. The FAST exam is also limited in several other ways.[rx] It is unable to identify an intraperitoneal fluid volume less than 200ml, nor can it differentiate between different types of fluid such as blood, urine, or stool.[rx]
Also common in the trauma bay are the routine cervical, chest, and pelvic roentgenograms. A pelvic fracture may be the clinician’s first indication that the inferior retroperitoneum is involved; however, outside of the trauma setting, roentgenograms lack usefulness. The computed tomographic (CT) cross-sectional imaging of the abdomen with intravenous contrast has been identified as the superior study for narrowing the broad differential associated with retroperitoneal bleeding. CT can easily, rapidly, and repeatedly identify the specific hematomal location, concomitant injuries, and determine if bleeding is ongoing.[rx][rx]
CT examination is not without its negatives. The scan utilizes ionizing radiation, which can predispose a patient to develop cancer at a later date. The use of intravenous contrast can cause an acute kidney injury. Evaluation of pregnant women and children should emphasize studies that forgo the use of ionizing radiation such as abdominal ultrasonography and magnetic resonance imaging.
Treatment of Wunderlich Syndrome
The treatment of retroperitoneal bleeding can be thought of in a stepwise approach from nonoperative management to angioembolization or, in severe cases, surgical intervention. The determination of the specific treatment strategy is dependent on several factors, including the location of the retroperitoneal bleeding, the patient’s stability, and the etiology of the bleeding. In all cases, regardless of whether or not trauma has proceeded the initiation of bleeding, initial resuscitation must begin with the use of the “ABCDE’s” of trauma. Simultaneous to the implementation of this algorithmic resuscitative approach, the search for the etiological cause and assessment of patient stability is undertaken. If at any time, the patient is noted to be hemodynamically unstable or demonstrate peritoneal signs, an urgent surgical intervention must be considered. Other indications that warrant immediate surgical intervention in trauma are based on the location of bleeding, which will be discussed here.
The retroperitoneum has been described as having three anatomical zones. Zone 1 is centrally located in the upper retroperitoneum and includes the aorta, inferior vena cava, pancreas, esophagus, and duodenum; zone 2 is located laterally to zone 1 and includes the ascending and descending colon, the kidneys and their ureters, and the adrenal glands; finally, zone 3 is located across the inferior portion of the retroperitoneum and contains the iliofemoral vessels as well as the rectum.
When retroperitoneal bleeding has been identified in trauma, the mechanism guides management. With penetrating trauma, zones 1 and 3 should be immediately surgically explored in all cases. Zone 2 is explored if bleeding is noted to be ongoing. In blunt trauma, zone 1 is always explored, and zone 2 is explored if bleeding is noted to be ongoing; however, if zone 3 bleeding is identified, surgical intervention should not be attempted in the setting of blunt trauma as the typical source is a pelvic fracture and associated venous plexus bleeding. Here, exploration can worsen bleeding by disrupting partially formed coagulum and removing hematomal tamponade.
Next, we will briefly discuss the primary survey of trauma resuscitation. The primary survey can be easily remembered using the “ABCDE” mnemonic. This begins with an assessment of the patient’s airway, “A,” to determine if it is patent. This is followed immediately by confirmation that the patient is breathing, “B.” If either a patient’s airway or breathing has been compromised, rapid intubation should be performed to maintain sufficient oxygenation and ventilation. Next, “C” stands for the obtainment of vascular access and maintenance of circulation. Massive transfusion protocol should be initiated early if bleeding is profound, especially if a patient’s presentation is concerning for hemorrhagic shock. Fluid resuscitation should follow trauma guidelines with no more than two liters of crystalloid fluid administration before transfusing packed red blood cells, platelets, and plasma in a 1 to 1 to 1 ratio.[rx]
Assessment of patient disability, “D,” then occurs and includes performing a Glasgow coma scale, spine sweep, and assessing pupils for size and reactivity to light. Finally, one must completely expose “E,” the patient’s skin, and remove their clothing to complete a comprehensive physical exam. In the context of retroperitoneal bleeding, the emphasis is paid to the abdominal exam. The astute clinician must also elucidate a focused history, typically described with the acronym “SAMPLE,” consisting of the patient’s symptoms, allergies, medications, past medical history, last meal, and events proceeding their presentation. Reversal of anticoagulation, administration of blood products, and identification of concurrent pathology are paramount to the initial assessment.
If bleeding is ongoing and the patient is sufficiently stable, angioembolization may be employed. During this procedure, a catheter is introduced into a more peripheral artery, most commonly the femoral artery, and advanced in a retrograde manner to the area of suspected bleeding. Radiopaque contrast is infused, and image processing, such as digital subtraction angiography, allows the vascular flow and potential extravasation of contrast to be identified. The benefit of defining vascular injury with interventional angiography over CT angiography or magnetic resonance angiography is the possibility for immediate intervention. Once an injury is identified, hemostasis is achieved through the use of coil or particle embolization. Repeat contrast investigation demonstrates cessation of bleeding.[rx][rx]
For all patients with retroperitoneal bleeding, especially those managed nonoperatively, frequent reassessments are paramount to successful outcomes. Nonoperative management includes the reversal of anticoagulation, crystalloid and blood product resuscitation, serial abdominal examinations, and etiological identification of bleeding. Once an underlying cause is known, it should be reversed or corrected if possible. During all stages of the resuscitative process, careful attention must be paid to the patient’s vital signs, particularly blood pressure and heart rate, as retroperitoneal bleeding can be a dynamic process from which patients may deteriorate despite resuscitative efforts.[rx]
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