Spontaneous Retroperitoneal Hemorrhage - A Rare Presentation of Polyarteritis Nodosa: Role of Angiography in Diagnosis and Management

Case 1

A 46-year-old female, initially, presented with right upper quadrant pain associated with nausea without a history of diarrhea, fever, or dysuria. Physical examination revealed right upper quadrant tenderness with positive Murphy sign, bilateral costovertebral angle tenderness, and epigastric tenderness. Computed tomography (CT) scan revealed right renal infarct. Magnetic resonance angiography of abdomen performed a few days later revealed a new left renal infarct. The differential diagnosis of atrial fibrillation, atherosclerotic embolism, hypercoagulable state, and fibromuscular dysplasia were considered. Her antineutrophil cytoplasmic antibody (ANCA) was negative. Transesophageal echocardiography, Doppler of lower extremities, and other laboratory hypercoagulable state workup were negative; however, the patient was started on anticoagulation. Subsequently, 2 weeks later, she presented with right lower quadrant (RLQ) abdominal pain, diaphoresis, and hypotension. CT scan revealed fractured right kidney with retroperitoneal pericapsular hematoma and multiple hepatic and splenic infarcts (Figure 1). Interventional radiology was consulted for possible embolization of bleeding vessel. The digital subtraction angiography (DSA) performed by experienced interventional radiologist demonstrated fractured right kidney with a large area of devascularization. Multiple areas of vessel wall irregularity with multifocal areas of stenosis, dilatations, and microaneurysms were also seen in the right renal artery (Figure 2). Mild beading of the origin of the left renal artery was also noted. No active extravasation of IV contrast was demonstrated. She was treated conservatively and started on pulsed steroids and cyclophosphamide. On the 6 months follow-up, the patient was stable and was on medical management with methotrexate and folic acid.

Close

Figure 1

A 46-year-old female with abdominal pain. The axial and coronal computed tomography images of abdomen demonstrate fractured right kidney with a large perinephric retroperitoneal hematoma (white solid arrow) and also noted are multiple wedge-shaped hypodensities in liver consistent with infarcts (white hollow arrow).

Export to PPT

Close

Figure 2

A 46-year-old female with abdominal pain. Right renal angiogram demonstrates severe irregularity with multifocal areas of vascular stenosis (white hollow arrow), dilatations, and microaneurysms (white solid arrow) in the right arteries with large areas of devascularization shown by only the upper pole of kidney is opacified with contrast seen as a faint blush of contrast (Curved arrows). The lower pole and the lateral aspect of the upper pole of the right kidney are not visualized, likely devascularized. Incidental note is made of Greenfield Inferior vena cava filter.

Export to PPT

Case 2

A 61-year-old female with hypertension and chronic lower quadrant pain presents to the emergency department with RLQ abdominal pain, nausea, vomiting, and anterior chest pain for 3 days. She had a history of left common iliac vein stent for May-Thurner syndrome (Iliofemoral deep venous thrombosis secondary to compression of the left common iliac vein by the right common iliac artery). CT scan of the abdomen revealed hemorrhage in the right anterior pararenal region. She was hemodynamically stable, but there was a hemoglobin drop from 14.2 mm of Hg to 12.2 mm of Hg. Serum lactate at the time of presentation was 5.2 which later dropped to 2.7 with resuscitation. Her ANCA was negative. CT angiography (CTA), performed subsequently, revealed multiple visceral aneurysms and focal areas of stenosis in the branches of the celiac artery and superior mesenteric artery without active contrast extravasation. Notably, there was a 9 mm pseudoaneurysm in the inferior pancreaticoduodenal artery in the center of the retroperitoneal hematoma (Figure 3). The DSA performed by experienced interventional radiologist revealed multifocal areas of irregularity and strictures in the pancreaticoduodenal branches of the superior mesenteric artery and inferior pancreaticoduodenal pseudoaneurysm (Figure 4). The opacified celiac artery and its branches were irregular with multifocal areas of luminal stenosis and post-stenotic dilatation. Embolization of pseudoaneurysm was then carried out using metallic coils using a sandwich technique (Figure 5). Post-embolization there was no flow through the inferior pancreaticoduodenal artery with good flow through the collaterals to the pancreaticoduodenal bed.

Close

Figure 3

A 61-year-old female with abdominal pain. The axial and coronal computed tomography images of abdomen demonstrating large right anterior perirenal space retroperitoneal hematoma with inferior pancreaticoduodenal pseudoaneurysm (white solid arrow) within the hematoma without active extravasation of IV contrast. Volume rendered three-dimensional image showing the inferior pancreaticoduodenal pseudoaneurysm (white solid arrow) with proximal vascular irregularity and also note multiple areas of luminal dilatation and stenosis (White hollow arrow) involving multiple other arteries such as left hepatic artery, gastroduodenal branches, and renal artery (curved arrows).

Export to PPT

Close

Figure 4

A 61-year-old female with abdominal pain. Superior mesenteric artery angiogram reveals multifocal areas of irregularity and strictures in the pancreaticoduodenal branches and inferior pancreaticoduodenal pseudoaneurysm (White solid Arrow) with proximal luminal stenosis (White hollow arrow).

Export to PPT

Close

Figure 5

A 61-year-old female with abdominal pain. Successful post-embolization of inferior pancreaticoduodenal pseudoaneurysm with metallic coils using the sandwich technique (White solid arrow). Partially visualized is left common iliac vein stent (Black solid arrow).

Export to PPT

Imaging

CTA is the initial modality of choice. Due to an increase in abdominal angiography, some asymptomatic cases are diagnosed by angiography. Knowledge of the angiographic findings is of importance in diagnosing those. The most common angiographic findings are aneurysms, ectasia, and stenosis in 40–90% patients. Other findings include irregular narrowing of the arteries and pseudoaneurysms. However, these findings are not specific as other vasculitis such as rheumatoid vasculitis, Churg-Strauss syndrome, and systemic lupus erythematous may have similar findings. Thus, clinical evaluation is necessary to confirm the diagnosis.^[5] Embolization of the bleeding aneurysms is the treatment of choice. Prophylactically large aneurysms (>2 cm) may be embolized due to the risk of rupture.^[6]

Treatment

Transarterial angioembolization of the pseudoaneurysms is now the preferred treatment modality of choice as it is safe, effective, and less invasive and can be both diagnostic and therapeutic. Usually, visceral artery aneurysms require treatment only when the size is >2 cm, which are symptomatic or show interval growth.^[6] However, pancreaticoduodenal aneurysms are associated with a high risk of rupture and require immediate treatment with embolization or surgery. Transarterial embolization involves radial or femoral access using 4 or 5 Fr sized arterial sheath with selective catheterization of involved arteries performed using Cobra, or Shepherd hook-shaped 4–6 Fr sized catheters, with microcatheters (2.8 Fr) used for super selective embolization to reduce morbidity and the size of infarcted area.^[4,7]

The embolization procedures are performed under moderate sedation or general anesthesia. The choice of agent depends on the site of embolization. Metallic coils (0.035 in or 0.018 in pushable Vortx™ microcoils by Boston Scientific Corporation, Massachusetts, USA and Tornado™ by Cook Medical LLC, Indiana, USA) are commonly used to induce permanent proximal occlusion. These are relatively easy to use and can be retrieved if non-targeted. Sometimes, vascular plugs (Amplatzer™, St. Jude Medical Inc., New Jersey, USA) are used to induce permanent proximal occlusion. They consist of thrombogenic polyester filaments in the detachable nitinol cage. They are used for large vessel embolization with high flow state such as arteriovenous fistulas or aneurysms (splenic or renal).^[8]

Complications

Transcatheter arterial embolization is a relatively safe procedure with low complication rates compared to surgical procedures. The non-specific complications of embolization are related to procedure rather than embolization and include groin hematoma, arterial dissection or thrombosis, contrast reactions, and contrast-induced nephropathy.^[8,9] The specific embolization related complications include non-targeted embolization causing infarctions in kidney, liver, spine, and bowel or pulmonary embolism.^[8] Coil migration can occur but can be removed using snares. Post-embolization syndrome can occur due to necrosis of the masses causing low-grade fever, pain, vomiting, and leukocytosis and can be reduced with pre- and post-embolization corticosteroids.^[7,10]