Spontaneous dissection of the celiac artery with concurrent dissection of the splenic artery without abdominal aortic dissection is very rare [1]. Spontaneous visceral artery dissection was first reported in 1947, which occurs most frequently in the superior mesenteric artery and rarely in the celiac trunk [1]. In the past, celiac artery dissection could only be confirmed by autopsy. However, advances in diagnostic tools such as contrast-enhanced computed tomography (CT), CT angiography, magnetic resonance angiography, and Doppler ultrasonography have improved the rate of diagnosis. Disease management includes medical and surgical treatment and endovascular intervention.

We report two similar cases of isolated spontaneous splenic artery dissection in patients presenting to the emergency department (ED) and review the etiology, epidemiology, diagnosis, and treatment of this entity.

Isolated celiac artery dissection is rare but can be fatal [2]. Naganuma et al. [2] found 72 case reports of isolated celiac artery dissection from 1964 to 2011. The most important etiology is atherosclerosis. Other known risk factors are trauma, pregnancy, cystic medial degeneration (Marfan syndrome), connective tissue disease, periarterial inflammation, fibromuscular dysplasia, congenital disorders of the vascular wall (such as Ehlers-Danlos syndrome), syphilis, polyarteritis nodosa, and fungal infections [3-5]. Naganuma et al. [2] suggested that hypertension and smoking may also be associated with isolated dissection of abdominal aortic branches. However, in more than half of all cases, none of the risk factors mentioned above were found, similar to the cases presented here [3]. Spontaneous dissection of visceral arteries occurs predominantly in males. About 83% to 88% of cases occur in men, with most cases occurring between 45 to 87 years (average age, 55 years) [4,6,7]. About half of splanchnic artery dissections present asymptomatically because there is little involvement of the small intestine [4,5,8]. The most common symptom of celiac trunk dissection is abdominal pain. Other symptoms associated with celiac trunk dissection are obstructive jaundice and postprandial abdominal pain. These symptoms are often related to the dissected lesions. For example, malabsorption is caused by dissection in the superior mesenteric artery, jaundice in the hepatic artery, and pancreatitis in pancreatic arterial arcades [7,8]. Chronic dissection may present as intestinal angina, including postprandial abdominal pain and weight loss [4,5,9].

In previous studies, diagnosis of isolated celiac artery dissection was possible only through autopsy [7,9]. Recently, more advanced diagnostic techniques have been developed, such as contrast-enhanced CT, CT angiography, magnetic resonance angiography, Doppler ultrasonography, and conventional angiography [3,4,9]. Among these techniques, enhanced CT and CT angiography are considered to be the diagnostic imaging modalities of choice due to their convenience and reliability. Doppler ultrasonography is also widely used because it is the least invasive without exposure to radiation [3,7]. The key to diagnosis of visceral artery dissection is to find an intimal flap [10]. However, this intimal flap is not always easily found, and a mural thrombus may be the only indicator of splanchnic artery dissection [4,11,12]. The acute inflammatory process causes a high attenuation of fat around the dissection in symptomatic patients, though high attenuation is thought to be a nonspecific sign [13].

Celiac trunk dissection can be managed by medical treatment, surgery, or endovascular intervention. Conservative management includes anticoagulation and antihypertensive drug regimens to prevent thrombotic complications such as visceral infarction and ischemia, and prevent progression of artery dissection [4,11]. Surgical or endovascular treatment is indicated for complications such as arterial rupture and liver or bowel ischemia, and when artery dissection progresses despite medical treatment [1,3,4]. Surgical treatments include small bowel resection, thrombectomy, resection of the dissected artery with anastomosis, and artery bypass grafting. Endovascular interventions consist of insertion of a bare stent or stent graft, fenestration, and transcatheter embolization [4,10,13,14]. Splenic infarction is uncommon and incidence of splenic infarction due to celiac artery dissection is even rarer [15]. As in our cases, medical treatment is indicated for cases of splenic infarction with concurrent celiac trunk dissection without any complications. However, if there are complications, such as abscesses or rupture, the patient should undergo surgical treatment [15].

Isolated celiac artery dissection is uncommon, and can be misdiagnosed initially in the ED. If a patient persistently suffers from unexplained acute abdominal pain, care providers should consider performing contrast-enhanced CT, CT angiography, or Doppler ultrasound to exclude vascular events. Once a diagnosis has been made, ED providers should consult a vascular surgery specialist to tailor the treatment modality to the patient. If the patient is hemodynamically stable without complications, conservative management is recommended as first-line treatment. However, if the patient becomes hemodynamically unstable, complications arise, or medical treatment fails, caregivers should consider surgical or endovascular treatment.