Spontaneous Renal Artery Thrombosis and Common Iliac Artery Dissection in a Previously Healthy Young Adult

D Eli Penn, MD; Amber Gist, MD, Cd; R Neal Axon, MD

Disclosures

South Med J. 2008;101(12):1263-1265. 

In This Article

Abstract and Case Report

Abstract

A previously healthy 20-year-old male with a history of easy bruising presented to the emergency department complaining of intermittent left lower quadrant abdominal pain for one week. He was diagnosed with vascular (type IV) Ehlers-Danlos syndrome (EDS) and attendant defects in type III collagen leading to spontaneous left renal artery thrombosis and common iliac artery dissection. Treatment was conservative. The types of EDS and their general management are discussed.

Case Report

A previously healthy 20-year-old male presented to the emergency department complaining of intermittent left lower quadrant abdominal pain for one week. The first episode was mild and lasted only a few minutes, but two days later the pain returned with more intensity, lasted several hours, and was associated with nausea and vomiting. On the day of presentation the pain became 10/10 in severity and radiated straight through to his left back. There were no exacerbating factors, but the pain was temporarily relieved by urination. Review of systems was positive for decreased appetite and chronic constipation, but was otherwise negative.

The patient's past medical history was remarkable only for easy bruising and a weak stomach. He had no prior hospitalizations or episodes of major illness, no surgeries or blood transfusions. As a child he had varicella. All of his immunizations were up to date. He was on no medications and had no known allergies. His family history was remarkable for his mother dying in her 40s to 50s from an unknown vascular problem.

The patient was a tall, thin man in severe pain. His vital signs were unremarkable except for a mildly elevated blood pressure. His skin appeared pale with prominent subcutaneous vasculature. A small hematoma was noted on his posterior left shoulder, and there was a subungual hematoma of the first right toe. Femoral, popliteal, posterior tibial, and dorsalis pedis pulses were full and symmetric. His abdomen was extremely tender to palpation in the left lower quadrant. Bowel sounds were hypoactive. His extremities were notable for arachnodactyly (Fig. 1). Mild leukocytosis and microscopic hematuria were reported. A basic metabolic panel and liver function tests were within normal limits.

Figure 1.

Arachnodactyly and translucent-appearing skin.

Computed tomographic imaging of the abdomen and pelvis revealed an inferolateral infarct of the left kidney with a thrombus in a lower pole branch of the left renal artery. There was also a dissection of the distal left common iliac artery extending into the left external iliac, and what appeared to be another, incompletely occluding thrombus. Magnetic resonance angiography (Figs. 2 and 3) was done for better visualization of the lesions.

Figure 2.

Magnetic resonance angiography demonstrating inferolateral infarct (seen as perfusion defect) of the left kidney.

Magnetic resonance angiography showing dissection of the left common iliac artery.

Further patient history revealed that several of his family members had been diagnosed with Ehlers-Danlos syndrome (EDS). His mother had a spontaneous carotid-cavernous fistula and died during surgery to repair it. The patient's maternal aunt had been diagnosed with type IV, or vascular EDS.

In the present case, the patient was not anticoagulated. A beta blocker was used to control his blood pressure, hoping that its reduction of arterial wall stress might prevent progression of the iliac artery dissection. The patient recovered without sequelae. At discharge we advised him to avoid contact sports and emphasized the necessity of prompt medical attention if he were to develop any new symptoms. Genetic testing and counseling were recommended for the patient and his relatives.

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