Introduction: Spinal cord infarction (SCI) is a rare event accounting for about 2% of all neurological vas-cular diseases. There have been numerous cases re-ported as complications of acute aortic dissection and aorta surgery, but few caused by intramural hematoma (IMH). We report a rare case of intramural aortic he-matoma resulting in ischemia of the spinal cord.
Methods: A 62-year-old Caucasian man presented to the emergency department with sudden onset of lower back pain during intense physical exertion. Within mi-nutes from the onset of pain, bilateral loss of inferior limbs motor function appeared. Physical examination revealed preserved mental status and cognition. Blood pressure was 190/100 mmHg, without significant di-fference between arms. Neurological examination re-vealed complete paralysis and paranesthesia in both legs. The pain and temperature sensory examination revealed a sensory level at T6, although touch, vibra-tion, and position senses were normal. Deep tendon reflex was normal in both upper limbs but was absent in both lower limbs, without plantar response. Compu-ted tomography contrast revealed dilated thoracic aor-ta (maximum diameter 44 mm) and abdominal aorta (maximum 58 mm) with extended intramural hemato-ma. Inward displacement of intimal calcifications was noted and no evidence of intima tear, dissection flap or penetrating ulcers was present at the time of the CT examination. Also, there was no aortic regurgitation or pericardial effusion on transthoracic echocardio-graphy. Magnetic resonance imaging (MRI) excluded other causes of anterior spinal artery syndrome such as acute disc herniation and epidural hematoma. We established the diagnosis of type A intramural aortic hematoma complicated with paraplegia through spinal malperfusion. The patient was assessed by vascular and cardiothoracic surgery teams. Considering the exten-sive aortic disease, surgical repair was not considered feasible in our patient. The patient was treated conser-vatively and ten days after the event was released from the hospital with no improvement of the neurological deficiencies.
Results: Particularities SCI is a well-documented com-plication of open and endovascular thoracic aortic sur-gery, but its association with IMH is very rare. There are 6 other cases described in literature besides our case. Our patient had an extensive Type A IMH associated with vascular compromise of the intercostals and lumbar arteries as a result of a shearing effect on these vessels. The maximum diameter of the thoracic aorta was less than 45 mm; therefore, conservative therapy was preferred. Treatment of IMH aims to prevent pro-gression to dissection and aortic rupture. The primary goal of medical therapy is to reduce aortic wall stress by decreasing the force of left ventricular contraction and systemic blood pressure, the main determinants of dissection extension and rupture. β-Blockers are the first line of treatment, with a target of systolic blood pressure <120 mmHg and heart rate <60 bpm. Simi-lar to aortic dissection, surgery is advocated in patients with type A IMH, and initial medical therapy in pati-ents with type B IMH. Indications for open or endo-graft surgery depend on the anatomy of the lesion, cli-nical presentation, and patient comorbidities. Althou-gh reversal of paraplegia has been reported following open surgical repair of aortic dissections there is mi-nimal role of open surgery and endovascular stenting for the treatment of spinal cord ischemia in the setting of IMH.
Conclusions: Spinal cord infarction is a rare condition commonly associated with aortic diseases and proce-dures. The diagnosis is challenging and usually based on clinical examination as the sensitivity of MRI is li-mited, especially within the first few hours of presen-tation. In patients presenting with suggestive clinical setting, evaluation of the aorta should be included in the diagnostic workup.