Aortic dissection presented as paraparesis: a case report with literature
review
主動脈夾層動脈瘤表現為下肢輕癱：病例報告與文獻回顧
CL Hui許正力and PY Lau婁培友
One case of acute type B aortic dissection presented with paraparesis and retrosternal chest pain was treated conservatively with warfarin and blood pressure control with good recovery of symptoms. A literature review including 25 cases of aortic dissection presented with paraparesis/paraplegia from year 1980 to 2010 is also presented. (Hong Kong j.emerg.med. 2013;20:312-316)
一例急性B型主動脈夾層瘤表現為下肢輕癱和胸痛，通過華法林和血壓控制的保守治療症狀恢復良好。 本文也回顧了從1980年到2010年包括25例主動脈夾層瘤表現為下肢輕癱/截癱的文獻。
Keywords: Aortic disease, dissecting aneurysm, paraplegia, spinal cord ischemia
關鍵詞：主動脈病、夾層動脈瘤、截癱、脊髓缺血
Correspondence to: Hui Ching Lik,MBChB
United Christian Hospital,Department of Orthopaedics and Traumatology, 130 Hip Wo Street, Kwun Tong, Kowloon, Hong Kong
Email: clhuialex@yahoo.com.hk
Lau Pui Yau,FHKAM(Orth), FRCS(Edin), FHKCOS
Introduction
Aor tic dissection is one of the most cr ucial cardiovascular emergencies which can be life threatening. Autopsy data showed an annual incidence of 3.5 per 100,000.1Although most cases associate with
cardinal features of chest pain, rarely it presents with paraparesis due to cord ischaemia. Spinal cord ischaemia has been reported as one of the complications of aortic dissection. We report one patient presented with sudden onset of chest pain followed by paraparesis, subsequently diagnosed to have acute aortic dissection. This emphasizes the importance of careful history taking with subtle signs and symptoms,
which helps in making a correct diagnosis with prompt management. A comprehensive literature review is also presented.
Case
A 70-year-old chronic smoker enjoying good past health, presented to accident and emergency (A&E) department for sudden onset of bilateral lower limbs weakness while walking from toilet at home. At first consultation in A&E department, he also complained of abdominal and back pain without preceding injury. He could still barely walk with assistance without breathlessness, palpitation, dizziness or sphincter disturbance. He had normal appetite without recent weight loss. Bedside ultrasonography (USG) was performed in A&E department which showed gallstones without signs of acute cholecystitis, aortic diameter was measured 1.9 cm and no intra-abdominal free fluid was noted. Physical examination showed bilateral lower limbs weakness with absent knee and ankle jerks and equivocal Babinski's test on both sides
was noted. Abdominal examination showed no palpable mass. The initial impression was acute cord compression and was admitted to the orthopaedics and traumatology department.
On admission, he was stable and afebrile with blood pressure 179/94 mmHg over right arm and 180/80 mmHg over left arm. Physical examination showed paraparesis with Medical Research Council (MRC) grade 3/5 over bilateral L4 to S1 myotomes with intact lower limbs sensation. There was no tenderness over back with normal anal tone and sensation. Straight leg raise test was 90 degrees bilaterally and femoral stretch test was negative over both sides. Electrocardiogram (ECG) showed sinus rhythm without ischaemic change. All blood parameters including cardiac enzymes were normal and bedside abdominal USG was repeated which showed no abnormality except presence of gall stones. Plain X-ray of whole spine showed no bony lesion.
Further history taking revealed that the patient complained of retrosternal chest pain with radiation to the back. Therefore, urgent computered tomogram (CT) scan of thorax and abdomen was arranged 6 hours after initial presentation, which showed type B aortic dissection extending from distal to the left subclavian artery to the left common iliac artery with impaired
left renal perfusion (left renal artery arose from false lumen). Subsequent magnetic resonance imaging of spine showed expansion of distal end of spinal cord and abnormal signal (9 x 6 x 43 mm) in central aspect of spinal cord extending from T11/12 to L1 levels suggesting an area of ischaemia or infarction, related to aortic dissection involving the spinal cord supply (artery of Adamkiewicz) (Figure 1).
Physician and vascular surgeon were consulted and suggested conservative treatment by control of blood pressure using amlodipine and metoprolol. Warfarin was also started with international normalised value maintained between 2 to 3. After 4 weeks of rehabilitation, he was discharged walking with frame independently. He could walk independently with a quadripod after 6 months. His lower limbs power gradually improved with MRC grade 5/5 from L2 down to L5 over both sides and grade 4+/5 over both S1 myotome with intact sensation.
Discussion
There are numerous differential diagnoses for paraparesis, such as central nervous system infection, stroke, autoimmune diseases, spinal cord tumour, spinal cord injur y, spondylosis, etc. Clues to distinguish different causes are always present at first
Figure 1. Magnetic resonance image showed expansion of distal end of spinal cord and abnormal signal (9 x 6 x 43 mm) in central aspect of spinal cord extending from T11/12 to L1 levels suggesting an area of ischaemia or infarction, related to aortic dissection involving the spinal cord supply (artery of Adamkiewicz).
presentation, however, subtle signs and symptoms may easily be missed during our nowadays busy A&E department setting.
Retrosternal chest pain radiating to the back is a cardinal feature of aortic dissection, so paraparesis together with chest or back pain should alarm us the diagnosis of aortic dissection with spinal cord ischaemia. However, paraparesis can be the only clinical presentation making it even more difficult to arrive at a correct diagnosis with subsequent delay in appropriate treatment.
After literature search using MEDLINE with keywords including aortic dissection, paralysis, paraplegia and paraparesis, we identified 25 cases of aortic dissection presented with paraparesis/paraplegia in literature from year 1980-2010 (Table 1). There were 14 (56%) type A and 11 (44%) type B aortic dissections. Mean age was 61.8 years old (range 32-92). Only 10 out of 25 cases (40%) presented with chest, back or abdominal pain. Fifteen out of the 25 cases had history of cardiovascular diseases, and most commonly, hypertension. Five cases received surgical intervention. Ten (40%) patients died and 8 of them were due to type A dissection. Eleven patients enjoyed recovery of symptoms.Literature review of these 25 identified cases showed that only 40% of patients with spinal cord ischaemia presented with paraparesis complaint of chest or back pain. It suggested that more than half of the cases would be missed if other symptoms or signs not being respected. Therefore, in addition to chest or back pain, the onset of paraparesis is important in differentiating different causes. Sudden onset of paraparesis should alert us on causes like spinal cord injury or vascular causes including stroke and spinal cord ischaemia. However, there should be obvious trauma or history of fall when spinal cord injury is suspected. Nevertheless, we may easily overlook this hint as many patients with paraparesis usually present with history of fall. As a result, the sequence of events should be well documented in order to differentiate whether paraparesis is the cause or result of the injury.
Past history of cardiovascular disease also increases the risk of aortic dissection. Physical examination reveals
flaccid paraplegia/paraparesis and paraesthesia with intact lower limbs perfusion in most cases, which makes it difficult to differentiate from cord compression. Although lower limbs vascular deficiency and discrepancy in blood pressure over arms could be present in aortic dissection, most cases presenting with paraplegia/paraparesis in previous literature did not show vascular compromise. Incidence of aortic dissection in a local study in Hong Kong was 2.1 per 100,000.2 The sensitivity of diagnosing aortic
dissection was only 54% in A&E department, and the mortality rate was higher in those with delayed diagnosis.2Aortic dissection presented with paraplegia
is rare. DeBakey et al reported that only 2% of patient with aortic dissection presented as paraplegia.3Aortic
dissection is caused by blood entering through intima of aorta creating a false lumen within the media. It is classified by Stanford classification according to whether the ascending aorta is involved (type A) or not (type B). Patients with type B aortic dissection could recover well after conservative treatment; however, type A aortic dissection would carry a much higher mortality rate and requires urgent operation in most cases.4
Paraplegia was thought to be due to spinal cord ischaemia associated with occlusion of the arterial supply to the spinal cord.5The artery of Adamkiewicz
or great radicular artery gives the dominant arterial supply to the thoracolumbar portion of the spinal cord. Impairment of this artery due to thrombosis or aortic dissection may cause spinal ischaemia. Kieffer et al reported that 77.6% of the artery of Adamkiewicz originates from intercostal or lumbar artery between T9-L1 (86.2% from left side, 13.8% from right side).6
Aortic dissection with a left sided false lumen lying at thoracolumbar junction may be more likely to cause impairment of the artery of Adamkiewicz, which is compatible to our case.
Malperfusion is one of the common reasons for surgical intervention including fenestration, extra-anatomical bypass, or graft endovascular treatment. However, surgical intervention is seldom required and considered in spinal cord ischaemia. Altuwaijri et al reported a case of known type B aortic dissection presented with
Table 1. Reported cases of aortic dissection presented with paraparesis/paraplegia
Author Year Age/Sex PMH Presentation Imaging Type Treatment Outcome
Waltimo & Karli8 1980 52/M HT Paraplegia Angiography A Medical Death
Waltimo & Karli8 1980 46/M N/A Paraparesis and Angiography B Medical Recovery chest pain
Zull & Cydulka9 1988 63/F N/A Paraplegia and Autopsy A Medical Death
chest pain
Zull & Cydulka9 1988 67/M N/A Paraplegia and Autopsy A Medical Death
chest pain
Rosen10 1988 67/F N/A Transient paraplegia MRI A Medical Death
Tanaka et al11 1990 66/M HT Paraplegia, back pain CTA B Medical Recovery
Holloway et al12 1993 92/F N/A Paraplegia MRA A Medical Death
Krishnamurthy et al13 1994 80/M Good past Paraplegia, Thoracic B N/A Death health paraesthesia, back aortography
and bilateral legs pain
Kellett et al14 1997 65/M IHD, MI Paraparesis, CT chest B Medical Partial
back pain recovery
Donovan et al15 2000 77/F MVP, HT, Paraplegia CT chest A Conservative, Partial
angina, complicate recovery
emphysema with stroke
Inamas et al16 2000 50/M Small lacunar Paraplegia, MRI A BP control N/A
stroke, HT parasthesia
Joo & Cummings17 2000 63/F HT, RA Paraplegia, MRI B BP Full
paraesthesia control recovery
Killen et al18 2000 57/M HT, brain stem Paraparesis, CT B CSF Full
stroke with back pain drainage recovery
complete recovery
Syed & Fiad19 2002 32/M Non-Hodgkin's Paraparesis, CT abdomen A Surgery Satisfactory
lymphoma paraesthesia, and chest recovery
lower limbs pain
Blacker et al7 2003 66/F HT Paraplegia and MRA B Medical and Recovery
paraparesis CSF drainage,
stent graft repair
Hsu & Lin20 2004 55/M HT Paraparesis, CT abdomen A Conservative Persistent paraesthesia, back pain and chest paraplegia
Hsu & Lin20 2004 64/F HT Paraparesis CT abdomen A Graft Recovery
and chest replacement
Hsu & Lin20 2004 67/M HT, congestive Paraparesis, CT chest A Death while Death heart failure paraesthesia waiting CT result
Hsu & Lin20 2004 68/M HT, DM, Paraparesis, CT chest B Medical Death
old MI paraesthesia, abdominal pain radiating to back
Chiang et al21 2005 74/F Good past Paraplegia, CT chest A Surgery Death after
health paresthesia sternotomy
Altuwaijri et al5 2006 51/F HT, peptic ulcer Paraparesis CTA B Open aortic Recovery
with Roux-en-Y bypass fenestration
Aktas et al22 2008 54/M Good past health Paraplegia CT A N/A N/A
Aktas et al22 2008 54/M HT Paraplegia CT B N/A N/A
Karacostas et al23 2010 46/M HT, ankylosing Paraparesis, MRI A Died before Death
spondylitis paraesthesia surgery
Current study 2010 70/M Good past Paraplegia, CT, MRI B Medical Recovery
health retrosternal pain radiating to back
N/A: not available; PMH: past medical history; HT: hypertension; IHD: ischaemic heart disease; MI: myocardial infarction; MVP: mitral valve prolapsus; RA: rheumatoid arthritis; DM: diabetes mellitus; MRI: magnetic resonance imaging; CTA: computed tomography angiography; MRA: magnetic resonance angiography; CT: computed tomography; BP: blood pressure; CSF: cerebrospinal fluid
transient paraparesis, aortic fenestration was performed in order to reduce flailing of intimal flap which may jeopardise blood flow to the artery of Adamkiewicz.5
Blacker et al reported a case of type B aortic dissection with paraplegia treated with cerebrospinal fluid drainage followed by stent-graft repair, aiming at increasing the spinal perfusion pressure and resulted in good recovery.7Nevertheless, there is no guideline
for indications of surgery in case of spinal cord ischaemia due to aortic dissection.
Conclusions
Although aortic dissection presented with paraparesis is so uncommon that we may easily overlook especially in our busy A&E department setting, it warrants early diagnosis and treatment as it carries a high mortality rate especially for type A aortic dissection. By starting with a careful history taking and physical examination, one would differentiate the possible causes of paraparesis, and treatment delay could be avoided.
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