Article

Spinal cord ischemia and spontaneous epidural hematoma caused by spinal epidural arteriovenous malformation: a warning

Case Report

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American Journal of Emergency Medicine

journal homepage: www. elsevier. com/ locate/ajem

Spinal cord ischemia and spontaneous epidural hematoma caused by spinal epidural arteriovenous malformation: a warning?

Abstract

Spinal epidural arteriovenous malformations (AVMs) are rare dis- eases. In a pediatric emergency setting, spinal epidural AVMs should be considered as part of the differential diagnosis for acute extremity weak- ness and numbness. The authors report herein a case of spinal cord ische- mia and Spontaneous spinal epidural hematoma (SSEH) arising from an epidural AVM. A 13-year-old Adolescent boy presented with recurrent se- vere interscapular pain followed by complete motor and sensory paraly- sis. The magnetic resonance imagingT2 sequences demonstrated increased signal within the bilateral anterior horns involving the anterior spinal artery territory. The patient was misdiagnosed as having transverse myelitis until epidural hematoma occurred and postoperative pathologi- cal studies confirmed an AVM. AVMs are recognized as 1 reason for spinal cord ischemia and have been identified as underlying lesion for SSEH Howell et al. (1987), Aminoff et al. (1974) [1,2]. Although suspected, there were no vascular malformations identified as the underlying lesion for spinal cord ischemia and hematoma in the same patient. To our knowledge, this represents the first case of spinal cord ischemia and SSEH caused by a histologically proven epidural AVM.

A 13-year-old previously health adolescent boy was awakened with severe interscapular pain which radiated around the chest wall. Complete paraplegia with total sensory and motor function loss was developed at the level of the T4 dermatome within 30 minutes, without bladder or rec- tal incontinence. Initially, he was admitted to a local children hospital emergency department and had noncontrast magnetic resonance imag- ing (MRI) of spine, which demonstrated abnormal signal within the spinal cord from C7 to T2 (Fig. 1). A misdiagnosis of transverse myelitis was made. Before treatment, his symptoms had improved; then he received intravenous methylprednisolone and immunoglobulin for 6 days. Within 2 days, his sensory symptoms completely resolved; in 7 days, he could walk with assistance; and 2 months later, he made Full recovery.

The patient was well until 5 months later, at which point the same symptom developed. One hour later, the symptom partially resolved; 3 hours later, he could walk slowly, but numbness persisted. As the symptom resolved rapidly, he was not brought back for immediate medical attention. Two days after, he experienced a third episode when he was sleeping. The symptoms were similar to the previous episode but combined concurrent uri- nary incontinence. Three hours later, urinary incontinence completely recov- ered and back pain was relieved. He was taken back to the emergency department. On admission, the patient was afebrile, awake, and alert, and neurologic evaluation revealed complete paralysis of lower extremities with loss of sensation below the T4 dermatome. Deep tendon reflexes in the

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lower extremities were present, and Babinski response was positive. A lum- bar puncture was performed; results of Cerebrospinal fluid analysis (white blood cell count, Red blood cell count, chloride, glucose, bacteria, antibodies, oligoclonal bands) were normal. Results of blood tests, including platelet count and coagulation profiles, were all within normal limits. Result of cranial computer tomography was normal. Six hours later, neurologic evaluation re- vealed lower extremities muscle power scored as 4/5 and hypesthesia below the T4 dermatome, 2+ deep tendon reflexes in upper extremities, 3+ deep tendon reflexes in lower extremities, and positive Babinski response. The next day, noncontrast MRI of the spine demonstrated that the spinal cord became narrow from C7 to T2 (Fig. 2). At noon on the seventh day after admission, he experienced a fourth episode. The symptoms were similar to previous epi- sode, but he could not relieve spontaneously. Emergency spinal MRI showed an epidural hematoma on the dorsal side of the Spinal canal extending from C7 to T3 (Fig. 3). Emergent Surgical decompression was undertaken, and pathological studies confirmed the AVM (Figs. 4-8). The postoperative recov- ery was uneventful, and he regained full muscle strength and sensation; but he had Urinary retention with postvoid residual volume of about 100 mL and required self-catheterization every day. He underwent spinal angiogra- phy at 2 months after discharged, which revealed no abnormality (Fig. 9). At 6-month follow-up, after receiving Hyperbaric oxygen therapy, his bladder function recovered.

Spinal arteriovenous lesions are classified by their nidus location, vascular supply, and drainage pattern. The majority of AVMs within spi- nal canal are intradural; epidural arteriovenous malformations are rare, especially in pediatric patients. There are few cases reported [1-4].

Acute spinal cord ischemia is an uncommon and urgent occurrence, and accounts for less than 1% of all strokes [5]. Given the rarity, it may be misdiagnosed as other pathological processes such as transverse mye- litis. AVMs in the epidural region may cause venous hypertension, which can lead to reduced perfusion, and thus spinal cord ischemia with trans- verse syndrome [6]. Noncontrast MRI is insensitive for the early detection of AVMs. Furthermore, some abnormal scans were read as normal in real- world practice [7]. In retrospect, we speculate that our patient presented with recurrent spinal cord ischemia of transverse syndrome caused by epidural AVM with increased T2 signal of the cord and no flow voids.

The first spontaneous spinal epidural hematoma (SSEH) case was report-

ed in a 14-year-old adolescent girl in 1869 [8]. The typical clinical presenta- tion of SSEH consists of acute preceding severe neck or back pain followed by sudden onset of Neurological deficits. The etiopathogenesis of SSEH is not clear. There are many Predisposing factors including anticoagulation thera- py, intense piano playing, vascular malformation, sneezing, trauma, introgenic manipulations, and hypertension; some even deny any predispo- sition [9-15]. Because of improvement of radiology, myelography has been replaced by MRI as the most suitable diagnostic method, but digital subtrac- tion angiography remains the definitive test [6,15].

0735-6757/(C) 2016

Fig. 1. Emergency MRI showed inhomogeneous hyperintense signal changes in spinal cord on T2WI (sagittal, axial) beginning at C7 to Th2.

The transverse syndrome following spinal cord ischemia caused by AVMs is rare and nonspecific, but it is a really urgent condition. Symp- tom and abnormal MRI signals of the spinal cord caused by AVMs are similar to acute transverse myelitis, so AVMs may be misdiagnosed as myelitis. Epidural AVMs should be diagnosed early because it may cause SSEHs. If emergency physicians keep this disease process in their differential diagnosis of transverse syndrome, they may be able to make an early diagnosis, decrease morbidity, and improve prognosis.

Hai-Feng Wang, MD1

Department of neurosurgery, Beijing Hospital National Center of Gerontology, No. 1 DaHua Rd, Dong Dan

Beijing 100730, China Graduate School of Peking Union Medical College, No. 9 Dongdansantiao, Dongcheng District, Beijing 100730, China E-mail address: [email protected]

1Tel.: +86 10 85 132 621; fax: +86 10 65 237 928

Fig. 2. MRI of the spine cord from C7 to T2 became narrow and was interpreted as high signal intensity on T2-weighted images.

Jun Lu, MD2 Li-Jun Wang, MD2 Peng Qi, MD2

Jun-Jie Wang, MD2 Shen Hu, MD2

Xi-Meng Yang, MD2 Kun-Peng Cheng, MD2

Department of Neurosurgery, Beijing Hospital, National Center of Gerontology, China, No. 1 DaHua Rd, Dong Dan, Beijing 100730, PR China E-mail addresses: [email protected] (J. Lu), [email protected] (L.-J. Wang), [email protected] (P. Qi), [email protected]

(J.-J. Wang), [email protected] (S. Hu), [email protected] (X.-M. Yang), [email protected] (K.-P. Cheng)

2Tel.: +86 10 85 136 282; fax: +86 10 65 237 928

Fig. 3. Sagittal T1-weighted images showed spinal epidural hematoma extending from C7 to T3 level, displacing the spinal cord anteriorly and slightly to the left.

Da-Ming Wang

Department of Neurosurgery, Beijing Hospital National Center of Gerontology, No. 1 DaHua Rd, Dong Dan

Beijing 100730, China Graduate School of Peking Union Medical College, No. 9 Dongdansantiao, Dongcheng District, Beijing 100730, China Corresponding author at: Department of Neurosurgery, Beijing Hospital National Center of Gerontology, China, No. 1 DaHua Rd, Dong Dan

Beijing 100730, PR China Tel.: +86 10 85136283; fax: +86 10 65237928

E-mail addresses: [email protected]

[email protected]

http://dx.doi.org/10.1016/j.ajem.2016.09.042

Fig. 4. Scanning magnification shows irregularly dilated and thickened, hyalinized wall vessels separated by connective tissue and fibroadipose tissue; the dark red thrombus could be seen in lumen of vessels; and there was additional small vessel component consisting of capillaries and venules (hematoxylin and eosin, x100).

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    Fig. 5. Irregularly dilated and thickened wall vessels in fibroadipose tissue; immunohistochemically, the Endothelial cells were consistently positive for CD34. (CD34, x100).

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    Fig. 6. Scanning magnification shows irregularly dilated and proliferated vessels; the cells of vessel wall were positive for smooth muscle actin (smooth muscle actin, x100).

    Fig. 8. Note decreased vascular smooth muscle cell (red) density in dilated vessel wall and collagenization of the smooth muscle cells (green) (Masson trichrome, x100).

    Fig. 7. Elastic stain shows thick wall vessels that lack elastic fiber and internal elastic lamina (Verhoeff-van Gieson, x100).

    Fig. 9. Digital subtraction angiography with catheter in the right subclavian artery showing no vascular malformation.

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