Article, Neurology

Spontaneous spinal epidural hematoma presenting with quadriplegia after sit-ups exercise

Case Report

Spontaneous spinal epidural hematoma presenting with quadriplegia after sit-ups exercise

Abstract

Spontaneous spinal epidural hematoma (SSEH) repre- sents 0.3% to 0.9% of spinal epidural space-occupying lesions, and most surgeons advocate aggressive and early surgical intervention. In this article, we describe a patient with SSEH with sudden quadriplegia after sit-ups exercise.

This 25-year-old man in otherwise good health experi- enced an acute pain syndrome in the interscapular region radiating to both upper and lower limbs, followed by quadriplegia. The patient had complete quadriplegia, analge- sia below the C5 level, and Urinary retention. He had no anticoagulant agent and no coagulopathic disease. He was submitted to magnetic resonance imaging, which revealed a dorsally located epidural hematoma extending from the C6 through T1 level with spinal cord compression and intramedullary hyperintensity. He then underwent laminec- tomies from C6 through T1 and posterior fixation with lateral mass screws of T1 about 16 hours after the onset of the symptom. In postoperative time, the patient presented partial sensorial recovery and motor force grade III. The patient was directed to a neurorehabilitation program; and 1 month later, he presented recovery without any neurologic deficits.

As evidenced in the literature, outcome depends on time to operation; and prognosis is impacted by age and preoperative deficit. Because of the high risk of poor outcome without treatment, SSEH should always be a diagnostic consideration in patients of hyperacute spinal cord compression. Rapid, appropriate treatment of these patients can often lead to Complete recovery of function, whereas any delay in appropriate treatment can be catastrophic.

Acute spontaneous spinal epidural hematoma (SSEH) is an extremely rare hemorrhagic spinal pathology [1-7] defined as the presence of blood in the epidural space in the absence of an underlying hematologic or hemostatic disorder, systemic or local disease, vascular malformation, or trauma [5,8]. It represents 0.3% to 0.9% of all epidural space- occupying lesions [1]. First described in 1869 by Jackson [9], approximately 460 have been reported. Many potential

causes have been discussed [10-15]. In our review of the literature, only 5 cases of SSEH after exercise have been reported [12,14-18]. Some of these patients had additional risk factors, such as repeated Valsalva maneuvers [14,15,17] or antiplatelet treatment.[16,18] Because SSEH is uncom- mon, numerous differential diagnostic tests often delay diagnosis and treatment, resulting in increased risk of permanent neurologic deficit [1,3,5]. The differential diagnoses include pathologic fractures, metastatic disease, intervertebral disc prolapse, dissecting aortic aneurysm, anterior spinal artery syndrome, transverse myelitis, spinal Epidural abscess, spinal subarachnoid hemorrhage, hemato- myelia, and spinal epidural lipomatosis [1,2,5]. The standard treatment of these hematomas is prompt surgical evacuation [11,15,18-20], although spontaneous recovery has been reported [12-14,16,17].

A 25-year-old previously healthy man presented to the emergency department with a complaint of sudden onset of interscapular pain radiating to both upper and lower limbs, followed by quadriplegia. He denied any trauma or medications. On further inquiry, he admitted feeling sharp interscapular pain during sit-ups exercise. There was no medical history of any recent trauma or illness, and he was on no medication.

On presentation, his vital signs were stable. He was in apparent distress due to interscapular and back pain. Neurologic examination showed a moderate bilateral ankle and patellar clonus and Babinski sign. The anal wink reflex was diminished, and the bulbocavernosus reflex was present. The calcaneal and patellar tendon reflexes were classified as 3 bilaterally. The muscle power was grade 0 in lower limbs and grade 2 in upper limbs (Medical Research Council grading). Sensory deficits were found bilateral to lower extremity anesthesia below the C5 dermatome. Routine laboratory and coagulation parameters were within normal limits. C-reactive protein and erythrocyte sedimentation rate were also normal. Afterward, he underwent Spinal magnetic resonance imaging . Magnetic resonance imaging with gadolinium showed a crescent-shaped-like mass, dorsally to the spinal cord, extending from C6 through T1, with well- defined contours. Epidural location was assessed by the presence of cerebrospinal fluid around the spinal cord at the compressed levels. The mass was isointense on T1-weighted

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images and hyperintense on T2-weighted images, with spinal cord compression and increased signal intensity of the cervical cord on T2-weighted image (Fig. 1). Intravenous pulse prednisolone (bolus of 30 mg/kg and maintenance of 5.4 mg/kg per hour for 23 hours) therapy was initiated, and he was admitted to the neurosurgery ward. Surgical decompres- sion was performed the following day. The interval since the onset of symptoms was almost 36 hours (surgery was performed 16 hours after hospitalization). Posterior C6 through T1 laminectomies were performed without abnormal intraoperative bleeding, and the hematoma was completely evacuated. Internal fixation with lateral mass screws of C6-7 and transpedicle screws of T1 was done at the end of surgery (Fig. 2). Samples of tissues and hematoma were sent for bacteriologic culture (including Mycobacterium and myco- logic agents) and anatomopathologic analysis. The post- operative period was uncomplicated. In postoperative time, the patient presented partial sensorial recovery and motor

force grade III. The patient was directed to a neurorehabil- itation program; and 1 month later, he presented recovery without any neurologic deficits.

Spontaneous spinal epidural hematoma is a relatively rare but important neUrologic emergency. Spontaneous spinal epidural hematoma is defined as bleeding outside the dura mater, within the Spinal canal, without an obvious precipitat- ing cause. Bleeding due to trauma, anticoagulants, bleeding diatheses, or tumor is excluded. The incidence of SSEH is

0.1 patient per 100 000 [21,22] and, thus defined, has been estimated as less than 1% of all spinal epidural lesions. Idiopathic cases account for approximately 40% to 60% of all spinal epidural hematoma [23,24]. The cervicothoracic and thoracolumbar regions are most commonly affected, with more than one third of cases in the C5 through T2 levels [17,25]. It has been reported in patients from 14 months to 90 years of life, and most cases occur after the fourth or fifth decade [22,26]. The Potential causes include undiagnosed

Fig. 1 Sagittal and axial T1-weighted (A and B), T2-weighted (C and D), and gadolinium-enhanced T2-weighted (E) MR images showing an isointense fusiform intraspinal lesion at the C6 through T1 levels (on T1-weighted images), a hyperintense (on T2-weighted images) and mildly Gadolinium peripheral enhancing epidural mass located in the right posterior aspect of the spinal canal with compression of the spinal cord and intramedullary hyperintense signal were noted.

Fig. 2 Sagittal (A) and axial (B) T2-weighted images of the same area 4 weeks later showing complete resolution of the collection after the patient underwent C6 through T1 laminectomies and evacuation of the intraspinal lesion, which was proven to be a fresh blood clot (C).

acquired or congenital coagulopathies, hypertension, increased venous pressure (during coughing, sneezing, vomiting, training to void or stool), seemingly trivial daily events leading to minor trauma (bending, twisting, lifting), and cocaine or alcohol use [10,12,13-22]. Anatomic lesions predisposing to SSEH include venous angiomas, hemangio- mas, arteriovenous malformations, unclassifiable venous abnormalities, arterial aneurysms, vascular neoplasms, and Paget disease [14]. In those cases in which there is no underlying vascular abnormality, the bleeding is believed to be venous in origin [9], although an arterial etiology has also been suggested [25]. Proof of either is difficult because, in most cases, there is lack of angiographic documentation of the lesion and, even with open surgical exploration and clot removal, the specific cause is often not evident [16,27]. Epidural venous plexus has been considered the source of hemorrhage for SSEH by many authors. The spinal epidural space contains an extensive internal venous plexus, which communicates with the external vertebral venous plexus. Together, they form the Batson plexus, which is a valveless

low-pressure system [17,28]. This system provides an alternate route for venous return from other areas of the body and assists in regulating volume and pressure changes between the intrathoracic, intraabdominal, and intraspinal systems [27,28]. Valsalva maneuvers, such as straining, coughing, or lifting, increase intraabdominal and intrathor- acic pressure [27,28]. This, in turn, is transferred to the spinal epidural Venous system [27] and is thought to contribute to the rupture and hemorrhage of the epidural venous vessels [17]. The basic pathophysiologic cause is presumed to be a weakened vessel in a preexisting abnormal venous plexus. Sit-ups exercise may involve a Valsalva maneuver, which may be accentuated by improper breathing. Although athletes or soldiers are taught to exhale with contraction, many do not use this technique. Many untrained people wanting to perform faster, as in our patient, will often take sit-ups exercise without proper knowledge or guidance and may be more susceptible to accentuated Valsalva maneuvers [15,17]. In the absence of any other Predisposing factors, it was felt that the SSEH in our patient was caused by the sit-

ups exercise. The typical presentation of SSEH is that of the acute onset of localized back, neck, or interscapular pain followed by radicular pain in the extremities and signs and symptoms of spinal cord or cauda equina compression [17,25]. The initial pain is usually localized and is referable to the level of the lesion [11,15-17,27]. Progressive sensory and motor deficits usually occur within minutes or hours, but occasionally after many days. The differential diagnosis of SSEH includes the following: acutely ruptured intervertebral disc, epidural neoplasia, transverse myelitis, a dissecting aortic aneurysm, congenital cysts, spondylitis, infection, spondylitis, toxic exposure, Guillain-Barre syndrome, and pathologic vertebral fractures [14,16,]. Acute or progressive painful paraparesis and/or paraparesis with sphincter dys- function is a well-known indication for an emergency MRI. Magnetic resonance imaging is the most useful method for diagnosis of SSEH. It provides information about differential diagnoses with the pathologies that most frequently present with spinal pain and neurologic deficit [1,2,7]. It can evaluate the location, extent, and compressive effects of hemorrhage. The epidural hematoma appears as a long segmental fusiform axial mass compressing or displacing the spinal cord. It may affect any segment of the spinal canal; and its size is variable, typically multisegmental. In the Hyperacute phase, MRI demonstrates an isointense lesion on T1-weighted images and hyperintense lesion on T2-weighted images. In the late or subacute phase, the hemorrhage demonstrates a hyper- intense lesion on T1- and T2-weighted images [29]. A computed tomography scan with or without myelography is an alternative. It is less specific, and it can delay diagnosis and treatment. Alexiadou et al [1] reported 2 cases of false- negative diagnosis in a series of 5 patients with SSEH. Angiography and a computed tomography scan angiography may show vascular malformations. Although some cases of spontaneous recovery have been reported, the most appro- priate treatment of SSEH is Surgical decompression. Laminectomy or alternating hemilaminectomy and evacua- tion of the hematoma are the most effective methods for rapid decompression of the spinal cord. The most important predictors of outcome for neurologic improvement are the time interval between the onset of the symptoms and surgical evacuation and the preoperative neurologic status [1,2,5,7,18,20,22]. Kreppel et al [5] found that patients taken to surgery within the first 12 hours had the best rate of Neurologic recovery. They found the worst outcome in patients with complete paralysis and loss of sensory functions and associated urinary/intestinal disturbances. Other predictors of outcome have been proposed, including age, hematoma extension, segmental localization, and rapidity of symptom development; but Kreppel et al found no statistical correlation between these factors and neurolo- gic outcome. In a report of 15 consecutive cases of SSEH, Borm et al [4] found no correlation between surgical timing and neurologic outcome; but most of their patients were treated within the first few hours (mean time to surgery, 17.9 hours). Therefore, operation should be considered as

soon as possible. Even if increased spinal intensity is present, intensive therapy should be applied because functional recovery may be expected, as in our case.

We report a young patient with SSEH who has no history of any trauma, recent medication, or iatrogenic manipula- tions. Spontaneous spinal epidural hematoma was diagnosed using MRI, and he was operated on immediately. early diagnosis and treatment were the major factors that contributed to the complete recovery of our patient. Spinal epidural hematomas can cause dramatic neurologic deficits, which can be successfully reversed if diagnosed and treated without delay. Emergency physicians and neurosurgeons should be aware of spinal epidural hematoma in adolescents and young patients presenting with back pain and new-onset neurologic deficits, which may be possible complication of sit-ups exercise or other forms of exercise that might be associated with significant Valsalva maneuvers.

Chun-Lin Chen MD Ching-Hsiang Lu MD Nan-Fu Chen MD Division of Neurosurgery Department of Surgery

Kaohsiung Armed Forces General Hospital

Kaohsiung 802, Taiwan, ROC E-mail address: [email protected]

doi:10.1016/j.ajem.2009.01.003

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