85
Clinical Brief
SCIWORA-Spinal Cord Injury Without Radiological Abnormality Veena Kalra, Sheffali Gulati, Mahesh Kamate and Ajay Garg1 Department of Pediatrics and 1Department of Neuroradiology, All India Institute of Medical Sciences, New Delhi
ABSTRACT Following trauma, the commonly used radiological investigations, plain radiographs and computed tomography (CT) studies do not rule out injury to the spinal cord. This is especially true for children, as an entity known by the acronym SCIWORA (spinal cord injury without radiological abnormality) exists and the changes may be picked up only on magnetic resonance imaging (MRI). Early treatment (within 6 hours) with high dose methylprednisolone improves the outcome. Spinal trauma being common it is possible that the burden of neurological handicap following this can be reduced by increasing awareness and early treatment with steroids. In the community, pediatricians are often the first medical contact after spinal trauma and awareness of the lacune of conventional imaging techniques is important especially if clinical symptoms pertaining to the spine are present. The community pediatrician is hereby made aware of the need to investigate spinal trauma with a MRI for possible SCIWORA situation as it generates a possibility for therapeutic intervention to alter the outcome positively. [Indian J Pediatr 2006; 73 (9) : 829-831] E-mail :
[email protected]
Key words : Spinal cord; Trauma; MRI
Spinal cord injury without radiological abnormality (SCIWORA) is defined as the occurrence of acute traumatic myelopathy despite normal plain radiographs and normal computed tomography (CT) studies. Though common in children compared to adults, overall incidence is less. As both the radiograph and CT scan be normal and early treatment with high dose methylprednisolone improves the outcome, pediatrician who comes across such a patient should be aware of such condition, its treatment and outcome. We report here a case of a two and a half year old child with SCIWORA who presented to us late, 3 days after trauma with complete flaccid paraparesis with bladder involvement. CASE REPORT A two and a half year old boy presented to us with 2 day history of paucity of movement of both legs, inability to bear weight on his legs, and inability to pass urine. Previous day in the afternoon he had fallen from a tractor. There was no history of any injury to head, unconsciousness, bleeding from ear nose or throat or any seizures. Child was moving his legs after he fell and there
Correspondence and Reprint requests : Prof. Veena Kalra, Head, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi-110029, India; Fax No. : 91-11-26588663, 26588641.
Indian Journal of Pediatrics, Volume 73—September, 2006
was no deformity of legs or spine. Next day when the child woke up, the parents noted that the child was not moving his legs and was not able to sit without support. There was no history of fever or vomiting, no history of any paucity of movement or weakness in upper limbs or any history suggestive of cranial nerve involvement. There was no breathing difficulty or bowel incontinence. On general examination, there was pallor. There was no evidence of any fracture of limb bones, lacerations or deformity or tenderness over the spine. Neurological examination revealed a conscious child with normal cranial nerves and upper limbs. There was gross hypotonia in the lower limbs, 0/5 power and areflexia. Abdominal reflex, cremasteric, anal reflex were absent. Bladder was palpable and urine could be expressed out on abdominal pressure. There were no meningeal or cerebellar signs. CECT brain was normal. There was no evidence of any fracture or displacement of vertebra on radiograph of the spine and CT scan of the spine done on day 1 of illness. Hemogram, LFTs, RFTs, electrolytes were within normal limits. CSF done on day 2 had many RBCs, 10 polymorphs, protein of 80 mg/dl and sugar of 30 mg/dl. MRI of spine done on day 4 showed edema of the cord with expansion from C6 to the lower end of cord (fig 1). In view of history of significant trauma, complete paraplegia, normal radiograph and CT spine, a diagnosis of SCIWORA was made and this was confirmed by MRI of the spine. Proper physiotherapy was advised and 829
86
Veena Kalra et al 66% of all spinal cord injuries (mostly around 10-20% of all pediatric spinal trauma). 1 Although Lloyd 2 first proposed the concept of SCIWORA and Burke 3 was the first to report it, Pang and Wilberger4 were the first to coin the acronym SCIWORA and define it as a clinicoradiological entity. Pathogenesis1,2,5: In young children, the pathogenesis of SCIWORA may be related to the mismatch in the elasticity of the tissue of the vertebral column and spinal cord.
Fig. 1. MR T2 saggital image of the spinal cord shows increased signal intensities within the cord extending from C6 to D3 level. No evidence of any spinal cord compression or haemorrhage in the spinal cord. Vertebrae and ligaments are normal
parents were taught clean intermittent catheterization. In view of young age, complete flaccid paraplegia the parents were counseled regarding the poor outcome. As the child presented to us beyond 6 hours, high dose i.v. methylprednisolone infusion was not given. Spinal Cord Injury WithOut Radiological Abnormality (SCIWORA) : Spinal cord injury without radiological abnormality (SCIWORA) is defined as the occurrence of acute traumatic myelopathy despite normal plain radiographs and normal computed tomography (CT) studies. This occurs predominantly among the pediatric population, where its reported incidence ranges from 4%-
Neurological presentation: SCIWORA can have a wide spectrum of neurological dysfunction, ranging from mild, transient spinal cord concussive deficits to permanent, complete injuries of the spinal cord. The incidence and severity of injury are related to the patient’s age. Young children have a higher incidence of SCIWORA; this age group accounts for two-thirds of all reported cases. Until the age of 8 years, neurological injuries tend to be severe. Three quarters of the injuries in this group are complete. Over half of the injuries in young children occur in the thoracic spine; almost all of these thoracic injuries are complete. Adolescents sustain less severe, typically incomplete injuries. A delay in the onset of neurological deficits or a delayed neurological deterioration had been reported. Brief transient motor or sensory symptoms are often associated with the initial injury. An asymptomatic period usually intervenes. The delays in deficits can range from hours to 4 days after injury.1,5,6 MRI is preferred for acute assessment because it is non invasive, delineates spinal cord and soft tissue abnormalities, and can assess compressive pathology. If MRI facility is unavailable or not possible and acute assessment is indicated then, CT myelography should be performed. Differential diagnosis : The possible differential diagnosis include, traumatic compressive myelopathy (compression by fractured vertebrae, disc herniation etc), and if trauma is not very significant then acute disseminated encephalomyelitis, transverse myelitis are
Mechanisms of injury1, 5 1.
Direct spinal cord traction
2.
Direct spinal cord compression
3. Indirect spinal cord injury 4.
830
Vascular/ischemic injury
a. Longitudinal cord traction b. Root traction/avulsion a. Transient compression i. Ligamentous bulging ii. Reversible disc protrusion iii. Transient subluxation of vertebrae b. Persistent compression (potentially requires operative intervention) i. Occult fracture with cord compression ii. Spinal epidural hematoma iii. Persistent disc herniation iv. Occult subluxation/instability Transmission of externally applied kinetic energy to spinal cordSpinal cord concussion (SCC) a. Vascular occlusion, dissection, cord infarction b. Vasospasm c. Hypotension, impaired cord perfusion.
Indian Journal of Pediatrics, Volume 73—September, 2006
87
SCIWORA-Spinal Cord Injury with Out Radiological Abnormality other diagnostic possibilities. Treatment : SCIWORA involving the cervical spine should be treated by immobilization with a collar or a more rigid brace until neurological deficits have resolved. After the acute phase of injury, it is advisable to repeat the flexion/extension views of the spine to rule out ligamentous instability that may have been masked by paravertebral muscle spasm during the initial evaluation. Once deficits have resolved range of motion is gradually increased. However, to avoid the risk of recurrent injury, activity should be strictly limited for at least 3 months. Patients with thoracic or lumbar myelopathy (SCIWORA) also are initially treated with bed rest and subsequent gradual mobilization. 1 High dose steroidsMethylprednisolone bolus of 30 mg/Kg iv within 8 hrs of injury, followed by infusion at 5.4 mg/Kg/hr for the next 23 hrs is beneficial in improving the outcome. 7 When given over 48 hrs outcome at 6 wks and 6 months was better in a recent study.8 Role of stem cell transplant is emerging. Outcome : The prognosis is related to the severity of the spinal cord dysfunction. Young children tend to sustain complete injuries with permanent deficits; the rate of functional recovery after complete neurological injuries is reported to range from 0-10%. Outcome after incomplete injuries in older children in excellent.1 REFERENCES 1. Dickman CA, Zabramski JM, Hadley MN, Rekate HL, Sonntag
Indian Journal of Pediatrics, Volume 73—September, 2006
2.
3. 4.
5.
6.
7.
8.
9.
VKH. Pediatric spinal cord injury without radiographic abnormalities: Report of 26 cases and review of literature. J Spinal Disorders 1991; 4 : 296-205. Pang D, Sahrarkar K, Sun PP. Pediatric spinal cord and vertebral column injuries. In: Youman JR, editor. Neurological Surgery, 4th ed. Philadelphia: WB Saunders; 1996. p 1991-2037. Burke DC. Traumatic spinal paralysis in children. Paraplegia 1974; 11 : 268-276. Pang D, Wilberger Jr JE. Spinal cord injury without radiological abnormality in children. J Neurosurg 1982; 57 : 114 129. Pang D, Pollack IF. Spinal cord injury without radiographic abnormality in children-The SCIWORA syndrome. J Trauma 1989; 29: 654-664. Tiwari MK, Gifti DS, Singh P, Khosla VK, Mathuriya SN, Gupta SK et al. Diagnosis and prognostication of adult spinal cord injury without radiographic abnormality using magnetic resonance imaging analysis of 40 patients. Surgical neurology 2005; 63: 204-209. Bracken MB, Shepard MJ, Collins WF et al. A randomized, controlled trial of methylprednisolone or naloxone in the treatment of acute spinal cord injury. Results of the second national acute spinal cord injury study. N Engl J Med 1990; 322: 1405-1415. Bracken MB, Shepard MJ, Collins WF et al. A randomized, controlled trial of methylprednisolone or naloxone in the treatment of acute spinal cord injury. Results of the second national acute spinal cord injury study. N Engl J Med 1990; 322: 1405-1415. Bracken MB, Shepard MJ, Holford TR et al. Administration of mehtylprednisolone for 24 or 48 hours or tirilazad mesylate for 48 hours in the treatment of acute spinal cord injury. Results of the Third National Spinal Cord Injury Randomized Controlled Trial. National Acute Spinal Cord Injury Study. JAMA 1997; 277 : 1597-1610.
831