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Official Journal of the European Paediatric Neurology Society
Review article
Spontaneous spinal epidural hematoma in infancy: Review of the literature and the “seventh” case report An-Sofie Schoonjans a,b, Jozef De Dooy b,c, Sandra Kenis a, Tomas Menovsky d, Stijn Verhulst b, Johan Hellinckx f, Ingrid Van Ingelghem a,f, Paul M. Parizel e, Philippe G. Jorens c, Berten Ceulemans a,* a
Department of Neurology-Pediatric Neurology, Antwerp University Hospital (UZA), University of Antwerp, Wilrijkstraat 10, B-2650 Edegem, Belgium b Department of Pediatrics, Antwerp University Hospital, University of Antwerp, Belgium c Pediatric Critical Care Unit, Antwerp University Hospital, University of Antwerp, Belgium d Department of Neurosurgery, Antwerp University Hospital University of Antwerp, Belgium e Department of Radiology, Antwerp University Hospital, University of Antwerp, Belgium f Department of Pediatrics, KLINA Regional Hospital, Brasschaat, Belgium
article info
abstract
Article history:
Spontaneous spinal epidural hematomas (SSEH) are a rare cause of spinal cord compres-
Received 30 November 2012
sion in childhood and especially in infancy. We reviewed the literature and describe a case
Received in revised form
of an 8-month-old boy with a large spontaneous cervico-thoracic epidural hematoma. With
14 May 2013
this review we want to detail the importance of early investigation, diagnosis and treat-
Accepted 25 May 2013
ment in infants with SSEH. In our case the infant presented with irritability and crying and an ascending paralysis within four days. Magnetic resonance imaging (MRI) of the spine
Keywords:
demonstrated an extensive epidural hematoma between C5 and L1, serious medullar
Spontaneous spinal epidural hema-
compression and secondary cervical and thoracic medullar edema and hydromyelia. An
toma
emergency laminectomy was performed with evacuation of a well organized hematoma.
Infancy
There was a partial recuperation of the neurologic symptoms.
Pediatric Child
Based on the scarce literature which only concerns seven case reports, SSEH is a rare cause of spinal compression in infancy. The presentation is often not specific and neurological symptoms are often lacking in the beginning. However early diagnosis with MRI and prompt neurosurgical intervention are important to improve outcome. ª 2013 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.
* Corresponding author. Tel.: þ32 3 821 34 23; fax: þ32 3 8214312. E-mail address:
[email protected] (B. Ceulemans). 1090-3798/$ e see front matter ª 2013 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ejpn.2013.05.012
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Contents 1. 2. 3.
4.
1.
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Case report . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . SSEH in infancy: overview . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3.1. Definition . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3.2. Pathogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3.3. Clinical presentation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3.4. Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3.5. Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3.6. Outcome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Introduction
Spontaneous spinal epidural hematomas (SSEH) are an extremely rare neurological emergency. The overall incidence is estimated to be 0.1/100 000/year,1 most frequently seen in the fourth and fifth decades of life.2 The presentation in childhood and certainly under the age of one year (infancy) is, in contrast to adults, often nonspecific with irritability, crying and mild neurological deficits. MRI has been proven to be the modality of choice for diagnosing these lesions with a typical appearance of the hematoma. Prompt diagnosis is essential but difficult in children due to the nonspecific signs and limitations in clinical assessment. We describe such a case in an 8-month-old baby boy and reviewed the six previously reported cases of SSEH in infancy.3e8
2.
Case report
A previous healthy 8-month-old boy, born at term following an uncomplicated pregnancy and delivery, was transferred to our pediatric intensive care unit with recent unexplained progressive hypotonia. There was no history of trauma or bleeding diathesis. Four days before admission he presented with irritability and crying without fever. The general practitioner prescribed amoxicillin for a presumed angina. As the crying and the irritability continued, the parents presented to the emergency department. The infant was found to be ill and hypotonic. Meningitis was assumed and antibiotics were given intravenously. In spite of proper antibiotics there was a progression of his neurologic symptoms with an ascending paralysis. Analysis of the cerebrospinal fluid obtained by lumbar puncture showed an extreme high protein concentration (390 mg/dl, reference value 15e45 mg/dl) and an elevated white blood cell count (220 red blood cells/mm3 with 85 white blood cells/mm3 of which 53% monocytes, 28% lymphocytes, 19% neutrophils). Due to progression of the hypotonia and the appearance of respiratory distress he was transferred to a university hospital. On admission we saw an irritable boy with a flaccid paralysis (lower limbs bilateral 0/5 according to the British Medical Research Council Scale (BMRC)), an absent sphincter tonus,
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urinary retention and a bilateral upper limb weakness (left 4-/5, right 3/5). Deep tendon reflexes of the lower limbs and plantar reflexes were absent. His vital signs were stable with increased respiratory effort and a paradoxical breathing pattern. Consciousness and cranial nerves’ function were normal. A provisional diagnosis of GuillaineBarre´ syndrome was initially considered in view of the high protein concentration of the cerebrospinal fluid. An emergent spinal and cerebral magnetic resonance imaging (MRI) was requested to rule out cord compression. The MRI demonstrated a large fusiform mass, compressing the spinal cord, in the posterior epidural space extending from C5 to L1 (Fig. 1) consistent with an acute epidural hematoma. There were no flow voids suggesting an underlying arteriovenous malformation or fistula. The cerebral MRI was normal. Chest X-ray showed an atelectasis of the upper lobe of the right lung. Laboratory studies showed an anemia (Hb 8.9 g/dl). An extensive work up of the coagulation profile was normal (platelet count, PT, APTT, fibrinogen, platelet aggregation studies, von Willebrand antigen assay and activity, protein C, protein S, factor VIII, factor IX and factor XIII). The patient underwent an emergency laminectomy (T1, T5 and T7) with evacuation of the underlying hematoma. During the operation no dural arteriovenous malformation was seen. A control MRI after 20 h showed a marked diminution of the epidural hematoma with a remainder epidural hematoma from C5 till T6. A second neurosurgical revision was performed with additional removal of the rest hematoma. Histopathological examination of both resected tissues showed well organized hematomas without evidence of vascular malformations, tumor or other abnormal structures. Culture of the “fluid” from the hematomas was negative. A skeletal survey and ophthalmologic examination to rule out non-accidental injury was executed and did not show any abnormalities. The postoperative course was uneventful. There was a full recuperation of spontaneous voiding and sphincter tonus. The strength in the upper limbs gradually regained to 4/5 and the lower limbs to 2/5. At one year follow up he retained an incomplete quadriplegia and chronic respiratory insufficiency with non-invasive ventilation during the night. There is a kyphotic deformity of the thoracal spine. Six months postoperative MRI showed a remaining epidural collection and a medullopathy (Fig. 2).
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Fig. 1 e MRI examination of the cervico-thoracic spine performed on the day of admission with sagittal T2-weighted (a) and T1-weighted turbo spin echo images (b). The examination reveals a fusiform mass in the posterior epidural space, containing heterogeneous signal intensities. On the T2-weighted images, the epidural collection is predominantly hypointense. This finding is consistent with an acute epidural hematoma containing deoxyhemoglobin. The epidural hematoma occupies the posterior 2/3rds of the spinal canal from C5 to L1, and compresses the thecal sac from behind. There is intramedullary edema, seen as an ill-defined intramedullary region of increased signal intensity in the cervical and thoracic spinal cord.
Fig. 2 e Follow-up examination of the cervico-thoracic spine, 6 months after acute presentation, with sagittal T2-weighted (a) and T1-weighted turbo spin echo images (b). There is persistent epidural blood, seen as hypo-intense signal components in the anterior and posterior epidural space. The posterior epidural hemorrhage causes anterior displacement of the spinal cord from C6 to Th5. The spinal cord is thin and atrophic, presumably due to extrinsic compression.
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3.
SSEH in infancy: overview
3.1.
Definition
Spinal hematomas are clinical important due to permanent neurological deficit or even death. They can be divided into subdural, epidural, subarachnoidal and intramedullar hematomas. Epidural hematomas are by far the most common type of spinal hematomas (75%).2 The so called spontaneous spinal epidural hematomas are those not caused by trauma: these are a rare and hardly reported cause of spinal compression in childhood and more particularly during infancy. Review of the literature showed only 6 other cases under the age of one year (Table 1). Most authors refer SSEH to all the spinal epidural hematomas of non-traumatic origin, while others only speak of SSEH if there is no identifiable etiology or predisposing factor. The described predisposing factors so far include bleeding diatheses, vascular malformations, a medical procedure (lumbar puncture, spinal anesthesia, vertebral surgery), tumor bleeding, minor trauma or an infectious state.2,9 In all age groups the largest category (38.2%) consists of cases with no identifiable etiology. The second and third categories are respectively related to bleeding diathesis and vascular malformations. In younger patients (0e15 year) vascular malformations are even the second most common etiology (15.6%).2
3.2.
Pathogenesis
The pathogenesis of SSEH still remains unknown. Most consider the bleeding is venous in origin and arises from rupture of a ‘locus minoris resistentiae’ in the epidural valve less venous plexus.5 Due to the lack of valves in the epidural venous plexus a sudden increase in intra-abdominal and intrathoracic pressure induced by crying, cough, voiding, straining and trauma may cause a bulk of backflow resulting
in a sudden increase in pressure. When the pressure rises at a locus minoris restistentiae2,5,10,11 the vein can rupture. SSEH are usually restricted to the posterior aspect of the epidural spinal space2,12 SSEH can be localized at the cervical, thoracic or lumbar region. In children the cervicothoracic level is most affected, whereas in adults most hematomas are located at the thoracolumbar segment.1,2,13 SSEH are usually localized to two to three vertebral segments2 although in childhood more extensive hematomas are reported.
3.3.
Clinical presentation
The usual clinical presentation of SSEH in adults is sudden severe back pain. This is, hours or even days later followed by progressive motor and sensory deficits with (usually flaccid) paralysis below the affected spinal level.2,14 In children the initial presentation is more nonspecific with signs such as irritability, crying and torticollis. The neurological deficit may be minimal and is often harder to recognize.5,15 Due to the nonspecific presentation and the limitations at the neurological examination there is often a greater delay in diagnosis in childhood. Review of the case reports in infancy confirms the nonspecific presentation. All the infants presented with irritability, only after a while the neurological symptoms became clear. Consequently there was a great delay until treatment was initiated.
3.4.
Diagnosis
The differential diagnosis is broad and includes inflammatory, infectious and metastatic lesions as well as congenital malformation of the spinal cord. Investigation of the cerebrospinal fluid is not specific for spinal hematomas.2 The protein content is usually increased (even to more than 1 g/l), but may as well be normal.2,16 In view of the fact that there are now more exact and safer diagnostic tools, lumbar puncture is no longer recommended. Due to the combination of the albumin-cytologic dissociation
Table 1 e Summary of cases of SSEH under the age of 1 year. Author
Gendera/age (months)
Poonai et al.3
F/8
Min et al.4
M/8
Lee et al.5
M/4
Ramelli et al.6 Liao et al.7
M/7 F/7
Hosoki et al.8
M/11
Current case
M/8
Delayb
Treatment
None
2 months
Laminoplasty
Vascular malformation None
40 days
Laminectomy
5 days
Laminectomy
14 days 7 days
Laminectomy Laminectomy
T9eL1
None Upper respiratory tract infection (cough) None
3.5 days
C5eL1, posterior
None
4 days
Hemi-partial laminectomy Laminectomy
Symptoms
Location
Irritability, motoric regression Irritability, paraplegia
C4eT3, posterior C7eT3, posterior
Irritability, fever, neck stiffness Irritability, paraplegia Irritability, complete paraplegia
C4eT4, posterior
Irritability, reluctance to stand and walk Irritability, paraplegia
a M ¼ male, F ¼ female. b Delay between start of the symptoms and treatment.
C6eT7, posterior C7eT7
Etiology
Outcome Partial recovery Complete recovery Complete recovery Paraplegia Paraplegia
Partial recovery Incomplete quadriplegia
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and the ascending flaccid paralysis differential diagnosis with GuillaineBarre´ syndrome is obvious. This is also in line with our case. Magnetic resonance imaging (MRI) is considered the imaging modality of choice in diagnosis of SSEH. MRI is able to evaluate the location, extent, age and compressive effects of the hemorrhage. The sagittal images usually show a biconvex mass in the dorsal epidural space with well-defined contours and tapering of the superior and inferior margins. The axial planes provide more information on the location and help to differentiate from spinal subdural hematomas. Like brain hematomas, spinal hematomas show signal intensities that vary over time which is helpful in determining the age of the hematoma.17,18 MRI may also reveal enlarged vessels suggesting the presence of a vascular malformation. When there is a suspicion of a vascular malformation, a spinal angiography is the best investigation for the definitive diagnosis. In the presented case the epidural hematoma was seen as a fusiform mass in the posterior epidural space with heterogeneous signal intensities. On the T2-weighted images, the epidural collection was predominantly hypo-intense. On the T1-weighted images the collection was slightly more intense than the spinal cord. This is consistent with an acute hematoma, aged 1e3 days, containing paramagnetic deoxyhemoglobin.18 In our case a modest circumferential (gadolinium) contrast enhancement was seen as well (not shown) which can be observed in the acute stage of an SSEH.17 Due to the compression of the extensive SSEH there was intramedullary spinal edema, seen as an ill-defined intramedullary region of increased signal intensity on the T2-weighted images. The follow up MRI after one year shows residual epidural collections, buckling of the spinal cord and myelomalacia which is consistent with the clinical picture.
3.5.
Treatment
Emergency surgical decompression of the spinal cord is widely accepted as the treatment of first choice.5,19,20 Laminectomy of the involved segment and removal of the hematoma is the main surgical modality in most cases. However some authors are concerned that laminectomy will limit normal development in young children, resulting in spinal deformity or tethered cord.5,21,22 In our case, after a laminectomy of three vertebrae, a kyphotic deformity was observed one year after treatment. The decompression preferable takes place within 48 h, but for patients with complete sensorimotor deficit the critical time is reduced to 12 h.23 Although surgical decompression is the treatment of choice for progressive neurological deficits, there are reports of positive outcomes with conservative treatment especially in mild cases or patients with coagulopathy.24
3.6.
Outcome
The neurological recovery after surgery depends on the neurological deficit prior to surgery and the interval between clinical presentation and surgery. In children the relation is less obvious. There seems to be a more favorable outcome after surgery in children compared to adults.5,16,25,26 However, as illustrated by this case, the combination of an extensive hematoma with serious spinal compression and a delay of the
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diagnosis and thus surgery may have contributed to the permanent deficit. Unlike adults, delayed recovery of neurological function can eventually occur in children.26 Therefore in children without coagulopathy, surgical decompression should always be considered despite poor neurological status or delayed presentation as the benefits of surgery far outweigh the morbidity occurring following non-operative treatment.15
4.
Conclusion
SSEH is a rare cause of compression of the spinal cord in children and especially in infants. Until now only seven cases under the age of one year have been published. The presentation is usually nonspecific and the neurological deficit is often not obvious in the beginning. However, prompt diagnosis with MRI and early neurosurgical intervention is important to improve the outcome. Although rare SSEH should always be considered in infants whose presentation is only slightly suggestive.
references
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