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REVIEW ARTICLE
Year : 2020  |  Volume : 15  |  Issue : 3  |  Page : 171-174
 

Rare presentations of spontaneous spinal epidural hematomas in children


Department of Pediatrics, Safdarjung Hospital, New Delhi, India

Date of Submission17-Aug-2016
Date of Decision16-Feb-2017
Date of Acceptance25-May-2020
Date of Web Publication06-Nov-2020

Correspondence Address:
Dr. Smita Ramachandran
R 524, 2nd Floor, New Rajinder Nagar, New Delhi.
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpn.JPN_137_16

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   Abstract 

Spontaneous spinal epidural hematomas are a rare presentation in children with very few case reports of spontaneous spinal hemorrhage in the pediatric age-group. There has to be a high index of suspicion of spontaneous spinal bleed in patients with acute-onset quadriparesis with no prior history of bleeding or trauma, as early diagnosis and surgical decompression is associated with better neurological outcomes.


Keywords: Laminectomy, pediatric age-group, spontaneous spinal hematoma


How to cite this article:
Ramachandran S, Mishra V, Gera R. Rare presentations of spontaneous spinal epidural hematomas in children. J Pediatr Neurosci 2020;15:171-4

How to cite this URL:
Ramachandran S, Mishra V, Gera R. Rare presentations of spontaneous spinal epidural hematomas in children. J Pediatr Neurosci [serial online] 2020 [cited 2020 Nov 24];15:171-4. Available from: https://www.pediatricneurosciences.com/text.asp?2020/15/3/171/300039





   Introduction Top


Incidence

Spinal epidural hematoma is a rare presentation in children with an incidence of about 0.1 per 100,000 patients in the general population, and this incidence is significantly lower in pediatric population,[1] and it can be misleading as a meningitis mimic. Majority of the cases reported are in the adult population, with very few incidences of spontaneous bleed in the pediatric population reported.[2],[3] The youngest case report in a newborn, by caesarian section, was confused with birth-related spinal injury.[4] Studies have reported the spontaneous hemorrhage having a bimodal age of occurrence at 15-20 years and 65-70 years. They are commonly develop at C-6 and T-12.[5]

Pathogenesis and clinical presentation

Spinal hematomas are categorized into four groups: subdural, epidural, subarachnoid, and intramedullary.

However, 40%–50% cases are spontaneous with no associated cause.[1] It can be associated with trauma, surgery, coagulation defects, arteriovenous malformations, infection, hypertension, pregnancy, thrombolysis for myocardial infarct, factor IX deficiency, long-term aspirin use, neoplasms, and spinal catheterizations.[1],[2]

In children, it occurs most commonly in the cervical and thoracic region (46.3%). Other sites were thoracic (20%), cervical (15.4%), thoracicolumbar (8.1%), cervicolumbar (6.3%), and lumbar (3.6%).

These bleeds can be of both arterial and venous origin. The posterior epidural venous network is the most common source of the hematoma. This is primarily due to the absence of valves in spinal epidural veins, which are prone to rupture and hemorrhage following the abdominal or thoracic pressure changes associated with coughing, sneezing, and lifting of weights.[6] Recent theories have attributed pressure difference in arteriovenous network and epidural space as the cause for arterial bleeds.[7]

Epidural bleeds most commonly present as backache ranging from dull aching to severe radicular, associated with weakness, paresis, sensory deficits, and bladder bowel involvements depending on the age, size, and site of the hematoma.[7] In adults, the most common presenting complaint is back pain of varying intensity, and motor and sensory deficits below the level of bleed progress over hours to days.[8]

Infants present with nonspecific symptoms such as irritability, torticollis, gait disturbance, excessive crying, and urinary retention mimicking meningitis, whereas older children have neck pain and back pain of varying intensity.[9],[10]

Spinal hematomas presenting as acute-onset quadriparesis is very uncommon. It is important to have a high index of suspicion in such cases with no previous antecedent events, in which neurological signs and symptoms maybe absent at presentation and subtle points of differentiation may not be present in the acute spinal shock stage.


   Case Report Top


A 10-year- old male child weighing 25kg presented to the hospital with complaints of inability to walk and get up from the bed due to acute onset of bilateral lower limb and trunk weakness since 4 days, followed by bowel and bladder incontinence on the second day of illness. On examination, he was conscious, oriented, with heart rate 90 beats/min, respiratory rate 24 breaths/min, and blood pressure 100/60 mm Hg. He had severe pain in both shoulders extending up to the hands. Systemic examination revealed bilateral lower limbs with power of 0/5; bilateral upper limb power was normal but intrinsic muscles power was 3/5. Superficial reflexes bilateral abdominal and anal were absent, plantar were equivocal, and deep tendon reflexes: biceps (2+), triceps (+), supinator (1+), and lower limb deep tendon reflexes were absent. There was absent sensation to pain and touch below the trunk, and there was sensory loss below level of fourth thoracic dermatome. There was no history of trauma, surgery, infections, prolonged bleeding, injections, lumbar puncture, or any drug intake. The patient was started on injectable third-generation cephalosporin, and blood investigations were ordered. Hemoglobin (Hb) was 9.0 g/dL, total leukocyte count was 8000/cumm, differential leukocyte count was neutrophils (63%), lymphocytes (34%), monocytes (2%), eosinophils (1%), platelet count was 3.67 × 105/dL, serum sodium 140 meq/L, potassium 4.2 meq/L, and liver function tests, prothrombin time (PT) (13.6s), partial thromboplastin time (PTT) (33s), and international normalized ratio (INR) (1.2), and bleeding time and clotting time were normal. A peripheral blood smear showed no evidence of any hemolysis. A magnetic resonance imaging (MRI) of brain and spine revealed a collection in the spinal epidural space hypointense in T1-weighted (T1W) and hyperintense in T2-weighted (T2W) extending from lower margin of cervical 7 to mid third of dorsal 4 causing extramedullary compression, suggesting a posterior spinal epidural hematoma [Figure 1].
Figure 1: MRI of the patient preoperatively

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The child was operated on day 3 of admission, the hematoma was drained by dorsal laminectomy, and 50mL of clotted blood was drained. A repeat computed tomography (CT) angiography done 4 weeks after the surgical decompression ruled out any arteriovenous malformation. The patient regained bowel and bladder control on the second post-operative day, and by 2 weeks later he regained power in the lower limbs (2/5), and the deep tendon reflexes had become normal. The child was discharged, and he is on regular follow-up and physiotherapy and is gradually regaining power in both the legs.


   Diagnosis Top


Magnetic resonance imaging (MRI) is the neuroimaging modality of choice to diagnose epidural hematomas; it helps to accurately delineate the size and margins of the hematomas in the epidural spaces.

The images on MRI of acute epidural bleed reveal isointense or hypointense on T1W images and hyperintense on T2W images. 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. MRI also detects the signal changes in the bleed, which can detect the age of the bleed.[11] An important feature for this is the early detection of deoxyhemoglobin. It gives rise to low signal intensity due to a local magnetic susceptibility effect, and this is most obvious on T2W gradient echo sequences. By 48h, the hematoma gives an increased signal on T1W images and remains hyperintense on T2W sequences [Table 1].[12]
Table 1: Stages of hematoma

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CT scan can also diagnose bleeds but may miss it in the initial 24h, which is very crucial in epidural bleed for immediate surgical intervention. Contrast enhancement pattern and morphological findings on MRI images can differentiate acute epidural hematoma from spinal epidural neoplastic mass, abscess, disc herniation, spinal tumor, spinal tuberculosis, and pyogenic spondylitis.[3] It is highly sensitive in diagnosing vascular malformations.


   Management Top


The two ways to manage this condition are as follows:

  1. Surgical


  2. Conservative


Surgical

Spontaneous epidural bleeds are often accompanied by acute neurological deficits, hence needs to be diagnosed and managed rapidly. Decompressive surgery has been the mainstay of treatment for epidural hematoma in majority of case studies.[1],[2],[3],[4],[8]

Laminectomy with hematoma evacuation is the most effective decompressive approach for epidural hematomas. It can be single level or more depending on the extent of the hematoma.[4],[8]

However, due to progressive kyphotic deformity in children in the following years, hemilaminectomy, laminotomy, and laminoplasty has been used in pediatric population.[3],[7],[10] The prognosis of the hematoma depends on the site, size, and time interval between onset of symptoms and surgical intervention.

Long hematomas and prolonged duration between onset of symptoms and surgical decompression are associated with poor outcome.[13],[14] There is however no definite consensus on the best timing of the surgery and outcome, various studies have reported early surgery ranging from 12 to 48h from the onset of symptoms for better neurological outcome.[13],[14],[15],[16]

Liao et al.[13] and Lawton et al.[14] in different series have reinforced better outcomes in surgeries performed within 12h of onset of symptoms. Patients taken to surgery within 12h had better neurological outcomes than patients with identical preoperative Frankel grades whose surgery was delayed beyond 12h.

Conservative

Conservative management has been reported in few cases, this has been postulated due to the size of the hematoma. García et al.[17] stressed that patients with coagulation defects when given immediate replacement therapy defer surgical intervention, these patients were given aspirin, clopidogrel, or warfarin or a combination of these. Groen et al.[5] hypothesized that this was due to the hematoma remaining liquefied for a long period, hence spreading slowing in the spinal epidural space.

A high dose methylprednisolone have been useful in some studies of acute spinal injury, however the data is limited to make recommendations about the same.

Medical management is usually reserved for cases with early spontaneous neurological recovery.[7]

Some studies have not shown any significant difference in the neurological outcome of various treatment modalities or to the site of the involved segments.[17]


   Prognosis Top


Liao et al.[13] reviewed a large number of cases over a period of 5 years and reported predictors of poor outcome: presence of coagulopathy, hematoma extending over several vertebral segments, and complete spinal cord dysfunction before surgery.[13]

Old age, high levels of spinal cord dysfunction, severe preoperative neurological dysfunction, and complete spinal injury are indicators of poor outcome.[3],[18],[19]

Incomplete spinal injury, small hematomas, and surgery in children have a favorable outcome.[1],[19],[20]


   Conclusion Top


Early diagnosis with immediate surgical decompression singularly affects the neurological outcome in spinal epidural hematomas, hence prompt diagnosis should be made in children presenting with acute pain and signs of spinal cord compression.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Patel H, Boaz JC, Phillips JP, Garg BP Spontaneous spinal epidural hematoma in children. Pediatr Neurol 1998;19:302-7.  Back to cited text no. 1
    
2.
Sklar EML, Post JMD, Falcone S MRI of acute spinal epidural hematomas. J Comput Assist Tomogr 1999;23:238-43.  Back to cited text no. 2
    
3.
Vallée B, Besson G, Gaudin J, Person H, Le Fur JM, Le Guyader J Spontaneous spinal epidural hematoma in a 22-month-old girl. J Neurosurg 1982;56:135-8.  Back to cited text no. 3
    
4.
Blount J, Doughty K, Tubbs RS, Wellons JC, Reddy A, Law C, et al. In utero spontaneous cervical thoracic epidural hematoma imitating spinal cord birth injury. Paediatr Neurosurg 2004;40:23-7.  Back to cited text no. 4
    
5.
Groen R Non-operative treatment of spontaneous spinal epidural hematomas: a review of the literature and a comparison with operative cases. ACTA Neurochir 2004;146:103-10.  Back to cited text no. 5
    
6.
Akimoto T, Yamada T, Shinoda S, Asano Y, Nagata D Spontaneous spinal epidural hematoma as a potentially important stroke mimic. J Central Nervous System Dis2014;6:15-20.  Back to cited text no. 6
    
7.
Motamedi M, Baratloo A, Majidi A, Rahmati F, Shahrami A Spontaneous spinal epidural hematoma; a case report. Emerg (Tehran) 2014;2:183-5.  Back to cited text no. 7
    
8.
Kreppel D, Antoniadis G, Seeling W Spinal hematoma: a literature survey with meta-analysis of 613 patients. Neurosurg Rev 2003;26:1-49.  Back to cited text no. 8
    
9.
Jumani DB, Littlewood R, Iyer A, Fellows G, Healey A, Abernethy L, et al. Spontaneous spinal epidural haematoma mimicking meningitis in a 2-year-old child—a case report and literature review. Childs Nerv Syst 2013;29:1795-8.  Back to cited text no. 9
    
10.
Babayev R, Şakir Ekş M Spontaneous thoracic epidural hematoma: a case report and literature review. Childs Nerv Syst 2016;32:181-7.  Back to cited text no. 10
    
11.
Chang FC, Lirng JF, Chen SS, Luo CB, Guo WY, Teng MM, et al. Contrast enhancement patterns of acute spinal epidural hematomas: a report of two cases. AJNR Am J Neuroradiol 2003;24:366-9.  Back to cited text no. 11
    
12.
Avrahami E, Tadmor R, Ram Z, Feibel M, Itzhak Y MR demonstration of spontaneous acute epidural hematoma of the thoracic spine. Neuroradiology 1989;31:89-92.  Back to cited text no. 12
    
13.
Liao CC, Hsieh PC, Lin TK, Lin CL, Lo YL, Lee SC Surgical treatment of spontaneous spinal epidural hematoma: a 5 year experience. J Neurosurgery Spine 2009;11:480-6.  Back to cited text no. 13
    
14.
Lawton MT, Porter RW, Heiserman JE, Jacobowitz R, Sonntag VK, Dickman CA Surgical management of spinal epidural hematoma: relationship between surgical timing and neurological outcome. J Neurosurg 1995;83:1-7.  Back to cited text no. 14
    
15.
Liao CC, Lee ST, Hsu WC, Chen LR, Lui TN, Lee SC Experience in the surgical management of spontaneous spinal epidural hematoma. J Neurosurg 2004;100:38-45.  Back to cited text no. 15
    
16.
Bracken MB, Shepard MJ, Collins WF, Holford TR, Young W, Baskin DS, 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;322:1405-11.  Back to cited text no. 16
    
17.
García López A, Pérez Lara JM, Herrainz Hidalgo R, Puente Gonzalo E Spinal epidural hematoma following thrombolytic therapy for acute myocardial infarction. Orthopedics 1999;22:987-8.  Back to cited text no. 17
    
18.
Kim B-S, Lee S-B, Kim J-H, Lee T-G, Yoo D-S, Huh P-W, et al. Retrospective analysis of 14 cases of spinal epidural hematoma. J Korean Neurotraumatol Soc 2011;7:51-6.  Back to cited text no. 18
    
19.
Kim T, Lee C-H, Hyun S-J, Yoon SH, Kim K-J, Kim H-J Clinical outcomes of spontaneous spinal epidural hematoma: a comparative study between conservative and surgical treatment. J Korean Neurosurg Soc 2012;52:523-7.  Back to cited text no. 19
    
20.
Patel H, Garg BP Increasing irritability with sudden onset of flaccid weakness. Semin Pediatr Neurol 1996;3:192-7.  Back to cited text no. 20
    


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    Abstract
   Introduction
   Case Report
   Diagnosis
   Management
   Prognosis
   Conclusion
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