**4.3 Investigations**

A whole spine MRI scan is the investigation of choice. A CT myelogram or noncontrast CT may be required depending on the patient's compliance and compatibility with an MRI scan, although the ionising radiation risk should be recognised and exposure limited.

As with adults the typical location of these haematomas are the cervico-thoracic region, and the size of the haematoma may span one to 22 spinal levels [18].

#### **4.4 Treatment**

Similar to adults, neurological dysfunction determines surgical intervention, not the presence of back pain or the size of the haematoma. Thus, in patients with neurological dysfunction, the gold standard treatment is surgical decompression and evacuation of the haematoma. The surgical approach to achieve this depends on the haematoma size, location, consistency and surgeon familiarity. While partial or complete laminectomies as well as laminoplasties have been reported, the consequences of each approach on the long-term consequences on spinal growth and function in these patients remain unknown. Thus, the least invasive approach to achieve decompression and haematoma evacuation should be utilised, but patients should also be counselled of the longer-term risks that surgical intervention may impose.

In patients without neurological dysfunction or with improving neurology, non-operative care with close neurological monitoring is appropriate. In patients with a bleeding tendency, systemic pro-coagulant therapy should be initiated, and if neurological improvement rapidly occurs, then non-operative treatment should be continued, but if no improvement occurs, then surgical intervention should be undertaken [18].

With this approach most children (83%) are expected to make a complete (69%) or partial (14%) neurological recovery [18]. However, this condition can result in death, with two children having been reported to have died following a SSEH. One child died from respiratory failure after operative intervention and the other without a cause for death being defined, but with evidence of progressive neurological dysfunction after surgical decompression [3, 4].

#### **5. Vascular malformations**

While most SSEH are believed to occur in the absence of local vascular malformations, there remains debate as to the number of these haematomas that have a predisposing vascular pathology, most notably arterio-venous fistulas, malformations and aneurysms. This is predominantly due to a poor specificity of initial imaging modalities to identify small vascular pathologies in the presence of a epidural haematoma, the rarity of sending pathological specimens of the local vasculature for histological assessment and the lack of follow-up imaging. However, local vascular pathologies have been identified in spinal epidural haematomas that develop without an inciting event in all age groups, although it is more commonly reported in children [18].

Clearly, an area of vascular fragility, due to an underlying malformation can predispose to spontaneous bleeding. The location of the subsequent haematoma from the bleed depends on the location of the vascular disruption. In the case of an epidural haematoma, the pressure exerted on the neural structures may cause neurological dysfunction for which the treatment is the same as that of a SSEH. If surgical intervention is necessary then coagulation of the vascular anomaly at the time of surgery to prevent further bleeding is necessary. This is often inadvertently performed during the coagulation manoeuvres undertaken to stop bleeding at the time of surgery. Fortuitously, this typically treats the underlying vascular pathology and avoids recurrence.

#### **6. Summary**

To date, the major limitations of the literature reporting on SSEH are the low numbers, publication bias, the variance in imaging and reporting of vascular

#### *Spontaneous Spinal Epidural Haematomas DOI: http://dx.doi.org/10.5772/intechopen.89175*

malformations and the lack of randomised control trials assessing outcome. However, it is clear that all age groups can be affected by SSEH and that three subsets of patients exist, namely adults, pregnant women and children.

In all groups, SSEH can present with variable symptoms and signs but most have neck or back pain with neurological dysfunction. In adults, SSEHs can mimic ischaemic events and lead to inappropriate anticoagulation therapy, which can be avoided by clinical awareness of this condition and whole spine cross-sectional imaging. Similarly, in children the diagnosis can be challenging due to the problems conversing with children and the variable presentations, often mimicking neuromuscular disorders. It is further compounded by the challenges of attaining crosssectional imaging, which is again diagnostic. In pregnancy, the further difficulties lie in the protection of the unborn foetus, with the avoidance of ionising radiation, but cross-sectional imaging remains diagnostic.

In all groups, most SSEH affect the cervico-thoracic region and span multiple spinal segments. The gold standard of treatment for patients with neurological dysfunction is surgical decompression and haematoma evacuation. However, in patients without neurological dysfunction, close neurological monitoring without surgical intervention is appropriate. In adults with bleeding disorders, procoagulant therapy should be used as an adjunct to treatment, however in children with bleeding disorders pro-coagulant therapy may be trialled prior to surgical intervention. In pregnancy, surgical intervention involves surgical decompression with foetal monitoring in those of gestational age less than 32 weeks and a caesarean section followed by decompression in those of gestational age greater than 32 weeks. In children, the long-term effect of surgical intervention on spinal growth and function should be recognised.
