Introduction

Spinal epidural hematoma (SEH) is a rare neurosurgical emergency in respect of motor and sensory loss. SEH occurring without a trauma is called as spontaneous SEH (SSEH). Identifiable reasons for spontaneous hemorrhage are vascular malformations and bleeding disorders. Incidence of SSEH is 0.1/100,000, and SSEH is common in fourth and fifth decades of life [1, 2]. Prompt diagnosis is very important for timely intervention in children [3].

We present an 18-year-old female patient having SSEH and discuss the literature in respect of prevalence, diagnostic tools, treatment approaches, and outcomes of SSEH in pediatric patients.

Case report

An 18-year-old girl was admitted to our clinic for difficulty in walking and sensory disturbances that had developed 1 day ago. In her neurologic exam, she was alert and oriented. She was paraparetic (strength of lower extremities = 2/5), and her sensory level was at T3 dermatome. Her deep tendon reflexes were normoactive, and Babinski was negative, bilaterally. In her medical history, there was no recent trauma, no familial bleeding disorder, or no anticoagulation treatment. On MRI, posterior to the spinal cord, there was a mass lesion in the epidural space at T2–T3 levels, which was isointense on T1-weighted images and hypointense on T2-weighted images compared to cerebrospinal fluid intensity, consistent with acute hematoma (Fig. 1a, b). She was taken to surgery after immediate clinical and laboratory evaluations had been completed. T2 hemilaminectomy and T3 laminectomy were performed in the operation. A blood clot was observed and was aspirated completely. Upper and lower spinal levels were clear for any additional presence of hematoma mass. No identifiable vascular malformation was noticed during decompression of the epidural space. Surgery was uneventful, and she recovered completely after the operation (Fig. 1c).

Fig. 1
figure 1

Preoperative T2-weighted MRI depicts hypointense (a), and T1-weighted MRI depicts isointense posterior spinal epidural mass lesion, which is compatible with acute hematoma (b). No residual hematoma or myelopathy is present in postoperative T2-weighted MRI (c)

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Discussion

Spinal hematomas are categorized into four groups: subdural, epidural, subarachnoid, and intramedullary. Spinal hematomas could lead to devastating results such as neurologic deficits and even death [3]. Spinal epidural hematoma, which is the most common type of spinal hematomas, presents rarely in pediatric population [2, 3]. Although there is confusion in definition of SSEH, we determined SEH that occurs without any trauma as SSEH. Identifiable underlying disorders beside trauma are spinal interventions, bleeding disorders, spinal infections, spinal tumors, and spinal vascular malformations [2, 4].

Spinal epidural hematoma in pediatric population was first described by Cooper in 1832 [5]. Literature review depicted 112 pediatric cases (including the presented one) of spinal epidural hematoma. Of these 112 patients, 70 were male and 39 were female (F:M = 1.1:2, sex was not mentioned in three cases). Average age at presentation was 7.09 years (range = 0–18 years). Clinical presentations include loss of strength, sensory disturbance, bowel and bladder disturbances, neck pain, back pain, leg pain, abdominal pain, meningismus, respiratory difficulty, irritability, gait instability, and torticollis. Clinical diagnosis is hard to make in infants, who have usually presented with non-specific symptoms such as irritability [3]. Most common spinal level was cervicothoracic spine (46.3 %). Other sites were thoracic (20 %), cervical (15.4 %), thoracolumbar (8.1 %), cervicolumbar (6.3 %), and lumbar (3.6 %). Time interval from symptom onset to clinical diagnosis varied from immediate to 18 months. Spinal epidural hematoma happened spontaneously in 71.8 % of the cases. Hemophilia was the leading disorder (58 %) in the cases with a known disease. Partial or complete recovery is possible after surgical interventions and factor supplementations (Table 1) [3, 5–73].

Table 1 Pediatric spinal epidural cases in the literature
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Exact pathogenesis of SSEH is not clear, yet. Spinal epidural venous plexus has been accused of bleeding source by many authors [3]. Spinal epidural venous plexus has no valves, and a sudden increase in pressure due to straining, voiding, crying, and coughing could lead to backflow of blood in the plexus and rupture of the venous vessels [2, 16, 40, 74]. There are infectious, inflammatory, and metastatic diseases in differential of SEH. Magnetic resonance imaging is superior to other diagnostic modalities in delineating location, consistency, and duration of the hematoma. Status of the spinal cord and any underlying pathology such as vascular malformations could also be evaluated with MRI [3]. In case of vascular malformations, additional imaging with angiography is necessary to better delineate the feeding and draining vessels.

Surgical decompression is the first-line treatment modality for SSEH [3, 40, 75, 76]. Laminectomy is the most effective decompressive approach in SSEH, yet it has some conflicts in pediatric patients due to progressive kyphotic deformity in upcoming years. For this reason, hemilaminectomy, laminotomy, and laminoplasty have been used in this patient population [3, 6–11, 14–16, 18–20, 22–24, 26, 28, 30–41, 45, 46, 48, 50, 52, 54–66, 77]. In our case, we preferred single-level laminectomy with single-level hemilaminectomy due to limited SSEH extension between T2 and T3 levels. In some cases with mild neurologic deficits and/or bleeding disorders, conservative approaches were preferred over surgical decompression [12, 13, 21, 23, 25, 27, 29, 42, 44, 49, 51, 53, 60, 61, 67–69]. The outcome of surgical interventions depends on preoperative neurological status of the patient, and the time interval passed from the onset of symptoms to surgery [3]. Critical deadline for timely intervention is 48 h for incomplete and 24 h for complete neurologic deficits [78]. The success rate of surgery is more profound in pediatric cases than adults, and even complete recovery has rarely been reported after late presentation [14, 40, 71, 72]. For this reason, delayed presentation and severe neurologic deficits are not contraindications for surgery if the patient does not have any underlying bleeding disorders [3, 5].

Conclusion

Spontaneous spinal epidural hematoma is a rare neurosurgical emergency, especially in pediatric population. Response to surgery (in non-coagulopathy situation) is devastating in this patient population despite delayed surgical intervention and severity of neurologic deficits. However, surgery should not be delayed, as soon as bleeding disorders have been eliminated from differential diagnosis list, for a better and fast recovery.