Spontaneous spinal epidural hematoma (SSEH) is uncommon, especially during pregnancy. However, SSEH is a serious emergency that must be managed immediately to prevent permanent neurological deficit of the mother and stillbirth. The etiology and pathogenesis of SEEH remain unknown, although multiple theories and precipitating factors are described in the literature. In this report, we describe the case of a pregnant female who developed SSEH. The patient provided written and informed consent for the publication of her clinical information and associated images.
Here, we report a case of a 24-year-old woman at 31 weeks of gestational age, with a known factor S deficiency and positive lupus anticoagulant that was being treated with low molecular weight heparin (LMWH). It is possible that this treatment could have caused the hemorrhage. In addition, the patient reported a 10-day history of numbness and pain in her back, breasts and lower limbs. No history of trauma was reported. The patient presented with acute (12-hour) paraplegia and an magnetic resonance imaging (MRI) scan was immediately conducted in the local hospital, revealing acute hemorrhage in the dorsal epidural space at the T1–T3 level (Figs. 1, 2). Corticosteroids were administered, and the patient was transferred via helicopter to our emergency department, where a neurological assessment revealed impaired strength in both lower limbs (right: 3/5, left: 1/5), as well as a loss of light touch.
Cardiotocography and abdominal ultrasound revealed bradycardia with a median pulse of approximately 98 bpm on cardiotocography with reduced fetal movements. These conditions placed the fetus at immediate risk. Thus, even though gestation was preterm, we proceeded with an urgent cesarean section following pulmonary preparation with corticosteroids, resulting in live birth. Subsequently, as the patient remained hemodynamically stable, a decompressive laminectomy of the T1-T3 vertebra was performed in the prone position and under neurophysiological monitoring. An encapsulated epidural hematoma was recognized and evacuated, thus resulting in expansion of the spinal cord.
On the first post-operative day, the patient’s neurological condition improved significantly. The patient regained light touch and sensitivity in the perineum and anus and was able to manage the movement of her lower limbs against gravity. A follow-up MRI scan was performed on the day 19 postoperatively and again, after one year. These scans revealed decompressive laminectomy without indications of remaining hematoma in the epidural space (Fig. 3).
A search of the relevant literature revealed reports of 37 cases of SSEH during pregnancy between 1966 and 2020; 31 of these cases involved the thoracic spine. The most common clinical presentation was back pain (or neck/shoulder pain), leg weakness/paraplegia, and numbness. The management of choice was decompressive laminectomy immediately after delivery of the fetus; in all cases, cesarean section was selected over vaginal delivery. Complete restoration was reported in 18 cases; only a few patients suffered from remaining impairment or experienced no restoration at all. All deliveries resulted in live births, except for one abortion after surgery. See SDC Table 1 for more specific details (https://links.lww.com/MFM/A19).
Spinal epidural hematoma (SEH) is a rare and acute condition that can be traumatic or nontraumatic in origin and was initially described in the literature by Jackson in 1869 and Bain in 1897.1,2 SSEH represents approximately 0.3% to 0.9% of all epidural space-occupying lesions with an annual incidence of 0.1 cases/100,000 individuals. SSEH mostly affects those who are aged 40 to 50 years with a 1.5:1 male to female ratio. SSEH is frequently encountered in the thoracic, cervicothoracic, or thoracolumbar regions. The prominence of the epidural venous plexus in the dorsal space and the tight adherence of the posterior longitudinal ligament to the anterior dura leads to fewer ventral hematomas.1–4 Pregnancy-related SSEH, first reported by Bidzinski5 in 1966, is even rarer. The majority of cases occur during the third trimester and mostly affect the upper thoracic spine.4
Based on its pathogenesis, SEH can be classified as secondary, spontaneous or idiopathic; the term SSEH is used to describe both idiopathic and spontaneous conditions. In comparison to the spontaneous condition, no specific risk factors have been reported for the idiopathic condition, although it is not always possible to identify associated factors. Direct or indirect vasculature injury of traumatic or iatrogenic origin (spine surgery, lumbar puncture, epidural catheterization or anticoagulants) are known to contribute to secondary SEH.6–8
Although the etiology of SSEH is generally unknown, with an undetermined underlying cause in approximately 40% of patients,1,2,9 certain precipitating factors have been reported in the literature, including bleeding disorders,1–3,6,9 anticoagulants,1,3,6,9 vascular malformations,1,9 Paget disease,1,9 vasculitis,3,6,10 tumors,10 hypertension,3,9 Valsava maneuver/increased intrathoracic and intra-abdominal pressure/physical strain,3 and pregnancy.1,3,10
The most broadly accepted hypothesis for the cause of SSEH is venous bleeding when taking into consideration the predominance of posterior hematomas, the segmental distribution of SSEH, and the anatomical characteristics of the internal vertebral venous plexus. Another opinion supports the fact that epidural hematoma may be caused by rupture of the epidural arterial plexus due to certain movements in the highly mobile segments of the spine. A rise in central venous pressure (CVP) following increased intra-abdominal/intrathoracic pressure can easily be transmitted to the valveless epidural veins, thus causing spontaneous bleeding. During pregnancy, intra-abdominal pressure increases due to enlargement of the uterus as well as compression of the vena cava, thus resulting in the diversion of venous return and engorgement of the extradural venous plexus. Hemodynamic changes in pregnancy, such as increased blood volume, arterial and venous pressure, and structural vessel changes induced by progesterone and estrogen, can increase the potential for rupture. Although pregnancy is considered to be a hypercoagulable state, the incidence of SSEH can be high in the presence of predisposing factors.3,6,7
Typically, SSEH presents with severe localized back pain of spontaneous onset (within minutes to hours). Underlying spinal cord and nerve root compression cause the acute dysfunction of various types and extents depending on the spinal level and the degree of compression.3,6,9,10
In the presence of typical signs and symptoms, a coagulation profile and an imaging study should be performed.10 The rapid, accurate, and noninvasive evaluation that MRI offers makes it the gold-standard method for such investigations. MRI is a safe choice for the detection of hematoma during pregnancy as this technique avoids exposing the fetus to radiation. Furthermore, MRI provides information relating to the location, extent, and age of the hematoma along with the degree of cord compression or edema, while simultaneously allowing inspection of the spinal cord and its nerve roots.1,3,9 During the hyper-acute (the first 6 hours) and acute stages (7–72 hours), a hematoma will appear isointense on T1W (weighted) images; in contrast, in T2W images, hematomas first appear hyperintense and subsequently become hypointense. During the sub-acute stages, hematomas appear hyperintense on both T1W and T2W images.1,9 Upon the administration of gadolinium-diethylenetriamine penta-acetic acid (Gd-DTPA) contrast agents, enhancement may be seen in the hematoma due to hyperemia in the dura mater and thickening of the meninges; these effects are typically peripheral.1
MRI can also help with the differential diagnosis of intradural hemorrhages, epidural abscesses, inflammatory conditions, primary/metastatic epidural tumors, spinal epidural cavernous hemangiomas, spine fractures, spinal cord infarction, aortic dissection, disc herniation, and other conditions.8 In addition, MRI or spinal angiography may be performed preoperatively to investigate for the presence of an arteriovenous malformation.1,10
The choice of treatment depends on the degree of neurological impairment and the time interval from onset until presentation.1 Most patients develop serious and progressive neurological deterioration, and because there is limited time to reverse this condition, urgent surgical decompression is needed in such cases.2,3,9,11 Conservative management is limited to those with mild or no neurological deficit and without progression or improvement within the first 24 hours. Conservative management can also be administered to patients with a small noncompressive hematoma, as well as medically high-risk patients on anticoagulation, with extremely high surgical rates of mortality and morbidity.1,3 Potential conservative measures include immobilization, steroids, the treatment of coagulopathy, and percutaneous needle aspiration combined with neurological monitoring and surgery if progressive neurological symptoms develop or recovery is not rapid.3
Treatment during pregnancy depends on gestational age. In a full-term pregnancy, the fetus is delivered first with cesarean section in the supine position, followed by surgical decompression of the hematoma in the prone position1,3,6,9,10 with free space in the abdomen.12 This strategy reduces the incidence of aortocaval and uterine compression and lowers the risk of preterm delivery.13 The perioperative administration of tocolytics is also recommended to avoid contractions and rebleeding.14 In the case of an immature and nonviable fetus (less than 24 weeks gestation), conservative management can be administered with close neurological monitoring and the administration of steroids; this strategy increases fetal lung maturation and also has beneficial effects on the spinal cord.6,15 Early delivery facilitates the performance of decompression as this practice reduces epidural venous engorgement. Vaginal delivery is not recommended as uterine contractions can increase venous pressure and precipitate the expansion of a hematoma.1,6,9 In the early weeks of gestation, an epidural hematoma must be evacuated; once mature, the fetus must be delivered by cesarean section or by normal vaginal delivery.1
The most important outcome predictors for neurological improvement include the time interval between the onset of symptoms and surgery, preoperative neurological status, hematoma localization and the rate of symptom progression.2,3,6,9,10 Complete sensorimotor lesions, rapidly progressing symptoms, and cervical or thoracic hemorrhage have all been shown to be poor prognostic indicators.6 Early surgery, within 48 hours or 36 hours in incomplete or complete spinal cord dysfunction, respectively, or even 12 hours in a more aggressive approach, are recommended options that can achieve the best outcome.2,7,9 Dorsal epidural hematoma appear to be associated with the worst post-operative motor recovery due to relatively poor vascular supply. In contrast, lumbosacral hemorrhage is associated with a better prognosis due to the greater resistance of the cauda equina to compression injury. Surgical outcome has also been shown to be favorable in terms of the fact that epidural bleeding is limited to only one bony level.2 Factors such as gender, age, size, the extent or position of the hematoma, as well as the duration of signs and symptoms do not appear to affect post-operative outcome after SSEH.2,7
Although SEH is a rare condition, early diagnosis is essential, and urgent decompressive surgery is crucial if we are to improve the outcome and prevent permanent neurological deficits. In pregnant women, it is vital that we decide whether it is better to perform simultaneous cesarean section and laminectomy (in full term fetuses) or delay these procedures until after the embryo has matured.
Conflicts of interest
All data generated or analyzed during this study are included in this published article and its supplementary information files.
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