Spontaneous Extra-Dural Hematoma: A Rare Complication of Sickle Cell Disease

Introduction: Extradural hematoma is a serious complication of moderate and severe head traumas. Spontaneous occurrence in patients with sickle cell disease (SCD) with no history of trauma is extremely rare.

Case Presentation: We report the case of a 17-year-old boy who was treated for sickle cell disease. Symptoms included a headache with facial and left palpebral oedema without vomiting or neurological deficits, which occurred during hospitalisation for a vaso-occlusive crisis.

Clinical Discussion: The diagnosis was confirmed by a brain scan, and the patient was operated on through a parietal cranial flap. The postoperative course was marked by neurological deterioration, and the follow-up CT scan showed subdural and intraparenchymal bleeding probably related to vasoocclusive crisis.

Conclusion: This case report highlights the probable pathophysiological mechanisms involved in the occurrence of spontaneous extradural hematomas in sickle cell disease patients, the importance of imaging in the presence of the slightest suspicion of cerebral damage, and the need for rapid management.

Introduction

Sickle cell anaemia is a group of autosomal recessive genetic disorders characterised by the production of haemoglobin S (HbS) [1]. It combines chronic hemolytic anaemia with ischemic lesions in various tissues. Although the brain is particularly vulnerable to ischemic lesions, which may occur during a crisis, sickle cell disease can rarely cause spontaneous extradural bleeding. We report a case of non-traumatic extradural hematoma in sickle cell disease, and we discuss the two hypotheses that may explain it: haematopoiesis and rupture of the internal table [2], [3].

Case Presentation

The patient was 17 years old and had been treated for sickle cell disease since the six years old. He was admitted to the hematology department for vaso-occlusive sickle cell crisis, dominated by intense pain in all four limbs. Clinically, the patient was conscious with a Glasgow score of 15/15 and no sensory-motor deficit, with marked facial oedema in the left palpebral area. Symptomatology worsened with helmet headaches calmed by analgesics without vomiting or seizures, stable hemodynamic state, HR at 102 bpm and BP at 11/7, and polypnoea at 34 cycles per minute.

Blood tests showed anaemia of 9 g/dl with a mean corpuscular volume of 72, hyperleukocytosis of 21570/mm³, predominantly neutrophilic at 19629/mm³, thrombocytopenia of 73,000 with a normal prothrombin rate of 81%, and fibrinogenaemia of less than 1.

As part of the investigation into his facial oedema and headaches, a cerebral CT scan was carried out two days after his admission to the hospital, showing two extra dural haematomas on the right frontal side measuring 7.1 mm and the right parietal side measuring 27 mm, with no signs of involvement (Fig. 1) or associated bone fracture, and an abnormally thick diploid (Fig. 2).

Fig. 1. Parenchymal axial CT section showing a 27 mm right parietal extradural hematoma.

Fig. 2. Axial scans through a bone window showing thick bone with no visible fracture.

He underwent emergency surgery, and surgical evacuation of the hematoma after a right parietal flap was made. The bone was hemorrhagic, with no fractures or signs of soft tissue trauma. The patient was transfused with packed red blood cells and 5 platelet concentrates. The postoperative course was marked by neurological deterioration and the appearance of right anisocoria, prompting an emergency CT scan, which revealed an intraparenchymal hematoma associated with a 10 mm thick subdural fronto-parieto-occipital haematoma and diffuse cerebral oedema (Fig. 3), necessitating emergency surgery. The patient was transferred to the intensive care unit and died of multi-visceral failure. This work has been reported in line with the Surgical Case Report (SCARE) criteria [4].

Fig. 3. Post-operative CT scan showing an intra-parenchymal haematoma with a subdural haematoma lamina, diffuse cerebral oedema, and meningeal hemorrhage.

Discussion

Sickle cell disease (SCD) is a genetic disorder linked to hemoglobinopathy, and can lead to neurological complications in the form of ischemic strokes in 2/3 of cases. Compared to hemorrhagic strokes, these are less frequent [1], [5], [6]. Extradural haematomas are generally due to cranial trauma. The hemorrhage originates from the middle meningeal artery or one of its branches, fracture edges, torn venous sinuses, or dipliac veins [6], [7]. Spontaneous extradural hematoma remains a possible but rare complication of sickle cell disease, and was first described in 1951 by Schneider and Hegarty [7].

Various pathophysiological mechanisms have been proposed, including; either a microvascular diploid vaso-occlusive mechanism associated with occlusion of an outflowing vein [8], or an extensive hematopoiesis mechanism resulting from rapid deglobulation leading to an increase in the thickness of the diploid (in the case of our patient) and rupture of the internal tables of the cranial bone, which will cause bleeding [3], [7].

The classic clinical symptomatology of extradural hematoma in cranioencephalic trauma, with an initial loss of consciousness and a free interval, is dominated by headaches that may be masked by analgesics, delaying the diagnosis [3], [7], [8] as in our case. Facial oedema was described in our case as the reason for requesting imaging, which was an essential examination of certainty for all authors [5], [8], [9].

Other signs are described, such as disorders of consciousness with deterioration of the Glasgow score to coma, and hemiparesis of the contralateral side [3], [6]. Symptomatology occurs in patients in the midst of a vaso-occlusive crisis, as in the present case [8], [9].

Emergency neurosurgical management remains the option of all authors, whether for spontaneous or post-traumatic HED, except in cases of small hematomas with reduced thickness or small volume requiring only close clinical and radiological monitoring [3], [8].

The postoperative course is generally good except in cases of delayed diagnosis or management [3], [9] or hemorrhagic or infectious complications, as in our case.

Conclusion

Although exceptional, the occurrence of extradural hematoma in sickle cell disease remains a possibility and is often not mentioned as a complication of this hemoglobinopathy. Any neurological deterioration or headache in a patient with sickle cell disease should be considered an emergency, prompting the patient to undergo emergency neurosurgery, whether or not in the midst of a vaso-occlusive crisis, with emergency brain imaging as the only definitive test. Prompt treatment is a major advantage, as it improves the vital function.