Spontaneous Calvarial Infarction with Epidural Hematoma and Subgaleal Hemorrhage in a Teenager with Sickle Cell SS Disease and Glucose-6-Phosphate Dehydrogenase Deficiency

Abstract

Introduction: Sequelae of sickle cell disease (SCD) are often the result of vaso-occlusion (VOC) related to the dysmorphic shape of sickled red blood cells (RBC). Bone infarction occurs commonly in patients with sickle cell, most notably in long bones and axial skeleton. Infarct occurs much less frequently in the bones of the skull. Calvarial bone infarct results in MRI findings of T2 hyperintensity in areas of bone marrow edema with associated swelling or hemorrhage. Symptomatic headaches in children with SCD are more likely to be due to severe underlying cerebral pathologies than in those children without SCD. When evaluating a child with SCD presenting with headache, it is important to consider calvarial infarct. Case Description: A 15 year old male with a history of sickle cell disease (type SS) and G6PD deficiency presented with chief complaints of headache and swelling over the left parietal region of the skull for two days with associated left sided visual disturbance. He denied head trauma or seizure-like activity. Upon presentation he was febrile with tachycardia and a normal neurological exam. CT scan of his head was significant for a left subgaleal hemorrhage and bilateral epidural midline and left parietal fluid collections consistent with bone marrow infarcts and associated subacute epidural hematomas, without evidence of brain parenchymal injury or sinus venous thrombosis. A follow up MRI obtained during hospitalization indicated that the left subgaleal hematoma remained stable with breakdown of subgaleal blood products. The patient was treated primarily with supportive care including hydration, packed red blood cell transfusions and pain management. He did not require neurosurgical intervention. His hospital course was complicated by VOC, sickle cell hepatopathy and acute chest syndrome. Discussion: Rarely, cranial VOC has resulted in epidural hematoma. In this case, calvarial infarct with unilateral headache, acute vision loss and acute cranial swelling resulted in subgaleal hemorrhage. This clinically significant finding results from bleeding between the periosteum and scalp galea aponeurosis, which may lead to hypovolemic shock. Calvarial infarcts should be considered on the differential in sickle cell disease patients presenting with a headache as prompt diagnosis can improve morbidity outcomes. Disclosures No relevant conflicts of interest to declare.

Subgaleal and Epidural Hematomas Secondary to Calvarial Infarcts: An Uncommon Presentation of Sickle Cell Disease

“…In accordance with the literature [7][8][9][10][11][12][13], we recommend that patients be managed conservatively to minimize hematoma formation and expansion. Adequate pain control is a cornerstone in the management of such patients.…”

“…Our case documents a presentation of SCD that is rarely reported in the literature. Although subgaleal and epidural hematomas have been individually described in previous case reports in patients with SCD and other comorbid genetic disorders such as glucose-6-phosphate dehydrogenase deficiency, the presence of both is extremely uncommon in isolated SCD [8][9][10][11]. In these cases, hematomas tended to be associated with an adjacent bone infarction seen on imaging.…”

“…Infarction of the bone is generally due to occlusion of microvasculature from a variety of causes, including mechanical obstruction, thrombosis, or gas/fat emboli, ultimately leading to osteonecrosis [4][5][6]. In general, pediatric calvarial infarctions are highly likely to be due to SCD or other sickling disorders; related case reports describe this pathology in the context of SCD [7][8][9][10][11][12][13].…”