Objective:

To highlight a rare cause of headache in sickle cell disease due to spontaneous epidural hematomas, subgaleal hematoma and calvarium infarcts.  

Background:

NA

Design/Methods:

We present a 9-year-old with sickle cell disease (HbSS) and past complications of vaso-occlusive crises, acute chest syndromes, splenic sequestrations requiring splenectomy, and avascular necrosis of femur. He was first admitted for headache, vomiting and forehead swelling with features of upper respiratory illness. He was treated with intravenous fluids, antibiotics and discharged home. Skin findings and mild forehead swelling was treated as tinea. He then returned after one day with worsening headache and increased swelling which progressed to involve eyelids and scalp. A CT head was significant for bilateral epidural and multiple subgaleal collections. Differentials included hemorrhage, infective abscesses from prior infection, and sterile collections.  

Results:

The resulting work up showed a platelet level of 364 K/ul (normal), but prothrombin time of 15.4 (prolonged), activated partial thromboplastin time of 70.6 (prolonged), and further mixing studies which raised suspicion for possible factor VII deficiency. A follow-up MRI brain confirmed bilateral frontoparietal epidural hematomas, multiple scalp subgaleal hematomas and focal abnormal marrow signal of the calvarium likely secondary to bone ischemia. He was treated with analgesia, fluid resuscitation and PRBC transfusion to reduce the percentage of sickled cells.  

Conclusions:

Spontaneous epidural and subgaleal hematomas are rare neurologic complications of sickle cell disease that all clinicians and neurologists should be aware of. The median age of presentation is 16 years and is more prevalent in males. It can mimic other sickle cell sequelae such as stroke or migraines, and as a result can be difficult to diagnose. Treatment is primarily supportive care and close monitoring of neurological status. Surgical intervention is only needed for enlarging epidural hematomas.