A 4-year-old boy with sickle cell disease is brought to the emergency department by his parents due to a progressive headache and drowsiness. While coming down the stairs, the patient slipped and fell from a height of 1.5 m (5 ft), hitting his head on the tile floor. He lay on the ground for several seconds but got up and continued walking and talking normally. The patient initially had only mild pain at the injury site. A few minutes later, he developed a progressive headache and vomiting before becoming somnolent. The patient has been hospitalized multiples times for vasoocclusive crises. He takes hydroxyurea and penicillin daily. Blood pressure is 140/86 mm Hg and pulse is 66/min. The patient is sleepy and requires painful stimuli to remain awake. On neurologic examination, the left pupil is 3 mm with normal pupillary reflex, but the right pupil is 8 mm and unreactive to light. Which of the following is the most likely cause of this patient's acute decompensation?

Question

A 4-year-old boy with sickle cell disease is brought to the emergency department by his parents due to a progressive headache and drowsiness.  While coming down the stairs, the patient slipped and fell from a height of 1.5 m (5 ft), hitting his head on the tile floor.  He lay on the ground for several seconds but got up and continued walking and talking normally.  The patient initially had only mild pain at the injury site.  A few minutes later, he developed a progressive headache and vomiting before becoming somnolent.  The patient has been hospitalized multiples times for vasoocclusive crises.  He takes hydroxyurea and penicillin daily.  Blood pressure is 140/86 mm Hg and pulse is 66/min.  The patient is sleepy and requires painful stimuli to remain awake.  On neurologic examination, the left pupil is 3 mm with normal pupillary reflex, but the right pupil is 8 mm and unreactive to light.  Which of the following is the most likely cause of this patient's acute decompensation?

Quizplus · Accepted Answer

11ec25c8_d5a3_a0e3_bc81_7f72b998219c_MD0003_00 This patient most likely has an epidural hematoma (EDH), an accumulation of blood between the cranium and the dura mater, typically caused by middle meningeal artery laceration. In adolescents and adults, EDH most commonly occurs following high-impact head trauma (eg, motor vehicle collision). In contrast, low-velocity falls are more likely to cause EDH in young children. epidural hematoma (EDH) Patients classically have a brief loss of consciousness, followed by a lucid interval of minutes to hours. Rapid hematoma expansion leads to deterioration of consciousness (eg, somnolence) and symptoms (eg, headache, vomiting) of elevated intracranial pressure. Without treatment, worsening intracranial hypertension can result in uncal herniation. Signs include ipsilateral pupillary dilation (due to oculomotor nerve [CN III] compression), as seen in this patient, and contralateral hemiparesis (due to compression of the ipsilateral cerebral peduncle). As herniation progresses, compression of the contralateral cerebral peduncle may also occur, leading to ipsilateral hemiparesis (a false localizing sign). uncal herniation Signs A biconvex hyperdensity on CT scan of the head is diagnostic. Emergency craniotomy is required in patients with neurologic deficits to prevent brain herniation and death. biconvex hyperdensity (Choice A) Cerebral contusion is characterized by microhemorrhages ("bruising") within the superficial brain parenchyma due to coup-contrecoup injury. Symptoms of increased intracranial pressure may develop if significant cerebral edema occurs; however, this typically develops gradually over hours to days (vs minutes). (Choice C) Patients with sickle cell disease have increased risk for ischemic stroke, which may present with focal neurologic deficits (eg, anisocoria). However, this patient's rapid deterioration after head trauma and signs of herniation make EDH more likely. (Choice D) Subarachnoid hemorrhage (SAH) presents with a sudden-onset, severe headache most commonly due to a ruptured intracranial aneurysm or occasionally after trauma. This patient's headache was initially mild before progressively worsening, and a lucid interval followed by increasing somnolence is not typical of SAH. Subarachnoid hemorrhage (SAH) (Choice E) Acute subdural hematoma can occur from traumatic tearing of the bridging veins, with subsequent slow bleeding into the subdural space. However, this more commonly occurs in patients with cerebral atrophy (eg, elderly, chronic alcohol use), and symptoms (eg, headache, confusion) classically occur gradually (eg, over 1-2 days). subdural hematoma Educational objective: Epidural hematoma occurs when blood accumulates between the cranium and the dura mater after traumatic head injury. Patients classically have a brief loss of consciousness followed by a lucid interval. Rapid hematoma expansion can lead to elevated intracranial pressure and uncal herniation. References: Epidural hematoma. (http://www.ncbi.nlm.nih.gov/pubmed/30085524) Spontaneous epidural hemorrhage in sickle cell disease, are they all the same? A case report and comprehensive review of the literature. (http://www.ncbi.nlm.nih.gov/pubmed/31346480) Management and outcome of traumatic epidural hematoma in 41 infants and children from a single center. (http://www.ncbi.nlm.nih.gov/pubmed/27622712)

A 4-Year-Old Boy with Sickle Cell Disease Is Brought to the Emergency