Examkin

Question

Difficulty: MediumPediatric Hematology and Oncology

An 88-year-old boy with a known history of severe hemophilia A is brought to the emergency department by his parents after falling from a tree and striking his head. The parents report a brief loss of consciousness. He is now awake but complains of a severe headache and has vomited twice. His vital signs are temperature 37.1∘C37.1^\circ\text{C}, heart rate 110 /min110\text{ /min}, blood pressure 115/75 mmHg115/75\text{ mmHg}, and respiratory rate 20 /min20\text{ /min}. Physical examination reveals a large boggy hematoma over the right parietal region. Neurologic examination shows a Glasgow Coma Scale score of 1414 (confused conversation). Arrange the following management steps in the most appropriate chronological order for this patient.

  1. 1Administer intravenous recombinant factor VIII concentrate
  2. 2Perform an emergent non-contrast CT scan of the head
  3. 3Obtain an emergent neurosurgical consultation
  4. 4Admit the patient for serial neurological examinations

Answer

The correct sequence is to first administer intravenous recombinant factor VIII concentrate, followed by an emergent non-contrast CT scan of the head, then obtain a neurosurgical consultation, and finally admit the patient for serial neurological examinations.
In patients with bleeding disorders such as severe hemophilia, suspected life-threatening bleeding (e.g., intracranial hemorrhage following head trauma) requires immediate factor replacement therapy as the absolute first step in management. Factor activity must be replaced to a target of 100% immediately before any imaging or other diagnostic tests are performed. Delaying factor administration to obtain a CT scan is a classic medical error that can result in fatal expansion of the hemorrhage. Once hemostasis is supported with factor concentrate, an emergent non-contrast head CT should be obtained to confirm the diagnosis. Subsequent steps involve consulting neurosurgery for possible operative intervention and admitting the patient to a closely monitored intensive care setting for serial neurological checks.

Step-by-Step Solution

1
Identify the critical emergency.
The patient has severe hemophilia A with signs of increased intracranial pressure (headache, vomiting, altered mental status) following head trauma.
Intracranial hemorrhage is the leading cause of trauma-related death in hemophilia patients and must be assumed present until proven otherwise.
2
Determine the immediate life-saving intervention.
Factor VIII replacement must be administered immediately.
Hemostasis is the absolute priority. Diagnostic imaging must never delay factor administration, as the bleeding can rapidly expand in the interim.
3
Determine the appropriate diagnostic sequence.
A non-contrast head CT is performed next.
Once the patient is protected with factor replacement, imaging is required to confirm the diagnosis and determine the extent of the intracranial bleed.
4
Determine subsequent definitive management and disposition.
Neurosurgical consultation followed by admission for monitoring.
Surgical intervention may be necessary for large bleeds, and continuous neurological checks in a critical care setting are required due to the high risk of re-bleeding.

Key Concept

Prioritization of Factor Replacement in Hemophilia Trauma
Estimated Time:1m 15s
Rate this question

Topics

Pediatric Hematology and Oncology

More questions from this topic