What is the management for a 2-day-old infant with hemophilia A and a significant cephalohematoma?

Management of Hemophilia A in a 2-Day-Old Infant with Cephalohematoma

The correct answer is C - Factor VIII concentrate replacement therapy is the definitive treatment for this 2-day-old infant with hemophilia A and a significant cephalohematoma bridging sutures after vacuum extraction. 1

Immediate Management Approach

Administer Factor VIII concentrate immediately to achieve hemostatic levels of 50-100% (50-100 IU/dL) for this significant bleeding complication. 1, 2 The cephalohematoma bridging between sutures represents a substantial hemorrhage requiring urgent replacement therapy, not observation alone.

Initial Dosing Strategy

• Loading dose: 50 IU/kg of Factor VIII concentrate should be administered intravenously 2
• This achieves the target hemostatic level needed for significant bleeding episodes 1
• Subsequent doses of 20-50 IU/kg should be given every 8-12 hours until bleeding is controlled 3
• Continue monitoring for expansion of the hematoma and hemoglobin levels 2

Why NOT the Other Options

Vitamin K (Option B) is incorrect because hemophilia A is a Factor VIII deficiency, not a vitamin K-dependent coagulation factor deficiency. Vitamin K would not address the underlying Factor VIII deficiency causing this bleeding. 1

Fresh Frozen Plasma (Option A) is suboptimal because it contains insufficient Factor VIII concentration to achieve adequate hemostatic levels in severe hemophilia A. Factor VIII concentrates provide targeted, high-concentration replacement therapy that FFP cannot match. 1, 2

Critical Monitoring Requirements

• Monitor hemoglobin/hematocrit serially to assess for ongoing blood loss 2
• Measure Factor VIII levels after initial dosing to confirm adequate replacement (target >50%) 1
• Assess cephalohematoma dimensions clinically and consider ultrasound if expansion suspected 4
• Watch for signs of increased intracranial pressure or neurological changes 3

Special Considerations for Neonates

Emicizumab prophylaxis should be strongly considered after acute management to prevent future bleeding complications, including the devastating risk of intracranial hemorrhage that occurs in 6% of infants with hemophilia A. 3 The HAVEN 7 study demonstrated safety and efficacy in infants as young as the first week of life, with no intracranial hemorrhages reported in 55 treated infants. 3

Prophylaxis Initiation

• Subcutaneous emicizumab can be started within days of diagnosis 3
• This avoids the need for central venous access and frequent intravenous infusions 3
• Provides continuous hemostatic protection with lower treatment burden 3

Management of the Cephalohematoma Itself

Avoid aspiration or surgical intervention during the acute phase when coagulation is not optimized. 4 If the cephalohematoma remains large and persistent after Factor VIII levels are normalized, needle aspiration can be safely performed between day 15-30 of life under local anesthesia to prevent calcification and aesthetic complications. 4

Common Pitfalls to Avoid

• Do not delay Factor VIII replacement while awaiting confirmatory testing - treat based on clinical diagnosis when severe bleeding is present 1
• Do not use desmopressin (DDAVP) in neonates with severe hemophilia A - it is ineffective when baseline Factor VIII is <1% and carries risks of hyponatremia and seizures 3
• Do not perform invasive procedures (including cephalohematoma aspiration) without adequate Factor VIII coverage 3
• Do not assume the cephalohematoma will resolve without treatment - significant hemorrhages in hemophilia A require active replacement therapy 1

Long-Term Considerations

All infants with severe hemophilia A should receive regular prophylactic Factor VIII replacement or emicizumab to prevent spontaneous bleeding episodes and joint damage. 1 The American Society of Hematology recommends prophylactic therapy as the cornerstone of management for severe hemophilia A. 1