Bleeding and Thrombosis in Children

1
Bleeding and Thrombosis in Children

• Alice J. Cohen, M.D.
• Newark Beth Israel Medical Center

2
History

• 13 year old white male presented to an orthopedic
  surgeon for a second opinion for an injury to his
  left elbow that had occurred 8 weeks ago while
  playing basketball
• Physical therapy had been ongoing for 6 weeks
  without improvement of pain nor range of motion
  of this left elbow

3
Past Medical History

• Age 3 child reverted back to crawling for several
  weeks because his right ankle hurt
• Age 5 DYFS investigated the family because a
  kindergarten teacher had seen numerous bruises
• Age 7 epistaxis began
• Age 9 bleeding with loss of a tooth
• Age 10 episode of hematuria

4
Past Medical History

• Age 11 a large hematoma occurred when a soccer
  ball hit his left thigh
• Age 12 right hip injury while playing basketball
• No family history of bleeding disorders.

5
Physical Examination

• Left elbow boggy
• Unable to extend beyond 90 degrees
• Multiple scattered bruises
• Right forearm hematoma
• Right hip limited ROM with abduction and adduction

6
Radiography

• X-ray left elbow no joint destruction
• CT scan left elbow - fluid in the joint space
  possibly blood
• Diagnosis - left elbow joint hemarthrosis
• Referral made to pediatric hematology

7
Laboratory Testing

• PT 14 seconds
• aPTT 51 seconds
• Factor VIIIC 12
• Von Willebrand antigen 91
• Von Willebrand activity 90

8
Diagnosis

• Mild Hemophilia A

9
History

• 16 year old hispanic female presented complaining
  of left leg pain and swelling for 1 week.
• No history of trauma. No other medical problems.
• Venous Dopplers positive for DVT of left
  ileofemoral vein.
• Negative work up for underlying hypercoagulable
  state reported and she was treated with heparin
  and 6 months of warfarin therapy.

10
History

• She was well until age of 20 years (a doppler
  study was negative at age of 18) when she again
  presented with left lower leg pain and swelling
  for 1 week. The leg was so painful that she was
  unable to walk.
• Venous Doppler studies revealed DVT involving
  left common femoral, proximal superficial femoral
  and popliteal veins.

11
History

• No recent travel, trauma, use of oral
  contraceptives nor family history of thrombosis.
• Recent upper airway stenosis found.
• Nonsmoker.

12
Physical Examination

• Lungs revealed inspiratory wheezing
• Left lower extremity 1 nonpitting edema

13
Laboratory Testing

• Venous Dopplers revealed DVT involving common
  femoral, proximal superficial femoral, mid
  superficial femoral, distal superficial femoral,
  poplital veins.
• Activated protein C resistance 0.98 (neg)
• Negative Factor V Leiden mutation
• Negative Prothrombin Gene Mutation
• Protein C 149
• Free protein S 78
• Homocysteine lt5
• Negative lupus anticoagulant and ACAs
• FVIII 164
• ATIII activity 53 (88-125)

14
Diagnosis

• Antithrombin III deficiency
• Recurrent spontaneous venous thrombosis

15
Treatment

• Life-long anticoagulation with warfarin