Bleeding and Thrombosis in Children Alice J. Cohen, M. D. Newark Beth Israel Medical Center
History l 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 l Physical therapy had been ongoing for 6 weeks without improvement of pain nor range of motion of this left elbow
Past Medical History l Age 3 child reverted back to crawling for several weeks because his right ankle hurt l Age 5 DYFS investigated the family because a kindergarten teacher had seen numerous bruises l Age 7 epistaxis began l Age 9 bleeding with loss of a tooth l Age 10 episode of hematuria
Past Medical History l Age 11 a large hematoma occurred when a soccer ball hit his left thigh l Age 12 right hip injury while playing basketball l No family history of bleeding disorders.
Physical Examination l Left elbow boggy l Unable to extend beyond 90 degrees l Multiple scattered bruises l Right forearm hematoma l Right hip limited ROM with abduction and adduction
Radiography l X-ray left elbow no joint destruction l CT scan left elbow - fluid in the joint space possibly blood l Diagnosis - left elbow joint hemarthrosis l Referral made to pediatric hematology
Laboratory Testing l PT 14 seconds l a. PTT 51 seconds l Factor VIII: C 12% l Von Willebrand antigen 91% l Von Willebrand activity 90%
Diagnosis Mild Hemophilia A
History l l 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.
History l 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. l Venous Doppler studies revealed DVT involving left common femoral, proximal superficial femoral and popliteal veins.
History l No recent travel, trauma, use of oral contraceptives nor family history of thrombosis. l Recent upper airway stenosis found. l Nonsmoker.
Physical Examination l Lungs revealed inspiratory wheezing l Left lower extremity 1+ nonpitting edema
Laboratory Testing l l l l l 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 <5 Negative lupus anticoagulant and ACA’s FVIII 164% ATIII activity 53% (88 -125%)
Diagnosis l Antithrombin III deficiency l Recurrent spontaneous venous thrombosis
Treatment Life-long anticoagulation with warfarin
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