Hemophilia

Paralyzed

Step 1: View clinicals

A 3 month old boy presents with diminished upper and lower limb movements, absence of defecation, and dribbling micturition for 4 days. His perinatal history is significant for a prolonged labour with vacuum extraction. A caput succedaneum was present at birth, but resolved spontaneously in 3 days. His medical and family histories are unremarkable.


Step 2: Order all relevant investigations

Septic screen

Urine, blood and cerebrospinal fluid cultures are obtained; results will be available in 3 days. Gram stains are negative, and there are no ancillary findings suggestive of infection.

MRI Brain & Spine

There is a heterogeneous collection in the posterior spinal epidural space, extending from the C2 to D5 vertebrae, with anterior displacement of the dura and compression of the spinal cord. This is consistent with an acute epidural hematoma.

Coagulation profile

Bleeding time: 4 min (3-9 min) Clotting time: 14 min (3-7 min) PT: 11s (< 11s) APTT: 75s (< 26s)

Factor VIII and IX assays

Factor VIII levels: <1% of normal Factor IX levels: normal


Step 3: Select appropriate management

IV Antibiotics
Surgical Decompression
Factor VIII Concentrate
Platelet Transfusion


Score: ★★☆