Clin Pediatr Hematol Oncol.  2018 Oct;25(2):180-184. 10.15264/cpho.2018.25.2.180.

Management of Spontaneous Spinal Epidural Hematoma Developed in Children with Hemophilia

• Jang HN
• Yoon HS
• Park YS

• KMID: 2435412
• DOI: http://doi.org/10.15264/cpho.2018.25.2.180

Abstract

Spinal epidural hematoma (SEH) is a rare neurosurgical emergency in which pressure on the spinal cord leads to acute neurological deficits, and is a rare complication in children with hemophilia. We report three cases of SEH in severe hemophilia A. An 8-month-old boy who presented with non-traumatic acute-onset irritability was found to have SEH and was later diagnosed with hemophilia. The two other patients presented with neck pain and magnetic resonance imaging confirmed the diagnosis of SEH. Two patients who received conservative management fully recovered, however the patient who presented with progressive neurological abnormalities at the time of diagnosis, received surgery but later developed breathing difficulties and quadriplegia. Early diagnosis and immediate, aggressive, clotting factor replacement therapy are crucial when managing SEH in children with hemophilia. Immediate and aggressive factor replacement, accompanied by both neurological monitoring and early imaging, are essential for hemophiliac with suspected SEH.

Keyword

Hemophilia; Spinal; Epidural; Hematoma; Management

MeSH Terms

Child*
Diagnosis
Early Diagnosis
Emergencies
Hematoma
Hematoma, Epidural, Spinal*
Hemophilia A*
Humans
Infant
Magnetic Resonance Imaging
Male
Neck Pain
Quadriplegia
Respiration
Spinal Cord