First manifestation of severe haemophilia: acute flaccid paralysis due to spinal cord contusion with subdural hemorrhage

Abstract

Hemophilia A and hemophilia B are the most common and serious congenital coagulation factor deficiencies. Intracranial hemorrhages occur in 3-10%. Spinal epidural hematomas are rare. Even with severe hemophilia, only 90% have evidence of increased bleeding by 1 year of age. Only 2% of neonates with hemophilia sustain intracranial hemorrhages. Here we describe a case of a 5-month old boy with positive family history of hemophilia A on his maternal side who was admitted to the hospital because of retention of urine and decreased movement in both lower limbs following history of fall from height (approximately 2 feet high), two days prior to admission. Physical examination showed no skin lesion or hematoma. Neurological examination showed the infant in frog like posture with flaccid paralysis of both lower limbs without loss of sensation. There was associated bladder involvement with a Phantom hernia on the right side of his abdomen. MRI Spine revealed cord contusion in D4-D9 segment with spinal subdural hemorrhage. Coagulation profile was abnormal with a prolonged Activated plasma thromboplastin time. Factor VIII assay revealed a level of 1%. Treatment was conservative, and the infant was given factor VIII replacement. There was remarkable improvement within weeks. Thus, spinal hematomas being rare should still be considered and ruled out for prompt management of cases of suspected hemophilia. This case highlights the importance of a thorough family history which led to the ultimate diagnosis of severe Hemophilia A by coagulation profile and neuroimaging and further confirmed by factor VIII assay.

A Case Report of Acute Hemorrhage in Spinal Schwannoma Associated with Hemophilia B

“…Hemophilia is an inherited bleeding disorder characterized by improper blood clotting and intraarticular and intramuscular bleeding 1) . Although intracranial hemorrhage has been estimated to occur in about 3%-10% of hemophilia cases 2) , spinal intratumoral hemorrhage is rare 3) , and intraspinal hemorrhage is even rarer (0.001% of hemophilia cases) 4) . Spinal schwannomas often accompany minor hemorrhages; however, it is rare for hemorrhage schwannomas to exacerbate neurological symptoms 5) .…”