Conservative management of an acute spinal epidural hemorrhage in a child with hemophilia a with inhibitor

Abstract

Hemophilia A is an inherited, X-linked, recessive disorder caused by deficiency of clotting factor VIII. Central nervous system hemorrhage is an uncommon complication in patients with hemophilia. We report the case of a 5-year-old child who had intraspinal hematoma with nonsurgical, conservative management. It should be kept in mind that the optimal management is decided according to patient's condition because of high morbidity and mortality.

Spinal epidural hematoma in a child with hemophilia A with high titer inhibitors and follow-up with prophylactic emicizumab: case report and literature review

“…We excluded from the analysis one case of hemophilia B [15] and one with acquired hemophilia [16]. We included a total of 12 cases of patients diagnosed with hemophilia A that reported inhibitor presence from 1993 to date [17][18][19][20][21][22][23][24][25][26]. Most of these 12 patients had severe hemophilia.…”

Conservative therapy for spinal epidural hematoma in a child with hemophilia A with high-titer VIII inhibitors

“…Oymak et al 4 also successfully treated an infant with severe hemophilia A and inhibitors who presented with an SEH using recombinant activated factor VIIa. Erkutlu et al 5 reported the successful treatment of a 5-year-old child with an SEH using activated PCCs (aPCCs).…”

Atraumatic Spinal Epidural Hematoma as Initial Presentation of Hemophilia A in an Infant

“…1 Previous case reports have identified only 8 cases of atraumatic spinal hematoma leading to a diagnosis of hemophilia (Table 1), and between 1848 and 2015, an additional 35 published cases of spinal epidural hematoma in pediatric hemophilia patients. 8,[10][11][12][13] The presenting complaints of the atraumatic patients varied, but all included neurological findings of weakness or urinary/ bowel symptoms. [2][3][4][5][6][7][8][9] The cause of atraumatic spinal epidural hematoma in both hemophiliac and nonhemophiliac patients is not well established.…”

“…Known maternal carrier status, followed by bleeding events, was the most common reason for diagnosis 1 . Previous case reports have identified only 8 cases of atraumatic spinal hematoma leading to a diagnosis of hemophilia (Table 1), and between 1848 and 2015, an additional 35 published cases of spinal epidural hematoma in pediatric hemophilia patients 8,10–13 . The presenting complaints of the atraumatic patients varied, but all included neurological findings of weakness or urinary/bowel symptoms 2–9 …”