Child's Nervous System

, Volume 25, Issue 8, pp 987–991 | Cite as

Spinal epidural hematoma in hemophilic children: controversies in management

  • Pilar Varela RoisEmail author
  • Manuel Rodríguez López
  • Beatriz Cabanas López de Vergara
  • Adolfo de la Lama Zaragoza
  • Jesús González García
  • Jorge Mañas Uxo
Case for Discussion


Introduction and objective

Spinal epidural hematoma (SEH) is an uncommon complication in hemophilic children. It can produce rapidly progressive neurological deficits. We aim to discuss the different management options for these patients.

Case report

A 13-year-old boy with a history of hemophilia A was admitted with acute onset of localized spine pain and weakness. No trauma was reported on review of the history. Recombinant factor VIII aggressive replacement therapy was started. Spinal magnetic resonance imaging revealed an extradural mass lesion extending from D5 to D6 level. Emergency hemilaminectomies of D5 and D6 and evacuation of the clot were done. The patient made excellent recovery following surgery.


Early diagnosis and immediate aggressive replacement therapy are mandatory in the management of SEH. Prompt surgical decompression to avoid any permanent neurological deficit is a safe and effective treatment option for an SEH in selected hemophilic children.


Spinal epidural hematoma Hemophilia Magnetic resonance imaging Factor VIII deficiency Spinal hemorrhage 



The authors wish to thank Professor Rene de Costa for his assistance with the editing of this paper.


  1. 1.
    Balak N, Silav G, Kilic Y, Timur C, Elmaci I (2007) Successful surgical treatment of a hemophiliac infant with nontraumatic acute subdural hematoma. Surg Neurol 68:537–540PubMedCrossRefGoogle Scholar
  2. 2.
    Balkan C, Kavakli K, Karapinar D (2006) Spinal epidural haematoma in a patient with haemophilia B. Haemophilia 12:437–440PubMedCrossRefGoogle Scholar
  3. 3.
    Börm W, Mohr K, Hassepass U, Richter HP, Kast E (2004) Spinal hematoma unrelated to previous surgery. Analysis of 15 consecutive cases treated in a single institution within a 10-year period. Spine 29:555–561CrossRefGoogle Scholar
  4. 4.
    Cuvelier GD, Davis JH, Purves EC, Wu JK (2006) Torticollis as a sign of cervico-thoracic epidural haematoma in an infant with severe haemophilia A. Haemophilia 12:683–686PubMedCrossRefGoogle Scholar
  5. 5.
    Eftekhar B, Ghodsi M, Ketabchi E, Bakhtiari A, Mostajabi P (2003) Spinal subdural hematoma revealing hemophilia A in a child: a case report. BMC Blood Disord 3:2–5PubMedCrossRefGoogle Scholar
  6. 6.
    Heer JS, Enríquez EG, Carter AJ (2007) Spinal epidural hematoma as first presentation of hemophilia A. J Emerg Med 34:159–162PubMedCrossRefGoogle Scholar
  7. 7.
    Iwamuro H, Morita A, Kawaguchi H, Kirino T (2004) Resolution of spinal epidural haematoma without surgery in a haemophilic infant. Acta Neurochir (Wien) 146:1263–1265CrossRefGoogle Scholar
  8. 8.
    Kalina P, Morris J, Raffel C (2008) Spinal epidural hematoma in an infant as the initial presentation of severe hemophilia. Emerg Radiol 15:281–284PubMedCrossRefGoogle Scholar
  9. 9.
    Kreppel D, Antoniadis G, Seeling W (2003) Spinal hematoma: a literature survey with meta-analysis of 613 patients. Neurosurg Rev 26:1–49PubMedCrossRefGoogle Scholar
  10. 10.
    Liao CC, Lee ST, Hsu WC, Chen LR, Lui TN, Lee SC (2004) Experience in the surgical management of spontaneous spinal epidural hematoma. J Neurosurg 100:38–45PubMedGoogle Scholar
  11. 11.
    Pai SB, Maiya P (2006) Spontaneous spinal epidural hematoma in a toddler: a case report. Childs Nerv Syst 22:526–529PubMedCrossRefGoogle Scholar
  12. 12.
    Ravid S, St S, Maytal J (2002) Spontaneous spinal epidural hematoma: an uncommon presentation of a rare disease. Childs Nerv Syst 18:345–347PubMedCrossRefGoogle Scholar
  13. 13.
    Rodriguez-Merchant EC, Rocino A (2004) Literature review of surgery management in inhibitor patients. Haemophilia 10:22–29CrossRefGoogle Scholar
  14. 14.
    Steinbok P (2008) Multiple short-segment laminoplasties in children: a novel technique to avoid postoperative spinal deformity. Childs Nerv Syst 24:369–372PubMedCrossRefGoogle Scholar
  15. 15.
    Tailor J, Dunn IF, Smith E (2006) Conservative treatment of spontaneous spinal epidural hematoma associated with oral anticoagulant therapy in a child. Childs Nerv Syst 22:1643–1645PubMedCrossRefGoogle Scholar
  16. 16.
    Watts RG (2005) Successful use of recombinant factor VIIa for emergency fasciotomy in a patient with hemophilia A and high-titer inhibitor unresponsive to factor VIII inhibitor bypassing activity. Am J Hematol 79:58–60PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  • Pilar Varela Rois
    • 1
    Email author
  • Manuel Rodríguez López
    • 2
  • Beatriz Cabanas López de Vergara
    • 2
  • Adolfo de la Lama Zaragoza
    • 1
  • Jesús González García
    • 1
  • Jorge Mañas Uxo
    • 3
  1. 1.Department of NeurosurgeryXeral-Cies HospitalVigoSpain
  2. 2.Department of HematologyXeral-Cies University HospitalVigoSpain
  3. 3.Department of RadiologyXeral-Cies University HospitalVigoSpain

Personalised recommendations