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Case Report
Management of Spontaneous Spinal Epidural Hematoma Developed in Children with Hemophilia
Clin Pediatr Hematol Oncol 2018;25:180-4.
Published online October 31, 2018
© 2018 Korean Society of Pediatric Hematology-Oncology and Korean Society for Pediatric Neuro-Oncology

Han Na Jang, M.D., Hoi Soo Yoon, M.D., Ph.D. and Young Shil Park, M.D., Ph.D.

Department of Pediatrics, College of Medicine, Kyung Hee University, Seoul, Korea
Correspondence to: Young Shil Park
Department of Pediatrics, Kyung Hee University Hospital at Gangdong, 892 Dongnam-ro, Gangdong-gu, Seoul 05278, Korea
Tel: +82-2-440-6133
Fax: +82-2-440-7175
E-mail: pysmd@khnmc.or.kr
ORCID ID: orcid.org/0000-0002-6643-4245
Received August 29, 2018; Revised September 13, 2018; Accepted September 30, 2018.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
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.
Keywords: Hemophilia, Spinal, Epidural, Hematoma, Management
References
  1. Holtas S, Heiling M, Lönntoft M. Spontaneous spinal epidural hematoma: findings at MR imaging and clinical correlation. Radiology 1996;199:409-13.
    Pubmed CrossRef
  2. Eyster ME, Gill FM, Blatt PM, Hilgartner MW, Ballard JO, Kinney TR. Central nervous system bleeding in hemophiliacs. Blood 1978;51:1179-88.
    Pubmed
  3. de Tezanos Pinto M, Fernandez J, Perez Bianco PR. Update of 156 episodes of central nervous system bleeding in hemophiliacs. Haemostasis 1992;22:259-67.
    Pubmed
  4. Hoots WK. Emergency management of hemophilia. In: Lee C, Berntorp EE, Hoots WK, editors, Textbook of hemophilia. 3rd ed. Hoboken: Wiley-Blackwell, 2014;463.
    CrossRef
  5. Kreppel D, Antoniadis G, Seeling W. Spinal hematoma: a literature survey with meta-analysis of 613 patients. Neurosurg Rev 2003;26:1-49.
    Pubmed CrossRef
  6. Börm W, Mohr K, Hassepass U, Richter HP, Kast E. Spinal hematoma unrelated to previous surgery: analysis of 15 consecutive cases treated in a single institution within a 10-year period. Spine 2004;29:E555-61.
    Pubmed CrossRef
  7. Cuvelier GD, Davis JH, Purves EC, Wu JK. Torticollis as a sign of cervico-thoracic epidural haematoma in an infant with severe haemophilia A. Haemophilia 2006;12:683-6.
    Pubmed CrossRef
  8. Irwin SL, Attia MW. A traumatic spinal epidural hematoma in an infant with hemophilia A. Pediatr Emerg Care 2001;17:40-1.
    CrossRef
  9. Küker W, Thiex R, Friese S, et al. Spinal subdural and epidural haematomas: diagnostic and therapeutic aspects in acute and subacute cases. Acta Neurochir (Wien) 2000;142:777-85.
    CrossRef
  10. Liao CC, Lee ST, Hsu WC, Chen LR, Lui TN, Lee SC. Experience in the surgical management of spontaneous spinal epidural hematoma. J Neurosurg 2004;100:38-45.
    CrossRef
  11. Ravid S, Schneider S, Maytal J. Spontaneous spinal epidural hematoma: an uncommon presentation of a rare disease. Childs Nerv Syst 2002;18:345-7.
    Pubmed CrossRef
  12. Kiehna EN, Waldron PE, Jane JA. Conservative management of an acute spontaneous holocord epidural hemorrhage in a hemophiliac infant. J Neurosurg Pediatr 2010;6:43-8.
    Pubmed CrossRef
  13. Rois PV, Lopez MR, de Vergara BC, de la Lama Zaragoza A, Garcia JG, Uxo JM. Spinal epidural hematoma in hemophilic children: controversies in management. Childs Nerv Syst 2009;25:987-91.
    Pubmed CrossRef
  14. Bakker NA, Veeger NJ, Vergeer RA, Groen RJ. Prognosis after spinal cord and cauda compression in spontaneous spinal epidural hematomas. Neurology 2015;84:1894-903.
    Pubmed CrossRef
  15. Witmer C, Presley R, Kulkarni R, Soucie JM, Manno CS, Raffini L. Associations between intracranial haemorrhage and prescribed prophylaxis in a large cohort of haemophilia patients in the United States. Br J Haematol 2011;152:211-6.
    Pubmed CrossRef


October 2018, 25 (2)
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  • Young Shil Park