Torticollis in a haemophilic infant with inhibitor: a case of spinal epidural haematoma

Oymak Y., Muminoglu N., Ay Y., Karapinar T. H., Koker S. A., Toret E., ...More

BLOOD COAGULATION & FIBRINOLYSIS, vol.27, no.5, pp.576-579, 2016 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 27 Issue: 5
  • Publication Date: 2016
  • Doi Number: 10.1097/mbc.0000000000000465
  • Journal Name: BLOOD COAGULATION & FIBRINOLYSIS
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.576-579
  • Keywords: haemophilia, infant, inhibitor, spinal epidural haematoma, torticollis, MANAGEMENT, CHILDREN, SURGERY
  • Eskisehir Osmangazi University Affiliated: Yes

Abstract

Central nervous system bleeding, which can be a life-threatening complication, is seen in 2.7% of patients with haemophilia. Spinal epidural haematomas represent about one-tenth of such cases. Here, we report on a 10-month-old boy with severe haemophilia A, who presented with torticollis. Although administration of factor VIII at a dose of 50 U/kg, the patient developed flaccid paralysis of the upper extremities. Factor VIII inhibitor screen was positive. Magnetic resonance imaging of the spine revealed spinal epidural haematomas, extending from C-1 to the cauda equina. Treatment was continued with recombinant activated factor VIIa without surgery. After 1 month, complete neurological recovery was achieved and fully resolved haematomas were detected on spinal MRI. A prompt radiological evaluation of the cervical spine with MRI should be made in patients with haemophilia presenting with torticollis. In addition, in the case of life-threatening bleeding in patients with haemophilia, the possibility of an inhibitor should be kept in mind. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.