title: Torticollis in a haemophilic infant with inhibitor: a case of spinal epidural haematoma.
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.
authors.affiliation: aClinic of Hematology bDivision of Neurosurgery, Dr Behcet Uz Children's Hospital, Konak cAlsancak MR, Radiology Alsancak, Izmir, Turkey.
authors.forename: Yesim
authors.initials: Y
authors.lastname: Oymak
authors.forename: Neryal
authors.initials: N
authors.lastname: Muminoglu
authors.forename: Yilmaz
authors.initials: Y
authors.lastname: Ay
authors.forename: Tuba H
authors.initials: TH
authors.lastname: Karapinar
authors.forename: Sultan A
authors.initials: SA
authors.lastname: Köker
authors.forename: Ersin
authors.initials: E
authors.lastname: Töret
authors.forename: Aşiyan K
authors.initials: AK
authors.lastname: Ylmaz
authors.forename: Müge
authors.initials: M
authors.lastname: Gürçnar
authors.forename: Canan
authors.initials: C
authors.lastname: Vergin
mesh_terms: D005169:Factor VIII / Q000627:therapeutic use*; D015942:Factor VIIa / Q000627:therapeutic use*; D046748:Hematoma, Epidural, Spinal / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology; D006467:Hemophilia A / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D011994:Recombinant Proteins / Q000627:therapeutic use; D014103:Torticollis / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D011994:Recombinant Proteins; D005169:Factor VIII; C103587:recombinant FVIIa; D015942:Factor VIIa