title: Spontaneous epidural hematoma in a child with inherited factor XIII deficiency.
abstract: We report the case of a 2-year-old Lebanese male child, known to have congenital factor XIII (FXIII) deficiency, who presented to the emergency department with somnolence and projectile vomiting without any head trauma. He has been on a prophylactic dose of 10 IU/kg of FXIII concentrate every 4 weeks since birth, but he missed his last 2 doses due to shortage of supply. Imaging studies showed an epidural hematoma with a midline shift. The child was started on 20 IU/kg of FXIII replacement, and a left parietal craniotomy was performed immediately. He tolerated the surgery well with an uneventful postoperative course. Previous DNA analysis carried out for the family members detected a small deletion (c.1475-1476delGA) in exon 12 in this child and his eldest brother. This mutation has been previously reported once in another Lebanese child with FXIII deficiency who presented with spontaneous splenic rupture. To the best of our knowledge, this is the first case of acute nontraumatic spontaneous epidural hematoma in a child with congenital FXIII deficiency. Furthermore, patients on FXIII replacement therapy have less bleeding events, thus lifelong adherence to the prophylaxis is essential to decrease the morbidities and the mortalities associated with FXIII deficiency, most notably intracranial hemorrhages.
authors.affiliation: Departments of *Pediatrics ‡Laboratory Medicine §Neurosurgery, University Medical Center-Rizk Hospital, Beirut, Lebanon †Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, U.O.S. Dipartimentale per la, Diagnosi e la Terapia delle Coagulopatie, Fondazione IRCCS Cà Granda - Ospedale Maggiore, Policlinico, and Luigi Villa Foundation, Milan, Italy.
authors.forename: Roula A
authors.initials: RA
authors.lastname: Farah
authors.forename: Jad Z
authors.initials: JZ
authors.lastname: Al Danaf
authors.forename: Rita A
authors.initials: RA
authors.lastname: Chahinian
authors.forename: Nabil T
authors.initials: NT
authors.lastname: Braiteh
authors.forename: Naim F
authors.initials: NF
authors.lastname: Al Ojaimi
authors.forename: Andrea
authors.initials: A
authors.lastname: Cairo
authors.forename: Hussein
authors.initials: H
authors.lastname: Farhat
authors.forename: Joseph R
authors.initials: JR
authors.lastname: Mantoura
mesh_terms: D002675:Child, Preschool; D004632:Emergency Medical Services; D005176:Factor XIII / Q000235:genetics / Q000627:therapeutic use; D005177:Factor XIII Deficiency / Q000150:complications* / Q000188:drug therapy / Q000235:genetics; D006407:Hematoma, Epidural, Cranial / Q000097:blood* / Q000209:etiology* / Q000601:surgery; D006801:Humans; D008297:Male
chemical_list: D005176:Factor XIII