Spontaneous epidural hematoma in a child with inherited factor XIII deficiency

Roula A. Farah, Jad Z. Al Danaf, Rita A. Chahinian, Nabil T. Braiteh, Naim F. Al Ojaimi, Andrea Cairo, Hussein Farhat, Joseph R. Mantoura

Research output: Contribution to journalArticle

1 Citation (Scopus)

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.

Original languageEnglish
Pages (from-to)62-65
Number of pages4
JournalJournal of Pediatric Hematology/Oncology
Volume36
Issue number1
DOIs
Publication statusPublished - Jan 2014

Fingerprint

Factor XIII Deficiency
Hematoma
Factor XIII
Splenic Rupture
Spontaneous Rupture
Intracranial Hemorrhages
Craniotomy
Craniocerebral Trauma
Vomiting
Hospital Emergency Service
Siblings
Exons
Parturition
Hemorrhage
Morbidity
Mutation
Mortality
DNA

Keywords

  • Epidural hematoma
  • Factor XIII
  • Factor XIII deficiency
  • Factor XIII mutation
  • Prophylaxis
  • Spontaneous bleeding

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Oncology
  • Hematology

Cite this

Farah, R. A., Al Danaf, J. Z., Chahinian, R. A., Braiteh, N. T., Al Ojaimi, N. F., Cairo, A., ... Mantoura, J. R. (2014). Spontaneous epidural hematoma in a child with inherited factor XIII deficiency. Journal of Pediatric Hematology/Oncology, 36(1), 62-65. https://doi.org/10.1097/MPH.0b013e31828e52b6

Spontaneous epidural hematoma in a child with inherited factor XIII deficiency. / Farah, Roula A.; Al Danaf, Jad Z.; Chahinian, Rita A.; Braiteh, Nabil T.; Al Ojaimi, Naim F.; Cairo, Andrea; Farhat, Hussein; Mantoura, Joseph R.

In: Journal of Pediatric Hematology/Oncology, Vol. 36, No. 1, 01.2014, p. 62-65.

Research output: Contribution to journalArticle

Farah, RA, Al Danaf, JZ, Chahinian, RA, Braiteh, NT, Al Ojaimi, NF, Cairo, A, Farhat, H & Mantoura, JR 2014, 'Spontaneous epidural hematoma in a child with inherited factor XIII deficiency', Journal of Pediatric Hematology/Oncology, vol. 36, no. 1, pp. 62-65. https://doi.org/10.1097/MPH.0b013e31828e52b6
Farah, Roula A. ; Al Danaf, Jad Z. ; Chahinian, Rita A. ; Braiteh, Nabil T. ; Al Ojaimi, Naim F. ; Cairo, Andrea ; Farhat, Hussein ; Mantoura, Joseph R. / Spontaneous epidural hematoma in a child with inherited factor XIII deficiency. In: Journal of Pediatric Hematology/Oncology. 2014 ; Vol. 36, No. 1. pp. 62-65.
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