Maxillofacial extramedullary hematopoiesis in a child with sickle cell presenting as bilateral periorbital cellulitis

Int J Pediatr Otorhinolaryngol. 2014 Jul;78(7):1173-5. doi: 10.1016/j.ijporl.2014.03.028. Epub 2014 Apr 6.

Abstract

Review of a case of paraosseous extramedullary hematopoiesis (EMH) affecting the maxillary sinuses and retro-orbital spaces imitating bilateral orbital cellulitis. Maxillofacial EMH causes diagnostic/therapeutic challenges. This case report describes a 4-year-old African American male with sickle cell disease (HbSS) who presented with bilateral orbitofacial swelling. Diagnosis was made with imaging and confirmed with tissue sampling. Partial exchange transfusion was utilized to stop the progression of maxillofacial EMH and to treat the patient's chronic anemia. Follow-up MRI showed regression of orbital and retro-orbital involvement. Early treatment with conservative modalities and close observation may prevent need for more invasive treatments.

Keywords: Extramedullary hematopoiesis; Maxillary sinuses; Paraosseous; Retro-orbital space.

Publication types

  • Case Reports

MeSH terms

  • Anemia, Sickle Cell / complications*
  • Child, Preschool
  • Diagnosis, Differential
  • Exchange Transfusion, Whole Blood
  • Hematopoiesis, Extramedullary*
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Maxillary Sinus / pathology
  • Orbit / pathology
  • Orbital Cellulitis / diagnosis
  • Tomography, X-Ray Computed