Retinoblastoma presenting with orbital cellulitis

J AAPOS. 2013 Jun;17(3):282-6. doi: 10.1016/j.jaapos.2013.02.009.

Abstract

Purpose: To study the effectiveness of pre-enucleation steroids in reducing inflammation in patients with retinoblastoma presenting as orbital cellulitis.

Methods: Medical records of consecutive retinoblastoma patients presenting at a single tertiary eye care center during a period of 3 years were retrospectively reviewed. For those who presented with orbital cellulitis, clinical, radiological, and histopathological variables were assessed. The effect of pre-enucleation steroids was noted in this group of patients.

Results: Of 260 retinoblastoma cases reviewed, 14 had retinoblastoma-associated cellulitis (5.39%). Of these 14 patients, 4 received neoadjuvant chemotherapy and were excluded from the series. Of the remaining 10 cases (mean age at presentation, 14.2 months; mean follow-up, 16.4 months), 9 presented with orbital cellulitis and were included in the study. Radiological imaging depicted intraocular tumors occupying 80% to 100% of the globe in each case. All patients underwent enucleation. Five children received pre-enucleation systemic steroids (mean, 5.4 days), which resulted in a prompt decrease in inflammation. Postenucleation chemotherapy was administered in 4 (6 cycles) and external beam radiation therapy in 1 patient with high-risk histopathological characteristics.

Conclusions: Advanced necrotic retinoblastoma with anterior segment involvement may present as orbital cellulitis. Pre-enucleation systemic steroids can aid in the surgical management of these tumors.

MeSH terms

  • Chemotherapy, Adjuvant
  • Child, Preschool
  • Combined Modality Therapy
  • Diagnosis, Differential
  • Eye Enucleation
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Male
  • Orbital Cellulitis / diagnosis*
  • Orbital Cellulitis / therapy
  • Radiotherapy, High-Energy
  • Retinal Neoplasms / diagnosis*
  • Retinal Neoplasms / therapy
  • Retinoblastoma / diagnosis*
  • Retinoblastoma / therapy