• Raksha Rao
  • Santosh G. Honavar
Living reference work entry


Retinoblastoma is the most common malignant intraocular tumor in children. An estimated 5000 new cases are diagnosed every year, and of these, very few present as intraocular inflammation. Due to the rarity of the disease itself, which furthermore complicated by the rarity of the uveitis-like presentation, this small subset of cases with atypical manifestations is often misdiagnosed and inappropriately managed. Delay in primary management results in suboptimal life, eye, and vision salvage. Intraocular surgery in a child with viable retinoblastoma can lead to extraocular extension and consequent regional lymph node and systemic metastasis. Therefore, atypical manifestations of retinoblastoma have to be excluded in every child presenting with uveitis and hypopyon.


Anterior Chamber Trabecular Meshwork Retinal Ischemia Neovascular Glaucoma Orbital Cellulitis 
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Suggested Reading

  1. Shields CL, Ghassemi F, Tuncer S, Thangappan A, Shields JA. Clinical spectrum of diffuse infiltrating retinoblastoma in 34 consecutive eyes. Ophthalmology. 2008;115(12):2253–8.CrossRefPubMedGoogle Scholar
  2. Jijelava KP, Grossniklaus HE. Diffuse anterior retinoblastoma: a review. Saudi J Ophthalmol. 2013;27(3):135–9.CrossRefPubMedPubMedCentralGoogle Scholar
  3. Shields CL, Lally SE, Manjandavida FP, Leahey AM, Shields JA. Diffuse anterior retinoblastoma with globe salvage and visual preservation in 3 consecutive cases. Ophthalmology. 2016;123(2):378–84.CrossRefPubMedGoogle Scholar
  4. Yang J, Dang Y, Zhu Y, Zhang C. Diffuse anterior retinoblastoma: current concepts. Onco Targets Ther. 2015;8:1815–21.PubMedPubMedCentralGoogle Scholar
  5. Murthy R, Honavar SG, Vemuganti GK, Naik MN, Reddy VP. Systemic metastasis following hyphema drainage in an unsuspected retinoblastoma. J Pediatr Ophthalmol Strabismus. 2007;44(2):120–3.PubMedGoogle Scholar
  6. Honavar SG, Singh AD. Management of advanced retinoblastoma. Ophthalmol Clin North Am. 2005;18(1):65–73.CrossRefPubMedGoogle Scholar
  7. Honavar SG, Singh AD, Shields CL, Meadows AT, Demirci H, Cater J, Shields JA. Postenucleation adjuvant therapy in high-risk retinoblastoma. Arch Ophthalmol. 2002;120(7):923–31.CrossRefPubMedGoogle Scholar
  8. Shields CL, Honavar S, Shields JA, Demirci H, Meadows AT. Vitrectomy in eyes with unsuspected retinoblastoma. Ophthalmology. 2000;107(12):2250–5.CrossRefPubMedGoogle Scholar

Copyright information

© Springer (India) Pvt. Ltd. 2016

Authors and Affiliations

  1. 1.Ocular Oncology ServiceNational Retinoblastoma Foundation, Centre for SightHyderabadIndia

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