Last reviewed: November 2017
Last updated: October  2017

Summary

Definition

History and exam

Key diagnostic factors

  • age <3 years
  • positive family history
  • leukocoria (white pupillary reflex)
  • strabismus
  • pseudo-orbital cellulitis
  • 13q syndrome

Other diagnostic factors

  • visual disturbances
  • ocular pain
  • pinealoma

Risk factors

  • mutation in RB1 gene
  • HPV viral exposure
  • advanced paternal age

Diagnostic investigations

1st investigations to order

  • funduscopy and examination under anaesthesia
  • wide-field fundus photography and spectral domain optical coherence tomography (sdOCT)
  • ophthalmic A- and B-scan ultrasound
Full details

Investigations to consider

  • molecular testing
  • MRI head/orbit
  • bone marrow aspiration
  • lumbar puncture
Full details

Treatment algorithm

Contributors

Authors VIEW ALL

Founding Director/CEO

Murray Ocular Oncology and Retina

Miami

FL

Disclosures

TGM is an author of a number of references cited in this monograph.

Ophthalmologist/Ocular Oncologist

Bascom Palmer Eye Institute

Miami

FL

Disclosures

VMV is an author of a reference cited in this monograph.

Dr Timothy G. Murray and Dr Victor M. Villegas would like to gratefully acknowledge Dr Amy C. Schefler, a previous contributor to this monograph, and Dr Steven Houston, III, MD for his contribution to the monograph. ACS is an author of a number of references cited in this monograph. SH declares that he has no competing interests.

Peer reviewers VIEW ALL

Director

Pharmaceutical Clinical & Scientific Affairs

Bausch & Lomb

Rochester

NY

Disclosures

SS declares that she has no competing interests.

Professor and Head of Pediatric Ocular Oncology Unit

Jules Gonin Eye Hospital

Lausanne

Switzerland

Disclosures

FM declares that he has no competing interests.

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