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Post-chemoreduction cryptic optic nerve relapse in a patient with bilateral retinoblastoma
  1. Nandan G Shetye1,
  2. Asimina Mataftsi1,2,
  3. Philippe Maeder3,
  4. Alexandre P Moulin1,
  5. Marie-Claire Gaillard1,
  6. Aubin Balmer1,
  7. Maya Beck Popovic4,
  8. Francis L Munier1
  1. 1Department of Ophthalmology, Jules Gonin Eye Hospital, Lausanne, Switzerland
  2. 2IInd Department of Ophthalmology, Aristotle University of Thessaloniki, Thessaloniki, Greece
  3. 3Radiology Department, CHUV, University of Lausanne, Lausanne, Switzerland
  4. 4Pediatric Hemato-Oncology Unit, CHUV, University of Lausanne, Lausanne, Switzerland
  1. Correspondence to Professor Francis L Munier, Department of Ophthalmology, Jules Gonin Eye Hospital, Av.de France 15, Lausanne 1004, Switzerland; francis.munier{at}fa2.ch

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Introduction/case report

Retinoblastoma is the most common primary intraocular tumour in children with an incidence of 1 in 15 000 live births.1 In recent years, intravenous chemoreduction, followed by adjuvant focal treatment, has reduced the number of retinoblastoma cases needing external beam radiotherapy and enucleation.2 Optic nerve involvement is one of the most highly predictive factors for death from this disease.3 Postlaminar tumorous invasion, and tumour presence at the surgical margin of the optic nerve are both considered major prognostic factors for disease dissemination.3 ,4

A 20-month-old patient was diagnosed with sporadic bilateral retinoblastoma, Group C, or Vb endophytic, in the left eye (LE) with local vitreous seeds, and Group D, or Va exophytic, in the right eye (RE) with complete retinal detachment, subretinal and epiretinal seeding, due to a macular tumour adjacent to the optic disc (figure 1A,B).5

Figure 1

Right eye (RE) and left eye (LE) fundus at presentation (A, B) and after initial treatment (D, E). Right optic nerve imaging with MRI at presentation (C) and with B-scan ultrasonography after initial treatment (F). (A) Montage fundus photograph of the RE at presentation, showing exophytic tumour growth with complete retinal detachment (main tumour in the posterior pole measuring 13.6 mm of height upon ultrasonography). (B) Montage fundus photograph of the LE at presentation, demonstrating endophytic retinoblastoma tumours. (C) MRI of the right orbit at …

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