Associated Features of Strabismus in the Presentation of Retinoblastoma
Miles F. Kiernan, MD; Victoria Smith; Andrew Stacey, MD; Mandeep Sagoo, MD; Ashwin Reddy, MD
The Retinoblastoma Unit, Barts Health NHS Trust
Introduction: The common presenting features of intraocular retinoblastoma (RB) include Leukocoria (L), Strabismus (S) and inflammation (I). Leukocoria has been associated with poorer ocular survival (1). Exotropia has been shown to be more common than expected in infantile strabismus (2). The impact of the combination of L and S presenting together has not been assessed. The need for enucleation at presentation and the use of adjuvant chemotherapy post-enucleation are recognised indicators of advanced disease (3).
Methods: The medical notes of sporadically presenting patients referred to our unit between 2009 and 2015 were reviewed. Patients were assessed by an orthoptist on admission.
Results: 125 patients’ records were reviewed. 91% of patients were assessed by an orthoptist at presentation. 60% of patients presented with leukocoria only, whilst 13% had strabismus only, and 12% presented with LS together. 10% presented with inflammatory signs. 78% of patients with strabismus as a feature of their presentation (LS or S) were enucleated, compared to 50% who presented with L only (P = 0.005). 10 out of 11 children who presented with inflammation required enuncleation, and 50% underwent adjuvant chemotherapy, significantly more than if inflammation was not present (p = 0.005). 63% of the strabismic patients had exotropia, 20% had esotropia, and 17% had variable exotropia/esotropia.
Discussion: Strabismus and inflammation are prognosticators of advanced disease. Exotropia is more common than esotropia, at a much higher ratio than previously demonstrated. In patients with LS, the parents may be unaware of the strabismic component.
Conclusion: An accurate assessment of strabismus at presentation may be a prognostic indicator in patients with RB.
References: 1. Abramson DH et al. Screening for Retinoblastoma: Presenting signs as prognosticators of patient and ocular survival. Paediatrics. 2003; 112: 1248-1255.
2. Abramson et al. Presenting signs of retinoblastoma. J Paediatrics. 1998; 132 (3 pt 1): 505-8.
3. Goddard AG, Kingston JE, Hungerford JL. Delay in diagnosis of retinoblastoma: risk factors and treatment outcome. Br J Ophthalmol. 1999; 83 (12): 1320-3.