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Jelena Potic, Maja Beck Popovic, Marie-Claire Gaillard, Aubin Balmer, Francis L Munier, patients treated for bilateral sporadic retinoblastoma; SPONTANEOUS RESOLUTION OF MACULAR PUCKER IN TWO PATIENTS WITH RETINOBLASTOMA. Invest. Ophthalmol. Vis. Sci. 2014;55(13):3082.
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We report on two patients treated for bilateral sporadic retinoblastoma (RB), who presented a macular pucker (MP) that resolved spontaneously.
Fundus imaging was performed under anesthesia including RetCam photography, optical coherence tomography (OCT) (Bioptigen), B-scan ultrasonography (OtiScan), and fluorescein angiography.
Patient 1 presented with group D and group A disease at the age of 5 months. Patient 2 was diagnosed group C and group D disease at 15 months of age. Following neoadjuvant systemic chemotherapy, secondary enucleation was performed for the group D eye of both patients in order to prevent optic nerve invasion by the residual tumors and to avoid salvage treatment by conformal stereotactic radiotherapy of the posterior pole. The follow eye of both patients was characterized by a temporally located para-macular tumor,2.5 papillary diameters away from the fovea. In patient 1, tumor control (type IV regression) was achieved by combining transpupillary diode-mediated hyperthermia and argon laser photocoagulation.Five months after completion of the last treatment, thickening (970 micrometers) of the foveal area was observed secondary to the contraction of a MP. This lesion spontaneously resolved 24 months later, with flattening of the macula area as documented by OCT. At 6 years of age his visual acuity was 0.8. In patient 2, the paramacular lesion was treated by chemohyperthermia and hyperthermia resulting in a flat scar. Five months later, the OCT showed the formation of a MP creating a retinal fold across the fovea. Spontaneous resolution of the MP could be observed over the next 18 months,with restoration of the macular anatomy as shown by OCT. At last visit, visual acuity was 0.63, which is within the normal range for his age (2 years and 10 months).
Although indirect ophthalmoscopy remains the gold standard for the management of RB, OCT imaging has improved the sensitivity of detecting tumour recurrences and complications especially at the posterior pole. The threshold for macular pucker formation appears to be low in case of tumors located temporally to the fovea and aligned with the papillo-macular bundle. To our knowledge, this is the first time that spontaneous resolution of a macular pucker is documented in retinoblastoma patients. The regression occurred over a 18 to 24 months period, stressing the need to delay the indication to vitrectomy.
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