Publications by authors named "Ernesto Alemany-Rubio"

4 Publications

  • Page 1 of 1

Global Retinoblastoma Presentation and Analysis by National Income Level.

JAMA Oncol 2020 05;6(5):685-695

Imam Hussein Cancer Center, Karbala, Iraq.

Importance: Early diagnosis of retinoblastoma, the most common intraocular cancer, can save both a child's life and vision. However, anecdotal evidence suggests that many children across the world are diagnosed late. To our knowledge, the clinical presentation of retinoblastoma has never been assessed on a global scale.

Objectives: To report the retinoblastoma stage at diagnosis in patients across the world during a single year, to investigate associations between clinical variables and national income level, and to investigate risk factors for advanced disease at diagnosis.

Design, Setting, And Participants: A total of 278 retinoblastoma treatment centers were recruited from June 2017 through December 2018 to participate in a cross-sectional analysis of treatment-naive patients with retinoblastoma who were diagnosed in 2017.

Main Outcomes And Measures: Age at presentation, proportion of familial history of retinoblastoma, and tumor stage and metastasis.

Results: The cohort included 4351 new patients from 153 countries; the median age at diagnosis was 30.5 (interquartile range, 18.3-45.9) months, and 1976 patients (45.4%) were female. Most patients (n = 3685 [84.7%]) were from low- and middle-income countries (LMICs). Globally, the most common indication for referral was leukocoria (n = 2638 [62.8%]), followed by strabismus (n = 429 [10.2%]) and proptosis (n = 309 [7.4%]). Patients from high-income countries (HICs) were diagnosed at a median age of 14.1 months, with 656 of 666 (98.5%) patients having intraocular retinoblastoma and 2 (0.3%) having metastasis. Patients from low-income countries were diagnosed at a median age of 30.5 months, with 256 of 521 (49.1%) having extraocular retinoblastoma and 94 of 498 (18.9%) having metastasis. Lower national income level was associated with older presentation age, higher proportion of locally advanced disease and distant metastasis, and smaller proportion of familial history of retinoblastoma. Advanced disease at diagnosis was more common in LMICs even after adjusting for age (odds ratio for low-income countries vs upper-middle-income countries and HICs, 17.92 [95% CI, 12.94-24.80], and for lower-middle-income countries vs upper-middle-income countries and HICs, 5.74 [95% CI, 4.30-7.68]).

Conclusions And Relevance: This study is estimated to have included more than half of all new retinoblastoma cases worldwide in 2017. Children from LMICs, where the main global retinoblastoma burden lies, presented at an older age with more advanced disease and demonstrated a smaller proportion of familial history of retinoblastoma, likely because many do not reach a childbearing age. Given that retinoblastoma is curable, these data are concerning and mandate intervention at national and international levels. Further studies are needed to investigate factors, other than age at presentation, that may be associated with advanced disease in LMICs.
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May 2020

Confocal scanning laser ophthalmoscope in the retromode imaging modality in exudative age-related macular degeneration.

Graefes Arch Clin Exp Ophthalmol 2013 Jan 29;251(1):27-34. Epub 2012 Apr 29.

Department of Ophthalmology, University of Padova, via Giustiniani 2, 35128 Padova, Italy.

Purpose: To evaluate the ability of confocal scanning laser ophthalmoscope (cSLO) in the retromode imaging modality in detecting retinal changes secondary to exudative age-related macular degeneration (AMD).

Methods: Seventeen eyes of 13 consecutive patients affected by CNV secondary to AMD were evaluated with optical coherence tomography (OCT) to detect neuroretinal detachment (NRD), pigment epithelial detachment (PED), cystoid macular edema (CME), and epiretinal membranes (ERM). All eyes were examined with a cSLO equipped with infrared retromode (RM) imaging modality. Infrared and fundus autofluorescence images were also obtained (IR and FAF). The intermethod agreement between OCT and cSLO was evaluated considering single cSLO imaging modality separately (IR, FAF, and RM), and all imaging modalities together.

Results: Eight eyes (47 %) had NRD at OCT; intermethod agreement was poor for any single cSLO imaging modality considered separately (k: 0.14, 0.01, and 0.29 for cSLO IR, FAF, and RM, respectively). Four eyes had PED at OCT (24 %); intermethod agreement was mild for cSLO RM, poor for IR and FAF (k: 0.51, 0.16, and 0.00, respectively). CME was present in eight eyes (47 %); intermethod agreement was excellent for cSLO RM, poor for IR and FAF (k: 0.88, 0.38, and 0.26, respectively). ERM was present in three eyes (18 %); intermethod agreement was mild for cSLO IR, poor for FAF, and excellent for RM (k: 0.59, 0.00, and 0.76, respectively).

Conclusions: cSLO RM imaging is a useful and reproducible technique in detecting retinal features associated with CNV, particularly CME.
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January 2013

Short wavelength fundus autofluorescence versus near-infrared fundus autofluorescence, with microperimetric correspondence, in patients with geographic atrophy due to age-related macular degeneration.

Br J Ophthalmol 2011 Aug 24;95(8):1140-4. Epub 2010 Oct 24.

Department of Ophthalmology, University of Padua, via Giustiniani 2, Padua 35128, Italy.

Aim: To compare standard short-wavelength fundus autofluorescence (SW-FAF) and near infrared-wavelength fundus autofluorescence (NIR-FAF) in detecting geographic atrophy (GA) secondary to age-related macular degeneration, and its retinal sensitivity impairment.

Methods: Twenty-five consecutive patients (36 eyes) affected by GA were studied by means of fundus autofluorescence imaging, using both SW-FAF (excitation: 488 nm, emission >500 nm) and NIR-FAF (excitation: 787 nm, emission >800 nm). All patients underwent microperimetry to assess fixation characteristics and retinal sensitivity.

Results: In the extrafoveal region, the total hypoautofluorescent (hypo-FAF) area was significantly wider with NIR-FAF than with SW-FAF (8.03±6.68 mm(2) vs 7.37±6.34 mm(2) respectively; p=0.005). In the foveal area, the total hypo-FAF area was smaller with NIR-FAF than with SW-FAF (0.19±0.03 mm(2) versus 0.42±0.12 mm(2) respectively; p=0.008). Foveal sparing was larger at NIR-FAF compared with SW-FAF (p=0.021). In nine cases (25%) the site of fixation was hypoautofluorescent on SW-FAF, but normal on NIR-FAF with preserved retinal sensitivity.

Conclusions: Standard SW-FAF may overestimate GA in the foveal area, correctly detected by NIR-FAF. In the extrafoveal area, SW-FAF may underestimate GA. Standard SW-FAF should be integrated with NIR FAF when detecting and following GA to avoid inconsistent results and misinterpretation, from both a morphological and functional perspective. Microperimetry helps to quantify retinal sensitivity in GA.
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August 2011

Topical 1% 5-fluorouracil in ocular surface squamous neoplasia: a long-term safety study.

Br J Ophthalmol 2011 Mar 7;95(3):355-9. Epub 2010 Aug 7.

Department of Ophthalmology, University of Padua, Via Giustiniani 2, Padua 35128, Italy.

Background/aims: The aim of this study was to evaluate the long-term corneal toxicity of topical chemotherapy with 1% 5-fluorouracil (5-FU) as a sole or adjuvant treatment of ocular surface squamous neoplasia (OSSN).

Methods: Forty-one consecutive cases of OSSN were included in this prospective study. Patients underwent topical chemotherapy with 1% 5-FU four times/day for 4 weeks (one course). Adjunctive courses were repeated until clinical and cytological tumour regression. Clinical confocal microscopy was used to check for 5-FU long-term corneal toxicity.

Results: Mean follow-up was 89.7±14.4 months (range 63-122 months). Twenty-two patients (53.7%) underwent topical 5-FU as a sole treatment, and 19 patients (46.3%) as adjuvant and/or debulking therapy. The mean number of 5-FU cycles was 1.9 (range 1-5 cycles). Three tumours (7.3%) treated with 5-FU alone recurred during follow-up. Recurrences were successfully treated with additional 5-FU courses. Clinical confocal microscopy showed no long-term difference between the treated eye and fellow (control) eye in: endothelial cells count, pleomorphism and polymegatism, anterior stromal keratocyte density, sub-basal nerve plexus fibre number, density, and beadings and central cornea epithelium thickness (p=NS).

Conclusion: Topical 5-FU, as a sole or combined therapy, must be considered a long-term safe and effective treatment for patients affected by OSSN.
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March 2011