Author, year
Country
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Type of Study
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Population
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Intervention
|
Results
|
Public assistance |
|
|
|
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Hill, 2016
Kenya
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Cross-sectional study.
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Mean of ~ 60 delegates per meeting.
|
A nationally coordinated multidisciplinary (health workers, academics,
retinoblastoma survivors and families, members of government) drawing
input and expertise of professionals to optimize the cure of children
with retinoblastoma. The program was based on the following principles:
(1) Set the agenda together; (2) Interact with stakeholders; (3) Clarify
responsibilities; (4) Account to beneficiaries; (5) Promote mutual
learning; (6) Enhance capacities; (7) Share data and networks; (8)
Disseminate results; (9) Pool profits and merits; (10) Apply results;
(11) Secure outcomes.
|
The program fulfilled 9/11 of its objectives, with the last two
(applying results and securing outcomes) in progress.
They produced consensus national guidelines for care and supported
capacity-building initiatives to facilitate their implementation.
Outcomes of children with retinoblastoma will be evaluated in the long
term.
|
Surveillance |
|
|
|
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Ademola-Popoola, 2017
Nigeria
|
Cross-sectional study.
|
12 (2 RB cases).
|
Retinal imaging from a smartphone compared to traditional
fundoscopy.
|
In cases of retinoblastoma with a highly reflective tumor mass, the
images were not as clear as with a traditional fundus camera.
|
Bernard, 2022
Ethiopia
|
Cross-sectional study.
|
1200 individuals screened.
|
The EyeScreen® software, a smartphone application designed for use with
Android® devices, as an effective, easy-to-perform, community-based
screening tool.
|
Photographs obtained with inexpensive Android smartphones running the
EyeScreen® application were used to train an ImageNet (ResNet®) machine
learning model and to measure the performance of the app. Eighty percent
of the images were used in training the model, and 20% were reserved
for testing. The model showed a sensitivity of 87% and a specificity of
73%.
|
Kaliki, 2019
India
|
Prospective nonrandomized observational/
interventional case series.
|
262 parents and 23 siblings of 131 patients.
|
Routine ophthalmic examination of families (parents and siblings) in a
setting of the absence of genetic testing.
|
Spontaneously regressed RB in 8% of parents and active RB in 2% of
siblings indicate that routine fundus screening of siblings allows for
early detection of RB in otherwise asymptomatic children and detection
of spontaneously regressed RB in parents may act as a surrogate marker
for germline RB1 mutation.
|
Khedekar, 2019
India
|
Cross-sectional study.
|
34 eyes of 23 RB patients and 4 controls.
|
Two iPhone® apps (MDEyeCare® and CRADLE®) with modifications for early
detection of RB without anesthesia or pharmacological dilatation of the
pupil.
|
The modified MDEyeCare® app could detect the leukocoria in the early
stages of RB (50% of Group B, 83% of Group C, but none of Group A). In
the late stages (Group D and E), 100% of tumors were detected. The
CRADLE® app failed to provide adequate leukocoria detection except for
four late-stage RB eyes.
|
Vargas-Cuentas, 2019
Peru
|
Cross-sectional study.
|
35 participants:
15 RB cases
20 controls.
|
The use of a mobile app (Retino App®) associated with an algorithm for
imaging processing for the detection of RB.
|
The system correctly identified 93.33% of the cases and 95% of the
controls.
|
Yousef, 2020
Jordan
|
Retrospective, clinical case series.
|
32 families, 76 children.
34 presented signs of RB;
42 were enrolled in the screening program.
|
Screening routine with examination under anesthesia and non-sedated
exams. The frequency of evaluations was scheduled based on a clinical
assessment of pre-test risk for relatives to carry the mutant RB1
allele, in the absence of genetic testing.
|
Out of the 76 children enrolled, 46 children were diagnosed with Rb (12
by screening and 34 had signs of Rb). Patients diagnosed by screening
were younger (mean: 2.4 months vs 15.8 months), had significantly
earlier tumor stage at diagnosis (p = .0001), had higher eye salvage
rate (p = .0001), less need for systemic chemotherapy (p = .022), and
better visual outcome (p = .0017). None of the eyes were group D or E,
enucleated or irradiated. Six patients were cured without chemotherapy,
and the visual acuity was 0.5 or better in 55% of eyes.
|
International partnership |
|
|
|
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Al-Haddad, 2019
Lebanon
|
Retrospective case series.
|
40 (after) and 20 (before) RB cases.
|
The formalization of a multidisciplinary RB program (monthly meetings,
centralization of care, clinical nurse) with associated financial
support.
|
Reduced enucleation after the institution of the program (5% after
versus 13% before); increased number of diagnostics (52 after versus 20
before); increased reception of patients from neighboring countries.
However, even within a multidisciplinary setting, most patients with
retinoblastoma still presented with advanced intraocular disease, and
eye salvage rates were poor for patients with Group D and E
tumors.
|
Chantada, 2016
Argentina
|
Public policy study.
|
(mean/year)
1987-1994: 18.
1995-2002: 35.
2003-2008: 30.
2009-2015: 36
2016-2020: 40 (estimated).
|
20-year cooperation between Garrahan Hospital in Argentina with Memorial
Sloan Kettering Cancer Center in New York. The program initially
concentrated on medical education and facilitated the research and
treatment protocol for retinoblastoma in Latin America, incorporation of
the first center capable of performing intra-arterial
chemotherapy.
|
Improvement in the survival and ocular preservation of patients (3y
probability of disease-free survival from 0.84 to 0.97), centralization
of care, and participation in clinical studies and publications of the
group.
|
Howard, 2018
10 index countries (Venezuela, Honduras, Ukraine, Egypt,
Morocco, Senegal, Tanzania, Philippines, Vietnam, Bangladesh). 32
additional countries.
|
Public policy study.
|
201 RB cases.
|
The My Child Matters program supported diverse projects. Among them, the
African Retinoblastoma Network, which had as its goals to raise
awareness about retinoblastoma and childhood cancers with public
campaigns to promote early diagnosis; to train oncologists and
ophthalmologists to manage retinoblastoma; to create infrastructure and
expertise for vision conservation in children diagnosed at early stages;
to provide ocular prostheses, rehabilitation, and psychosocial support
to patients with advanced disease.
|
Decrease in patients presenting with extraocular disease from 17 (65%)
of 26 in 2011 to 19 (42%) of 45 in 2016; 1-year survival for patients
with the unilateral intraocular disease increased from 3 (43%) of 7 in
2011 to 10 (71%) of 14 in 2016; 1-year survival for patients with
bilateral intraocular disease increased from 0 (0%) of 2 in 2011 to 5
(42%) of 12 in 2016; eye preservation for patients increased from 0
(0%) of 4 in 2011 to 5 (21%) of 24 in 2016.
|
Qaddoumi, 2008
Jordan
|
Prospective cohort.
|
36 patients, 58 eyes.
|
A collaborative RB program was established with the International
Outreach Program at St. Jude Children’s Research Hospital in Memphis,
Tennessee. Mentoring included Internet consultations, videoconferences,
and exchange visits.
|
Twinning has positively impacted survival and ocular salvage in Jordan.
Thirty-three children with retinoblastoma (20 bilateral) were treated. A
total of 66 consultations with 29 patients were analyzed. New cases were
compared to previously treated cases. The success of the program was
evident in bilateral cases, with low enucleation (25%) and irradiation
rates (17%).
|
Wilimas, 2009
Central America (Guatemala, Honduras, El Salvador)
|
Public policy study.
|
196 (after) and 167 (before).
|
A RB program focused on developing early diagnosis strategies, treatment
protocols suited to local conditions, building local networks of
oncologists and ophthalmologists, training local healthcare providers,
using the modern donated equipment for diagnosis and treatment, and
teleconsultation to further education and share expertise.
|
Patients abandoning/refusing treatment decreased in Guatemala from 21%
to 11% and in Honduras from 35% to 19%.
|
Yousef, 2020b
Jordan
|
Cross-sectional study.
|
478 patients
813 eyes.
|
Implementing a telemedicine-based program with St. Jude Children’s
Research Hospital compared to before implementation.
|
After the program implementation, the mortality rate decreased from 38%
to 5% (P < 0.0001), and the overall eye salvage rate
increased from 4% to 61% (98% for group A, 93% for group B, 81% for
group C, and 48% for group D; P < 0.0001). Initially, all
cases were discussed via telemedicine, but as knowledge transfer
increased, the proportion of cases that required discussion decreased to
less than 3% 10 years later. Similarly, treatment changes based on
consultations decreased from 70% to 7% after 10 years. Both survival
and eye salvage rates were comparable at the early and later stages of
implementing the twinning program.
|
Education |
|
|
|
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Al-Nawaiseh, 2017
Jordan
|
Retrospective, clinical case series.
|
Seventy six eyes of 44 consecutive familial RB.
|
Screening for RB and raising awareness in non-probands versus
probands.
|
The eye salvage rate was significantly higher in the non-probands than
in the probands in this series (p = 0 002). Patients diagnosed by
screening (38%) had excellent visual outcomes, and both eyes were
saved. The authors concluded that awareness of families of the
possibility of retinoblastoma and adequate screening led to a
significantly higher rate of eye salvage in patients with familial
retinoblastoma.
|
Antoneli, 2004
Brazil
|
Cross-sectional study.
|
105 patients (before the educational program) and 152 (after).
|
Educational campaigns: articles in non-medical magazines and billboards
placed at strategic points in the city. The medical population received
training through courses and publications in a medical journal.
|
Patients who were referred within a period of less than 6 months had a
higher frequency of intraocular disease when compared to patients with a
referral time longer than six months (75% vs. 25% p< .001).
There was no statistically significant difference for extraocular
tumors. The mean referral time was 7.5 months (SD 7.79) before the
educational program versus 5.3 months (SD 6.84) after the
intervention.
|
Elfalah, 2022
Jordan
|
Cross-sectional study.
|
289 medical students.
|
Implementing modifications on teaching curriculum of medical students on
ophthalmology rotation that focuses on the red flags of RB.
|
Most participants considered leukocoria an abnormal sign. Medical
students of the control group had significantly lower knowledge about
the diagnosis of RB (p = .0001), while the intervention group scored
higher in tests about critical questions, such as knowing that RB is a
fatal disease (p = .041) that needs urgent treatment (p = .042). Only
2% of students adopted the “watch and wait” strategy in the
intervention group, compared to 12% in the control group. Proficiency
score in the test (≥ 90%) was achieved by 8% of students in the
intervention group versus 2% in the control group. In the intervention
group 27% of students, compared to 65% of students in the control
group, failed to obtain a sufficiency score (≥70%) in the
questionnaire.
|
Hill, 2015
Kenya
|
Cross-sectional study.
|
38 health workers.
|
A workshop for RB genetics education. Attendees: ophthalmologists,
pathologists, oncologists, ophthalmic clinical officers, and
nurses.
|
Knowledge increased significantly post-workshop, driven by increased
knowledge of RB causative genetics. One-year post-workshop, participant
knowledge had returned to baseline, indicating that knowledge retention
requires more frequent reinforcement. Participants reported feeling more
confident discussing genetics with patients and had integrated more
genetic counseling into patient interactions.
|
Leander, 2007
Honduras
|
Cross-sectional study.
|
23 patients diagnosed after the campaign
59 patients (before).
|
RB education program linked to a national vaccination campaign in
Honduras. Posters and flyers accessible to poorly educated readers, to
convey the severity of RB, and to provide contact information.
|
Extraocular disease at diagnosis was 73% before versus 35% after the
campaign (p = .002). Leukocoria was the presenting symptom in 54% of
patients before versus 83% after the campaign (p = .02). The median age
at diagnosis and the median time between the first sign or symptom and
diagnosis were 3.8 (p = .26) and 1.7 (p = .6) months after the
campaign.
|
Genetic counseling |
|
|
|
|
Joseph, 2004
India
|
Cross-sectional study.
|
25 patients.
|
Presymptomatic genetic screening (RB1) compared to the clinical
screening of RB proband and relatives.
|
The cost of genetic testing and clinical examination for a proband was
$US152.27 and 535.60, respectively, while for the nuclear family, it
was $US174.51 and $US1071.20, respectively.
|
Joseph, 2006
India
|
Diagnostic model proposal.
|
-
|
An efficient diagnostic model with a logical and practical flow of
various genetics tests (karyotyping, loss of heterozygosity analysis,
molecular deletion, linkage analysis (familial cases), mutation
screening of - CGA exons first and then non-CGA exons, methylation
screening of RB1 and essential promoter regions screening in a
laboratory) to reduce the overall health care costs.
|
In the proposed model, LOH analysis costs around US$ 9.72 (5.3%), RB1
gene mutational screening US$ 146.46 (79.88%), methylation analysis
US$ 3.67 (2.0%), and cytogenetic analysis US$ 22.37 (12.8%).
Assuming the patient did not exit until the last step in the genetic
testing algorithm and did the entire spectrum of the tests with some of
them being carried out simultaneously, about 84 days are required to
exit from the diagnostic model.
|
Thirumalairaj, 2015
India
|
Cross-sectional study.
|
21 patients (13 bilateral and 8 unilateral RB).
|
Rapid genetic screening strategy by prioritizing the order of exons to
be analyzed, based on the frequency of nonsense mutations, deletions,
and duplications reported in the RB1-Leiden Open Variation Database and
published literature on Indian patients.
|
Mutations were identified in 76% of patients in half the usual time and
one-third of the cost.
|