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ABSTRACT
Introduction. La mortalité liée au rétinoblastome demeure élevée dans les pays à faibles revenus. L’objectif de notre travail était de décrire l’itinéraire clinique des enfants atteints de rétinoblastome au Gabon, afin d’en dégager les différents obstacles. Méthodes. Il s’agissait d’une étude rétrospective et descriptive sur les dossiers médicaux des cas de rétinoblastome entre 2016 et 2020 à l’institut de cancérologie de Libreville. Résultats. L’effectif était de 4 filles et 2 garçons. La moyenne d’âge était de 9,83 mois au moment de la constatation des premiers symptômes et de 41,33 mois au moment du diagnostic. Les premiers symptômes constatés étaient la leucocorie (3 patients) et le strabisme (3 patients), négligés par l’entourage familial de 3 enfants. Chez trois enfants, le diagnostic de cataracte congénital avait initialement été évoqué. Trois enfants n’avaient pas été référés vers un centre de diagnostic. Le délai moyen entre le premier symptôme et le diagnostic était de 31,5 mois. Les distances parcourues entre le lieu de résidence et celui de la prise en charge allaient de 533 à 4 963 kilomètres. Dans cette population, aucun patient n’avait survécu. Conclusion. La méconnaissance des symptômes inauguraux du rétinoblastome, l’éloignement des centres de diagnostic et de traitement et le manque de qualification du personnel médical, sont autant de facteurs qui ont retardé la prise en charge.
RÉSUMÉ
Introduction. Retinoblastoma-related mortality remains high in low-income countries. The objective of our work was to describe the clinical itinerary of children with retinoblastoma in Gabon, in order to identify the various obstacles. Methods. This was a retrospective and descriptive study on the medical records of retinoblastoma cases between 2016 and 2020 at the Libreville Cancer Institute. Results. The enrolment was 4 girls and 2 boys. The mean age was 9.83 months at the time of first symptoms and 41.33 months at diagnosis. The first symptoms observed were leucocoria (3 patients) and strabismus (3 patients), neglected by the family entourage of 3 children. In three children, the diagnosis of congenital cataract was initially mentioned. Three children had not been referred to a diagnostic centre. The mean time from first symptom to diagnosis was 31.5 months. The distances travelled between the place of residence and that of care ranged from 533 to 4,963 kilometres. In this population, no patients had survived. Conclusion. The lack of knowledge of the inaugural symptoms of retinoblastoma, the distance from diagnosis and treatment centers and the lack of qualification of medical staff, are all factors that have delayed management.
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References
- Dimaras H, Kimani K, Dimba EA, Gronsdahl P, White A, Chan HS, Gallie BL. Retinoblastoma. Lancet 2012 ; 379 : 1436 – 46.
- Gauthier-Villars M, Stoppa Lyonnet D. Les prédispositions génétiques aux cancers de l’enfant en 2011. Bull Cancer 2011; 98(5): 459 – 475.
- Ali MJ, Parsam VL, Honavar SG, Kannabiran C, Vemuganti GK, Reddy VA. RB1 gene mutations in retinoblastoma and its clinical correlation. Saudi J Ophthalmol 2010; 24(4):119 – 23.
- Al-Nawaiseh I, Ghanem AQ, Yousef YA. Familial retinoblastoma: Raised awareness improves early diagnosis and autcome. Hindawi Jr ophtalmol 2017; 5p
- Canadian retinoblastoma society. National retinoblastoma strategy Canadian guidelines for care. Can J Ophtalmol 2009 ; 44 (2) : S1 – 88.
- Lacour B, Guyot-Goubin A, Guissou S, Bellec S, Desandes E, Clavel J. Incidence of childhood cancer in France: National Children Cancer Registries, 2000-2004. Eur J Cancer Prev. 2010;19(3) :173-81.
- Abba Kaka HY, Sylla F, Ali MH, Amza A. Les particularités du rétinoblastome au Niger. Eur Sci J. 2016; 12(3): 84 – 91.
- Epée E, Moukouri E, Koki G, Pondy A, Mbassi K. Clinical features and prognosis of retinoblastoma at the University Teaching Hospital of Yaounde Cameroon. Health Sci Dis 2014; 15(3): 1 – 6.
- Lau CSL, Choy KW, Fan DSP, BO Yu C, Wong CY, Lam DSC, Pang CP. Prenatal screening for retinoblastoma in Hong Kong. Hong Kong Med J 2008:14(5): 391 – 394.
- Rotter T, Kinsman L, James E, et al. Clinical pathways: Effects on professional practice, patient outcomes, length of stay and hospital costs. Cochrane Database of Systematic Reviews 2010 ; 6(3) : 2104.
- Lumbroso-Le Rouic L, Savignoni A, Levy-Gabriel C, Aerts I, Cassoux N, Salviat F et al. Treatment of retinoblastoma: The Institute Curie experience on a series of 730 patients (1995 to 2009). J Fr Ophtalmol 2015 ; 38(6) : 535 – 41.
- Aziz HA, Lasenna CE, Vidoga M, Fernandes C, Feuer W, Aziz-Sultan MA, Murray TG. Retinoblastoma treatment burden and economic cost: impact of age at diagnosis and selection of primary therapy. Clin Ophthalmol 2012; 6: 1601 – 6.
- Traore F, Togo B, Sylla F, Cheick TB, Diakité AA, Dicko-Traore F et al. Retinoblastoma: inventory in Mali and program to develop early diagnosis, treatments and rehabilitation. Bull Cancer 2013; 100(2):161 – 5.
- Lukamba RM, Yao JJA, Kabesha TA, Budiongo AN, Monga BB, Mwembo AT et al. Retinoblastoma in Sub-Saharan Africa: Case Studies of the Republic of Côte d’Ivoire and the Democratic Republic of the Congo. JGO 2018; 4:1-8.
- Abramson DH, Beaverson K, Sangani P, Vora RA, Lee TC, Hochberg HM, Kirszrot J, Ranjithan M. Screening for retinoblastoma: presenting signs as prognosticators of patient and ocular survival. Pediatrics 2003; 112 (6 Pt 1):1248 – 1255.
- Kagmeni G, Nguefack F, Monebenimp F, Kouogang G, Ngounou F, Kengne Kamga S, Moukouri E. Le Rétinoblastomedans la Région de l’Ouest Cameroun:Aspects Cliniques, Histologiques et Thérapeutiques. Health Sci. Dis 2013; 14(2) : 1 – 4.
- Leander C, Fu LC, Pena A, Howard SC, Rodriguez-Galindo C, Wilimas JA, Ribeiro RC, Haik B. Impact of an education program on late diagnosis of retinoblastoma in Honduras. Pediatr Blood Cancer 2007; 49(6): 817 – 819.
- Qaiser S, Limo A, Gichana J, Kimani K, Githanga J, Waweru W, Dimba EAO, Dimaras H. Design and Implementation of the Retinoblastoma Collaborative Laboratory. Cul Oncol Pathol 2017; 3:73–82.
- Skalet AH , Gombos DS, Gallie BL , Kim JW, Shields CL , Marr BP , Plon SE , Chévez-Barrios P. Screening Children at Risk for Retinoblastoma: Consensus Report From the American Association of Ophthalmic Oncologists and Pathologists. Ophthalmology 2018; 125(3):453-458.
- Ashwin Reddy, Kahaki Kimani. A national retinoblastoma network: experiences in Kenya and the UK. Community Eye Health J 2018; 31(101): 5-6.
- Hill JA, Kimani K, White A, Barasa F, Livingstone M, Gallie BL, Dimaras H et al. Achieving optimal cancer outcomes in East Africa through multidisciplinary partnership: a case study of the Kenyan National Retinoblastoma Strategy group. Global Health 2016; 12(23): 7p
- El Kettani A, Aderdour S, Daghouj G, Knari S, Zaghloul K, Zafad S, HarifEl Kettani M. Rétinoblastome : résultats préliminaires du protocole national de prise en charge. J Fr Ophtalmol 2014 ; 37(2) :115 – 124.
References
Dimaras H, Kimani K, Dimba EA, Gronsdahl P, White A, Chan HS, Gallie BL. Retinoblastoma. Lancet 2012 ; 379 : 1436 – 46.
Gauthier-Villars M, Stoppa Lyonnet D. Les prédispositions génétiques aux cancers de l’enfant en 2011. Bull Cancer 2011; 98(5): 459 – 475.
Ali MJ, Parsam VL, Honavar SG, Kannabiran C, Vemuganti GK, Reddy VA. RB1 gene mutations in retinoblastoma and its clinical correlation. Saudi J Ophthalmol 2010; 24(4):119 – 23.
Al-Nawaiseh I, Ghanem AQ, Yousef YA. Familial retinoblastoma: Raised awareness improves early diagnosis and autcome. Hindawi Jr ophtalmol 2017; 5p
Canadian retinoblastoma society. National retinoblastoma strategy Canadian guidelines for care. Can J Ophtalmol 2009 ; 44 (2) : S1 – 88.
Lacour B, Guyot-Goubin A, Guissou S, Bellec S, Desandes E, Clavel J. Incidence of childhood cancer in France: National Children Cancer Registries, 2000-2004. Eur J Cancer Prev. 2010;19(3) :173-81.
Abba Kaka HY, Sylla F, Ali MH, Amza A. Les particularités du rétinoblastome au Niger. Eur Sci J. 2016; 12(3): 84 – 91.
Epée E, Moukouri E, Koki G, Pondy A, Mbassi K. Clinical features and prognosis of retinoblastoma at the University Teaching Hospital of Yaounde Cameroon. Health Sci Dis 2014; 15(3): 1 – 6.
Lau CSL, Choy KW, Fan DSP, BO Yu C, Wong CY, Lam DSC, Pang CP. Prenatal screening for retinoblastoma in Hong Kong. Hong Kong Med J 2008:14(5): 391 – 394.
Rotter T, Kinsman L, James E, et al. Clinical pathways: Effects on professional practice, patient outcomes, length of stay and hospital costs. Cochrane Database of Systematic Reviews 2010 ; 6(3) : 2104.
Lumbroso-Le Rouic L, Savignoni A, Levy-Gabriel C, Aerts I, Cassoux N, Salviat F et al. Treatment of retinoblastoma: The Institute Curie experience on a series of 730 patients (1995 to 2009). J Fr Ophtalmol 2015 ; 38(6) : 535 – 41.
Aziz HA, Lasenna CE, Vidoga M, Fernandes C, Feuer W, Aziz-Sultan MA, Murray TG. Retinoblastoma treatment burden and economic cost: impact of age at diagnosis and selection of primary therapy. Clin Ophthalmol 2012; 6: 1601 – 6.
Traore F, Togo B, Sylla F, Cheick TB, Diakité AA, Dicko-Traore F et al. Retinoblastoma: inventory in Mali and program to develop early diagnosis, treatments and rehabilitation. Bull Cancer 2013; 100(2):161 – 5.
Lukamba RM, Yao JJA, Kabesha TA, Budiongo AN, Monga BB, Mwembo AT et al. Retinoblastoma in Sub-Saharan Africa: Case Studies of the Republic of Côte d’Ivoire and the Democratic Republic of the Congo. JGO 2018; 4:1-8.
Abramson DH, Beaverson K, Sangani P, Vora RA, Lee TC, Hochberg HM, Kirszrot J, Ranjithan M. Screening for retinoblastoma: presenting signs as prognosticators of patient and ocular survival. Pediatrics 2003; 112 (6 Pt 1):1248 – 1255.
Kagmeni G, Nguefack F, Monebenimp F, Kouogang G, Ngounou F, Kengne Kamga S, Moukouri E. Le Rétinoblastomedans la Région de l’Ouest Cameroun:Aspects Cliniques, Histologiques et Thérapeutiques. Health Sci. Dis 2013; 14(2) : 1 – 4.
Leander C, Fu LC, Pena A, Howard SC, Rodriguez-Galindo C, Wilimas JA, Ribeiro RC, Haik B. Impact of an education program on late diagnosis of retinoblastoma in Honduras. Pediatr Blood Cancer 2007; 49(6): 817 – 819.
Qaiser S, Limo A, Gichana J, Kimani K, Githanga J, Waweru W, Dimba EAO, Dimaras H. Design and Implementation of the Retinoblastoma Collaborative Laboratory. Cul Oncol Pathol 2017; 3:73–82.
Skalet AH , Gombos DS, Gallie BL , Kim JW, Shields CL , Marr BP , Plon SE , Chévez-Barrios P. Screening Children at Risk for Retinoblastoma: Consensus Report From the American Association of Ophthalmic Oncologists and Pathologists. Ophthalmology 2018; 125(3):453-458.
Ashwin Reddy, Kahaki Kimani. A national retinoblastoma network: experiences in Kenya and the UK. Community Eye Health J 2018; 31(101): 5-6.
Hill JA, Kimani K, White A, Barasa F, Livingstone M, Gallie BL, Dimaras H et al. Achieving optimal cancer outcomes in East Africa through multidisciplinary partnership: a case study of the Kenyan National Retinoblastoma Strategy group. Global Health 2016; 12(23): 7p
El Kettani A, Aderdour S, Daghouj G, Knari S, Zaghloul K, Zafad S, HarifEl Kettani M. Rétinoblastome : résultats préliminaires du protocole national de prise en charge. J Fr Ophtalmol 2014 ; 37(2) :115 – 124.