Orbital Retinoblastoma KNH Experience DR Nyenze E M
Orbital Retinoblastoma, KNH Experience DR Nyenze E. M University of Nairobi
Orbital retinoblastoma, frequency 1 Malawi 44. 1% 1 Congo 28%2 Kenya 37%3, 18%4, 42%5 Tanzania 30%6 Nyaka AS, Kimani K, Kollmann MK, The pattern of Retinoblastoma at Queen Elizabeth Central Hospital, Malawi. University of Nairobi. M. Med Ophthalmology Thesis, 2010 2 Kaimbo WA, Kaimbo D, Mvitu MN, Missotten L. Presenting signs of retinoblastoma in Congolese Patients. Bull Soc Belge Ophthalmol 2002; 283: 37 -41 3. Nyawira G, Kahaki K, Kariuki M. Survival among Retinoblastoma patients at KNH, Kenya. Journal of Ophthalmology of Eastern Central and Southern Africa August 2013; 17(1): 15 -19 4. Nyamori JM, Kimani K, Njuguna MW, Dimaras H. The incidence and distribution of retinoblastoma in Kenya. no. 1; pp. 141– 3, s. l. : The British Journal of Ophthalmology, Jan 2012, Vols. vol. 96. 5. Kimani K, Ilako D, Kollmann M, A review of retinoblastoma, presentation, diagnosis and management at Kenyatta National Hospital. University of Nairobi, 2000. Unpublished 6 Bowman RJC, Mafwiri M, Luthert P, et al. Outcome of Retinoblastoma in East Africa Pediatr Blood Cancer 2008; 50: 160– 162
Orbital retinoblastoma, challenges Cure or palliative treatment Exenteration or chemoreduction Primary orbital implant or not Chemotherapy/ radiotherapy or both
cure or palliative treatment? There is little information in literature on 5 year survival of retinoblastoma patients with orbital disease Anecdotal data from India show with combined modalities, it is possible to cure orbital Retinoblastoma 1 Our experience with combined modalities is also encouraging although we have not done a survival study since we started protocol based treatment 1 Honavar SG, Reddy VAP, Murthy R, Naik M, Vemuganti GK: Management of orbital retinoblastoma Proceedings of the XI International Congressof Ocular Oncology, Hyderabad, India, 2004
Exenteration or chemoreduction Exenteration alone does not achieve complete surgical clearance Exentaration also leaves the patient with a bad scar and a prothesis can not be fixed We therefore no longer perform exentaration for orbital retinoblastoma
Primary orbital implant or not Primary orbital implants do not interfere with the management of Retinoblasoma PMMA implants are particulary good because they do not interfere with radiotherapy Implants are good for bone growth, for improved movement and better prosthesis We encourage the myoconjunctival technique
Chemotherapy or radiotherapy Chemotherapy alone does not eradicate residual orbital disease Radiotherapy alone on the other hand does not prevent systemic metastasis Combined therapy therefore is the best treament of orbital retinoblastoma 1234 4 1. Pratt CB, Crom DB, Howarth C. Theuse of chemotherapy in extraocularretinoblastoma. Med and Pediatr Oncol 1985; 13: 330 -333. 2. Kiratli H, Bilgic S, Ozerdem U. Managementof massive orbital involvementof intraocular retinoblastoma. Ophthalmology 1998; 105: 322 -326. 3. Goble RR, Mc. Kenzie J, Kingston JE, et al. Orbital recurrence of retinoblastomasuccessfully treated by combined therapy. Br J Ophthalmol 1990; 74: 97 -98.
Orbital retinoblastoma Rule out intracranial spread and distant metastasis Chemoreduction With 2 or 3 courses of HD VEC chemotherapy (vincristine, etoposide, and carboplatin) Enucleation with primary orbital implant, EUA the other eye Continue with chemotherapy upto 6 courses radiotherapy
After chemoreduction and after enucleation
Good cosmesis on followup
Challenges Difficulty enucleation after chemoreduction Contracted socket after enucleation/radiotherapy Unreliable histology after chemoreduction Eye lid problemsectropion/entropion ptosis
Challenges: contracted socket due to pre enucleation chemotherapy
Challenges: Ptosis post enucleation
conclusion Combined therapy (chemoreduction, chemotherapy, enucleation and radiotherapy) is recommended for orbital retinoblastoma Orbital exenteration for orbital RBL should be avoided because it leaves a poor cosmetic effect and has no clinical advantage over the above modality Primary orbital implants do not interfere with the future management of retinoblastoma
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