Orbital Retinoblastoma,

KNH Experience
DR Nyenze E. M
University of Nairobi

Orbital retinoblastoma,
frequency

1
2
3.
4.
5.
6

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
Kaimbo WA, Kaimbo D, Mvitu MN, Missotten L. Presenting signs of retinoblastoma in Congolese Patients. Bull Soc
Belge Ophthalmol 2002; 283: 37-41
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
Nyamori JM, Kimani K, Njuguna MW, Dimaras H. The incidence and distribution of retinoblastoma in Kenya. no. 1;
pp. 1413, s.l.: The British Journal of Ophthalmology, Jan 2012, Vols. vol. 96 .
Kimani K, Ilako D, Kollmann M, A review of retinoblastoma, presentation, diagnosis and management at Kenyatta
National Hospital. University of Nairobi, 2000. Unpublished
Bowman RJC, Mafwiri M, Luthert P, et al. Outcome of Retinoblastoma in East Africa Pediatr Blood Cancer
2008;50:160162

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
Retinoblastoma1
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

1. Pratt CB, Crom DB, Howarth C. Theuse of chemotherapy in extraocularretinoblastoma. Med and Pediatr
Oncol1985;13:330-333.
2. Kiratli H, Bilgic S, Ozerdem U. Managementof massive orbital involvementof intraocular retinoblastoma. Ophthalmology
1998;105:322-326.
3. Goble RR, McKenzie J, Kingston JE,et al. Orbital recurrence of retinoblastomasuccessfully treated by combined
therapy. Br J Ophthalmol 1990;74:97-98.
4. Doz F, Khelfaoui F, Mosseri V, etal. The role of chemotherapy in orbitalinvolvement of retinoblastoma. The experience
of a single institution with 33patients. Cancer 1994;74:722-732.
5. Antoneli CB, Steinhorst F, de CassiaBraga Ribeiro K, et al. Extraocularretinoblastoma: a 13-year experience.Cancer
2003;98:1292-1298.

Orbital retinoblastoma

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
6courses

radiotherapy

Rule out intracranial

spread and distant
metastasis

After chemoreduction and

after enucleation

Good cosmesis on
followup

Challenges
Difficulty enucleation
after chemoreduction
Contracted socket
after
enucleation/radiothera
py
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