COECSA, Journal, Ophthalmology
A 5 year’s retrospective case series on the clinical profile and management of retinoblastoma at Maputo Central Hospital, Mozambique
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Maputo Central Hospital (MCH)
Modalities of management

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Dimande LSA, Ilako D, Nyenze EM, Zambujo Y. (2023). A 5 year’s retrospective case series on the clinical profile and management of retinoblastoma at Maputo Central Hospital, Mozambique. The Journal of Ophthalmology of Eastern, Central and Southern Africa, 15(01). Retrieved from


Background: Retinoblastoma is the commonest intraocular malignancy in childhood. The national
epidemiological characteristics of retinoblastoma in Mozambique are not clearly known. Early diagnosis
and appropriate treatment leads to a favourable outcome while a delayed diagnosis and inappropriate
treatment can be fatal.

Objective: To determine the pattern of presentation and management of retinoblastoma patients at
Maputo Central Hospital during a 5 year period (1st January 2010 to 31st December 2014).
Methodology: The study was a retrospective case series carried out at Maputo Central Hospital
(Ophthalmology and Paediatric Oncology Services). Records of all retinoblastoma patients were retrieved
with the help of the specific file number. Demographic characteristics, clinical presentation, investigations
and management modalities of retinoblastoma patients were retrieved. Data was extracted and entered
into a structured questionnaire and analysed.

Results: A total of 57 patients from 7 different provinces of Mozambique had clinical, histological or both
clinical and histological diagnosis of retinoblastoma. The overall mean age at presentation was 30.5 months
(CI 24.65 - 36.05) (SD 21.5); 82.4% of cases had unilateral retinoblastoma while 12.3% of cases had bilateral
(mean age at presentation was 30.2 months and 26.4 months respectively). The female: male ratio was
1.2:1. The main presenting complaint was white reflex (62.5%) followed by eye swelling (50%), eye redness
(40%) and poor vision (32.5%) while the main clinical signs were leukocoria (55%) followed by proptosis
(50%) and eye redness (27.5%). Out of the 51 eyes who had surgery: 96% did not have documentation
of the choroid and 98% of the scleral, but 54.9% of the eyes had optic nerve involovement, 33.3% had
resection margin involvement and 31.4% had periocular tissue involvement. The main modalities of
management were enucleation (93.9%), chemotherapy (24.5%) and exenteration (18%). The eligible
patients for chemotherapy were 67.7% but only 35.3% of the patients received chemotherapy.

Conclusion: Many patients presented at older ages (with a mean age at presentation of 30.5 months) and
many presented late with advanced orbital disease. Majority of the patients had unilateral retinoblastoma.
Significant number of patients had optic nerve, resection margin and periocular tissue involvement noted
on histology. The main modalities of management were enucleation, exenteration and chemotherapy,
although there were no clear eligibility criteria for chemotherapy in retinoblastoma patients.

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Ophthalmologists, American Academy of

Ophthalmic Pathology and Intraocular Tumours:

Section 4 – BCSC: AAO, 2012 – 2013

MacCarthy A, Birch JM, Draper GL, et al.

Retinoblastoma: treatment and survival in Great

Britain 1963 to 2002. Br J Ophthalmol. 2009;

(1): 38–39.

Nyawira G, Kahaki K, Kariuki M. Survival

among retinoblastoma patients at KNH, Kenya.

J Ophthalmol Eastern Central and Southern Afr.

; 17(1): 15-19.

Honavar SG. Retinoblastoma: They Live and

See! AIOS ... MD, FACS Head, Ocular Oncology

Service, LV Prasad Eye InstituteLV Prasad Marg,

Banjara Hills, Hyderabad 500034, India.

Shields CA, Shields JA. Recent development

in the management of retinoblastoma. J Pediat

Ophthalmol Starbismus. 1999; 36: 8-18.

Shields JA, Parsons H, Shields CL, et al. The

role of cryotherapy in the management of

retinoblastoma. Am J Ophthalmol. 1989; 108:

- 264.

Shields CL, Mashayekhi A, Sun H. Iodine 125

plaque radiotherapy as salvage treatment for

retinoblastoma recurrence after chemoreduction

in 84 tumors. Ophthalmology. 2006; 113(11):


Demirci H, Shields CL, Meadows AT, et al. Longterm

visual outcome following chemoreduction

for retinoblastoma. Arch Ophthalmol. 2005; 123

(11): 1525-30.

Antoneli CB, Ribeiro KC, Steinhorst F, et al.

Treatment of retinoblastoma patients with

chemoreduction plus local therapy: Experience

of the AC Camargo Hospital, Brazil. J Pediat

Hematol Oncol. 2006; 28 (6): 342-345.

Chan HS, Gallie BL, Munier FL, Popovic BM.

Chemotherapy for retinoblastoma. Ophthalmol

Clin North Am. 2005; 18 (1):55-63.

Shields CL, Shields JA, Needle M, et al. Combined

chemoreduction and adjuvant treatment for

intraocular retinoblastoma. Ophthalmology. 1997;

(12): 2101-2111.

Friedman DL, Himelstein B, Shields CL, et al.

Chemoreduction and local ophthalmic therapy for

intraocular retinoblastoma. J Clin Oncol. 2000;

(1): 12-17.

Wilson MW, Rodriguez-Galindo C, Haik

BG, et al. Multiagent chemotherapy as

neoadjuvant treatment for multifocal intraocular

retinoblastoma. Ophthalmology. 2001; 108 (11):


ElZomor H, Taha H, Aleieldin A, et al. High

risk retinoblastoma: Prevalence and success of

treatment in developing countries. Ophthalmic

Genetics. 2015; 36(3): 287-289.

Uusitalo MS, Van Quill KR, Scott IU, et al.

Evaluation of chemoprophylaxis in patients with

unilateral retinoblastoma with high-risk features

on histopathologic examination. Arch Opthtalmol.

; 119 (1) 41-48.

Nyamori JM, Kimani K, Njuguna MW, Dimaras H.

The incidence and distribution of retinoblastoma

in Kenya. Br J Ophthalmol. 2012; 96: 141-143.

Bowman RJC, Mafwiri M, Luthert P, et al.

Outcome of retinoblastoma in East Africa Pediat

Blood Cancer. 2008; 50:160–162.

Makite I, Kimani K, Njuguna MW. Delay in

presentation and management of retinoblastoma

patients at Kenyatta National Hospital.


Dimaras H, Kimani K, Dimba EAO, et al.

Retinoblastoma. The Lancet. 2012; 379 (9824):


Dimaras H, Dimba E, Gallie BL. Challenging the

global retinoblastoma survival disparity through

a collaborative research effort. Br J Ophthalmol.

; 94: 1415–1416.

Chang CY, Chiou TJ, Hwang B, et al.

Retinoblastoma in Taiwan: survival rate and

prognostic factors. Japan J Ophthalmol. 2006;


Nyaka AS, Kimani K, Kollmann MK. The pattern

of retinoblastoma at Queen Elizabeth Central

Hospital, Malawi. University of Nairobi. M. Med

Ophthalmology Thesis, 2010.

Waddell KM, Kagame K, Ndamira A, et al.

Clinical features and survival among children

with retinoblastoma in Uganda. Br J Ophthalmol.


Broaddus E, Topham A, Singh AD. Incidence

of retinoblastoma in the USA: 1975-2004. Br J

Ophthalmol. 2009; 93:21-23.

Owoeye JFA, Afolayan EAO, Ademola-Popoola

DS. Retinoblastoma – a clinicopathologic study

in Ilorin Nigeria. Afr J Health Sci. 2005; 12(3-4):


Kimani K, Ilako D, Kollmann M. A review of

retinoblastoma, presentation, diagnosis and

management at Kenyatta National Hospital.

University of Nairobi, 2000 (Unpublished).

Roth DB, Scott IU, Murray TG, et al. Echography

of retinoblastoma: histopathologic correlation

and serial evaluation after globe-conserving

radiotherapy or chemotherapy. J Pediat

Ophthalmol Strabismus. 2001; 38:136–143.

Graaf P, Göricke S, Rodjan F, et al. Guidelines

for imaging retinoblastoma: imaging principles

and MRI standardization. Pediat Radiol. 2012;

: 2–14.

Khelfaoui F, Validire P, Auperin A, et al.

Histopathologic risk factors in retinoblastoma:

A retrospective study of 172 patients treated in a

single institution. Cancer. 1996; 77:1206–13.

Magramm I, Abramson DH, Ellsworth RM.

Optic nerve involvement in retinoblastoma.

Ophthalmology. 1989; 96: 217–222.

Chan HS, DeBoer G, Thiessen JJ, et al.

Combining cyclosporin with chemotherapy

controls intraocular retinoblastoma without

requiring radiation. Clin Cancer Res. 1996; 2 (9):



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