Six stages, three levels of care
Tap any stage to expand. The gold nodes are performed at Kenyatta National Hospital (KNH); the earlier steps happen closer to the child, where speed matters most.
The two presenting signs that must trigger urgent action:
- Leukocoria — a white pupillary reflex ("white eye" / cat's-eye reflex), often first noticed in photographs or dim light.
- Squint (strabismus) — new-onset misalignment in a young child.
Community health workers and primary staff should be trained to recognise leukocoria and to refer same-week. Lag time — not tumour biology — is the dominant driver of death in Kenya.
Any child with leukocoria = urgent referral, no watchful waitingThe ophthalmologist rules out the differential diagnoses of leukocoria before committing to a retinoblastoma diagnosis:
- Coats disease · persistent fetal vasculature (PHPV)
- Toxocariasis · congenital cataract · retinal detachment
- Retinopathy of prematurity · coloboma · astrocytic hamartoma · endophthalmitis
If retinoblastoma is suspected: examine the fellow (healthy) eye, then proceed by laterality — enucleate a unilateral affected eye, or refer bilateral disease to KNH. A trained ophthalmologist performing prompt enucleation locally can prevent extraocular spread and reduce loss-to-follow-up from onward referral.
Rule out mimics → confirm laterality → act- B-scan ultrasound of both eyes.
- Full haemogram and electrolytes.
- Confirm the child is fit for general anaesthesia.
Bilateral disease and any eye considered for salvage are referred here, where the multidisciplinary team (ophthalmology, paediatric oncology, pathology) is available.
Under GA, each eye is examined and staged separately using the International Intraocular Retinoblastoma Classification (Groups A–E). Group determines whether the eye is treated for salvage or enucleated.
Go to the Decision Maker tab to stage each eye interactively- Group A — focal therapy alone: posterior pole → transpupillary thermotherapy (diode laser); peripheral → triple freeze-thaw cryotherapy.
- Groups B / C / D — chemoreduction (Vincristine + Etoposide + Carboplatin), then focal consolidation as above.
- Group E — enucleation with orbital implant + histopathology.
Enucleated eyes are staged pathologically (pTNM). High-risk features decide adjuvant therapy:
- pT1 / pT2a / pT2b — no adjuvant chemotherapy; surveillance.
- pT3a / pT3b — adjuvant chemotherapy.
- pT4 (a–d) — adjuvant chemotherapy and radiotherapy.
Stage the child, get the plan
Answer each step. Every eye is staged independently; enucleated eyes then run through pathological staging. A printable management summary builds at the bottom.
Why the pathway is built this way
The design of this tool follows the Kenyan evidence: late presentation and loss-to-follow-up — not a lack of treatment options — are what kill children here.
Late presentation is the core problem
The mean lag time from first sign to diagnosis in Kenya is 6.8 months, versus 0.5 months in the UK and 1.5 in the USA. The commonest reason for delay is not cost — it is families and health workers not recognising leukocoria. Coast Province recorded the longest lag (mean 13.8 months). This is why the pathway front-loads community recognition and same-week referral.
Age at diagnosis is falling
Under the Kenya National Retinoblastoma Strategy (KNRbS, from 2008), mean age at diagnosis fell from 24.4 → 16.8 months for bilateral disease and 35.9 → 24 months for unilateral disease — approaching high-income-country figures. The Lancet series reported Kenyan means of 25 months (bilateral) and 36 months (unilateral).
Referral delay and loss-to-follow-up
82% of children were referred from their first facility; of those, 35% were lost before reaching treatment (highest in Nyanza, 52%). Mean referral lag added another 1.7 months. Building enucleation and histology capacity at district level — so simple cases are managed locally and only complex cases travel to KNH — is the strategy's central recommendation, and the reason the ophthalmologist can enucleate a unilateral eye without onward referral.
Enucleate first — do not shrink Group E eyes with chemo
Delaying enucleation of a Group E eye by more than 3 months increases the risk of death. Pre-enucleation chemotherapy on severely affected eyes masks high-risk histological features and increases metastatic death (Zhao et al., J Clin Oncol 2011). Primary radiotherapy for salvage is avoided because it raises the risk of second primary cancers in these genetically predisposed children.
Prognosis is stage-related
Intraocular, promptly treated disease is highly curable; survival collapses once the tumour reaches the optic nerve resection margin, sclera or beyond. Kenya's rise from 26% to 70% survival came almost entirely from earlier presentation and completed treatment — not new drugs. The remaining gap to the ~97% seen in high-income countries is a lag-time and follow-up gap.
- Gichigo EN, Kariuki-Wanyoike MM, Kimani K, Nentwich MM. Retinoblastoma in Kenya: survival and prognostic factors. Ophthalmologe 2014;112:255–260.
- Nyamori JM, Kimani K, Njuguna MW, Dimaras H. Retinoblastoma Referral Pattern in Kenya. Middle East Afr J Ophthalmol 2014;21(4):321–327.
- Dimaras H, Kimani K, Dimba EA, et al. Retinoblastoma. Lancet 2012;379:1436–1446.
- Hill JA, Kimani K, White A, et al. Achieving optimal cancer outcomes in East Africa… the KNRbS. Global Health 2016;12:23.
- Hudani A, et al. Navigating familial retinoblastoma in Kenya. Pediatr Blood Cancer 2024.
- Zhao J, Dimaras H, et al. Pre-enucleation chemotherapy… increases death from metastasis. J Clin Oncol 2011;29:845–851.
- Murphree AL. Intraocular retinoblastoma: the case for a new group classification. Ophthalmol Clin North Am 2005 (IIRC / Groups A–E).