From Leukocoria to Orbital Retinoblastoma: Consequences of Delayed Treatment in a 3-Year-Old Child

Eva Imelda; Sarra Mutiara Adev; Navneet Shamsundar Toshniwa

doi:10.60084/ijcr.v4i1.421

Authors

Eva Imelda Department of Ophthalmology, General Hospital Dr. Zainoel Abidin, Banda Aceh 23126, Indonesia
Sarra Mutiara Adev Faculty of Medicine, Brawijaya University, Malang 65145, Indonesia
Navneet Shamsundar Toshniwa Navneet Hospital, Solapur, Maharashtra, India

DOI:

https://doi.org/10.60084/ijcr.v4i1.421

Keywords:

Retinoblastoma, Leukocoria, Enucleation, Delayed treatment, Phthisis bulbi

Abstract

Retinoblastoma is the most common primary intraocular malignancy of childhood, and delayed treatment can shift management from globe preservation to survival-oriented intervention. We report a 3-year-and-4-month-old girl with bilateral advanced retinoblastoma who re-presented after an 18-month default from recommended treatment. She had progressive left-eye proptosis and severe cancer-related cachexia, weighing 9.2 kg, below the 3rd percentile for age. She had initially been evaluated at 1 year of age for bilateral leukocoria, when urgent enucleation was advised but declined by caregivers. On re-presentation, contrast-enhanced CT of the brain and orbits showed a 24 × 18 mm retrobulbar soft-tissue mass surrounding a collapsed left globe. At the same time, the right eye contained an advanced calcified intraocular tumor filling the vitreous cavity, consistent with ICRB Group E disease. Systemic staging confirmed locoregionally confined disease. The patient received four cycles of neoadjuvant pediatric VEC chemotherapy. Subsequent assessment showed no light perception in either eye, tumor- and chemotherapy-induced phthisis bulbi in the left eye, and an unsalvageable Group E tumor in the right eye. Because severe malnutrition and systemic frailty made simultaneous bilateral surgery high risk, sequential bilateral enucleations were performed under general anesthesia with a four-week interval to optimize physiological recovery. Histopathology confirmed poorly differentiated bilateral retinoblastoma, with transmural scleral invasion in the left eye and massive choroidal invasion in the right eye; both elongated optic nerve margins were tumor-free. Dermis-fat graft reconstruction was not feasible due to the absence of subcutaneous adipose tissue. At 6 months, the sockets were stable with no clinical or radiological recurrence. This case highlights the consequences of treatment delay and supports individualized staged enucleation in fragile pediatric patients.

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