Papilledema and Benign Intracranial Hypertension in Two Children with Intraocular Retinoblastoma

Abstract

Abstract: : Purpose:To describe two boys with intraocular retinoblastoma with papilledema and benign intracranial hypertension and no other medical condition. Methods:Case 1 was diagnosed with bilateral retinoblastoma at 2.5 months of age. The patient was treated by enucleation of one eye and laser and cryotherapy of the other. Papilledema was seen at 6 months of age. Case 2 was diagnosed with unilateral retinoblastoma at 13 months of age. The patient was treated by enucleation of the involved eye. Papilledema was seen at the time of presentation in the other eye. Both patients were healthy males. They never had any constitutional symptoms or other medical problems and never received either chemotherapy or radiotherapy. Lumbar puncture and MRI of the head and orbits were performed in each case because of their papilledema. Results:The initial opening pressure on lumbar puncture in Case 1 was 42 cm H₂O and in Case 2 was 33 cm H₂O. MRI and CSF cytology were negative in both cases. In Case 1, the patient’s occipital frontal circumference (OFC) increased from the 50^th percentile at 3 months of age to the 90^th percentile at 6 months of age. His papilledema, OP (47 cm H₂O) and OFC (95^th percentile) were increased at 1 month from initial presentation with papilledema. He was treated with oral acetazolamide with a decrease in OP (30 cm H₂O). In case 2, there was a gradual decrease in papilledema and OP (23 cm H₂O) over 3 months without treatment. Serial MRIs and CSF cytology were negative with more than 14 months of follow–up. Conclusions:The differential diagnosis of papilledema and increased opening pressure on lumbar puncture in a child with retinoblastoma now includes benign intracranial hypertension in addition to extraocular retinoblastoma and pineal tumor.

Keywords: retinoblastoma • tumors • neuro–ophthalmology: optic nerve