Glaucomatous optic disc changes despite normal baseline intraocular pressure in a child

Purpose: We describe a case of normal tension glaucoma in the setting of sickle cell disease in a 9-year-old patient with a five-year follow up.

Observations: A 9-year-old male patient with a history of sickle cell disease presented initially at the age of 4 years for evaluation of a brief episode of nonspecific eye pain that had spontaneously resolved prior to the clinic visit. Over the course of several years, the patient was noted to have progressive optic disc cupping bilaterally, retinal nerve fiber layer thinning bilaterally, and has developed a corresponding inferior arcuate defect on automated visual field testing in the right eye, all without elevated intraocular pressures (IOP). After neuro-ophthalmic pathologies were ruled out, the patient was diagnosed with glaucoma associated with sickle cell disease and normal baseline IOP, and brimonidine therapy was initiated.

Conclusions and importance: To our knowledge, this is the first reported case of normal-tension glaucoma in a pediatric patient. Normal-tension glaucoma may be a consideration in the evaluation of pediatric glaucoma suspects, but remains a diagnosis of exclusion.