Silent sicklers and traumatic hyphema: the importance of maintaining a high index of suspicion.

Abstract

A previously healthy 6-year-old white boy presented emergently after trauma to his right eye. On examination, intraocular pressure (IOP) was 49 mm Hg, and visual acuity was 20/50. He was diagnosed with a 3 mm layering hyphema. Despite medical therapy and lifestyle modifications, the patient's IOP continued to rise, peaking at 77 mm Hg on day 3 after presentation. Given his elevated IOP despite the addition of new topical medications on daily follow-up, the patient was screened for sickle-cell disease to assess for an underlying pathology that could be exacerbating his elevated IOP. After testing positive for sickle cell trait, the patient was promptly taken to the operating room for anterior chamber washout. The following day, IOP normalized to 14 mm Hg, and visual acuity was 20/25. Hyphemas generally respond well to medical therapy, but patients with sickle cell hemoglobinopathy are at greater risk for permanent vision loss from pressure spikes. This case reinforces the need to collect thorough past medical and family histories and consider early sickle cell screening in all patients failing to respond to IOP-reducing medication or if hemoglobin status is unknown on initial presentation.