Is occlusive retinal vascular disease linked to hereditary spherocytosis postsplenectomy? A case series

To present two separate cases of occlusive retinal vascular disease with secondary cystoid macular edema in patients with a past medical history significant for hereditary spherocytosis and splenectomy.

We present two patients with atypical ocular changes in the setting of a distant history of splenectomy with underlying hereditary spherocytosis. The first patient is a 52-year-old Caucasian male with findings consistent with adult-onset Coats disease, predominantly presenting as type 1 macular telangiectasia. The second patient is a 79-year-old Caucasian female with acute branch retinal vein occlusion with a remote history of bilateral nonarteritic anterior ischemic optic neuropathy.

There is limited literature exploring the association between retinal vascular disease, hereditary spherocytosis, and splenectomy. These cases highlight that the link between vascular events and a remote history of splenectomy remains inconclusive, especially in the absence of evidence to differentiate it from other potential diagnoses. Both patients in our cases had secondary macular edema, which responded to treatment with intravitreal antivascular endothelial growth factor medications.