Acute retinal vein occlusion and cystic fibrosis

Abstract

BackgroundThe ocular manifestations of cystic fibrosis typically present with surface irritation or nyctalopia due to Vitamin A deficiency, however, there have been two previous reports of patients with cystic fibrosis that developed retinal vein occlusions. These reports hypothesized that either elevated fibrinogen levels due to chronic infections or elevated homocysteine levels have predisposed patients with cystic fibrosis to develop retinal vein occlusions.Case presentationWe present a case of a 35-year-old male with cystic fibrosis complicated by chronic sinusitis with no history of organ transplantation or chronic pulmonary infections who presented with an acute branch retinal vein occlusion in his left eye with associated macular edema. Evaluation revealed an elevated fibrinogen level, while the rest of his workup was relatively unremarkable including a normal homocysteine level. His vision remained 20/20 throughout his care and he did not require treatment of his macular edema.ConclusionsPatients with cystic fibrosis are at an increased risk of developing retinal vein occlusions likely due to a variety of systemic thrombogenic factors rather than a single risk factor which had been reported previously. Elevated fibrinogen levels in these patients may not be due to chronic infections, but inherent to the cystic fibrosis.

Corneal Refractive Surgery Considerations in Patients with Cystic Fibrosis and Cystic Fibrosis Transmembrane Conductance Regulator-Related Disorders

“… Note : Data from these studies 15 , 19 , 20 , 23 , 30 , 36–39 , 41 , 42 , 47 , 61 , 62 . Abbreviations : CF, cystic fibrosis; CFTR-RD, cystic fibrosis transmembrane conductance regulator-related disorder; DED, dry eye disease; pRNFL, peripapillary retinal nerve fiber layer; SPEED, Standard Patient Evaluation of Eye Dryness; OSDI, Ocular Surface Disease Index; TBUT, tear break-up time; MMP-9, matrix metalloproteinase-9; ELISA, enzyme-linked immunosorbent assay; IL-8, interleukin-8; IFN-γ, interferon-γ; MIP-1α, macrophage inflammatory protein-1α; MIP-1β, macrophage inflammatory protein-1β.…”

“…[50][51][52][53][54][55][56] AMEED and Regener-Eyes ® are not currently FDA approved but have promising therapeutic potential as they leverage their anti-inflammatory, anti-angiogenic, and nonimmunogenic properties to promote limbal stem cell proliferation, facilitate corneal epithelial healing, and treat DED. [54][55][56] Note: Data from these studies 15,19,20,23,30,[36][37][38][39]41,42,47,61,62…”

Ocular Changes in Cystic Fibrosis: A Review

“…The most common vascular changes were venous engorgement, tortuosity, and dilatation—at least one of them was seen in 20/23 patients, while retinal hemorrhages were found in 9 patients [ 77 ]. The case report of branch retinal vein occlusion (BRVO) with secondary macular edema in a 35-year-old male CF patient with elevated serum fibrinogen levels was described by Starr et al [ 92 ]. Hiscox et al described a similar case of a 35-year-old male CF patient with BRVO and hyperfibrinogenemia [ 93 ].…”

Advantages and Disadvantages of Using Magnetic Nanoparticles for the Treatment of Complicated Ocular Disorders

“…Night blindness is the first sign of vitamin A deficiency with further symptoms after prolonged periods of deficiency, such as Bitot’s spots; triangular, perilimbal grey plaques of keratinized conjunctival debris; and xerosis and dry granular patches. Furthermore, patients with CF have been reported to develop retinal vein occlusions [ 20 , 53 , 54 ]. It has been hypothesized that elevated fibrinogen levels due to chronic infections or increased homocysteine levels predispose patients with CF to develop retinal vein occlusions.…”

“…Another clinical study carried out by Seliger et al demonstrated that 91.1% of CF patients were diagnosed with cataract disease [ 18 ], which is in line with another clinical study conducted in the US evaluating the risk of cataracts of pediatric patients with cystic fibrosis [ 19 ]. More aggressive ocular problems were described by Starr et al in a 35-year-old male with CF that presented intraretinal hemorrhages along the nerve fiber layer with associated retinal thickening in the inferior macula, which means an acute branch retinal vein occlusion in his left eye with associated macular edema [ 20 ]. Rottner et al showed that CF patients are at high risk of developing retinal vein occlusions due to several systemic thrombogenic factors, such as diabetes, hypertension, hyperlipidemia, an elevated level of fibrinogen, hyperhomocysteinemia, or hypergammaglobulinemia [ 21 ].…”