Branch Retinal Vein Occlusion in an Asymptomatic Adult with Cystic Fibrosis

Hiscox, Rachel; Purslow, Christine; North, R. V.; Ketchell, Ian; Evans, Katharine

doi:10.1097/opx.0000000000000186

Cited by 3 publications

(3 citation statements)

References 18 publications

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“…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 ]. A case of CRVO recurrence in the contralateral eye four years after the first episode in a 31-year-old CF patient with a hypercoagulopathy secondary to increased serum fibrinogen and gammaglobulin levels has also been reported [ 94 ].…”

Section: Changes Of the Retinal And Choroidal Vasculaturementioning

confidence: 99%

Ocular Changes in Cystic Fibrosis: A Review

Liberski,

Confalonieri,

Cofta

et al. 2024

IJMS

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Cystic fibrosis (CF), also known as mucoviscidosis, is the most common autosomal recessive genetic disease in the Caucasian population, with an estimated frequency of 1:2000–3000 live births. CF results from the mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) gene localized in the long arm of chromosome 7. The product of CFTR gene expression is CFTR protein, an adenosine triphosphate (ATP)-binding cassette (ABC) transporter that regulates the transport of chloride ions (Cl−) across the apical cell membrane. Primary manifestations of CF include chronic lung and pancreas function impairment secondary to the production of thick, sticky mucus resulting from dehydrated secretions. It is well known that CF can cause both anterior and posterior ocular abnormalities. Conjunctival and corneal xerosis and dry eye disease symptoms are the most characteristic manifestations in the anterior segment. In contrast, the most typical anatomical and functional changes relating to the posterior segment of the eye include defects in the retinal nerve fiber layer (RNFL), vascular abnormalities, and visual disturbances, such as reduced contrast sensitivity and abnormal dark adaptation. However, the complete background of ophthalmic manifestations in the course of CF has yet to be discovered. This review summarizes the current knowledge regarding ocular changes in cystic fibrosis.

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Section: Changes Of the Retinal And Choroidal Vasculaturementioning

confidence: 99%

Ocular Changes in Cystic Fibrosis: A Review

Liberski,

Confalonieri,

Cofta

et al. 2024

IJMS

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“…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.…”

Section: Ocular Disorders Derived From Cystic Fibrosis Diseasementioning

confidence: 99%

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

Schneider‐Futschik

Reyes-Ortega

2021

Pharmaceutics

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Nanomaterials provide enormous opportunities to overcome the limitations of conventional ocular delivery systems, such as low therapeutic efficacy, side effects due to the systemic exposure, or invasive surgery. Apart from the more common ocular disorders, there are some genetic diseases, such as cystic fibrosis, that develop ocular disorders as secondary effects as long as the disease progresses. These patients are more difficult to be pharmacologically treated using conventional drug routes (topically, systemic), since specific pharmacological formulations can be incompatible, display increased toxicity, or their therapeutic efficacy decreases with the administration of different kind of chemical molecules. Magnetic nanoparticles can be used as potent drug carriers and magnetic hyperthermia agents due to their response to an external magnetic field. Drugs can be concentrated in the target point, limiting the damage to other tissues. The other advantage of these magnetic nanoparticles is that they can act as magnetic resonance imaging agents, allowing the detection of the exact location of the disease. However, there are some drawbacks related to their use in drug delivery, such as the limitation to maintain efficacy in the target organ once the magnetic field is removed from outside. Another disadvantage is the difficulty in maintaining the therapeutic action in three dimensions inside the human body. This review summarizes all the application possibilities related to magnetic nanoparticles in ocular diseases.

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“…The ocular manifestations of cystic fibrosis (CF) typically present with ocular surface irritation or nyctalopia due to Vitamin A deficiency secondary to malabsorption; additionally decreased contrast sensitivity, oculosympathetic paresis, decreased lenticular transparency, and optic nerve dysfunction have been reported [ 1 – 3 ]. There have been, however, only two reports of patients with cystic fibrosis with retinal vein occlusions (RVO) [ 4 , 5 ]. We report a third case of a branch retinal vein occlusion (BRVO) in a young male with CF who was found to have elevated fibrinogen levels on systemic evaluation without an elevation in his homocysteine levels.…”

Section: Introductionmentioning

confidence: 99%

Acute retinal vein occlusion and cystic fibrosis

et al. 2018

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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.

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Branch Retinal Vein Occlusion in an Asymptomatic Adult with Cystic Fibrosis

Abstract: It is hypothesized that BRVO occurred secondary to raised fibrinogen levels, a common feature in CF resulting from chronic pulmonary infection and inflammation. Practitioners should be aware of the possible link between BRVO and CF.

Cited by 3 publications

References 18 publications

Ocular Changes in Cystic Fibrosis: A Review

Ocular Changes in Cystic Fibrosis: A Review

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

Acute retinal vein occlusion and cystic fibrosis

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