Published online Nov 16, 2022. doi: 10.12998/wjcc.v10.i32.12000
Peer-review started: July 29, 2022
First decision: September 5, 2022
Revised: September 22, 2022
Accepted: October 11, 2022
Article in press: October 11, 2022
Published online: November 16, 2022
Processing time: 101 Days and 21.4 Hours
Amyloidosis is caused by misfolding of proteins and is characterized by formation of extracellular aggregates of insoluble fibrin. The primary effects in the eye include sharp deterioration of visual acuity as a result of vitreous opacity. According to the local and systemic distribution characteristics of amyloid deposits and their fibrin components, amyloidosis can be classified as primary, secondary or familial. Therefore, we report a typical case of vitreous amyloidosis in hereditary transthyretin amyloidosis (hATTR) to improve ophthalmologists’ understanding of the disease and reduce misdiagnosis and recurrence.
The patient was a 49-year-old man who complained of progressive visual decline in both eyes over a 2-mo period. No systemic diseases such as diabetes or hypertension were reported, and no obvious family history of disease was identified. The patient’s visual acuity was HM/10 cm in the right eye and 0.06 in the left eye. He had a transparent cornea in both eyes, with a normal anterior depth, clear aqueous humor, no obvious iris abnormalities, round pupils of approximately 3 mm in diameter, normal direct and indirect light reflexes, and normal intraocular pressure. After various examinations, the patient was diagnosed with binocular vitreous amyloidosis secondary to hATTR associated with a Lys55Asn variant in TTR. The binocular visual acuity recovered to 1.0 after binocular vitrectomy.
Vitreous amyloidosis is rare in the clinic and gene testing can assist the diagnosis accurately and effectively.
Core Tip: We report a case of binocular vitreous amyloidosis. Eye changes existed in isolation and no systemic lesions were present. Genetic sequencing revealed that the patient was diagnosed with binocular vitreous amyloidosis secondary to the presence of hereditary transthyretin amyloidosis associated with a transthyretin Lys55Asn genetic variant. Our study demonstrates that the accurate diagnosis of vitreous amyloidosis is very important and vitrectomy can effectively treat this particular disease phenotype.