Amiloidosis vítrea y glaucoma secundario en paciente con polineuropatía amiloidótica familiar variante Ala36Pro: un reporte de caso y revisión

Camila Casas; Nicolás Frank; María Belén Buzzi; María Angélica Moussalli; Federico Fernandez; Esteban Virguez

doi:10.70313/2718.7446.v18.n2.429

Authors

Camila Casas Hospital Bernardino Rivadavia
Nicolás Frank Hospital Bernardino Rivadavia
María Belén Buzzi Hospital Bernardino Rivadavia
María Angélica Moussalli Hospital Bernardino Rivadavia
Federico Fernandez Hospital Bernardino Rivadavia
Esteban Virguez Hospital Bernardino Rivadavia

DOI:

https://doi.org/10.70313/2718.7446.v18.n2.429

Keywords:

vitreous amyloidosis, secondary glaucoma, familial amyloidotic polyneuropathy, ala36pro

Abstract

Objective: To describe a case of ocular amyloidosis with vitreous involvement and secondary glaucoma, with Familial Amyloidotic Polyneuropathy (FAP), Ala36Pro variant.

Case report: This is a 30-year-old female patient with a genetic diagnosis of familial amyloidotic polyneuropathy. She was under neurological follow-up for neuropathy and involvement of various organs, in addition to ocular amyloidosis. Our case was associated with vitreous humor and trabecular deposits, resulting in decreased visual acuity (VA) due to cataracts and secondary glaucoma, in addition to vitreous turbidness. Phacoemulsification was performed with intraocular lens implantation plus a complete vitrectomy in the left eye (LE). The patient’s vision improved and she did not return to the prescribed controls. Eight months later she returned with decreased VA of the LE, showing new retrolental amyloid deposition and IOP in the right eye (RE) 34 mmHg and in the LE 22 mmHg. Open angle, goniodigeneses and amyloid material were detected in both eyes. Ultrabiomicroscopy (UBM) showed hyperreflective material in the anterior and posterior iris and material in the trabecular meshwork, compatible with amyloid deposit. Hypotensive treatment with latanoprost and dorzolamide/timolol is indicated, and the patient is currently able to regulate his IOP, maintaining a watchful waiting follow-up.

Conclusion: Vitreous amyloidosis can be frequently associated with secondary glaucoma, as in our case. The visual impairment could be partially resolved.

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Author Biographies

Camila Casas, Hospital Bernardino Rivadavia

Servicio de oftalmología del Hospital Bernardino Rivadavia.
Nicolás Frank, Hospital Bernardino Rivadavia

Hospital Bernardino Rivadavia; Ciudad de Buenos Aires, Argentina.
María Belén Buzzi, Hospital Bernardino Rivadavia

Servicio de oftalmología del Hospital Bernardino Rivadavia; Buenos Aires, Argentina
María Angélica Moussalli, Hospital Bernardino Rivadavia

Servicio de oftalmología del Hospital Bernardino Rivadavia; Ciudad de Buenos Aires, Argentina.
Federico Fernandez, Hospital Bernardino Rivadavia

Servicio de oftalmología del Hospital Bernardino Rivadavia; Ciudad de Buenos Aires, Argentina.
Esteban Virguez, Hospital Bernardino Rivadavia

Servicio de oftalmología del Hospital Bernardino Rivadavia; Ciudad de Buenos Aires, Argentina.

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