Apolipoprotein AI-Derived Vitreous Amyloidosis: An Elusive D
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A 56-year-old female presented with gradual visual disturbance in her right eye of 1-year duration for a second opinion. The patient was prescribed nonsteroidal and steroidal eye drops for a “nonspecific uveitis” for 3–6 months before presentation, without any improvement. Non-Hodgkin’s lymphoma had been diagnosed and treated 15 years before and was in remission without further treatment.

On initial examination, best-corrected visual acuity (BCVA) was 20/200 in her right eye and 20/25 in her left eye. Intraocular pressure was 18 mm Hg bilaterally. Slit-lamp examination of the anterior segment was within normal limits bilaterally, including the absence of cells and flare. On fundoscopy, the media was hazy with the typical glass-wool-like appearance her right eye. B-scan ultrasound indicated that the vitreous was full of middle-echo spots, vitreous opacities, and posterior vitreous detachment occurred. The patient underwent vitreous biopsy and a standard 25-gauge pars plana vitrectomy (diagnostic and therapeutic PPV). Intraoperatively, severe diffuse debris was noted along with very strong vitreoretinal adhesions. A mechanical surgical posterior vitreous detachment was difficult.

The diseased vitreous was very “gummy,” adherent to the retina, and resistant to separation from the retina and was removed as thoroughly as possible. Vitreous samples were sent for histopathologic evaluation. The cytospin smears prepared from the vitreous aspirate revealed amorphous acellular material that stained positively with Congo Red and showed apple green birefringence on polarized microscopy. These features were consistent with the diagnosis of amyloidosis. A genetic evaluation on tongue tissue demonstrated apolipoprotein AI-derived amyloidosis. The right eye underwent an additional PPV to remove residual vitreous that was difficult to remove during the first procedure due to the strong adhesion and “gummy” and adherent characteristics mentioned previously. Intravitreal triamcinolone was used to stain the vitreous during the second PPV. At the 3-month follow-up, the BCVA was 20/25 OU. Six months later, there was no recurrence. No perivascular deposits were seen during the vitrectomies or the follow-up period. The patient was transferred to the internal medicine service for further evaluation and follow-up.

Source: https://www.karger.com/Article/FullText/508065