Background: Gaucher disease is a congenital disorder of glycolipid metabolism that results in progressive accumulation of glucocerebroside in the body. Although various ocular complications have been reported, there are very few reports of tractional retinal detachment (TRD). In this study, we report a case of TRD associated with Type 3 Gaucher disease that required a pars plana vitrectomy, which was followed up for more than two years.
Case: A 29-year-old, diagnosed with Type 3 Gaucher disease at the age of 2 years, was referred to our hospital in February 2018 due to the aggregation of vitreous opacity and TRD in the left eye, identified in April 2015 at a local clinic. At initial presentation, decimal best-corrected visual acuity was 1.2 in the right eye and 0.05 in the left eye. Vitreous opacity and numerous irregular white deposits were observed on the retina's surface in both the eyes and extensive TRD was observed in the left eye; hence, a pars plana vitrectomy was performed. During the surgery, marked liquefaction of the vitreous gel and tight adhesion of the thickened posterior vitreous membrane to the retina were observed. An encircling procedure and a silicon oil tamponade were also performed. Postoperatively, the retina was positioned under the silicone oil, and a decrease in the white deposits was observed. Ninety-eight days after the initial surgery, silicone oil removal was performed. More than two years have passed since the surgery, with no recurrence of TRD and white deposits so far. Visual acuity in the left eye remains at 0.7. In the right eye, increased white deposits were observed, but no TRD was observed.
Conclusions: In Type 3 Gaucher disease, TRD requiring surgery may occur in the eyes. Vitreoretinal adhesion can be very strong, and special attention should be paid during the surgery.
Nippon Ganka Gakkai Zasshi (J Jpn Ophthalmol Soc) 125: 591-595, 2021.