Abstract
ObjectiveSecondary glaucoma is a serious complication in patients with transthyretin (TTR)-related familial amyloidotic polyneuropathy (FAP). We assessed the long-term outcomes and complications of trabeculectomy with mitomycin C (MMC) for secondary glaucoma associated with FAP.MethodsMedical case records of Kumamoto University Hospital were retrospectively reviewed. Twenty-one eyes of 13 patients (10 with FAP ATTR Val30Met; 3 with FAP ATTR Tyr114Cys) underwent trabeculectomy with MMC and follow-up of at least 2 years. The primary outcome measure was Kaplan-Meier survival, with failure of this treatment being defined as an intraocular pressure (IOP) of ≤5 mm Hg or ≥22 mm Hg on two consecutive visits or as additional operations needed to reduce IOP. Secondary outcome measures included complications, bleb characteristics, and additional postoperative interventions required.ResultsThe mean postoperative follow-up period was 5.7 years (range, 2.2–12.7 years). Kaplan-Meier analysis indicated probabilities of success of 0.76, 0.67, and 0.53 at 1, 2, and 3 years after operation, respectively. Significant complications included ocular decompression retinopathy in 7 eyes (33%) and bleb encapsulation in 10 eyes (48%). Twelve eyes (57%) needed additional surgery, such as bleb revision or trabeculectomy with MMC, to reduce IOP.ConclusionsTrabeculectomy with MMC may not be optimal for patients with FAP-related glaucoma and may have several significant complications.
Highlights
Transthyretin (TTR)-related familial amyloidotic polyneuropathy (FAP), which is characterized by systemic accumulation of mutant amyloidogenic TTR (ATTR) in organs and peripheral nerves, is a fatal amyloidosis that is inherited in an autosomal dominant fashion [1]
We previously reported on the short-term outcomes of surgical treatment of patients with secondary glaucoma associated with TTR-related FAP and the clinical features of these patients [5]
Subjects and Data Collection We retrospectively reviewed the medical records of patients with secondary glaucoma associated with FAP who underwent trabeculectomy with mitomycin C (MMC) at the Department of Ophthalmology, Kumamoto University Hospital, between 1987 and 2011
Summary
Transthyretin (TTR)-related familial amyloidotic polyneuropathy (FAP), which is characterized by systemic accumulation of mutant amyloidogenic TTR (ATTR) in organs and peripheral nerves, is a fatal amyloidosis that is inherited in an autosomal dominant fashion [1]. In Portugal and Japan, the penetrance is higher and symptoms typically develop before the age of 40. Patients with TTR-related FAP commonly have ocular manifestations, especially vitreous opacity and glaucoma, which are troublesome and can restrict the daily lives of patients; these ocular involvements occur more frequently during the course of the illness [2,3,4]. We previously reported on the short-term outcomes of surgical treatment of patients with secondary glaucoma associated with TTR-related FAP and the clinical features of these patients [5]. Glaucoma occurred in 12 (24%) of 49 patients; amyloid deposition at the pupillary border, a pupillary border with irregularity, and vitreous opacity were strongly related to the occurrence of glaucoma; and intraocular pressure (IOP) was well controlled during the short follow-up period (mean, 1.2 years; range, 0.2–6.5 years). During a longer follow-up period, certain patients required additional operations, and several specific complications developed after surgery
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