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. 2022 Dec;11(6):2271-2284.
doi: 10.1007/s40123-022-00580-1. Epub 2022 Oct 12.

Combination of Trabeculectomy and Primary Pars Plana Vitrectomy in the Successful Treatment of Angle-Closure Glaucoma with BEST1 Mutations: Self-Controlled Case Series

Yuxin Fang  1 , Xiaoming Duan  1 , Lin Chen  1 , Jie Shi  1 , Jie Liu  2 , Yunxiao Sun  1 , Jin Wang  1 , Yang Li  1 , Xin Tang  3
Affiliations

Combination of Trabeculectomy and Primary Pars Plana Vitrectomy in the Successful Treatment of Angle-Closure Glaucoma with BEST1 Mutations: Self-Controlled Case Series

Yuxin Fang et al. Ophthalmol Ther. 2022 Dec.

Abstract

Introduction: This study aimed to illustrate the efficacy of the combination of lens extraction, trabeculectomy, and anterior vitrectomy in patients with secondary angle-closure glaucoma (ACG) with autosomal recessive bestrophinopathy or Best vitelliform macular dystrophy.

Methods: This is a retrospective self-controlled case series study. Five patients undergoing a single trabeculectomy in one eye and triple surgery in the other eye were enrolled. All patients underwent a complete ophthalmic examination that included best-corrected visual acuity (BCVA), intraocular pressure (IOP), ultrasound biomicroscopy, and static gonioscopy. Multimodal fundus imaging was performed, including color fundus photography, fundus autofluorescence, and optical coherence tomography. Genetic testing was also analyzed.

Results: Among the 10 eyes, the mean IOP was 31.4 ± 4.7 mmHg before surgery. The mean axial length (AL) was 21.53 mm and the anterior chamber depth (ACD) was 2.31 mm. There were no statistically significant differences in preoperative IOP, BCVA, ACD, and AL between the two groups (all P > 0.05). The mean follow-up time was 64.0 months. All five eyes with a single trabeculectomy developed malignant glaucoma (MG). No complications were found in the other five eyes with triple surgery, and the anterior chamber was deepened and stable after surgery until the last visit. The mean IOP at the last visit was normalized to 16 mmHg without using any medications.

Conclusions: Triple surgery is superior to single trabeculectomy for patients with ACG and BEST1 mutation, effectively bypassing MG complications. The vitreous may play a vital role in the mechanism of ACG in those patients and the high incidence of MG after filtering surgery.

Keywords: Angle-closure glaucoma; Anterior vitrectomy; Autosomal recessive bestrophinopathy; Malignant glaucoma; Trabeculectomy.

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Figures

Fig. 1
Fig. 1
Fundus photograph, fundus autofluorescence (FAF), and spectral-domain optical coherence tomography (OCT) of both eyes in each patient. a Multimodal imaging of a 37-year-old woman (patient 1). The fundus photograph showed yellowish deposits around the vascular arcades with corresponding hyperautofluorescence in the FAF images in both eyes. Well-defined yellowish lesions were also seen in the macular area in both eyes. OCT revealed hyperreflective subretinal materials and subretinal fluid in the macula. b Multimodal imaging of a 23-year-old man harboring BEST1 mutation p.E35K (patient 2). Fundus photographs showed tiny yellowish-white spots located in the macula, near the inferior temporal vascular arcade and the nasal to the optic disc in the right eye with corresponding hyperautofluorescence in the FAF image. Retinal pigment epithelium (RPE) hyperplasia and scar in the macular area were seen in the left eye. The OCT image shows retinal thinning and diffuse photoreceptor degeneration with conspicuous cystoid macula edema and mild neurosensory retinal detachment. c Multimodal imaging of a 43-year-old man carrying the BEST1 mutation p.L40P (patient 3). Fundus photographs in both eyes showed multiple yellowish depositions around the fovea with corresponding hyperautofluorescent spots in FAF. The OCT revealed subretinal fluid with cystoid macular edema, which is more obvious in the left eye. d Multimodal imaging of a 46-year-old woman with the BEST1 mutation p.V81M (patient 4). Fundus photographs showed a vertical vitelliform lesion centered on the fovea with hyperautofluorescence in both eyes. The OCT revealed the presence of hyperreflective subretinal material in the left eye and subretinal fluid in both eyes. e Multimodal imaging of a 17-year-old woman with the BEST1 mutation p.R202W (patient 5). Fundus photographs showed a diffuse yellowish lesion in the macula. The FAF images show a widespread area of hypoautofluorescence in the posterior pole. The OCT imaged showed disruption of the ellipsoid zone in the foveal and numerous intraretinal cysts in each macula with mild subretinal fluid
Fig. 2
Fig. 2
Representative case of pre- and postoperative anterior segment imaging in eyes with different surgical approaches in the same patient (patient 4). The axial length was 20.51 mm in her right eye and 20.23 mm in her left eye. a Anterior segment photograph of right eye showed flat anterior chamber that did not respond to cycloplegia at 7 months after single trabeculectomy. The intraocular pressure (IOP) was 54 mmHg. b Ultrasound biomicroscopy (UBM) revealed anterior rotation of the ciliary body against the peripheral iris, indicating malignant glaucoma was present. The filtering passage was also seen. c After an additional operation of phacoemulsification–intraocular lens (IOL) implantation–anterior vitrectomy, the anterior chamber was deep (3.33 mm) with mild dilated pupil and localized atrophic iris due to uncontrolled IOP without regular IOP-lowering medication usage. Intraocular pressure was normalized to 16 mmHg using three IOP-lowering medications at the last visit. d Preoperative anterior segment photograph of the left eye showed a shallow anterior chamber. The preoperative IOP was 31 mmHg with full medication. e UBM showed angle closure and anterior chamber depth was 1.87 mm. f After triple surgery, phacoemulsification, IOL implantation–trabeculectomy–anterior pars plana vitrectomy, the anterior chamber was deep and stable (3.86 mm). The IOP was normalized to 15 mmHg without any medication
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