PMC9160387_01
General appearance of the patient. (A): Facial hypertrophy and thickening of the nasal soft tissue can be seen since we first saw him till now. Bilateral mild ptosis before the blepharoptosis correction surgery 3 years ago. (B): Mild ptosis still existed in OS, and incomplete eyelid closure existed in OD after the surgery.
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F1
He had bilateral ptosis since birth (Figure 1A) and underwent blepharoptosis correction surgery 3 years ago. Incomplete eyelid closure existed in OD (Figure 1B). Systemic physical examination was notable for facial hypertrophy and thickening of the nasal soft tissue (Figure 1). Fortunately, the hemorrhage was spontaneously resolved within 2 weeks, without IOP elevation and any intervention (Supplementary Figure 1).
Clinical presentation. (A): Examinations of the patient before surgery. Slit-lamp examination showed 360-degree ectropion uveae in the right (A1) and left (A2) eyes. (B): Gonioscopy showed anterior insertion of iris in the right (B1) and left (B2) eyes. (Red arrow) (C): Fundus photos of the right (C1) and left eyes (C2) showed marked cupping, overpass phenomenon, notching of blood vessels, and thinning of neuroretinal rim at the upper and lower poles. (D): Visual field testing showed temporal islands in right (D1) and left eyes (D2). (E): Retinal nerve fiber layer (RNFL) thickness in the right (E1) and left eye (E2) measured by OCT examination.
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F2
Slit-lamp examination (SL-D8Z; Topcon Corporation, Tokyo, Japan) showed transparent cornea with normal size, clear anterior chamber (AC) with normal depth, and bilateral ectropion uveae in both eyes (Figures 2A1,A2). Gonioscopy revealed abnormal angles with anterior insertion of the iris in both eyes (Figures 2B1,B2). Corporation, Tokyo, Japan) showed significant pallor of the neuroretinal rim and the vertical C/D ratios of 1.0 in both eyes (Figures 2C1,C2). Central corneal thickness was 519/522 mum, measured by a solid-tip, ultrasonic pachymeter (Tomey SP-3000, Tokyo, Japan). Visual field testing (Octopus 900, Haag-Streit, USA) showed bilateral temporal islands, and <5  central vision in OS (Figures 2D1,D2). The mean retinal nerve fiber layer (RNFL) thickness was 51 mum in OD and 56 mum in OS, measured by optical coherence tomography (OCT) examination (ZEISS CIRROS HD-OCT4000, Germany, Figures 2E1,E2). A diffuse avascular bleb had been maintained in OS since then (Supplementary Figure 2), and the IOP ranged from 8 to 10 mm Hg without any medication (Figure 3).
Changes of intraocular pressure (IOP) in both eyes during the 2-year follow-up period. A CO2 Laser-assisted Sclerectomy Surgery (CLASS) was first performed in the left eye (OS) and then a Microcatheter-assisted Trabeculotomy (MAT) surgery was performed in the right eye (OD). A fixed combination anti-glaucoma medicine (Azarga ) was added at 1-month post-CLASS, and a YAG laser goniopuncture (LGP) was performed to enhance the IOP-lowing effect at 3-month after CLASS. D: day; W: week; M: month.
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F3
A diffuse avascular bleb had been maintained in OS since then (Supplementary Figure 2), and the IOP ranged from 8 to 10 mm Hg without any medication (Figure 3). During the follow-up period, there was no obvious progression in visual field defect (Supplementary Figure 3).
Clinical presentation at 2-year after surgery. (A): Slit-lamp examination of the right (OD, A1) and left (OS, A2) eye. Shallow anterior chamber and complicated cataract were found in OS. (B): No bleb was found in the right eye (B1) and a low-lying diffuse bleb was found in the left eye (B2). (C): Ultrasound biomicroscopy (UBM) examination showed a normal anterior chamber depth in the right eye (C1) and a shallow anterior chamber in the left eye (C2). (D): UBM examination showed no obvious bleb in the right eye (D1); a diffuse subconjunctival bleb and a deep scleral lake was found in the left eye (D2). Optical coherence tomography (OCT) examination in OD (E1) and OS (E2). There was no obvious hypotony maculopathy in OS.
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F4
There was no obvious hypotony maculopathy in OS by OCT (Heidelberg Engineering, Heidelberg, Germany) examination (Figure 4).