A 73 year-old male, presented with painless, gradual dimness of vision, in the right eye for the last 4 years. He also gave history of no vision in left eye since birth and no history of trauma or surgery to his left eye, verified by his brother, who brought him for eye check-up. There was no history of consanguineous marriage and other sibling was normal, no other chronic medical or systemic illness was present. There were no clinical signs of systemic connections such as facial abnormalities, webbed hands, lid coloboma, preauricular skin tags, cleft lip, hydrocephalus, microcephaly, or convulsions.
On ocular examination, his left eye showed severe ptosis (Figure 1A), best corrected visual acuity in the right eye was 6/36 (with -1.75 DSph) and no perception of light (NPL) in the left eye. Central corneal thickness (CCT: 563 micron) corrected intraocular pressure was 30 mmHg in the right eye by Goldmann applanation tonometry.
On slitlamp examination, right eye showed grade 3 nuclear sclerosis with cortical changes in the lens and a sectoral anterior capsular fibrosis (Figure 1B). Left eye showed microphthalmia (Figure 1C), but ocular adnexa was in normal condition. Gonioscopy of the right eye showed Grade-4 open anterior chamber angle in all four quadrants with no notable pathological changes (Figure 1D).
Dilated fundus examination of the right eye showed advanced glaucomatous optic nerve changes with tigroid fundus (Figure 1E), supported by Humphrey visual field change (dense superior arcuate scotoma; (Figure 2A) and optical coherence tomography (OCT) of the optic nerve head showed extensive thinning of the retinal nerve fiber layer (RNFL) (Figure 2B).
B-scan ultrasonogram of the left eye showed dense vitreous opacities with thickened sclera which persisted in both high and low gain and short axial length of 9.77 mm (Figure 2C and 2D).
Patient was prescribed with topical combination of Timolol maleate (0.5%) and Brimonidine tartrate (0.2%) twice daily, and Brinzolamide (1%) thrice daily in his right eye. After 15 days of initiating antiglaucoma medications (AGM), IOP dropped down to around 22 mmHg and patient underwent combined phaco-trabeculectomy augmented with 0.1 ml of 0.2mg/ml of mitomycin-C (MMC) (Kyowa Hakko Kirin Co, Ltd., Tokyo, Japan) for 30 seconds.
Postoperative treatment regime was topical moxifloxacin (0.5%) 4 times daily for 1 month, Prednisolone (1%) 2 hourly for 7 days, then 4 hourly for 7 days, 6 hourly for 7 days and 8 hourly for 7 days, and Atropine sulphate (1%) 8 hourly for 7 days.
Postoperative day (POD) 1 showed, visual acuity of 6/24 with clear cornea, pharmacologically (Atropine sulphate 1%) dilated pupil, well-centered single-piece intraocular lens (IOL) in the bag, surgical peripheral iridectomy at 12 o’clock position and diffuse bleb formation (Figure 3A and 3B) with IOP of 12 mmHg without any AGM.
He was on regular follow-up for the last 1 year, maintaining good vision at distance (BCVA 6/18 with -0.75DCyl@90°) and near (N6 with +2.75DSph) with IOP ranging from 12 - 14 mmHg without any topical antiglaucoma drops. Patient was also advised for left eye evisceration followed by prosthetic eye implant, but he refused to do so.