Elderly man awakes with acute monocular vision loss
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An 87-year-old Russian man presented for routine postoperative retina follow-up 1 month after exchange of a dislocated IOL in the left eye. He mentioned he awoke with painless vision loss in the better-seeing right eye.
Review of systems was negative for giant cell arteritis symptoms, and the left eye felt stable from his baseline of light perception only.
Source: Daniel B. Azzam, MD, and Susan Liang, MD
Ocular history in the right eye was notable for pseudoexfoliation, and the left eye was significant for severe pseudoexfoliative glaucoma status post selective laser trabeculoplasty 5 years ago and Kahook Dual Blade (New World Medical) 4 years ago. He was pseudophakic with a posterior chamber IOL implanted in both eyes many years prior; however, due to a dislocated IOL in the left eye, he recently underwent pars plana vitrectomy (PPV) with IOL exchange for an anterior chamber IOL with peripheral iridectomy 1 month ago. Of note, intraoperatively, a small suprachoroidal hemorrhage inferiorly in the periphery was encountered and stabilized, with a postoperative course complicated by diffuse persistent vitreous hemorrhage and IOP spike to the 40s mm Hg with a hyphema in the left eye that was now resolved. Eye drops in the left eye only included postoperative prednisolone acetate taper, dorzolamide/timolol, latanoprost and brimonidine.
The patient’s medical history was significant for atrial fibrillation, coronary artery disease and hypertension, with systemic medications including metoprolol, apixaban, amlodipine and chlorthalidone. His family history was negative for ocular disease, including glaucoma. His was a retired mathematics professor in Russia, and he denied alcohol use or smoking.
Examination
Visual acuity without glasses measured hand motion in the right eye (down from 20/30) and light perception in the left eye (stable from prior; was 20/50 before IOL dislocation). The right pupil was mid-dilated and nonreactive with no relative afferent pupillary defect by reverse, while the left pupil had a stable mild relative afferent pupillary defect. IOP was 43 mm Hg in the right eye and 38 mm Hg in the left eye by Goldmann applanation tonometry. Prior maximum IOP was mid-30s mm Hg in the left eye. Previous central corneal thickness measurements were 569 µm in the right eye and 567 µm in the left eye.
Slit lamp exam of the right eye was significant for mild conjunctival injection, diffuse corneal microcystic edema, a diffusely flat anterior chamber with iridocorneal apposition temporally, posterior chamber IOL against the pupillary border with no iris capture and no IOL-cornea touch centrally, and previously noted mild pseudophacodonesis. The view to the fundus was poor but demonstrated an intact red reflex and visible disc. The left eye exam was stable with deep anterior chamber, anterior chamber IOL in place, resolved hyphema, an inferior iridotomy, improving vitreous hemorrhage, a flat retina, and a large cup with thin rims. Gonioscopy evaluation revealed no visible angle structures in the right eye and scleral spur visible 360° in the left eye.
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Flat anterior chamber and elevated IOP
The clinical picture was suspicious for pupillary block secondary to an anteriorly dislocated IOL given the pseudophacodonesis noted on prior exams with a history of pseudoexfoliation and recently dislocated IOL in the opposite eye. Malignant glaucoma was also considered; however, given no pain and no recent filtration or incisional surgery, this case did not match the typical clinical presentation that would be expected, specifically red eye with vision loss after recent incisional surgery. Suprachoroidal hemorrhage was on the differential given the history of recent suprachoroidal hemorrhage in the other eye, as well as anticoagulation with apixaban, although this would typically be a perioperative diagnosis, and the red reflex was still present in this case. Additionally, choroidal detachment with anterior rotation of the ciliary body and a mass lesion causing secondary angle closure were also considered. For the left eye, the IOP spike with an open angle was thought to be most likely a steroid response in the postoperative period rather than uveitis-glaucoma-hyphema syndrome from the anterior chamber IOL.
Further workup
B-scan ultrasonography of the right eye was negative for any choroidal effusion, suprachoroidal hemorrhage or mass (Figures 1a and 1b). The patient was urgently referred to the glaucoma clinic where IOP-lowering drops and oral acetazolamide were initiated. He underwent laser peripheral iridotomy in the right eye with three patent iridotomies made with overlying hemorrhage: The first at 4:30 had release of bubbles, and the second and third at 3:00 and 2:30 had good fluid waves. Mild improvement was noted in the peripheral anterior chamber depth nasally, but there was still iris-cornea touch temporally. The IOP was improved to 28 mm Hg in the right eye and 11 mm Hg in the left eye after multiple topical and oral antihypertensive medications were given to both eyes.
Ultrasound biomicroscopy (UBM) performed both supine and seated upright demonstrated persistently shallow anterior chamber both centrally and peripherally (Figures 1c to 1h), with anterior displacement of the lens-iris diaphragm and a small amount of supraciliary fluid suggesting occult mild ciliary body detachment (Figure 1e). However, there was no posterior shift in IOL position with the effect of gravity when supine (Figures 1c to 1e), contrary to what would have been expected in angle closure due to a dislocated IOL. Furthermore, the initial episode happened while the patient was asleep lying down, which would be unlikely if this were secondary angle closure from a subluxed IOL.
Management
At this point, a presumptive diagnosis of malignant glaucoma was made based on persistently flat anterior chamber both centrally and peripherally despite patent peripheral iridotomies and no evidence of IOL shift on UBM. Treatment in the right eye was initiated with atropine drops and maximum medical therapy with IOP-lowering drops and oral acetazolamide.
After a few days of atropine therapy, there was no improvement in the diffusely flat anterior chamber. The retina team elected for surgical intervention of PPV with hyaloidectomy and possible IOL exchange. Intraoperatively, the vitreous was debulked to release posterior pressure; subsequently, balanced salt solution and viscoelastic material were injected into the anterior chamber with immediate deepening of the anterior chamber. Next, core PPV and hyaloidectomy were performed, and two peripheral iridotomies inferonasally and inferotemporally were made using the vitrector. The anterior chamber was noted to be deep, and the IOL was in a good position without the need for an IOL exchange.
Discussion
Malignant glaucoma was first described by von Graefe in 1869 as an aggressive postoperative glaucoma resistant to treatment and is now also referred to as aqueous misdirection, ciliary block glaucoma, lens block glaucoma or vitreous block glaucoma. The mechanism is secondary angle-closure glaucoma due to posterior misdirection of aqueous humor into or behind the vitreous, creating positive vitreous pressure that leads to a flat peripheral and central anterior chamber with elevated IOP despite patent peripheral iridotomies, according to Salim.
On presentation, patients typically report pain, redness, vision loss and headache with nausea, usually after recent glaucoma surgery or any intraocular or laser surgery, including cataract surgery, scleral buckle, PPV, laser cyclophotocoagulation, laser iridotomy, laser capsulotomy or scleral flap suture lysis, Foreman-Larkin and colleagues reported. Malignant glaucoma has also been reported with the use of miotics and even may occur spontaneously in an eye with no antecedent miotics or operation. Diagnosis of malignant glaucoma is characterized by a flat peripheral and central anterior chamber despite patent peripheral iridotomy, which is a necessary component to make the diagnosis. B-scan ultrasonography or dilated fundus exam should rule out suprachoroidal hemorrhage and choroidal detachment. UBM and/or anterior segment OCT can be considered to evaluate the angle, iris and lens configuration.
Malignant glaucoma has been reported perioperatively in 0.4% to 6% of incisional surgery cases for primary angle-closure glaucoma, according to Shahid and Salmon. Other risk factors include female gender, hyperopia, history of angle closure, eye trauma, inflammation and pseudoexfoliation.
Management typically begins with medical therapy for 3 to 5 days, as it is effective in roughly half of cases, according to Foreman-Larkin and colleagues. This includes aqueous suppressants to decrease IOP, cycloplegics to reverse the anterior rotation of the ciliary body and tighten the zonules to pull the lens back, mydriatics to decrease the lens-iris apposition, hyperosmotics to shrink the vitreous cavity and decrease the volume posteriorly, and steroids to reduce inflammation and prevent peripheral anterior synechiae. Laser treatment options may be attempted, including Nd:YAG laser of the anterior vitreous face to create a new pathway for flow between the vitreous and anterior chamber (in pseudophakic eyes this can be through the pupil or via a large peripheral iridotomy, or if phakic through a surgical iridectomy if present) and argon laser through peripheral iridotomy to shrink the ciliary processes or cyclophotocoagulation to decrease posterior aqueous flow production. Surgical options include transcorneal needling through a patent peripheral iridotomy to disrupt the anterior vitreous; posterior sclerotomy to disrupt the anterior hyaloid with aspiration of fluid from the vitreous; PPV with hyaloidectomy; iridectomy-hyaloidotomy-zonulectomy with anterior vitrectomy if pseudophakic; and vitrectomy-phaco-vitrectomy, whereby in a phakic patient a limited core vitrectomy is done before phacoemulsification of the cataract followed by vitrectomy and anterior hyaloidectomy, according to AlQahtani and colleagues. Ninety-six percent of eyes on average eventually require surgery within 3 months for definitive management. Specific treatment may vary case by case. Liebmann and colleagues published a malignant glaucoma case series in 1998 suggesting two clinical subtypes of malignant glaucoma: aqueous misdirection predominant, with minimal ciliochoroidal detachment, and ciliary body detachment predominant, with minimal aqueous misdirection. Accordingly, four out of their six cases were treated medically, but two of the six required drainage of the supraciliary fluid.
Spontaneous malignant glaucoma is far more rare than classical postoperative malignant glaucoma, with only a handful of spontaneous cases reported in the literature. A 2019 literature review by González-Martín-Moro and colleagues reported 10 cases of spontaneous malignant glaucoma, with no pseudoexfoliation, no gender predilection, young mean age of 47 years, and 40% bilateral, with five of 14 eyes (36%) successfully managed medically rather than surgically.
Case resolution
After surgical treatment of the spontaneous malignant glaucoma, the patient’s vision improved from hand motion to 20/200 in the right eye by postop month 3 and remained hand motion in the left eye. IOP remained in the low teens in both eyes; however, increased cupping was noted in both eyes. It was felt that there may have been more damage to both optic nerves due to the recent IOP spikes. Furthermore, the postoperative course was complicated by pseudophakic bullous keratopathy in both eyes for which the patient was referred to the cornea service in addition to regular follow-ups with the retina and glaucoma services. The most recent vision 1 year later was 20/80 in right eye and light perception in the left eye.
In summary, the final diagnosis in the right eye was acute angle-closure glaucoma due to spontaneous malignant glaucoma associated with occult mild ciliary body detachment. This case highlights the utility of UBM in demonstrating occult ciliary body detachment that was not seen on B-scan, as well as in differentiating malignant glaucoma from a dislocated IOL. In this clinical scenario, the malignant glaucoma resolved with surgical PPV, with the aqueous misdirection posterior pressure being the main component compared with the relatively small supraciliary fluid noted on UBM. Furthermore, malignant glaucoma many years after surgery is exceedingly rare in the literature but has been reported up to 16 years after cataract surgery, according to Wilde and colleagues. It was interesting that our case occurred just weeks after incisional surgery in the opposite eye, but there is no known association.
- References:
- AlQahtani RD, et al. Clin Ophthalmol. 2023;doi:10.2147/OPTH.S385864.
- Foreman-Larkin J, et al. J Ophthalmol. 2015;doi:10.1155/2015/283707.
- González-Martín-Moro J, et al. Saudi J Ophthalmol. 2019;doi:10.1016/j.sjopt.2018.11.005.
- Liebmann JM, et al. Arch Ophthalmol. 1998;doi:10.1001/archopht.116.6.731.
- Salim S. Aqueous misdirection mechanisms and management. EyeTube. Published March 1, 2018. Accessed Aug. 2, 2024.
- Shahid H, et al. J Ophthalmol. 2012;doi:10.1155/2012/852659.
- von Graefe A. Archiv für Opthalmologie. 1869;doi:10.1007/BF02721215.
- Wilde C, et al. Oman J Ophthalmol. 2018;doi:10.4103/ojo.OJO_34_2016.
- For more information:
- Edited by William W. Binotti, MD, and Julia Ernst, MD, PhD, of New England Eye Center, Tufts University School of Medicine. They can be reached at william.binotti@tuftsmedicine.org and julia.ernst@tuftsmedicine.org.
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