Woman with amblyopia presents with unilateral eye pain and headache
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A 56-year-old woman presented to the ophthalmology office at the Lahey Clinic with 1 week of left eye pain associated with burning, injection and a left-sided headache without any vision changes.
She had been seen 1 week prior in the emergency room for severe left-sided headache associated with nausea and vomiting but without ocular symptoms. She had a CT scan of her brain during that visit, which was unremarkable, and her headache improved with a migraine cocktail of diphenhydramine, intravenous fluids, ketorolac, magnesium and metoclopramide. She was discharged home on naproxen for presumed migraine headache. The eye pain started shortly after her emergency room visit and prompted her to make an appointment with her ophthalmologist.
She denied any history of trauma to either eye. Her medical history included Crohn’s colitis with a previous bowel resection, anemia, anxiety and depression. Her ocular history included strabismic and refractive amblyopia of the left eye, exotropia, myopia, anterior basement membrane dystrophy and cataract extraction of the right eye 12 years before presentation. She had a dense cataract in the left eye with hand motions vision that had been monitored for the past few years, but given her significant amblyopia, she had decided not to pursue cataract extraction in the left eye.
Examination
Upon examination in the office, the patient’s best corrected visual acuity was 20/30 in the right eye and hand motions only in the left eye. She was noted to have anisocoria, with her left pupil larger than the right and minimally reactive to light. IOP was 12 mm Hg in the right eye and 36 mm Hg in the left eye. Extraocular movements were intact bilaterally, and alignment testing showed a large angle left exotropia.
Anterior segment examination of the right eye was notable for anterior basement membrane dystrophy and a well-centered posterior chamber IOL. Fundus exam of the right eye showed a small cup-to-disc ratio, a healthy-appearing nerve and mildly tortuous retinal vessels, with macula and retinal periphery within normal limits. Left eye anterior segment examination revealed 1+ conjunctival injection with limbal flush, diffuse corneal stromal edema and inflammatory debris on the endothelium of the inferior cornea without keratic precipitates or a hypopyon. The left anterior chamber appeared deep, and although the corneal edema limited the view, there was anterior chamber flare and a very dense opaque cataract without a view to the posterior segment.
The patient was started on timolol and brimonidine drops (patient had a sulfa allergy) and topical prednisolone every 2 hours while awake. She returned the next day, with exam of the left eye revealing stable hand motions vision, IOP of 46 mm Hg, and improved conjunctival injection and corneal edema. The anterior chamber still had inflammatory debris on the endothelium inferiorly, and there were new diffuse mutton-fat keratic precipitates inferiorly as well. She had 3+ flare and rare cells in the anterior chamber. With the improved corneal edema, it was apparent that there was an anterior capsular irregularity inferotemporally in the opaque lens (Figure 1). Gonioscopy was performed, and the patient had an open angle without any abnormalities noted.
Source: Chelsea Gottschalk, MD, and Carolyn Anderson, MD
Imaging
The patient had a B-scan ultrasound of the left eye as there was no posterior view due to the dense cataract. The scan did not show any masses, vitreous debris or retinal detachment.
What is the diagnosis?
See answer below.
Eye pain, headache
The differential diagnosis for this patient included lens-induced uveitis, either phacolytic or phacoantigenic, herpetic uveitis, sarcoidosis, tuberculosis, sympathetic ophthalmia and HLA-B27-associated anterior uveitis, as the patient had a diagnosis of Crohn’s disease.
Given that the patient never had any prior episodes of uveitis and the inflammation was granulomatous, it seemed less likely that this uveitis was related to her diagnosis of inflammatory bowel disease (patient never had HLA testing). While herpetic uveitis also typically presents with high IOP, the presence of a disruption and possible tear in the anterior lens capsule in the setting of a hypermature cataract made phacoantigenic uveitis the most likely diagnosis. Although phacoantigenic glaucoma/uveitis classically occurs in the setting of trauma or post-surgically, spontaneous rupture of the anterior capsule may occur with a hypermature cataract, leading to granulomatous inflammation. The inflammatory debris noted on the endothelium inferiorly (separate from the keratic precipitates) may have represented lens debris from the open capsule. If the keratic precipitates and anterior capsular disruption were absent, the presentation would have been more consistent with phacolytic glaucoma. The patient was relatively young for a mature cataract and had cataract extraction of her right eye 12 years prior. She denied a history of trauma or significant corticosteroid treatment, although it is possible that she received steroids at some point during her Crohn’s treatment, leading to an early cataract in both eyes.
Workup and management
The patient admitted that, while she used timolol twice a day and brimonidine twice a day as prescribed, she did not use the prednisolone that was prescribed to her at her initial visit, likely explaining why the inflammation worsened. She was subsequently continued on timolol twice a day and brimonidine was increased to three times a day. Latanoprost was added at bedtime in the left eye, and prednisolone was continued every 2 hours. As the patient had a listed non-anaphylactic sulfa allergy, oral acetazolamide was avoided; however, dorzolamide was added three times a day. Her IOP on exam 2 days later was 9 mm Hg.
As the inflammation and elevated IOP were likely the result of a violated lens capsule of a hypermature cataract, it was felt that the best option would be to plan for cataract extraction to control the IOP and intraocular inflammation. The patient agreed with this plan and underwent A-scan and topography studies for preoperative planning. Her surgery was less than 1 week after her presentation. Intraoperatively, the inferotemporal anterior capsular tear was incorporated into a continuous curvilinear capsulorrhexis. The nucleus was found to be extremely dense and rubbery, requiring significant phacoemulsification energy. After cortical cleanup, there appeared to be a residual white posterior subcapsular cataract; however, upon further inspection, this was felt to be retrolental cortical or inflammatory material in Berger’s space (Figure 2). The posterior capsule appeared intact without loss of vitreous at the conclusion of the case.
At the initial postoperative visits, the retrolental inflammatory material remained, blocking the view to the fundus, and appeared as white liquid material that moved upon blinking. It was unclear if this represented inflammatory material or leaked cortex that migrated posterior to the zonules. The patient continued to have anterior chamber inflammation and inferior inflammatory debris; however, the IOP remained controlled on topical medications. She was continued on topical prednisolone. At the 2-month visit, the retrolental material had dissipated completely, the anterior chamber was quiet, and there was a view to the fundus (Figure 3). Best corrected visual acuity improved to 20/400. Fortunately, the optic nerve and peripheral retina appeared healthy; however, she did have cystoid macular edema (CME) on OCT of the macula (Figure 4) as a result of either the initial intraocular inflammation or the complex cataract surgery. After an additional 5 weeks on topical prednisolone and ketorolac, the CME resolved, IOP remained stable, and the patient’s vision improved to 20/200 (Figure 5).
Discussion
Lens-induced uveitis can be broken down into phacoantigenic and phacolytic subtypes based on whether the lens capsule is macroscopically intact (phacolytic) or disrupted (phacoantigenic).
In phacolytic glaucoma, there is microscopic leakage of lens proteins from a hypermature cataract, which are then phagocytosed by macrophages, clogging the trabecular meshwork and leading to increased IOP. There has been more debate over the etiology of phacoantigenic uveitis/glaucoma (formerly known as phacoanaphylactic endophthalmitis). Initially, the lens was thought to be under immune privilege; however, it is believed that phacoantigenic glaucoma/uveitis is a result of a complement-mediated sensitization against one’s own lens proteins from a tear in the capsule, which can result in granulomatous (classically) or non-granulomatous inflammation. There may be lens debris in the anterior chamber and in the angle leading to elevated IOP. Both entities are underdiagnosed and can lead to significant morbidity if not appropriately recognized and treated.
While cataract surgery is the definitive treatment, topical steroids, cycloplegics and IOP-lowering therapy can be used to control inflammation and pressure until surgery is an option. There is a higher risk for intraoperative and postoperative complications in these cases, as most of these cataracts are either hypermature or post-traumatic.
- References:
- Ahmad SS. J Acute Dis. 2017;doi:10.12980/jad.6.2017JADWEB-2016-0065.
- Federici T, et al. Lens-induced uveitis. In: Zierhut M, et al, eds. Intraocular Inflammation. Springer-Verlag Berlin Heidelberg; 2016:985-990.
- Nche EN, et al. Graefes Arch Clin Exp Ophthalmol. 2020;doi:10.1007/s00417-019-04598-3.
- Thach AB, et al. Int Ophthalmol. 1991;doi:10.1007/BF00171031.
- Yanoff M, et al. Phacogenic uveitis. In: Ophthalmology, 5th ed. Elsevier; 2018:767-769.
- For more information:
- Chelsea Gottschalk, MD, and Carolyn Anderson, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.
- Edited by Christine Benador-Shen, MD, and Malgorzata Dymerska Peterson, MD. They can be reached at New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.