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Woman presents with bilateral severe eye pain and sudden decrease in vision
The patient also experienced a myopic shift and elevated IOP.
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A 27-year-old woman of Hispanic descent presented to an outside ophthalmologist with sudden-onset bilateral decreased vision, photophobia and severe, boring retrobulbar pain worse with eye movement. She had no medical or ocular history but did note 3 months of menorrhagia. She took no medications, had no known allergies, and denied any use of illicit drugs or other substances. Her family history was positive for cardiovascular disease and diabetes.
Examination
On examination, the patient’s vision was 20/200 bilaterally, correctable to 20/25 with –6.75 D and –7.5 D of myopic correction in the right and left eyes, respectively. Pupils were reactive bilaterally, without an afferent pupillary defect. IOPs were elevated to 55 mm Hg in each eye. External and adnexal exam was within normal limits; there was no proptosis. Extraocular movements were full and symmetric. The conjunctivae had mild chemosis but were not injected, and corneas were clear bilaterally. The anterior chamber in each eye was very shallow with one corneal thickness depth centrally and 360° of peripheral iris-cornea touch. On gonioscopy, no angle structures were visible. There were no anterior or posterior synechiae noted, and the lenses were clear. Undilated view of the posterior segment revealed optic nerves with a 0.3 cup-to-disc ratio and sharp margins (Figure 1). The maculas appeared flat bilaterally.
Source: Astrid C. Werner, MD, and Michelle Liang, MD
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Acute bilateral angle closure
Acute bilateral angle closure with a myopic shift can occur in situations in which the lens-zonular complex is displaced anteriorly. This can occur if the lens becomes dislocated or if there is a process pushing the lens-zonular complex forward. Simultaneous bilateral lens dislocation is rare and would likely involve a preceding history of trauma, systemic conditions predisposing to zonular weakness such as Marfan syndrome or homocystinuria, or microspherophakia. Ciliochoroidal effusions secondary to conditions such as uveal effusion syndrome, posterior scleritis and use of certain medications are causes of anterior rotation of the ciliary body leading to angle closure and a myopic shift. Aqueous misdirection is another important consideration but is exceedingly unlikely.
Upon evaluation at Tufts NEEC, the patient was found to have IOPs in the mid-20s and shallow anterior chambers; angle structures were now visible on gonioscopy, although with a steep approach. OCT revealed a thick choroid in both eyes without subretinal fluid, and ultrasound biomicroscopy (UBM) showed bilateral ciliary body detachments secondary to effusions (Figures 2 and 3). Further testing was obtained, including fluorescein angiography (FA), indocyanine green angiography (ICG), B-scan and A-scan (Figures 4 to 6). FA revealed inferior areas of hypofluorescence (“leopard spotting”) but no disc leakage or abnormalities. ICG revealed areas peripheral to the arcades with mixed hypofluorescence and hyperfluorescence in the late series. A-scan revealed normal axial length of 23.8 mm and 23.58 mm in the right and left eyes, respectively. B-scan revealed bilateral choroidal detachments with mildly thickened sclera and suggestion of a “T-sign” in the right eye.
The primary differential diagnostic considerations were posterior scleritis, uveal effusion syndrome or vascular abnormality such as arteriovenous fistula. There were no arterialized vessels to suggest arteriovenous fistula, and B-scan findings combined with symptoms of severe eye pain were most consistent with a diagnosis of posterior scleritis.
Discussion
Ciliochoroidal effusions occur when there is a disruption of the normal mechanisms to remove protein and fluid from the uveal circulation. Fluid from the choriocapillaris extravasates into the surrounding potential space, causing engorgement and thickening of the choroid with detachment of the choroid and ciliary body. Effusions extending to the ciliary body may cause an ensuing myopic shift.
In many cases, uveal effusion is secondary to underlying inflammatory conditions or hydrostatic abnormalities. In inflammatory conditions such as posterior scleritis or uveitis, the effusion is caused by increased choroidal permeability. Infectious causes, most notably HIV, herpes simplex virus, syphilis and tuberculosis, as well as certain medications, classically topiramate and sulfa drugs, have been known to cause effusions by a similar mechanism. In contrast, ocular or systemic conditions that disrupt normal hydrostatic forces can also produce effusions. These conditions include hypotony, myxedema, amyloidosis, multiple myeloma, nephropathy and arteriovenous fistula. When no obvious cause of the effusion exists, the term uveal effusion syndrome (UES) is applied. UES is itself divided into two categories, nanophthalmic and idiopathic, and should be considered a diagnosis of exclusion. Histologic examination of scleral specimens from cases of true UES often reveals underlying abnormalities in the arrangement of collagen fibrils and glycosaminoglycans.
While most causes of effusions are painless, posterior scleritis is a notable exception. In fact, pain is the most common presenting symptom in posterior scleritis (64%), followed by blurred vision (34%), headache (13%) and photophobia (4%). Clinical findings at presentation are quite variable, but conjunctival chemosis is the most common (32%), followed by conjunctival hyperemia, choroidal folds, serous retinal detachment, anterior chamber reaction, macular edema and anterior scleritis in 20% to 30% of patients. Posterior scleritis is more common in women (70% of cases). Bilateral disease is unusual but does occur in approximately 16% of patients. In contrast to unilateral disease, bilateral posterior scleritis is more often associated with headache as a presenting symptom (28%), and optic nerve edema is the most common presenting sign (33%).
There are several classic findings on ancillary testing that are associated with posterior scleritis. The “T-sign” on B-scan ultrasound is a squaring off of the interface of the optic nerve and sclera due to fluid accumulation beneath Tenon’s capsule. According to one large cohort study published in 2016, the T-sign is present in up to 52% of cases of unilateral cases. This study also reported a non-statistically significant finding that the classic T-sign was less apparent in bilateral cases (11%). B-scan remains the gold standard in diagnostic imaging for this condition; however, ultrasonic findings can be variable and the diagnosis may be missed. CT and MRI have diagnostic value as ancillary studies in these cases. A recent case series found scleral enhancement on CT and MRI in 100% of patients with posterior scleritis, and scleral thickening and focal periscleral cellulitis in upward of 75% of cases. FA typically reveals disc staining and leakage, as well as staining and leakage overlying areas of active scleritis. Similarly, ICG angiography shows diffuse zonal choroidal hyperfluorescence due to pinpoint leakage overlying active lesions in approximately 50% of cases.
An associated systemic disease is identified in 40% of cases of posterior scleritis, the most frequent of which is rheumatoid arthritis (12%). Other less commonly associated systemic inflammatory diseases are systemic lupus erythematous, granulomatosis with polyangiitis, sarcoidosis and polyarteritis nodosa. Infectious etiologies include syphilis, tuberculosis, herpes simplex, varicella zoster and Lyme disease. Initial diagnostic workup should be targeted to these entities; however, in most patients with posterior scleritis (up to 60%), no underlying systemic disorder is ever identified.
Unlike the treatment of anterior scleritis, there is no role for NSAIDs in the treatment of posterior scleritis. For infectious etiologies, treatment is targeted at the underlying pathogen; for noninfectious etiologies, the mainstay of therapy is oral prednisone, typically dosed at 1 mg/kg per day and gradually tapered weekly according to clinical response. If there is no response after several weeks or if the scleritis recurs with repeated attempts to wean steroids, then immunomodulatory therapy (IMT) with antimetabolites, alkylating agents or T-cell inhibitors is utilized next. The relative efficacy of the various IMT options was determined in the Systemic Immunosuppressive Therapy for Eye Diseases study. Patients who fail IMT or require long-term steroid-sparing treatment but are refractory to IMT can be treated with biologic agents such as TNF-a inhibitors and CD20 monoclonal antibodies. The clinical course of posterior scleritis can be prolonged; the median time to remission is around 200 days, and relapses occur in up to one-third of patients. Therefore, early consideration of steroid-sparing medications may be warranted.
Ocular hypertension is an increasingly recognized early complication of posterior scleritis, with reported incidences at presentation ranging from 7% to 46%. There are several possible mechanisms at play: inflammatory debris blocking the trabecular meshwork causing a resultant rise in IOP, elevated episcleral venous pressure due to inflammation in the sclera causing vascular outflow resistance, and ciliochoroidal effusion causing non-pupillary block angle closure. Of patients presenting with ocular hypertension, up to 20% will go on to develop true glaucoma during the course of follow-up, although some proportion of these cases are likely steroid-induced.
Clinical course continued
The patient underwent testing for possible underlying systemic diseases as outlined above, all of which were negative. She was started on 60 mg of oral prednisone and topical cyclopentolate three times a day. At 1-week follow-up, her vision returned to 20/25 with marked improvement in the myopic shift. Her anterior chambers had deepened, gonioscopy revealed open angles, and IOP was in the mid-teens bilaterally. Repeat UBM and B-scan revealed resolution of the ciliochoroidal effusions, and OCT showed interval thinning of the choroid. At this point, the cyclopentolate was discontinued and prednisone was tapered by 10 mg per week without recurrence of symptoms.
Summary
Our patient presented with bilateral angle closure with a myopic shift and increased IOP due to ciliochoroidal effusions. While laser peripheral iridotomy will prove ineffective in relieving angle closure, it is reasonable to attempt it both as a diagnostic technique and to relieve any possible contribution from pupillary block. In such cases, pilocarpine is contraindicated because it can worsen the situation, and treatment with cycloplegics is warranted to induce posterior movement of the ciliary-lens apparatus. Effusions can result from a myriad of underlying disorders; in the case presented above, bilateral effusions occurred as the result of posterior scleritis. Posterior scleritis frequently presents with severe pain, and B-scan ultrasound reveals thickened sclera with a T-sign due to fluid beneath Tenon’s capsule. Posterior scleritis can be associated with underlying systemic infectious and inflammatory diseases in 40% of cases, and a complete diagnostic workup is indicated. The mainstay of therapy is high-dose oral steroids, but IMT and biologic agents may be necessary.
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- For more information:
- Astrid C. Werner, MD, and Michelle Liang, MD, can be reached at New England Eye Center, Tufts University School of Medicine. 800 Washington Street, Box 450, Boston, MA 02111; website: www.neec.com.
- Edited by Aubrey R. Tirpack, MD, and Astrid C. Werner, MD. They can be reached at the New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.