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A 45-year-old man with spontaneous hyphema of the right eye
Digital Journal of Ophthalmology 2017
Volume 23, Number 3
August 9, 2017
DOI: 10.5693/djo.03.2017.02.003
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Jay C. Wang, MD | Department of Ophthalmology, Massachusetts Eye and Ear, Boston, Massachusetts
Maggie B. Hymowitz, MD | Department of Ophthalmology, Massachusetts Eye and Ear, Boston, Massachusetts
Diagnosis and Discussion
The occurrence of a spontaneous hyphema with an anterior uveitis is uncommon. One must first rule out other more common causes of hyphema, such as trauma. In addition, the iris should be carefully inspected for any signs of neovascularization from proliferative diabetic retinopathy, retinal vein occlusion, anterior segment ischemia, or other causes, such as a mass or tumor in the anterior segment. Once these causes for hyphema have been eliminated, one can be more confident that the hyphema is more likely associated with a uveitis. Our patient did not report a history of trauma, and there was no evidence of a mass in the anterior segment. The prominent dilated iris vessels observed were within the stroma and radial in orientation, differentiating them from neovascularization of the iris, which appears as small, fine, disorganized vessels on the anterior surface of the iris. With immunomodulatory treatment, the vessels were no longer prominently noted.

Fuchs heterochormic iridocyclitis was initially considered, given the presence of fine keratic precipitates and what appeared to be fine blood vessels traversing the angle. There was also an asymmetry in the color of the iris, although the iris was darker in the affected eye. A hyphema can occur due to bleeding of fine vessels traversing the iris and angle during anterior chamber paracentesis, or even spontaneously, also known as Amsler’s sign. However, the anterior uveitis in Fuchs is typically mild and rarely presents with visual acuity worse than 20/40.(1)

In our patient, the anterior chamber inflammation was quite significant and could not be adequately controlled with topical difluprednate. Due to the possibility of a viral infection, the patient was empirically started on oral antiviral therapy. It was important to obtain a sample of the aqueous humor to rule out a viral infection with PCR tests for herpes simplex and varicella zoster virus, because these have been implicated in keratouveitis and spontaneous hyphema.(2,3) In children, juvenile xanthogranulomatosis and juvenile idiopathic arthritis can also present with uveitis and spontaneous hyphema, but our patient is out of this age group.(4,5) Lens-induced uveitis and hyphema with resolution upon cataract extraction has been reported, but there was not a significant cataract in our patient.(6)

Labwork revealed our patient to be HLA-B27 positive. The uveitis associated with HLA-B27 is typically unilateral and can be severe. Spontaneous hyphema has been reported, especially in patients with ankylosing spondylitis.(3,7) It is thought that hyphema occurs in the setting of severe anterior uveitis secondary to breakdown of the blood–aqueous barrier and increase in capillary permeability.(8) Our patient did not carry a diagnosis of ankylosing spondylitis, although he did have a history of intermittent mild joint pains, improving with movement. It is not known whether recent spinal imaging has been performed to evaluate for this, but it should be considered.

Our patient’s uveitis was severe and could not be controlled with topical steroids. He required systemic corticosteroids in the acute phase and additional long-term steroid sparing therapy with methotrexate and adalimumab. Recent studies have shown anti-TNF-α agents such as infliximab, adalimumab, and etanercept to be effective in the treatment of uveitis associated with HLA-B27.(9,10) However, these agents increase the risk of severe infection and should be used with caution. Coordination with a rheumatologist can be helpful in the management of these patients.