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Girl initially presents with open globe injury
Three weeks after successful surgery, the patient experienced intermittent eye pain and decreased visual acuity.
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The ophthalmology service was consulted for an otherwise healthy 6-year-old girl who was hit in the right eye by her brother with a pen 3 days before presenting to the emergency room. The patient’s mother thought her daughter seemed fine immediately after the event. However, over the next few days, the child complained of increasing irritation in her right eye. The mother took a closer look and was alarmed when she saw a “brown bubble” on the right eye.
Examination
On exam, the patient’s visual acuity was 20/70 in the right eye and 20/25 in the left eye. In the right eye, the pupil was peaked at 7 o’clock with iris prolapsing from a full thickness scleral laceration near the limbus. The anterior segment of the left eye was unremarkable. Fundus exam was deferred until surgery due to the patient squeezing, given the open globe injury. A CT scan of the orbits showed no intraocular metallic foreign body. She was then taken to the operating room that night for urgent repair of the open globe.
Intraoperatively, the scleral laceration was found to be approximately 1 mm posterior and parallel to the limbus and measured 2.5 mm. The prolapsed portion of iris tissue appeared necrotic and was amputated. The scleral wound was then sutured closed, followed by closure of the conjunctiva. The intraoperative fundus exam was unremarkable in both eyes.
The child did well over the next 2 weeks. The vision in the right eye improved to 20/30. IOP was normal, and the anterior chamber remained deep and quiet. There was no evidence of infection. However, at postoperative week 3, she complained of new intermittent right eye pain.
At postoperative week 3, the patient’s visual acuity had worsened to 20/70 in the right eye and 20/30 in the left eye. On anterior segment exam, there was an area of conjunctival injection in the right eye localized to an exposed limbal suture. The cornea and lens were otherwise clear in both eyes. There was 4+ cell and 3+ flare in the anterior chamber of both eyes. In addition, 1+ anterior vitreous cell was seen in both eyes. Fundus exam revealed no chorioretinal lesions as well as normal optic nerves and vessels (Figure 1).
Images: Moon J, Rifkin L, Yoo S
What is your diagnosis?
Worsening after surgery
Acute bilateral simultaneous onset uveitis tends to occur in a younger subset of the population. The differential includes post-infectious uveitis, drug-induced uveitis, tubulointerstitial nephritis and uveitis (TINU) syndrome, and HLA-B27-associated uveitis. These are the more typical etiologies, but one should also consider sarcoidosis, syphilis and tuberculosis. In the pediatric age group, juvenile idiopathic arthritis-associated uveitis is also in the differential diagnosis. There had been no prior diagnosis of arthritis or history of joint pain in this patient.
The most common causes of acute bilateral simultaneous onset uveitis are post-infectious and drug-induced. Within the post-infectious category is post-streptococcal uveitis. This is usually bilateral and can have posterior involvement as well. It is associated with elevated serum ASO titers in 95% of cases. Our patient denied any recent illness, but it is important to note that in studies of patients with post-infectious uveitis, a significant number of patients did not recall any illnesses.
Frequent offenders in the drug-induced category include oral antibiotics and NSAIDs. Our patient did not have a history of taking any oral medications.
TINU is a rare entity that is diagnosed based on the presence of anterior uveitis and acute interstitial nephritis, diagnosed on histopathology or clinically, in the absence of other respective causes. Urine 2-microglobulin can be used as a screening test; it was elevated in 11 of 12 patients in a series of patients with biopsy-confirmed TINU. Our patient did not have any urinary symptoms.
HLA-B27-associated uveitis is usually a recurrent uveitis occurring in one eye at a time or asymmetrically; a bilateral simultaneous presentation is rare.
Sympathetic ophthalmia is on the differential because of the patient’s history of eye trauma. It classically presents with a bilateral granulomatous panuveitis with mutton-fat keratic precipitates, vitritis and multiple yellow-white choroidal lesions known as Dalen-Fuchs nodules. Advanced cases can present with bilateral serous retinal detachments.
Diagnosis and management
Sympathetic ophthalmia has been reported to develop anytime between 5 days to 66 years after the inciting event. Ninety percent of cases occur between 3 months and 1 year.
Based on the patient’s history of penetrating trauma to the right eye, sympathetic ophthalmia was the most likely diagnosis. Still, she was sent for bloodwork: ANA, FTA-ABS, basic metabolic panel, QuantiFERON, ACE, lysozyme, ASO titer, urinalysis and urine 2-microglobulin were normal or negative.
Fluorescein angiography showed areas of pinpoint hyperfluorescence in the periphery of the right eye in the late phases (Figure 2). Both Vogt-Koyanagi-Harada syndrome and sympathetic ophthalmia classically show multiple areas of pinpoint hyperfluorescence with late leakage that usually correspond to areas of serous retinal detachments.
Based on the negative workup for other etiologies of bilateral acute uveitis, the patient’s history of eye trauma 3 weeks prior, and the findings on exam and fluorescein angiography, the patient was diagnosed with sympathetic ophthalmia. She did not present with many of the classic features. Her inflammation was not granulomatous, and no choroidal lesions were seen. It may be that her diagnosis of sympathetic ophthalmia was discovered in its very early stages due to the patient’s frequent postoperative visits, before development of more significant posterior segment involvement.
The patient was started on oral prednisone 1 mg/kg/day at postoperative week 4, 1 week after the initial presentation of intraocular inflammation. The patient tolerated the systemic steroids well. Two weeks after starting oral prednisone (postoperative week 6), the patient showed significant improvement. Visual acuity in the right eye had improved from 20/70 to 20/30, and anterior chamber inflammation decreased from 4+ cell and 3+ flare in both eyes to 0.5+ cell and 0.5+ flare in the right eye and 1+ cell and 1+ flare in the left eye. At this point, a slow taper of oral prednisone was started, and steroid-sparing methotrexate therapy was initiated.
Discussion
Sympathetic ophthalmia is a disease with deep historical roots. Text from A.D. 1000 states, “(t)he right eye, when diseased, often gives suffering to the left.” It is a disease that famously rendered Louis Braille blind by the age of 5 years. However, for all its infamy, it is actually quite rare; the incidence is reported to be one in 10,000,000. Historically, the most common cause has been penetrating ocular trauma; however, recent data has found that intraocular surgery is becoming a more common cause.
In 1854, Dr. William Mackenzie, a Scottish ophthalmologist, described placing leeches on the conjunctiva to treat sympathetic ophthalmia in his textbook A Practical Treatise on the Diseases of the Eye. Fortunately, this did not become a popular mode of therapy. The standard of care became enucleation of severely injured eyes within 14 days of injury to prevent the development of sympathetic ophthalmia. Today, recommended treatment for sympathetic ophthalmia is initiation of systemic steroids with transition to steroid-sparing immunomodulatory therapy such as methotrexate or azathioprine.
There is little literature regarding the management of sympathetic ophthalmia in the pediatric population. Two recent studies demonstrated effectiveness of TNF-alpha antagonist therapy in children with refractory sympathetic ophthalmia and a case in which the patient was intolerant of steroid therapy. Animal models of sympathetic ophthalmia have shown upregulation of TNF-alpha levels, and so medications such as infliximab or adalimumab may have a role as targeted therapy.
The most important prognostic factor in sympathetic ophthalmia is the prompt initiation of systemic steroids at aggressive and adequate doses. Retrospective studies have concluded that prompt initial treatment significantly affects visual acuity outcomes. In fact, sympathetic ophthalmia has a fairly good prognosis today; a retrospective case series at the National Eye Institute of 32 cases of sympathetic ophthalmia found that 50% of cases had a final visual acuity of 20/40 or better.
Follow-up
Currently, the patient is off all steroids and maintained on 12.5 mg of weekly methotrexate with guidance from a pediatric rheumatologist. Visual acuity has improved to 20/25 in both eyes. The anterior chamber is deep and quiet with very rare anterior vitreous cell without haze in both eyes.
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- For more information:
- Jessica Moon, MD, Lana Rifkin, MD, and Sylvia Yoo, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.
- Edited by Gregory D. Lee, MD, and Nora W. Muakkassa, MD. They can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St, Box 450, Boston, MA 02111; website: www. neec.com.