American Academy of Ophthalmology Web Site: www.aao.org
The Saddest Diagnosis Is Confirmed
When we first saw Jen Hyatt,* we had no idea what we would be facing in the months to come. On this first visit, the pleasant 30-year-old woman, accompanied by her guide dog, seemed perfectly reliable and reasonable. Her vision was severely limited to light perception in her right eye and counting fingers in her left, with IOPs of 26 and 18 mmHg, respectively. The slit-lamp examination showed a clear cornea in the right eye with anterior and posterior synechiae, with the posterior synechiae located between the lens capsule and the iris. The left eye had a linear central scar in the cornea and iris due to sector iridectomy.
Ms. Hyatt was aphakic in both eyes, secondary to congenital cataracts, which had been removed before her first birthday. Her optic discs were perfused bilaterally. The view of the peripheral retina was difficult in both eyes because of posterior synechiae and poor dilation. B-scan ultrasonography was performed; this revealed that both retinas were attached but that there was a vitreoretinal adhesion in the nasal retina of the right eye.
At this point, we referred Ms. Hyatt to a glaucoma specialist for further evaluation and consideration of a tube shunt in her right eye. The tube shunt was placed approximately two months later; a vitrectomy was performed at that time. The procedure was successful, and Ms. Hyatt remained stable for the next five months. However, at that point, she was referred back to our clinic because of increased pain, swelling and erythema of the right eye. According to the referring ophthalmologist, the pain had hit four to five days earlier and did not respond to treatment.
A Hard Call
When we examined her, we found that Ms. Hyatt’s vision was stable with 3+ swelling, erythema and chemosis of the right eye. A B-scan showed posterior scleral thickening of 1.5 mm. We also observed black specks on her hard palate and along her tongue. A CT scan revealed preseptal cellulitis.
Ms. Hyatt was admitted and started on IV antibiotics. We consulted our otolaryngology colleagues, but they were unable to find a source of infection. We also consulted our infectious disease service, but the infectious workup was negative. Ms. Hyatt’s swelling, erythema and chemosis improved after three days, but her severe pain remained.
At this time—and after a long discussion—we made the decision to enucleate Ms. Hyatt’s right eye for the twin purposes of alleviating her pain and pinning down a diagnosis. Although Ms. Hyatt tolerated the procedure well, a definitive diagnosis was not forthcoming, as the pathology report was negative for an underlying infectious or neoplastic condition. The antibiotics were stopped, and Ms. Hyatt was discharged.
One week after the enucleation, Ms. Hyatt returned to our clinic. While she was waiting to be examined, Ms. Hyatt cried out that she had just rubbed her left eye and that doing so caused intense, immediate pain and decreased vision.
When we examined her, we noted a hyphema as well as vitreous extending to the area previously noted as a linear scar. She was taken immediately to surgery and a full-thickness corneal laceration was repaired (Fig. 1).
Five days later, she returned to the clinic complaining again of severe pain. Her IOP was 53 mmHg, and a B-scan showed posterior scleral thickening measuring 2 mm. Ms. Hyatt was started on oral steroids, and her pressure drops were increased. She was unable to tolerate the steroids; her pain increased; and the exam showed significant anterior and posterior scleritis. At this time, Ms. Hyatt requested that we remove her left eye. We attempted to reassure her and administered an intraocular injection of triamcinolone, and she was sent home.
Ten days later, Ms. Hyatt showed up in our emergency room, again complaining of severe pain in her left eye. The exam showed mild swelling and erythema of the lids and 2+ chemosis of the left eye. A CT scan found preseptal cellulitis and gas in the left globe and surrounding tissue as well as retrobulbar fat stranding (Fig. 2). A B-scan again showed scleritis.
Ms. Hyatt was admitted and IV antibiotics were initiated. However, the infectious disease and rheumatology workups were negative, and our colleagues had no further recommendations. Ms. Hyatt’s symptoms improved; the IV antibiotics were stopped after 72 hours; and she was discharged home on oral antibiotics.
Collapse and Confrontation
Two days after this discharge, Ms. Hyatt’s husband found her unresponsive in their home and called an ambulance. She was initially taken to a different ER and later transferred to our hospital.
When we saw her, Ms. Hyatt complained of severe pain in her left eye. Her IOP was initially elevated at 46 mmHg; it dropped to 28 mmHg after we instilled medication. She had mild periorbital edema and erythema and mild chemosis. A B-scan showed scleritis and a CT scan showed resolution of global air but increased retrobulbar air as well as progression of fluid and fat stranding.
At this point, we readmitted her to our hospital, and IV antibiotics were initiated. We performed an orbital biopsy; the results showed only chronic inflammation. Once again, the infectious disease workup was negative; as a result, our infectious disease and rheumatology colleagues recommended stopping the antibiotics. IV steroids were started, and Ms. Hyatt’s symptoms again improved.
After her fifth day in the hospital, a home health aide—who had found a journal kept by Ms. Hyatt—alleged that our patient had been injecting her eye with an insulin syringe and needles used for her guide dog’s diabetes. Initially, Ms. Hyatt denied knowingly doing this but stated that she might have done it unknowingly. Eventually, she told the truth.
At this point, we requested a psychiatry consult. As the psychiatry service was unable to treat her as an inpatient, we discharged her from the hospital on a steroid taper and glaucoma medications after she agreed not to harm herself.
Ms. Hyatt began working with a community psychiatrist. At her eight-month follow-up visit with us, her vision was stable at hand motions, and her IOP remained under control.
Rare and Challenging
This case was a significant challenge for all involved. While self-inflicted eye injuries are relatively rare, a high degree of suspicion is warranted when, as in our case, the clinical picture does not correlate with the results of diagnostic testing.
Ocular self-mutilation encompasses a wide range of outcomes, from minor abrasions to self-enucleation. Any part of the globe may be affected, from the ocular surface and anterior segment to the posterior segment. Most self-inflicted ocular injuries occur in people who have acute or chronic psychoses, with chronic schizophrenia being the most common underlying condition.1 Young to early middle-aged males are most often affected, and the behavior usually occurs during hallucinations.1 Successful treatment often necessitates a team effort involving multiple specialists.
To date, the motivation behind our patient’s self-mutilation has not been revealed. She continues to see the psychiatrist.