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Leber's Hereditary Optic Neuropathy Masquerading As Retinal Vasculitis

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Leber's Hereditary Optic Neuropathy Masquerading As Retinal Vasculitis CASE REPORTS AND SMALL CASE SERIES of the choroid and retina, fluid The aspirate showed gram-positive Bacillus cereus posterior to the sclera, inflamed rods, and the culture revealed B ce- Endophthalmitis extraocular muscles, and a nondis- reus/Bacillus thuringiensis. Intrave- Secondary to Self-inflicted placed lens. nous vancomycin hydrochloride, Laser iridotomy, topical cy- ceftriaxone sodium, and clindamy- Periocular Injection cloplegics, and steroids failed to cin phosphate supplemented intra- reduce the intraocular pressure or vitreal injections of vancomycin and Endophthalmitis is an ocular emer- reverse the ocular inflammation ceftazidime. A normal echocardio- gency that can have a devastating out- (Figure 1). On the second day, an gram ruled out a cardiac source of come. The poor prognosis is often re- orbital computed tomography scan bacteria. On the fourth day, spon- lated to rapid progression of the demonstrated marked scleral thick- taneous scleral perforation oc- disease process and a relative delay ening, enlarged extraocular muscles, curred with extrusion of purulent in diagnosis due to the wide array of and subluxation of the lens uveal contents. The eye was eviscer- clinical symptoms and signs.1 Al- (Figure 2). Suspicion of endoph- ated. Weeks later, 2 prison guards though endophthalmitis is most of- thalmitis led to vitreous aspiration. reported to a case worker that prior ten related to surgical intervention, endogenous sources are identified in 2% to 15% of cases.1 Intravenous drug-related endophthalmitis is most commonly caused by Bacillus ce- reus.2,3 We report a case of B cereus endophthalmitis secondary to peri- orbital drug injection that resulted in spontaneous lens subluxation. This case underscores the difficulty in making an expeditious diagnosis when there is an uncertain medical history. Report of a Case. A 29-year-old male prison inmate had a 12-hour his- tory of vomiting associated with pain, redness, and impaired vision of the left eye. The patient denied trauma and drug abuse. Medical his- tory was remarkable for active in- terferon therapy for hepatitis C. The Figure 1. Scleritis (T sign) and normal lens position on day 1 (ultrasound courtesy of Laurie Barber, MD). right eye was normal. The left eye had light perception vision, 360° perilimbal injection, mid-dilated pu- A B pil, and dull red reflex. There were no signs of penetrating injury. The angle was closed with an intraocu- lar pressure of 61 mm Hg. A toxi- cology screen test result was posi- tive for amphetamine. An initial diagnosis of angle-closure glau- coma led to initiation of timolol ma- leate, brimonidine tartrate, acetazol- amide, and isosorbide dinitrate therapy that reduced the intraocu- lar pressure to 40 mm Hg. On the evening of admission, ocular ultra- sonography revealed thickening Figure 2. Axial (A) and coronal (B) computed tomographic scans show lens subluxation on day 2. (REPRINTED) ARCH OPHTHALMOL / VOL 118, NOV 2000 WWW.ARCHOPHTHALMOL.COM 1585 ©2000 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 to the episode the patient had been Corresponding author: Chris- nal blade to penetrate the arterial observed injecting drugs into the topher T. Westfall, MD, Department of wall at the central bifurcation of the periocular tissues. Ophthalmology, University of Arkan- retinal artery (Figure 1). No no- sas for Medical Sciences, 4301 W table hemorrhage was noted. A 50- Comment. To our knowledge, this MarkhamSt,MailSlot#523,LittleRock, gauge flexible stylet made of nickel is the first reported case of endoph- AR 72205 (e-mail: westfallchristopher titanium was extended from a 19- thalmitis secondary to presumed il- @exchange.uams.edu). gauge support shaft and used to licit periorbital drug injection and cannulate the CRA through the ar- 1. Okada AA, D’Amico DJ. Endogenous endoph- the first reported case of lens dislo- thalmitis. In: Albert DM, Jakobiec FA, eds. Prin- teriotomy site. Cannulation was con- cation associated with B cereus en- ciples and Practice of Clinical Ophthalmology. Vol firmed by the ease of passage of the dophthalmitis. Unreliable patient 5. Philadelphia, Pa: WB Saunders Co; 1994: 3120-3126, 3379-3380. stylet into the CRA. Approximately history delayed correct diagnosis and 2. Beecher DJ, Pulido JS, Barney NP, Wong AC. Ex- 3.5 mm of the stylet entered the contributed to a poor outcome, but tracellular virulence factors in Bacillus cereus en- CRA. The stylet was moved using retrospective review of the initial dophthalmitis: methods and implication of in- volvement of hemolysin BL. Infect Immun. 1995; forward, backward, and circular mo- signs and symptoms and subse- 63:632-639. tions, and then withdrawn. A small quent eyewitness reports led us to 3. Ullman S, Pflugfelder SC, Hughes R, Forster RK. amount of semiclotted blood Bacillus cereus panophthalmitis manifesting as suspect orbital injection. Inadvert- an orbital cellulitis. Am J Ophthalmol. 1987;103: emerged from the arteriotomy site. ent ocular penetration and intraocu- 105-106. The caliber of the superior retinal lar injection may have caused acute arteries increased partially after angle closure at the patient’s initial cannulation; however, the inferior visit. Periocular injection or direct retinal arteries remained severely inoculation of the vitreous body with narrowed. Because it was unclear a dirty needle in turn progressed to Vitreous Surgery how the procedure might affect the B cereus endophthalmitis that for Central Retinal risk of ocular neovascularization, pe- rapidly led to lens dislocation. Al- Artery Occlusion ripheral endophotocoagulation was though other mechanisms may re- performed prophylactically to mini- sult in endophthalmitis, the lack of Central retinal artery (CRA) mize the risk. systemic sepsis, other infectious occlusion is a devastating disease Postoperatively, the patient was sources, or obvious trauma near the for which conventional therapies given oral aspirin, 325 mg daily. Ex- globe make these etiologies less are often relatively ineffective.1 amination findings on day 2 showed probable. Our report emphasizes the Since CRA occlusion is usually a visual acuity of counting fingers at virulent nature of B cereus, which caused by a physical obstruction 1.8 m. The appearance of the reti- typically causes retinal necrosis and in the CRA, we propose a mechani- nal vessels had not changed signifi- retinal detachment1,2 due to the pro- cal means of relieving the obstruc- cantly since the procedure. By the duction of multiple toxins and en- tion. We postulate that cannula- next examination on day 10, the cali- zymes.2 The orbit should be recog- tion of the CRA with a stylet during ber of the retinal vessels had re- nized as a potential site for illicit drug vitreous surgery may disrupt the turned to normal (Figure 2). An- injection, a practice which carries the obstructive agent and restore blood giographic images revealed a normal risk of inadvertent intraocular in- flow.2 retinal circulation time. The latest oculation. Our report illustrates 3 follow-up examination findings at 4 important aspects of B cereus en- Report of a Case. A 68-year-old months showed a corrected visual dophthalmitis: (1) A high index of man with diabetes developed CRA acuity of 20/25 OS. The cherry-red suspicion is necessary for early de- occlusion in his left eye. On initial spot had disappeared. A laboratory tection; (2) B cereus must be con- examination, his best-corrected vi- evaluation revealed an atheroma- sidered in an individual who abuses sual acuity was counting fingers at tous plaque in the thoracic aorta. drugs; and (3) The rapid destruc- 0.5 m OS. Funduscopy revealed a tion caused by this highly virulent cherry red spot and severely nar- Comment. Cannulation of the CRA organism in endophthalmitis may rowed retinal arteries. At approxi- is a novel surgical approach that al- result in lens subluxation. mately 30 hours after the onset of lows direct mechanical access to the vision loss, conventional treat- site of obstruction. This method pre- Douglas M. Blackmon, BA ments had still resulted in no im- sents the advantage of avoiding the Harold M. Calvert, MD provement in vision. The patient risk of neurological complications Paul M. Henry, MD declined the option of selective associated with selective thromboly- Christopher T. Westfall, MD thrombolysis. The option of vitrec- sis.2-4 In this case, immediate im- Little Rock, Ark tomy with vessel cannulation and provement in blood flow was mod- thrombus disruption under local an- est. It seemed that the thrombus was esthesia was then offered. The pa- only partially disrupted. In the fu- We would like to acknowledge the con- tient understood the experimental ture, to relieve the obstruction more tributions of Rebecca Martin, MD, and nature of this treatment and gave his completely, one might consider us- the Infectious Disease Department at informed consent. ing a longer stylet or an infusion can- the University of Arkansas for Medi- After vitrectomy, one of us nula to deliver thrombolytic agents cal Sciences. (W.M.T.) used a microvitreoreti- into the CRA. The excellent recov- (REPRINTED) ARCH OPHTHALMOL / VOL 118, NOV 2000 WWW.ARCHOPHTHALMOL.COM 1586 ©2000 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 A B C Support Shaft Stylet Arteriotomy Site Figure 1. Intraoperative digitized photographs. A, An arteriotomy is made over the optic nerve with a microvitreoretinal blade. B,
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