Miliary Tuberculosis Terior Segment Examination Find- Corresponding Author: Emmett T

CASE REPORTS AND SMALL CASE SERIES

a neighbor, who dialed 911, and the 48 hours the wound failed to seal, Boa Constrictor Bite police arrived. The policeman who so the patient was taken to the op- to the Eye answered the call, however, was erating room where 2 No. 11-0 ny- ophidiophobic (fear of snakes) and lon sutures were sewn in place. The Ocular injury to the eye from snake was unable to lend assistance. The patient was discharged from the hos- bite is extremely rare with few cases fire department arrived shortly there- pital after receiving a 72-hour course being reported in the literature. We after, and a fireman, using a large of prophylactic antibiotics with no report the case of man who sus- knife, cut the snake’s head from its signs of infection. Three months tained a penetrating injury to the eye body. The multiple small recurved postoperatively, the patient’s best- from a snake bite. teeth could not be disengaged, so the corrected visual acuity is 20/25 OD patient was transferred to a nearby (Figure 3). Report of a Case. An 18-year-old emergency department with the On arrival at the hospital, cul- man was bathing his pet snake, a snake’s head attached to his eye. The tures were made from the teeth of 6-ft-long North Brazilian boa con- attending physician removed the the snake, and numerous species of strictor (Boa constrictor), when it at- snake’s head from the patient, gram-negative rods were identi- tacked him and bit him on the right and diagnosed a ruptured globe. fied. No further attempt to classify eye. The snake had infectious sto- Photographs were obtained before the bacteria was made, as the pa- matitis, a bacterial infection in the the snake’s head was removed tient did not develop an infection. mouth. When the snake struck, the (Figure 1). After receiving1gof patient partially blocked the attack the ampicillin and sylbactam so- Comment. In the United States, ap- with his right hand; however, the dium combination drug (Unasyn) proximately 50 000 people per year intravenously, the patient was trans- are bitten by snakes, most of which snake was able to engage the pa- 1 tient’s right eye with its lower teeth, ferred to our institution with a shield are nonvenomous. We reviewed and his hand with its upper teeth. over his right eye while awaiting de- MEDLINE from 1966 to the pre- finitive treatment. The head of the sent and found only 2 cases of snake- It would not release its bite and tried 2,3 to wrap around the patient’s neck. snake was sent in a specimen bag bites to the eye. Both patients were The patient managed to telephone (Figure 2). bitten by venomous snakes and both When the patient arrived, he were children. One patient eventu- was in minimal discomfort. His eye- ally required enucleation and the lids were mildly swollen on the right other recovered. The patient who re- side. There was a puncture wound covered was bitten on the medial in the right upper eyelid. Visual acu- canthus and did not suffer a pen- ity was 20/50 OD. Three small punc- etrating ocular injury. ture wounds were noted in the cor- Infectious stomatitis is a rela- nea; 2 were Seidel positive. A small tively common infection in captive conjunctival puncture wound was snakes.4 This disease is known to oc- also noted in the inferonasal quad- cur when snakes are stressed envi- rant. The anterior chamber was deep ronmentally by poor husbandry. The and fibrin was adherent to the in- most common predisposing cause is ternal surface of the wound. The not providing the snake its pre- retina and vitreous humor ap- ferred optimal temperature zone, peared normal. Initial management which decreases the effectiveness of consisted of a bandage contact lens the animal’s immune system and al- and topical and intravenous antibi- lows opportunistic pathogens to otics; ofloxacin was applied topi- cause disease. Gram-negative organ- cally every hour, and standard doses isms such as Pseudomonas, Salmo- of intravenous vancomycin hydro- nella, Klebsiella, and Peromonas spe- chloride, ceftazidime, and clinda- cies are frequently implicated. A mycin were given. The next day, the culture made from the boa constric- chamber had shallowed, and 1 tor’s mouth from this case yielded Figure 1. Patient with snake’s head attached to wound remained Seidel positive. multiple organisms consistent with eye and hand. Glue was applied, but over the next bacterial stomatitis.

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/28/2021 Figure 2. Close-up of snake’s head. Snakes have 6 rows of teeth: 1 row in Figure 3. Slitlamp photograph of cornea. Two No. 11-0 nylon sutures can be each of the lower jaws (since they have a left and a right mandible) and 2 seen (original magnification ϫ10). rows on each side of the maxillary jaw. All of the teeth are similar in size and shape. Snake teeth are continuously replaced (original magnification ϫ2).

Most snakebites probably do 4. Draper CS, Walker RD, Lawler HE. Patterns of Report of a Case. A 30-year-old man oral bacterial infection in captive snakes. JAm not need prophylactic antibiotics. In Vet Med Assoc. 1981;11:1223-1226. came to our institution with severe studies on snakebites, fewer than 5% pain and irritation in the right eye. of patients had resultant infec- One month previously he had sus- tions.1 Nevertheless, we treated our tained corneal abrasion to the right patient with a 72-hour course of pro- eye from fiberglass while working on phylactic antibiotics that was initi- Amebic Keratitis Due to his boat and subsequently self- ated almost immediately. The con- Vahlkampfia Infection irrigated the eye with tap water. He cern for infection in this case was Following Corneal Trauma was then seen by his private oph- considerable, because the snake had thalmologist and was treated with a bacterial stomatitis, the patient’s cor- Acanthamoeba keratitis occurs in as- combination ointment of neomy- nea was punctured, and endoph- sociation with contact lens use, mi- cin sulfate, polymyxin B sulfate, and thalmitis is potentially devastating nor corneal trauma, or contact of the bacitracin zinc (Neosporin, Glaxo to vision. We believe that prophy- eye with contaminated water. It is a Wellcome Inc, Research Triangle lactic antibiotics may be indicated for relatively uncommon but poten- Park, NC) after yields from a cor- snakebites when the development of tially sight-threatening keratitis. In neal culture were examined. No or- an infection would have very seri- mild to moderate cases, medical ganism was isolated from the ini- ous consequences, such as with a treatment alone can eradicate the in- tial corneal cultures. No notable bite to the eye. fection; however, in severe cases improvement of his symptoms and surgical treatment is usually re- corneal findings was noted after 1 1 David M. Kleinman, MD quired. Other members of the month. He was referred to our in- Eileen F. Dunne, MD ameba family rarely cause ocular institution for further evaluation. On Michael J. Taravella, MD fection. A case of contact lens– ophthalmic examination, his best- Denver, Colo related amebic keratitis due to a corrected visual acuity was 20/50 OD mixed infection of Vahlkampfia and and 20/20 OS. Intraocular pressure Hartmannella was recently re- was normal in both eyes. Slitlamp Reprints: Michael J. Taravella, MD, 2 University of Colorado School of Medi- ported. Both patients were contact examination of the right eye re- cine, 4200 E Ninth Ave, Campus Box lens wearers. vealed a midstromal infiltrate with B-204, Denver, CO 80262. We report herein the second an overlying epithelial defect and lo- case of amebic keratits secondary to- calized corneal edema (Figure 1). 1. Weed HG. Nonvenomous snakebite in Massa- Vahlkampfia infection. To our No other ocular abnormality was chusetts: prophylactic antibiotics are unneces- knowledge, this is the first case of noted. Corneal scrapings were ob- sary. Ann Emerg Med. 1993;22:220-224. 2. Gupta M, Sharma P, Jain A, et al. Unusual site amebic keratitis following minor cor- tained for culture and the patient was of a snake bite. Trop Doct. 1995;25:134-135. neal trauma in a patient who did not given empiric treatment with cipro- 3. Brandao EO, de Bastos HC, Nishioka S de A, et wear contact lenses , and the first re- floxacin under the assumption that al. Lance-headed viper (Bothrops moojeni) bite wounding the eye. Rev Inst Med Trop Sao port of a non-Acanthamoeba ame- he had bacterial keratitis. The pa- Paulo.1993;35:381-383. bic keratitis in the United States. tient continued to have persistent in-

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/28/2021 Figure 1. Clinical appearance of the Vahlkampfia keratitis. Figure 2. Giemsa stain of corneal scraping shows free-living ameba morphologically consistent with a Vahlkampfia cyst (original magnification ϫ1000).

traocular inflammation with stro- and it belongs to the same family as port in the United States of kerati- mal infiltrates. Two weeks later, a Naegleria.3 Two cases of encephali- tis due toan ameba other than Acan- second set of scrapings was ob- tis presumably due to Vahlkampfia thamoeba, and the first case not tained, and the patient’s treatment have been reported.2 The first case associated with contact lens usage. was switched to fortified vancomy- of Vahlkampfia keratitis was in a 24- cin hydrochloride and ceftazidime. year-old contact lens wearer from George Alexandrakis, MD Yields from routine culturing of both whom amebas were isolated from the Darlene Miller, MA, MPH sets of corneal scrapings we ob- corneal biopsy tissue, contact lens, Andrew J. W. Huang, MD, MPH tained were negative for organ- case, and home water supply.2 He Miami, Fla isms. Despite intensive antibiotic was treated with amphotericin B; the treatment, he remained symptom- combination ointment of neomy- Corresponding author: Andrew J. W. atic. One week later, yields from an cin sulfate, polymyxin B sulfate, and Huang, MD, MPH, Bascom Palmer agar-agar culture obtained from a bacitracin zinc, and propamidine. Eye Institute, 900 NW 17th St, Mi- third set of corneal scrapings re- However, eventual penetrating kera- ami, FL 33136 (e-mail address: vealed a moderate amount of cysts toplasty secondary to central cor- [email protected]). and trophozoites. The organisms neal scarring was required. In our 1. Auran JD, Starr MB, Jakobiec FA. Acantham- were also identified by Giemsa stain case, corneal abrasion and self- oeba keratitis: a review of the literature. Cornea. (Figure 2) in both scraping and cul- irrigation with tap water predis- 1987;6:2-26. 2. Aitken D, Hay J, Kinnear FB, Kirkness CM, Lee ture yields. They appeared smaller posed the patient to such an un- WR, Seal DV. Amebic keratitis in a wearer of dis- and morphologically distinct from usual infection. The Vahlkampfia was posable contact lenses due to a mixed Vahl- Acanthamoeba and were identified as finally isolated only after repeated kampfia and Hartmannella infection. Ophthal- mology. 1996;103:485-494. Vahlkampfia. The patient subse- corneal scrapings, emphasizing the 3. John DT. Opportunistically pathogenic free- quently began receiving routine an- difficulty in culturing these organ- living amebae. In: Kreier JP, Baker JR, eds. Para- tiamebic treatment that included isms. Given the difficulty growing sitic Protozoa. Vol 3. 2nd ed. Orlando, Fla: Aca- demic Press Inc; 1993:143-246. propamidine; polyhexamethyl bi- and identifying these amebic organ- 4. Byers TJ, Gast RJ. DNA probe design for Acan- guanide (Bacquacil); the combina- isms, it is impossible to know the thamoeba and other pathogenic free-living amoe- rion ointment of neomycin sulfate, prevalence of non-Acanthamoeba bas. Invest Ophthalmol Vis Sci. 1994;35:2150. polymyxin B sulfate, and bacitracin amebic keratitis. Special tech- zinc; and clotrimazole every 2 hours niques such as the polymerase chain with rapid resolution of symptoms. reaction or organism-specific im- He was maintained on a regimen of munohistochemistry may facilitate Postoperative promamidine (Brolene) and Bac- identifying this kind of unusual ame- Streptococcus pneumoniae quacil for a month. His final visual bic keratitis.4 Our patient’s rapid re- Endophthalmitis acuity was 20/20 with complete reso- sponse to the standard treatment for Complicated by Meningitis lution of the stromal infiltrate. Acanthamoeba keratitis indicates that Vahlkampfia is also sensitive to these The most common causes of bacte- Comment. Vahlkampfia is a free- medications. To the best of our rial endophthalmitis are ocular sur- living ameba found in water and soil, knowledge, this case is the first re- gery, usually cataract extraction, and

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/28/2021 endogenous spread from other infec- tamicin sulfate (0.4 mg), and dexa- lococcus epidermidis, Staphylococ- tions such as meningitis, abdomi- methasone phosphate (0.4 mg), as cus aureus, and various Streptococcus nal infection, endocarditis, and uri- well as topical vancomycin (50 mg/ species, including S pneumoniae.3,4 nary tract infection.1 The clinical mL), tobramycin sulfate (14 mg/ Endophthalmitis resulting from course is highly variable and de- mL), and 1% prednisolone acetate. gram-negative organisms and fungi pends on the virulence of the infect- No systemic antibiotics were given. generally predisposes to an unfavor- ing organism, the quickness of di- Ocular and orbital ultrasonogra- able clinical outcome. When com- agnosis and administration of phy were performed and showed pared with endophthalmitis due to antibiotics, and the patient’s under- that no retinal detachment had oc- other gram-positive organisms, in- lying medical condition. Accord- curred and no obvious extraocular fection by nonviridans Streptococ- ing to the Endophthalmitis Vitrec- inflammation was present. Cul- cus species appears to result in the tomy Study2 conducted between tures from the donor corneal rim and worst clinical outcome by a signifi- 1990 and 1994, which studied the the vitreous humour both grew S cant margin.4 This relatively poor role of immediate vitrectomy and of pneumoniae sensitive to penicillin, outcome may be due to the greater intravenous antibiotics in the man- clindamycin, and erythromycin. degree of inflammatory response agement of postoperative bacterial On the seventh hospital day, evoked by streptococcal exotoxins endophthalmitis, on post hoc data the patient suddenly became con- and enzymes. analysis, there was no difference in fused and agitated and was found to In addition, there may be in- visual outcome whether or not an have a stiff neck, a temperature of creasing reports of Streptococcus bac- immediate vitrectomy was per- 38.9°C, and to be tachycardic. Her teremia and secondary endophthal- formed, except in a selected sub- white blood cell count was 18.6 ϫ mitis, particularly in patients with group of patients. Furthermore, no 109/L. A lumbar puncture was per- underlying medical conditions such ocular benefit was derived from the formed that showed pink, turbid ce- as diabetes mellitus, malignant neo- administration of systemic antibiot- rebrospinal fluid containing high plasms, and human immunodefi- ics. We reviewed the medical rec- levels of protein and abundant neu- ciency virus infection.5 While the ord of a patient who developed men- trophils and erythrocytes, as well as Endophthalmitis Vitrectomy Study2 ingitis following a postoperative case gram-positive diplococci. A diagno- clearly showed no ocular benefit of Streptococcus pneumoniae endoph- sis of meningitis secondary to en- from systemic antibiotics overall, thalmitis. Since the advent of anti- dophthalmitis was made and a regi- there may have been specific causal biotics, no other cases have been re- men of intravenous penicillin was subgroups that would derive ben- ported of systemic spread from a started. Blood cultures obtained at efit from intravenous antibiotics; primary exogenous bacterial en- that time later confirmed the pres- however, data were insufficient for dophthalmitis, to our knowledge. ence of S pneumoniae sensitive to drawing statistical inferences. Fur- penicillin and chloramphenicol. Ce- thermore, the study was designed to Report of a Case. An 81-year-old rebrospinal fluid cultures yielded no determine if there were any ocular, woman with Fuch corneal dystro- bacterial growth. The patient recov- not systemic, benefits to using in- phy complained of severe pain in her ered quickly once the intravenous travenous antibiotics. left eye 1 day following a penetrat- antibiotics were given and was in her Patients with bacterial endoph- ing keratoplasty and cataract extrac- normal neurological state the next thalmitis should be observed closely tion. The donor cornea was from a day. for signs or symptoms of meta- 3-year-old child who had drowned. During the patient’s course in static spread. Although postopera- On examination, she had hand mo- the hospital, the vision in her left eye tive bacterial endophthalmitis is typi- tions/light perception only visual did not improve and her intraocu- cally confined to the eye, this case acuity, an intraocular pressure of 49 lar pressure continued to rise de- report indicates that it is possible for mm Hg, conjunctival chemosis, ke- spite maximal therapy. Magnetic the infection to spread to the cen- ratic precipitates on the corneal graft, resonance imaging of the head was tral nervous system as well as other a heavy cellular reaction in her an- performed and ruled out any fur- areas of the body. Aggressive treat- terior chamber, and a 4+ vitreous re- ther intraorbital or intracranial ab- ment of endophthalmitis, possibly action that obscured retinal details scesses. A repeated orbital ultrasono- including intravenous antibiotics, in her left eye. The results of the re- gram showed dense vitreous may be considered in cases of par- mainder of her physical examina- opacities, vitreous membrane forma- ticularly virulent pathogens and in tion were normal. At this time, it was tion, and partial posterior vitreous de- patients with high-risk medical con- thought that she had postoperative tachment. After she was pro- ditions. endophthalmitis, and after consul- nounced medically stable with her tation with physicians from both the meningitis resolving uneventfully, a Stanley M. Chan, BSc retina and uveitis services, she was vitrectomy was performed, al- William G. Hodge, FRCSC admitted for a vitreous tap and in- though her visual acuity never im- Brian C. Leonard, FRCSC travitreal injection as well as being proved. Ottawa, Ontario started on a regimen of fortified topical antibiotics. She was treated with Comment. Most cases of bacterial Corresponding author: William G. an intravitreal injection of vanco- endophthalmitis are caused by gram- Hodge, FRCSC, University of Ot- mycin hydrochloride (1 mg), gen- positive organisms, notably Staphy- tawa Eye Institute, 501 Smyth Rd,

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/28/2021 Figure 2. Computed tomographic scan of the brain shows multiple enhancing lesions (arrows).

improvement of mental status and visual acuity with continued Figure 1. Chest x-ray film shows fine miliary opacities involving all lung fields. treatment. Mycobacterium tuberculosis is the most common infectious cause of death worldwide, accounting for Ottawa, Ontario, Canada K1H 8L6 graphic scan of the head revealed almost 10 million fatalities per (e-mail: [email protected]). prominent meningeal vascularity year.1 Recent immigrants to the and multiple supratentorial and in- United States appear to be at par- 1. Greenwald MJ, Wohl LG, Sell CH. Metastatic bac- fratentorial enhancing lesions ticularly high risk of infection.2 terial endophthalmitis: a contemporary reap- (Figure 2). A lumbar puncture praisal. Surv Ophthalmol. 1986;31:81-101. Tuberculous multifocal choroiditis, 2. Endophthalmitis Vitrectomy Study Group. Re- specimen contained 618 white blood sults of the Endophthalmitis Vitrectomy Study: although uncommon, is well rec- cells, of which 0.84 were neutro- ognized,3 and can support the diag- a randomized trial of immediate vitrectomy and phils; 0.12, monocytes; and 0.04, of intravenous antibiotics for the treatment of nosis of miliary, or disseminated, postoperative bacterial endophthalmitis. Arch lymphocytes. The diagnosis was pre- disease as was observed in our Ophthalmol. 1995;113:1479-1496. sumed Mycobacterium tuberculosis 3. Han DP, Wisniewski SR, Wilson LA, et al. Spec- patient. trum and susceptibilities of microbiologic iso- infection and the patient was admit- lates in the Endophthalmitis Vitrectomy Study. ted for therapy with 4 drugs that Ajita Grewal, MD Am J Ophthalmol. 1996;122:1-17. included isoniazide, rifampin, eth- 4. Shrader SK, Band JD, Lauter CB, Murphy P. The Robert Y. Kim, MD clinical spectrum of endophthalmitis: inci- ambutol hydrochloride, and pyrizin- dence, predisposing factors, and features influ- Emmett T. Cunningham, Jr, imide. On the second hospital day, MD, PhD, MPH encing outcome. J Infect Dis. 1990;162:115-120. the ophthalmology service was asked 5. Nagelberg HP, Petashnick DE, To KW, Wood- San Francisco, Calif come HA Jr. Group B streptococcal metastatic to see the patient because of blurred endophthalmitis. Am J Ophthalmol. 1994;117: vision of 2 months’ duration. The pa- 498-500. tient was lethargic, with a best- This study was supported in part by corrected visual acuity of 20/40 in an unrestricted grant from Research each eye. No afferent pupillary de- to Prevent Blindness, Inc, New York, fect was present. External and an- NY. Miliary Tuberculosis terior segment examination find- Corresponding author: Emmett T. ings were normal. Fundus Cunningham, Jr, MD, PhD, The Fran- A 31-year-old male-to-female trans- examination findings revealed mul- cis I. Proctor Foundation, University of sexual prostitute, a recent immi- tiple choroidal infiltrates involving California, San Francisco, School of grant from Mexico, came to the the posterior pole in each eye Medicine, San Francisco, CA 94143- emergency department disoriented (Figure 3, A, B). Serial fluores- 0944 (e-mail: [email protected]). and with an elevated temperature. cein angiography showed early Medical history was notable for re- blockage and late staining of these 1. Estimates of future global tuberculosis morbid- cent purified protein derivative posi- lesions (Figure 3, C-G). Cultures ity and mortality. MMWR Morb Mortal Wkly Rep. tivity on skin testing. A chest x-ray from sputum and cerebrospinal fluid 1993;42:961-965. film at examination showed fine (Figure 4) grew M tuberculosis. The 2. Tuberculosis morbidity—1995. MMWR Morb Mortal Wkly Rep. 1996;45:365-370. miliary opacities in all lung fields patient showed slow resolution 3. Helm CJ, Holland CN. Ocular tuberculosis. Surv (Figure 1). A computed tomo- of the multifocal choroiditis and Ophthalmol. 1993;38:229-256.

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/28/2021 Figure 3. Color fundus photographs of the right (A) and left (B) eyes show bilateral, multifocal choroiditis (arrowheads). Serial fluorescein angiographic photographs (C-F) show early blocking hypofluorescence and late-staining hyperfluorescence corresponding to areas of choroidal infiltrate, as well as mild, late leakage from the optic nerve heads in each eye.

Figure 4. High-power, brightfield photomicrograph shows typical cording of Mycobacterium tuberculosis organisms grown in culture from cerebrospinal fluid (Kinyous acid-fast stain,original magnification ϫ400).

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/28/2021 Oculocardiac Reflex Caused by Orbital Floor Trapdoor Fracture: An Indication for Urgent Repair

The oculocardiac reflex is a triad of bradycardia, nausea, and syncope. The ocular causes are numerous.1 Orbital causes also exist.2-4 The ophthalmic division of the trigeminal nerve is the afferent limb. The im- pulses pass through the reticular for- mation to the vagus nerve’s visceral motor nuclei. The efferent limb mes- sage is carried by the vagus nerve to the heart and stomach. We report 3 cases of orbital Figure 1. Patient 1. Top, Preoperatively, the patient could not depress the right eye completely. Results of forced duction tests revealed a restrictive cause. Bottom, Postoperatively the patient was able to fully floor fractures that entrapped the in- depress the right eye and denied diplopia. ferior rectus muscle and/or the orbital connective tissue to immediately produce the oculocardiac reflex Postoperatively, his pulse rate in 1 case and was highly suggestive was 86/min with a blood pressure of in 2 others. To our knowledge, there 121/63 mm Hg. The nausea and have been no previous reports of vomiting subsided. Initially there oculocardiac reflex caused by incar- was mild limitation with depres- ceration of orbital soft tissue in an sion. Thirteen months after sur- orbital trapdoor fracture. gery the patient was orthophoric (Figure 1, bottom). Report of Cases. Case 1. An 8-year- Case 2. A 12-year-old boy was old boy who was pushed and hit the hit with a brick in the right perior- right side of his face, immediately de- bital region,which causied binocu- veloped diplopia, nausea, and vom- lar vertical diplopia, nausea, and Figure 2. Coronal computed tomographic scan iting. In the emergency depart- vomiting. In the emergency depart- for patient 2 showing a nondisplaced orbital ment, his pulse rate was 58/min with ment his pulse rate was 36/min floor fracture with incarceration of the inferior rectus muscle in the maxillary sinus. No air-fluid a blood pressure of 111/56 mm Hg. with a blood pressure of 115/70 level or submucosal hemorrhage is present. The results of ocular examination mm Hg. Results from an electro- were normal except for periorbital cardiogram revealed bradycardia ecchymosis and limited vertical gaze along with some QRS complexes but after delivery from the maxil- that was worse with depression. Mo- missing P waves. The ocular lary sinus, it became perfused. The tility measurements revealed a 3– examination findings were normal floor defect was repaired with a po- prism diopter (PD) right hypertro- except for ecchymosis on the right rous polyethylene sheet. Findings pia (Figure 1, top). The computed cheek, a 9-PD right hypotropia from repeated forced duction tests tomographic scan revealed a non- with limitation in upgaze, and revealed no restriction. displaced right orbital floor frac- abnormal vertical forced duction Postoperatively, his pulse rate ture with incarcerated orbital soft tis- test results. The maxillary nerve remained at 60 to 70/min and all sue in the maxillary sinus and sensation was symmetrically intact. QRS complexes were associated with entrapment of the inferior rectus The computed tomographic scan P waves. The nausea and vomiting muscle. revealed a nondisplaced right subsided. The extraocular motility The patient was taken to the orbital floor fracture with the right examination findings revealed mod- operating room 2 days later where inferior rectus muscle entrapped in erate inability to depress the right an abnormal forced duction was con- the maxillary sinus (Figure 2). globe. He was discharged and the firmed. During surgery the orbital The patient was taken to the diplopia resolved. connective tissue septae and fat were operating room 7 hours later. Intra- Case 3. A 20-year-old man freed from the fracture. The infe- venous atropine sulfate was given walked into a metal pipe striking his rior rectus muscle was not directly prior to induction. His pulse rate left cheek, which caused immedi- visualized. The fracture site was re- ranged from 70 to 110/min. A por- ate pain, nausea, and vertical bin- paired with a shave of cranial bone. tion of the orbital floor was re- ocular double vision. His ocular ex- The forced duction test was removed to allow extrication of the in- amination findings were normal peated at the completion of the sur- ferior rectus muscle. The inferior except for limitation of both up- gery and results were normal. rectus muscle appeared ischemic, gaze and downgaze in the left eye.

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/28/2021 In primary gaze, he demonstrated a tive tissue, septae, and/or the infe- the development of Leber’s heredi- 6-PD left hypotropia. The com- rior rectus muscle in the maxillary tary optic neuropathy (LHON) for puted tomographic scan revealed a sinus. This leads to restriction of mo- 6.5 years. Both brothers were found nondisplaced left orbital floor frac- tility, and the stimulation of the oph- to harbor the identical homoplas- ture with soft tissue herniation into thalmic division of the trigeminal mic 4216, 13708, and 11778 mito- the maxillary sinus that caused en- nerve triggers the oculocardiac re- chondrial DNA mutations. Twin A trapment of the inferior rectus flex. developed bilateral optic neuropa- muscle. A patient experiencing the triad thy at the age of 34 years and had a Three days later, the patient of bradycardia, nausea, and syn- visual acuity of 1/200 OU. At the was taken to the operating room. cope following orbital injury should time of the report, twin B was 41 His preoperative pulse rate was 58/ immediately undergo computed to- years old and still had a visual acu- min; and his blood pressure, mography with coronal sections. If ity of 20/15 OU with no signs or 132/72 mm Hg. An abnormal a trapdoor fracture with incarcera- symptoms of LHON. In this cur- forced duction was noted on test- tion of soft tissue is identified, the rent report, updated information on ing. Entrapped fatty connective tis- fracture should be repaired the same twin B is provided and indicates that sue as well as muscular tissue were day. Timely fracture reduction and twin B has subsequently developed released from the fracture line. The release of the muscle and/or con- bilateral LHON. muscle contained a hematoma nective tissue protect against not with signs of ischemic necrosis. only life-threatening cardiac arrhyth- Report of a Case. Twin B, now 45 The fracture was repaired with a mias but also ischemic necrosis of years old, had remained healthy and Silastic sheet. Forced duction test the muscle that may lead to fibrosis visually asymptomatic until he noted results were normal. His postop- and permanent restrictive strabis- painless, blurred vision while driv- erative pulse rate was 76/min with mus. ing on the morning of September 23, a blood pressure of 144/93 mm Hg. 1997. The visual loss first occurred The nausea resolved. Bryan S. Sires, MD, PhD in the left eye and then in the right Seven months after surgery, Robert B. Stanley, Jr, MD, DDS eye 1 hour later. The patient re- diplopia of more than 20° from pri- Seattle, Wash ported no trauma, recent illness, mary persisted in extreme upgaze Lawrence M. Levine, MD weight change, or any unusual en- and downgaze. Gainesville, Fla vironmental exposure. He has continued to smoke 1 pack of ciga- Comment. The association of an Supported in part by a departmental rettes daily and to drink 24 beers oculocardiac reflex with an orbital award from Research to Prevent Blind- weekly for the past 25 years. He was fracture is rare, but prompt identi- ness, Inc, New York, NY. a firefighter but had not worked in fication and treatment are impor- Reprints: Bryan S. Sires, MD, this capacity for 2 years. He has been tant. The risk of a fatal cardiac ar- PhD, Harborview Medical Center, a part-time painter and has used la- rhythmia exists (1:3500) with the tex paint for many years. His only 5 Ophthalmology, Box 359894, Se- oculocardiac reflex. In addition, the attle, WA 98104-9894 (e-mail: medication was Ex-Lax twice a release of the extraocular muscle is [email protected]). month for constipation since age 38. critical for functional outcome. Hy- He has experienced much stress in poxia of the muscle was noted in pa- 1. Anderson RL. The blepharocardiac reflex. Arch the past year due to an ongoing liti- tient 2 when intervention was un- Ophthalmol. 1978;96:1418-1420. gation against his former employer 2. Strortebecker TR. Posttraumatic oculocardiac dertaken 7 hours from time of the syndrome from a neurosurgical point of view. for a nonphysically related matter. injury. Ischemic necrosis of the J Neurosurg. 1953;10:682-686. His daily activity has remained un- 3. Garrity JA, Yeatts RP. The oculocardiac reflex muscle was seen in patient 3 with a with an orbital tumor. Am J Ophthalmol. 1984; changed except for starting an ex- 72-hour delay prior to repair. Pa- 98:818. ercise regimen 1 month prior to the tient 2 had no diplopia 6 weeks af- 4. Chesley LD, Shapiro RD. Oculocardiac reflex dur- visual loss. His typical routine was ing treatment of an orbital blowout fracture. ter surgery while patient 3 contin- J Oral Maxillofac Surg. 1989;47:522-523. a 1-mile light run, 45 sit-ups, and 30 ued to have diplopia in extreme 5. Mendelblatt FI, Kirsch RE, Lemberg L. Study push-ups twice a week. upgaze and downgaze 7 months comparing methods of preventing oculocar- On examination, best visual diac reflex. Am J Ophthalmol. 1962; 53: 506-512. later. This suggests that immediate acuity was count fingers at 4 repair leads to a better motility prog- inches OU. Pupillary reaction in nosis. both eyes was sluggish to light Orbital trapdoor fractures are with no relative afferent pupillary rare, occurring in children and defect. Goldmann perimetry dem- young adults. This may be because Identical Twins No Longer onstrated large central scotomas in of elasticity of the orbital bone in this Discordant for Leber’s both eyes. Intraocular pressures age group, which allows the bone to Hereditary Optic were 17 mm Hg OU. The optic snap into position after the soft tis- Neuropathy nerves were hyperemic with a cup- sue has prolapsed through the frac- disc ratio of 0.5 and no peripapil- ture. Typically, they are seen as In 1993, Johns and colleagues1 de- lary vascular telangiectasia. The small, nondisplaced fractures, with scribed 2 monozygous twin broth- maculae and retinal vasculature incarceration of the orbital connec- ers who had remained discordant for appeared normal.

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/28/2021 Comment. Updated information in- had not worked as a firefighter for 2 LHON are variable and may be nu- dicates that the discordance for years and reported no recent expo- merous. LHON reported previously for a pair sure to smoke. The only recent Byron L. Lam, MD of monozygous twin brothers1 is change in his routine was starting a Miami, Fla temporal and not absolute. The dis- light exercise regimen 1 month prior cordance of the onset of LHON is 11 to the visual loss. He did, however, Corresponding author: Byron L. Lam, years, and the phenotypic findings report experiencing much stress for MD, 900 NW 17th St , Miami, FL are similar. more than a year due to ongoing liti- 33136. Potential mitigating factors such gation. Interestingly, both twins as excessive tobacco and alcohol use noted the onset of visual loss while 1. Johns DR, Smith KH, Miller NR, Sulewski ME, have been suggested in the pheno- driving. However, this is a common Bias WB. Identical twins who are discordant for 2-4 Leber’s hereditary optic neuropathy. Arch Oph- typic expression of LHON. In this activity and has not been associated thalmol. 1993;111:1491-1494. case, both twins smoked 1 pack of with the onset of LHON. 2. Newman NJ, Lott MT, Wallace DC. The clinical cigarettes daily. However, twin A In short, no common identifi- characteristics of pedigrees of Leber’s hereditary optic neuropathy with the 11778 mutation. Am drank 6 to 12 beers weekly, which is able epigenetic factor for the clini- J Ophthalmol. 1991;111:750-762. less than half of the amount con- cal expression of LHON is appar- 3. Johns DR, Smith KH, Savino PJ, Miller NR. Leber’s hereditary optic neuropathy: clinical sumed by twin B who in fact had a ent for these 2 monozygous twins manifestations of the 15257 mutation. Ophthal- much later onset of LHON. Both who had a discordance for the on- mology. 1993;100:981-986. twins were firefighters, and twin A ex- set of LHON of 11 years. This sug- 4. Johns DR, Heber KL, Miller NR, Smith KH. Leber’s hereditary optic neuropathy: clinical perienced visual loss 2 weeks after ex- gests that potential epigenetic fac- manifestations of the 14484 mutation. Arch posure to smoke. In contrast, twin B tors for the clinical expression of Ophthalmol. 1993;111:495-498.

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