PEER
REVIEWED
Update
on Posterior Segment Emergencies
THUCANH
T. HO, MD, & MATHEW W. MACCUMBER, MD, PHD
Ophthalmologists, particularly retinal specialists,
are faced with a variety of vision-threatening emergencies that must be handled
in an expeditious but careful
manner in order to optimize visual outcome. In
1998, the field was covered in a text edited by one of us entitled Management
of Ocular Injuries and Emergencies (MacCumber MW, ed. Philadelphia, Pa: Lippincott,
Williams & Wilkins, 1998). This update highlights several areas where ocular
emergency management has since changed, focusing on the recent increase in blunt
trauma due to paintball war games and airbags, imaging, management of open-globe
injuries, medical management of choroidal neovascularization (CNV) and retinal vascular
occlusion, and endophthalmitis.
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Figure 1. High frequency ultrasound image of
a normal eye (top) and of the opposite eye that developed angle recession after
blunt trauma (bottom). |
BLUNT
AND PENETRATING TRAUMA
Worldwide, approximately 55 million eye injuries are believed
to occur yearly, leaving 1.6 million eyes blind.1
Globally, there are approximately 200000 open-globe injuries each year with an estimate
of 3.5/100000/year of perforating eye injuries.1
Schrader2
looked at 1026 open-globe injuries occurring between 1981 and 1999 and found that
the incidence of injuries sustained at work and in traffic accidents decreased over
time, with an associated decreased incidence of blindness and enucleations. Unfortunately,
the incidence of injuries related to hobbies increased during the same period. One
such recreation-related ocular injury that has become prevalent in recent years
is seen with paintball war games.
Paintball Injuries
Conn et al3
analyzed the paintball-related injury data from 1997 to 2001 from the National Electronic
Injury Surveillance System, a data bank of injuries that are reported from a probability
sampling of 100 US hospital emergency departments, and they found an average annual
rate of 4.5 injuries per 10000 participants, with the number treated in hospital
emergency departments more than 3 times higher in 2001 than in 1997. The eye
was found to be the most commonly affected body part (42.7%). At least 85% of those
suffering from eye injuries were either not wearing eye protection or had eye protection
that was temporarily removed due to fogging or paint splattering.
Paintball guns were originally developed for use by foresters
to mark trees for harvest. In the early 1980s, they were adapted for war games and
have increased in popularity over the past 2 decades. Paintball pellets have a face-diameter
of 17 mm (approximately the diameter of a dime) and are 3.5-g gelatin capsules filled
with nontoxic, water-soluble paint designed to rupture on contact. A paint ball
travels at a velocity of 300 feet per second when discharged from CO2-powered
guns and can cause devastating blunt ocular damage through coup and contrecoup effects
and anterior-posterior compression with equatorial expansion. The high velocity,
small size, and high mass of paintballs can result in severe trauma. Anterior segment
complications include lens trauma, angle recession, iridodiaylsis, and, most commonly,
hyphema. Posterior segment complications include choroidal rupture, retinal dialysis
and detachments, open globe injury, and, most commonly, commotio retinae and vitreous
hemorrhage.4 Listman5
found that 43% of patients had resulting vision of 20/200 or worse.
The American Society for Testing and Materials (ASTM) issued the
Standard Practice for Paintball Field Operations, specifying minimum safety requirements,
which are used voluntarily by most commercial facilities. The ASTM also issued standard
specifications for eye protection devices intended to minimize the risk of eye injuries.
These standards have significantly decreased the rate of paintball-related injuries
in commercial facilities. However, injuries occurring after 1995 were almost 6
times more likely to occur during noncommercial games than those sustained before
1995. This is due to increased availability of paintball equipment, underutilization
of eye protection, and no supervision in "backyard" games. Fineman4
recommended restriction on sales of paintball equipment, public education, and increased
adult supervision.
Airbag
Injuries
Overall, the most common sources of eye injuries during motor
vehicle accidents (MVAs) are windshields, frontal airbags, steering wheels, and
flying glass. Frontal airbags were mandated starting in 1993 and the combined use
of seat belts and frontal airbags decreased the rate of MVA-related mortality by
45% and severe injury by 50%.6
Before 1993, windshields were the main source of eye injuries, but this was surpassed
by frontal airbags with their availability.7
The major mechanisms of frontal airbag-related eye injuries include contusion with
transfer of energy from the vehicle to the passenger, lacerations, and chemical
burns from sodium hydroxide release. McGwin et al7
looked at the 1988 to 2001 National Automotive Sampling System Crashworthiness Data
System, finding an eye injury rate of 18 in 1000 occupants, with eyelid injuries
and corneal abrasions accounting for 94% of such injuries. Frontal airbag deployment
was associated with a twofold increased risk of eye injuries compared to the twofold
decrease risk associated with seat belt use. Using the same database, Duma et al8
compared the MVA-related eye injury rate with full-powered airbag deployment (1993-1997)
vs depowered airbag deployment (1998-2001) and found a 3.7% and 1.7% rate of eye
injuries, respectively. Pearlman et al6
reviewed the literature published between 1991 and 2000 on 101 patients who sustained
MVA-related eye injuries. They reported that 12.5% of injuries were bilateral and
4.2% were unilateral open-globe injuries. Vitreous or retinal hemorrhage was seen
in 9.9% and retinal tears or detachment was seen in 5.7% of cases. The median visual
outcome was 20/40.6 Lehto
et al9 reviewed the literature
published between 1991 and 2001 on 121 patients and estimated the risk of eye injury
to be 2.5% and serious eye injury to be 0.4%. They also found a threefold increased
risk of open-globe injuries in eyeglass wearers.
Imaging Studies
Eyes with possible open-globe injuries, especially when intraocular
foreign bodies (IOFBs) are suspected, usually require imaging to confirm the diagnosis
and determine the presence, location, and number of IOFBs. Noncontrast computerized
tomography (CT) scanning has replaced conventional radiography as the diagnostic
study of choice for all forms of ocular trauma. CT provides much more reliable information
on size, shape, and localization of the foreign body, whether in the anterior or
the posterior segment.10-13
Drawbacks of conventional CT include separate scanning in axial and coronal planes
leading to prolonged scanning time and radiation exposure. Reconstruction is limited
by stair-step artifact, compromising detection and localization of small and multiple
foreign bodies, especially those adjacent to the sclera or optic nerve. Volume averaging
also hinders detection and localization of small and multiple foreign bodies.14
Current spiral CT scanning with both 1-mm and
3-mm cuts can detect metallic
IOFB to as small as 0.5 mm with nearly 100% sensitivity.15
Spiral CT allows continuous scanning in less time with volumetric, overlapping data
acquisition, providing multiplanar reconstruction of high-quality coronal and sagittal
images. Thinner slices mean less volume averaging and better detection and localization
of small and multiple foreign bodies. Size and location of IOFBs detected on helical
CT corresponded with what was found during surgical and clinical follow-up.14
In eyes with small, stable wounds, judicious use of B-scan ultrasonography
over closed eyelids has proved useful in detecting retinal detachment (RD), double
perforation, and foreign bodies not otherwise seen on CT, such as vegetative IOFBs.16-18
Using high-frequency (50 MHz) sound waves, ultrasound
biomicroscopy is able to create high-resolution, 2-D cross-sectional anterior
segment images to a depth of 5 mm (Figure 1). It is a useful adjunct in the
detection of small foreign bodies, including those of nonmetallic composition, not
otherwise detected by CT and/or B-scan.19,20
Magnetic resonance imaging is only occasionally useful in the setting of ocular
trauma, such as the detection of radioopaque IOFBs in large, unstable wounds, but
its use must be avoided when iron-containing (ie, metallic) IOFBs are suspected.21-27
Optical
coherence tomography (OCT) has revolutionized our ability to detect and differentiate
blunt injuries involving the macula. For example, inner lamellar holes can be difficult
to differentiate from full-thickness macular holes on slit lamp biomicroscopy but
can now be readily diagnosed by OCT, thus improving our ability to advise patients
on prognosis and management (Figure 2).
Management of Open Globe Injuries
All patients with severe ocular injuries should have initial evaluation
to determine primarily whether the globe is open to an extent that requires surgical
intervention in the operating room, whether the patient is medically stable for
surgery, and whether there is an IOFB to plan the surgical approach (ie, primary
vitrectomy for posterior segment IOFB removal).28
The surgeon should take note of certain characteristics of the ocular injury that
are of particular prognostic significance: the mechanism of injury, the presenting
visual acuity, the presence or absence of relative afferent papillary defect, and
the posterior extent of the wound (zone).29
Patients without recent immunization should receive tetanus prophylaxis. In general,
patients should be given systemic antibiotics such as a fourth-generation fluoroquinolone
and, if necessary, antiemetics. In cases of vegetable matter, coverage against Bacillus
species with systemic clindamycin or vancomycin should also be considered. All protruding
foreign material should be left undisturbed and a shield should be placed over every
eye with a suspected open-globe injury until the time of surgical repair (Figure
3). Preoperative topical medication can be withheld if there is risk of disrupting
the wound, especially in the hands of individuals unfamiliar with the management
of eye trauma.
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Figure 3. Protruding intraocular foreign body should be shielded
(eg, with a plastic cup) and removed in a controlled fashion in the operating
room
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Traditionally, general anesthesia is utilized in the management
of ocular trauma. However, this is contraindicated in high-risk patients and is
associated with a longer recovery period. Recently, some surgeons have investigated
the use of intravenous sedation with local, or even topical, anesthesia in selected
patients. Scott et al30,31
analyzed 220 eyes with open-globe injuries managed at Bascom Palmer Eye Institute
in Miami between 1995 and 1999 and 238 cases managed between 2000 and 2003. Sixty-four
percent and 59% of these cases, respectively, were performed under intravenous sedation
and regional anesthesia, in the form of retrobulbar or peribulbar blocks with augmentation
after conjunctival cutdown. Patients managed under local anesthesia and intravenous
sedation were more likely to have better presenting visual acuity, IOFBs, and more
anteriorly located and shorter wounds. In addition to the well-known complications
of retrobulbar blocks, care must be taken to prevent expulsion of intraocular content
in the presence of larger wounds. Additionally, Boscia et al32
looked at the use of topical anesthesia and intravenous sedation in 10 open-globe
cases and found it to be a reasonable alternative in less severe injuries.
Some
controversy still exists in surgical management of severe posterior segment injuries.28
The use of prophylactic intravitreal antibiotics varies, but many retinal surgeons
have become more comfortable using such antibiotics in the setting of primary repair
and IOFB. Secondary vitrectomy procedures traditionally occurred at 10 to 14 days
after injury to allow for reduced choroidal congestion and posterior hyaloid separation;
however, technicological improvements can permit vitrectomy sooner and more safely
to prevent fibrous ingrowth and proliferative vitreoretinopathy.33
Personally we prefer vitrectomy within 1 week for severe posterior segment trauma
unless hemorrhagic choroidal detachment demands further delay. There are advocates
for and against prophylactic scleral buckling, but we usually choose to place a
prophylactic encircling scleral buckle for more severe injuries, particularly those
with retinal detachment and when the crystalline lens is preserved.34
CHOROIDAL NEOVASCULARIZATION
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Figure 4. Montage
of a perfused central retinal vein occlusion.
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Although usually not true ophthalmic "emergencies," sudden vision
loss due to CNV or retinal vascular occlusion (below) often present in an emergent
manner. Progress in the treatment of CNV over the past 10 years has been remarkable.
In 1998, the only proven therapy was photocoagulation, which stabilized or improved
vision primarily only in selected juxtafoveal and extrafoveal cases. Subsequently,
photodynamic therapy (PDT) with verteporfin (Visudyne, Novartis) became available
as the first treatment for subfoveal CNV to stabilize and occasionally improve vision.35
Interest in intravitreal triamcinolone acetonide (IVTA, Kenalog, Bristol-Myers Squibb)
added to our ability to treat both CNV and macular edema due to a variety of causes.36
Recently, intraocular antivascular endothelial growth factor (VEGF) inhibition has
become a reality, initially with pegaptanib (Macugen, OSI),37
then bevacizumab (Avastin, Genentech),38
and now ranibizumab (Lucentis, Genentech).39
The remarkable benefit of ranibizumab has been clear in randomized clinical trials
for age-related macular degeneration (AMD).38
We are still learning how to use these therapies alone or in combination for the
management of CNV due to different etiologies, including retinovascular emergencies.
Choroidal ruptures are seen in up to 5% to 10% of blunt ocular
injuries.40 Unlike the sclera
and retina, the retinal pigment epithelium-Bruch's membrane-choriocapillaris complex
is relatively inelastic and may break in a concentric pattern around the optic nerve.
CNV can arise at the site of choroidal ruptures from 1 month to 4 years after injury.41
Both laser photocoagulation and subretinal surgery have been attempted with limited
success. Recently, there have been a few reported cases of choroidal rupture-related
CNV responsive to PDT.42-44
Harrisi-Dagher et al43 looked
at 5 cases that required an average of 2 treatments and found the absence of or
decreased leakage on fluorescein angiogram, with stabilization or improvement in
visual acuity in 80% of cases.
Bevacizumab is a humanized monoclonal antibody that inhibits all
isoforms of VEGF and is Food and Drug Administration-approved only as an intravenous
therapy for metastatic colorectal cancer. There are numerous case reports and case
series on the off-label use of intravitreal bevacizumab for CNV secondary to AMD
and pathological myopia with dramatic results.45-53
Although, to the best of our knowledge, there are no specific reports on the effect
of bevacizumab on choroidal rupture-related CNV, we would expect that it may also
have a beneficial effect in these cases.
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Figure
5. OCT scan of cystic macular edema secondary to BRVO before (top) and 1 week after
injection of 1.0 mg bevacizumab (bottom); Snellen visual acuity improved from 20/70 to
20/50.
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RETINAL VASCULAR OCCLUSIONS
Over the past 10 years, there has been substantial improvement
in the understanding of the etiology of retinal vascular occlusion, particularly
in the younger patient population. Use of birth control pills, dehydration, and
embolic events are all potential contributors to retinovascular occlusion in children
and young adults. However, no clear etiology is evident by history and review of
system in many cases. This has often resulted in an extensive workup that can include
MRI/MRA and echocardiography as well as a myriad of blood studies, including complete
blood count, folic acid, vitamin B12,
antinuclear antibody, rheumatoid factor, erythrocyte sedimentation rate, and lipid
profile. In the past, we have been disheartened by the poor yield of these tests;
however, each passing year has improved our understanding of the immune and coagulation
systems, so that now we can detect a predisposing abnormality in many patients.
Newer tests include antiphospholipid antibody profiles, antithrombin III, protein
C, protein S, prothrombin, homocysteine, C-reactive protein, factor V Leiden, and
mutations in methylene tetrahydrofolate reductase. We hope that we will learn to
better target these tests to certain clinical scenarios and perhaps even devise
preventive measures.
Progress
in the treatment of retinal arterial occlusion has been significantly limited in
part due to delay in presentation to the ophthalmologist until after irreversible
damage has occurred. Anterior chamber paracentesis is still the only treatment that
has improved vision in our hands. However, there have been significant developments
in the treatment of retinal vein occlusion (RVO). In the late 1990s, treatment of
RVO had been largely limited to photocoagulation. The Central Vein Occlusion Study52
demonstrated no visual acuity benefit from grid laser photocoagulation for macular
edema associated with central retina vein occlusion (CRVO, Figure 4) while the Branch
Vein Occlusion Study53
demonstrated a treatment benefit with grid laser photocoagulation in some patients
with nonischemic branch retinal vein occlusion (BRVO) and less than or equal to
20/40 vision who had not improved after 3 months of observation.
Recently, there is considerable interest in new pharmacologic
treatments for RVO and secondary macular edema. Unlike macular laser treatment,
these do not require clearing of macular hemorrhages so they can be employed sooner.
Surgical and other laser therapies have also been investigated but, in our experience,
are less commonly effective. Several small retrospective as well as prospective
case series have shown at least a temporary beneficial effect with IVTA in terms
of improvement in visual acuity (VA) and reduction in central macular thickness
(CMT) on OCT for the treatment of macular edema secondary to BRVO54-60
and CRVO.61-67 Notably, VA
has not been shown to correlate with CMT. Ozdek et al61
looked at 11 nonischemic and 11 ischemic CRVO eyes and found at least a 3 line improvement
in 82% of the nonischemic group compared to only 18% of the ischemic group. Ramezani
et al62 found the therapeutic
effects of IVTA 4 mg in CRVO to be greatest in the first 2 months. In a prospective,
randomized trial involving 63 eyes with macular edema secondary to diabetic retinopathy
(DR) or RVO, Avitabile et al68
compared treatment with IVTA 4 mg, grid laser photocoagulation, or both. At each
of the 1-, 3-, and 9-month follow-up visits, VA improved 0.19 to 0.26 logMAR and
CMT decreased 29% to 37% in the IVTA alone and combination groups while the VA remained
unchanged and CMT decreased only 5% to 16% in the laser group. Hayashi et al69
performed a randomized clinical trial on 60 eyes with BRVO, comparing IVTA 4 mg
with 3 repeated retrobulbar injections of 40 mg of triamcinolone acetonide, both
groups injected 1 week after grid laser, and found IVTA to be significantly more
effective. Based on such results, the National Eye Institute is currently funding
the Standard Care vs COrticosteroid for REtinal Vein Occlusion (SCORE) Study, a
multicenter, randomized, controlled clinical trial to investigate the safety and
efficacy of IVTA for the treatment of macular edema secondary to CRVO and BRVO.
The associated ocular complication rate of IVTA is relatively
high when compared with other intravitreal injections. Forty percent develop ocular
hypertension with 1% to 2% uncontrolled by medical therapy.70
Cataract extraction is performed within 1 year of treatment in 15% to 20% of injected
eyes.70 Because of its ocular
safety profile, the use of intravitreal bevacizumab for macular edema secondary
to vein occlusions has recently gained popularity (Figure 5).38,71-73
Iturralde et al71 reviewed
the results of 16 eyes injected with an average of 2.8 injections on bevacizumab
1.25 mg. Nine patients were previously given IVTA but either failed treatment or
developed ocular hypertension. The mean VA improved from a baseline of 20/600 to
20/200 at 1 month and 20/138 at 3 months, and CMT improved from 887 μm to 372
μm at 1 month.
Both IVTA74
and, to a more profound extent, bevacizumab75-79
have also been reported to cause regression of iris neovascularization (NVI), making
them much needed additions to our armamentarium for neovascular glaucoma (NVG) management,
a condition known for its dismal prognosis. Jonas et al74
treated 14 eyes with NVG (9 diabetics, 5 CRVO) with IVTA 20 mg as the only procedure
in 4 eyes, with cryotherapy in 7 eyes, with goniosynechiolysis in 1 eye, and with
phacoemulsification in 2 eyes. The degree of NVI decreased from 2.6 (±1.3)
to 1.3 (±1.2) relative units post-injection. Both intracameral and intravitreal
bevacizumab have been shown to consistently be associated with dramatic regression
of NVI within 1 week of treatment with improvement in intraocular pressure control.
These case reports suggest that the effect can last for up to 2 months post-injection.75-79
ENDOPHTHALMITIS
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Figure 6. OCT scan of an eye with diabetic
macular edema before (top) and 2 weeks after an intravitreal injection of 4.0 mg trimacinolone
acetonide (bottom). There was transient, but marked non-infectious endophthalmitis starting
1 day after injection that cleared over the following week. Vitreous tap revealed
no organism growth. Snellen visual acuity improved from 20/70 to 20/50 despite the
transient inflammation. |
The explosion in the use of intravitreal injections has generated
significant concern about another wave of iatrogenic endophthalmitis. Jager et al80
reviewed the literature from 1966 to 2004, looking at a total of 14 866 intravitreal
injections for 4382 eyes and found the overall prevalence of infectious and noninfectious
endophthalmitis to be 0.3% per injection and 0.9% per eye. The rate of infectious
endophthalmitis was 0.2% per injection and 0.5% per eye. Regarding 1739 IVTA injections,
the overall prevalence of endophthalmitis was 1.4% per injection and 0.6% per injection
for infectious endophthalmitis. IVTA injections can cause a noninfectious and self-limited
endophthalmitis (Figure 6) that may be an immune response to endotoxin or preservatives
in certain IVTA preparations; this entity usually has milder inflammation, occurs
sooner (hours to days after injection), and is painless. Intravitreal pegaptanib
has a reported infectious endophthalmitis risk of 0.16% per injection; the risk
is lower with the stricter revised injection protocol.37,81
Derived in large part from data from the VEGF Inhibition Study in Ocular Neovascularization
(VISION) trial,37,81 we have
learned that proper sterile technique, including the use of a lid speculum and 5%
povidone-iodine, can keep the endophthalmitis risk acceptably low.82
Management would usually involve vitreous tap and injection of intravitreal antibiotics
if infectious endophthalmitis is suspected.
Taban et al83
performed a review of the literature from 1963 to 2003, pooling 3 140 650 cataract
extractions and found the overall incidence of postcataract endophthalmitis to be
0.128%. Incision type was implicated in the increased incidence of postcataract
endophthalmitis in recent years, with 0.189% incidence for clear corneal incisions
vs 0.074% incidence for scleral incisions between 1992 and 2003. Using OCT, McDonnell
et al84 demonstrated that
transient postoperative IOP reduction causes the clear cornea cataract wound edge
to gape, starting at the internal aspect of the wound. Ciulla et al85
reviewed the literature from 1966 to 2000 on endopthalmitis prophalaxis for cataract
surgery and found that 5% povidone-iodine had a particularly significant inhibitory
effect.
The results of the Endophthalmitis Vitrectomy Study (EVS)86
published in 1995 showed no benefit of vitrectomy and intravitreal antibiotics over
vitreous tap and intravitreal antibiotics for postcataract endophthalmitis patients
with better than light perception vision. With modern vitrectomy technology, including
panoramic viewing, better instrumentation, and silicone oil, Kuhn and Gini87
advocated the early use of complete vitrectomy for patients with no retinal details
or a poor red reflex on ophthalmoscopy, regardless of visual acuity. They reported
a consecutive series of 47 eyes that underwent vitrectomy for infectious endopthalmitis,
91% of which recovered at least 20/40 vision compared to 53% in the EVS.
Ng et al88
in the Endopthalmitis Population Study of Western Australia (EPSWA) reviewed 213
cases of endophthalmitis between 1980 and 2000 and found that management involved
increased use of intravitreal antibiotics and decreased use of subconjuctival and
intravenous antibiotics. Other studies confirm that subconjunctival antibiotics
are probably not required in the treatment of infectious endophthalmitis.89,90
CONCLUSIONS
The spectrum and management of posterior segment emergencies continues
to evolve. Recent advances have resulted in better diagnosis and management of several
posterior segment emergencies, including posterior segment trauma, retinal vascular
occlusions, and endophthalmitis. Improved imaging modalities, vitrectomy technology,
and anesthesia approaches have permitted more expeditious care of the traumatized
eye.
Pharmaceutical developments have added to the outpatient armamentarium;
in particular, anti-VEGF therapies have revolutionalized not only how we manage
exudative AMD, but also other causes of CNV, retinal vein occlusions, and neovascular
glaucoma. Endophthalmitis continues to be a concern in the era of clear corneal
cataract surgery and frequent use of intravitreal injections.
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ThucAnh T.
Ho, MD is a second year vitreoretinal fellow with Illinois Retina Associates/ Rush
University Medical Center (Chicago) and trained as a resident in the Wills Eye Hospital
Emergency Department in Philadelphia. Mathew W. MacCumber, MD, PhD is associate
professor, associate chairman for research, and vitreoretinal fellowship director
in the Department of Ophthalmology, Rush University Medical Center, and a partner
with Illinois Retina Associates in Chicago. Dr. MacCumber has consulted for and/or
received grant support from Genentech, Novartis, Eyetech/OSI, Allergan, Bausch &
Lomb, and Optos, Inc. The authors have no financial interest in information
presented in this article.
Retinal Physician, Issue: September 2006