13
PostScript .............................................................................................. LETTERS If you have a burning desire to respond to a paper published in BJO, why not make use of our ‘‘rapid response’’ option? Log onto our website (www.bjophthalmol. com), find the paper that interests you, and send your response via email by clicking on the ‘‘eLetters’’ option in the box at the top right hand corner. Providing it isn’t libellous or obscene, it will be posted within seven days. You can retrieve it by clicking on ‘‘read eLetters’’ on our homepage. The editors will decide as before whether to also publish it in a future paper issue. The use of magnetic resonance imaging in the diagnosis of suspected giant cell arteritis Giant cell arteritis (GCA) is a vasculitis of unknown origin that has a predisposition for the cranial arteries in the elderly. It has potentially devastating visually complications and produces a broad range of symptoms and signs that mimic many other medical and surgical conditions. Blood tests reflect the underlying inflammatory process, yet the erythrocyte sedimentation rate (ESR) may be normal in 8% of patients with biopsy proved GCA. 1 Nevertheless, making a defini- tive diagnosis has importance therapeutically as patients are committed to a lengthy oral corticosteroid regimen. Non-invasive techni- ques, such as colour Doppler or duplex ultrasonography, have been studied in an attempt to improve patient preselection for temporal artery biopsy (TAB). 2 3 Magnetic resonance imaging (MRI) has been shown to improve the diagnosis of early Takayasu arteritis. 4 More recently several case reports have described the diagnostic potential of MR angiography and gadolinium contrast MRI in demonstrating the vessel changes of GCA. 56 We compared the ability of MRI to detect changes in the temporal arteries with TAB in patients clinically suspected of having GCA. Methods and results A prospective, pilot, single masked study of seven female patients (age range 60–88 years, mean 76 years) with suspected giant cell arteritis, and two age matched healthy controls was undertaken. Local research ethical approval and informed written con- sent were obtained. All patients underwent a standard clinical examination including a detailed history and clinical examination. Investigations included ESR and C reactive protein (CRP). Each patient was given a GCA criteria ‘‘score’’ based on the 1990 ACR (American College of Rheumatology) classi- fication 7 (Table 1). Within 48 hours of presentation patients underwent a unilateral temporal artery MRI scan on a 1.5T scanner using a surface coil and small field of view. T 1 and T 2 weighted images perpendicular to the temporal artery and a time of flight sequence were obtained. The MRI visualised the location of the temporal artery that was subsequently biopsied in a standard manner within 24 hours of the scan. Two healthy age matched controls also underwent a medical assessment, ESR and CRP, and an MRI as detailed above, but a TAB was not performed. The MRI scans were reported by an indepen- dent, masked neuroradiologist. Each patient’s ACR criteria ‘‘score’’ and the results of the MRI scan and TAB are shown in Table 2. The finding of three out of five ACR criteria is associated with a 94% sensitivity and 91% specificity for the diagnosis of GCA. 7 There were two positive and one equivocal TAB result from the seven patients, but no positive MRI findings were identified. However, when using the ACR criteria as ‘‘gold standard,’’ there were two true nega- tive MRI scan results compared with three false negative scan results. The two remain- ing MRI scans were described as equivocal, in comparison with the ACR criteria—one patient was positive for GCA and the other patient’s ACR criteria ‘‘score’’ was negative for GCA. From the data the negative pre- dictive values of MRI scanning and TAB for GCA were 40% and 50%, respectively. Of the five patients who showed a prompt response to oral corticosteroid, the MRI scan was negative in four and equivocal in the other. Comment Although our study sample was small our findings suggest that MRI scanning was unable to distinguish between a normal and an affected artery. We conclude that there is no potential for the use of MRI scanning without contrast enhancement in the evalua- tion of patients with suspected GCA. S O Brannan, D Cheung, P I Murray Birmingham and Midland Eye Centre, Dudley Road, Birmingham B18 7QU, UK C Dewar Sandwell and West Birmingham Hospitals NHS Trust, City Hospital, Birmingham, UK P Guest Queen Elizabeth Hospital, University Hospitals Birmingham, NHS Trust, Birmingham, UK Correspondence to: Professor P I Murray, Academic Unit of Ophthalmology, Division of Immunity and Infection, Birmingham and Midland Eye Centre, Sandwell and West Birmingham Hospitals NHS Trust, City Hospital, Dudley Road, Birmingham B18 7QU, UK; [email protected] Accepted for publication 3 March 2003 References 1 Hayreh SS, Podhajsky PA, Raman R, et al. Giant cell arteritis: validity and reliability of various diagnostic criteria. Am J Ophthalmol 1997;123:285–96. 2 Schmidt WA, Kraft HE, Vorpahl K, et al. Color duplex ultrasonography in the diagnosis of temporal arteritis. New Engl J Med 1997;337:1336–42. 3 Wenkel H, Michelson G. Correlation of ultrasound biomicroscopy with histological findings in diagnosis of giant cell arteritis. Klin Monatsbl Augenheilkd 1997;210:48–52. 4 Tanigawa K, Eguchi K, Kitamura Y, et al. Magnetic resonance imaging detection of aortic and pulmonary artery wall thickening in the acute stage of Takayasu arteritis: improvement of Table 1 1990 American College of Rheumatology criteria for the classification of giant cell (temporal) arteritis (traditional format) Criterion Definition 1 Age at disease onset .50 years Development of symptoms or findings beginning at age 50 or older 2 New headache New onset of or new type of localised pain in the head 3 Temporal artery abnormality Temporal artery tenderness to palpation or decreased pulsation, unrelated to arteriosclerosis of cervical arteries 4 Elevated ESR .50 mm in the first hour by the Westergren method 5 Abnormal artery biopsy Biopsy specimen with artery showing vasculitis characterised by a predominance of mononuclear cell infiltration or granulomatous inflammation, usually with multinucleated giant cells Table 2 Summary of results Patient Age ACR criteria score Prompt response to steroids TAB MRI scan 1 78 5 Yes Positive Negative 2 67 4 Yes Negative Negative 3 83 2 Yes Equivocal Negative 4 88 3 Yes Negative Negative 5 60 2 No Negative Negative 6 67 2 No Negative Equivocal 7 87 4 Yes Positive Equivocal 8 67 NA NA NA Negative 9 69 NA NA NA Equivocal TAB = temporal artery biopsy; ACR = American College of Rheumatology, MRI = magnetic resonance imaging; NA = not applicable, * = control. Br J Ophthalmol 2004;88:1595–1607 1595 www.bjophthalmol.com on June 23, 2022 by guest. Protected by copyright. http://bjo.bmj.com/ Br J Ophthalmol: first published as 10.1136/bjo.2004.048413 on 17 November 2004. Downloaded from

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LETTERS

If you have a burning desire to respond toa paper published in BJO, why not makeuse of our ‘‘rapid response’’ option?

Log onto our website (www.bjophthalmol.com), find the paper that interests you, andsend your response via email by clicking onthe ‘‘eLetters’’ option in the box at the topright hand corner.

Providing it isn’t libellous or obscene, itwill be posted within seven days. You canretrieve it by clicking on ‘‘read eLetters’’ onour homepage.

The editors will decide as before whetherto also publish it in a future paper issue.

The use of magnetic resonanceimaging in the diagnosis ofsuspected giant cell arteritisGiant cell arteritis (GCA) is a vasculitis ofunknown origin that has a predisposition forthe cranial arteries in the elderly. It haspotentially devastating visually complicationsand produces a broad range of symptoms andsigns that mimic many other medical andsurgical conditions. Blood tests reflect theunderlying inflammatory process, yet theerythrocyte sedimentation rate (ESR) maybe normal in 8% of patients with biopsyproved GCA.1 Nevertheless, making a defini-tive diagnosis has importance therapeutically

as patients are committed to a lengthy oralcorticosteroid regimen. Non-invasive techni-ques, such as colour Doppler or duplexultrasonography, have been studied in anattempt to improve patient preselection fortemporal artery biopsy (TAB).2 3 Magneticresonance imaging (MRI) has been shown toimprove the diagnosis of early Takayasuarteritis.4 More recently several case reportshave described the diagnostic potential of MRangiography and gadolinium contrast MRI indemonstrating the vessel changes of GCA.5 6

We compared the ability of MRI to detectchanges in the temporal arteries with TAB inpatients clinically suspected of having GCA.

Methods and resultsA prospective, pilot, single masked study ofseven female patients (age range 60–88 years,mean 76 years) with suspected giant cellarteritis, and two age matched healthycontrols was undertaken. Local researchethical approval and informed written con-sent were obtained. All patients underwent astandard clinical examination including adetailed history and clinical examination.Investigations included ESR and C reactiveprotein (CRP). Each patient was given a GCAcriteria ‘‘score’’ based on the 1990 ACR(American College of Rheumatology) classi-fication7 (Table 1). Within 48 hours ofpresentation patients underwent a unilateraltemporal artery MRI scan on a 1.5T scannerusing a surface coil and small field of view. T1and T2 weighted images perpendicular tothe temporal artery and a time of flightsequence were obtained. The MRI visualisedthe location of the temporal artery that was

subsequently biopsied in a standard mannerwithin 24 hours of the scan. Two healthy agematched controls also underwent a medicalassessment, ESR and CRP, and an MRI asdetailed above, but a TAB was not performed.The MRI scans were reported by an indepen-dent, masked neuroradiologist.Each patient’s ACR criteria ‘‘score’’ and the

results of the MRI scan and TAB are shown inTable 2. The finding of three out of five ACRcriteria is associated with a 94% sensitivityand 91% specificity for the diagnosis of GCA.7

There were two positive and one equivocalTAB result from the seven patients, but nopositive MRI findings were identified.However, when using the ACR criteria as‘‘gold standard,’’ there were two true nega-tive MRI scan results compared with threefalse negative scan results. The two remain-ing MRI scans were described as equivocal, incomparison with the ACR criteria—onepatient was positive for GCA and the otherpatient’s ACR criteria ‘‘score’’ was negativefor GCA. From the data the negative pre-dictive values of MRI scanning and TAB forGCA were 40% and 50%, respectively. Of thefive patients who showed a prompt responseto oral corticosteroid, the MRI scan wasnegative in four and equivocal in the other.

CommentAlthough our study sample was small ourfindings suggest that MRI scanning wasunable to distinguish between a normal andan affected artery. We conclude that there isno potential for the use of MRI scanningwithout contrast enhancement in the evalua-tion of patients with suspected GCA.

S O Brannan, D Cheung, P I MurrayBirmingham and Midland Eye Centre, Dudley Road,

Birmingham B18 7QU, UK

C DewarSandwell and West Birmingham Hospitals NHS Trust,

City Hospital, Birmingham, UK

P GuestQueen Elizabeth Hospital, University Hospitals

Birmingham, NHS Trust, Birmingham, UK

Correspondence to: Professor P I Murray, AcademicUnit of Ophthalmology, Division of Immunity andInfection, Birmingham and Midland Eye Centre,

Sandwell and West Birmingham Hospitals NHS Trust,City Hospital, Dudley Road, Birmingham B18 7QU,

UK; [email protected]

Accepted for publication 3 March 2003

References

1 Hayreh SS, Podhajsky PA, Raman R, et al. Giantcell arteritis: validity and reliability of variousdiagnostic criteria. Am J Ophthalmol1997;123:285–96.

2 Schmidt WA, Kraft HE, Vorpahl K, et al. Colorduplex ultrasonography in the diagnosis oftemporal arteritis. New Engl J Med1997;337:1336–42.

3 Wenkel H, Michelson G. Correlation ofultrasound biomicroscopy with histologicalfindings in diagnosis of giant cell arteritis. KlinMonatsbl Augenheilkd 1997;210:48–52.

4 Tanigawa K, Eguchi K, Kitamura Y, et al.Magnetic resonance imaging detection of aorticand pulmonary artery wall thickening in the acutestage of Takayasu arteritis: improvement of

Table 1 1990 American College of Rheumatology criteria for the classificationof giant cell (temporal) arteritis (traditional format)

Criterion Definition

1 Age at disease onset .50 years Development of symptoms or findings beginning at age50 or older

2 New headache New onset of or new type of localised pain in the head3 Temporal artery abnormality Temporal artery tenderness to palpation or decreased

pulsation, unrelated to arteriosclerosis of cervical arteries4 Elevated ESR .50 mm in the first hour by the Westergren method5 Abnormal artery biopsy Biopsy specimen with artery showing vasculitis

characterised by a predominance of mononuclear cellinfiltration or granulomatous inflammation, usually withmultinucleated giant cells

Table 2 Summary of results

Patient Age ACR criteria score Prompt response to steroids TAB MRI scan

1 78 5 Yes Positive Negative2 67 4 Yes Negative Negative3 83 2 Yes Equivocal Negative4 88 3 Yes Negative Negative5 60 2 No Negative Negative6 67 2 No Negative Equivocal7 87 4 Yes Positive Equivocal8 67 NA NA NA Negative9 69 NA NA NA Equivocal

TAB = temporal artery biopsy; ACR=American College of Rheumatology, MRI =magnetic resonanceimaging; NA=not applicable, *= control.

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clinical and radiologic findings after steroidtherapy. Arthritis Rheum 1992;35:476–80.

5 Mitomo T, Funyu T, Takahashi Y, et al. Giant cellarteritis and magnetic resonance angiography.Arthritis Rheum 1998;41:1702.

6 Anders HJ, Sigl T, Sander A, et al. Gadoliniumcontrast magnetic resonance imaging of thetemporal artery in giant cell arteritis. J Rheumatol1999;26:2287–8.

7 Hunder GG, Bloch DA, Michel BA, et al. TheAmerical College of Rheumatology 1990 criteriafor the classification of giant cell arteritis. ArthritisRheum 1990;33:1122–8.

Bilateral ischaemic opticneuropathy and stroke aftermultiple bee stingsDespite the common occurrence of insectstings and local and systemic allergic reac-tions,1 there are few reports of optic neuro-pathy or stroke following bee or wasp stingsand, to our knowledge, there has been noreport of both cerebral infarction and opticneuropathy occurring in the same patientafter such an event. We report on a middleaged woman who sustained both a strokeand ischaemic optic neuropathy after multi-ple bee stings.

Case reportA 57 year old white woman reported beingstung by 30–40 bees, identified as Africanisedhoney (killer) bees, in the back of her neck,head, right eye, face, and right arm. She wastreated with intravenous antihistamines andantiemetics at a local emergency room andreleased.Two days later, the patient experienced a

severe headache with nausea and vomitingand noticed a left homonymous visual fieldloss. She went to see her primary doctor andwhile there became unresponsive, leading tohospitalisation. Head computed tomography(CT) showed a right occipital ischaemicinfarct.Shortly thereafter, the patient experienced

acute nausea and vomiting with neck rigidityand was readmitted. A head CT scan andbrain magnetic resonance image (MRI)/mag-netic resonance angiography (MRA) wereperformed showing a large right temporo-occipital haemorrhagic infarct (fig 1A, B). Anocular examination revealed best correctedvisual acuity (BCVA) of 20/20-1 right eye and20/30-2 left eye at distance and 20/20 righteye and 20/200 left eye at near, with lefthomonymous hemianopia, a left inferioraltitudinal defect, and bilateral arcuatedefects (fig 1C) with bilateral haemorrhagicdisc oedema.Past medical and surgical history are

significant only for controlled arterial hyper-tension and pseudophakia.Neuro-ophthalmic examination 5 weeks

after her sting episode showed BCVA of 20/15 right eye and 20/25 left eye at distance and20/20 right eye and 20/30+1 at near. Amslergrid and automated perimetry showed a lefthomonymous hemianopic defect with a rightinferior arcuate defect and a left inferioraltitudinal defect.Pupil examination showed isocoria with a

0.3–0.6 log unit relative afferent pupillarydefect in the left eye. Motility was unremark-able, as was anterior segment both eyes.Intraocular pressures were 20 mm Hg righteye and 18 mm Hg left eye. Funduscopicexamination showed bilateral disc oedemawith pallid swelling superiorly and tem-porally in both eyes and peripapillary

haemorrhage and cotton wool spots in botheyes consistent with anterior ischaemic opticneuropathy (AION). Both maculas were un-remarkable without exudative changes. Bothretinas were flat with normal vasculature outto the periphery.Three months after the sting event, the

patient reported some improvement ofperipheral vision, and repeat visual fieldsimproved slightly inferiorly but were other-wise unchanged. Both optic discs were nowflat and showed superior temporal pallorwith corresponding nerve fibre layer dropout.

CommentIn their literature review of five cases andreport of two additional cases of opticneuropathy occurring after bee and waspsting, Maltzman, et al2 describe commoncharacteristics, such as acute to subacuteonset of symptoms, moderate to severe visualloss followed by significant recovery (exceptin one case of a sting directly to the eye);oedematous and haemorrhagic optic discs,and central or caecocentral scotomas.Although our patient had subacute visionloss associated with haemorrhagic discoedema, her case differs because of minimalrecovery of vision and altitudinal visual lossconsistent with an ischaemic neuropathy,rather than a transient optic neuritis.Seven cases of wasp and bee sting

associated cerebral infarction were found inthe literature.3–9 Reported neurological

complications included seizure, hemiparesis,aphasia, apraxia, dysarthria, ataxia, andcoma, none of which were experienced byour patient. None of these patients had a fulleye examination, although in one patient8 aright homonymous superior quadrantanopiawas demonstrated (table 1).The pathophysiology explaining the asso-

ciated stroke is unknown. Hypotensioncaused by anaphylaxis may certainly inducecerebral and optic nerve ischaemia; however,this was not documented in our case. Similarto acute myocardial infarction after hyme-noptera stings, it has been suggested thatvasoconstriction secondary to mediatorsreleased after the sting, aggravated by exo-genous adrenaline, and platelet aggregationalso contribute to cerebral ischaemia.8 Beevenom itself contains histamine, thrombox-ane, leucotrienes, and other vasoactive andinflammatory mediators. In our patient, wepostulate that the systemic immunemediated reaction to the bee sting causedvasoconstriction and a prothrombotic statewith subsequent ischaemia leading to boththe stroke and AION. In addition, a neuro-pharmacological (sympathetic) mechanismof endothelial permeability involving thecerebral vasculature with a concurrent sys-temic thrombogenic or immune response hasalso been postulated.5 6

J S SchiffmanUniversity of Houston University Eye Institute, Houston,

TX, USA

Figure 1 MRI (A), MRA (B), and Humphrey 30-2 visual fields (C) of our patient.

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Table

1Re

portsof

cerebral

hypo

xiaan

dinfarctio

nfollowingbe

e/waspsting

Autho

r/ref

Age/

sex

Typeof

stings:

locatio

nOnset

ofne

urolog

icaldeficit

Examinatio

nfin

dingsand

symptoms

Eyeex

aminatio

nMRI/CTfin

dings

Trea

tmen

tRecov

ery

Day

336/M

Wasp:

multip

leon

neck,

face,an

darms

,1ho

urHea

dache,

seizure,

righ

the

miplegia,

coma

Equa

land

reactive

pupils

NR;

necrop

syshow

edleft

haem

orrhag

iccortical

infarct

Cortison

e,an

tihistamines,

phen

obarbital

Decea

sed

Starran

dBrashe

r437/M

Wasp:

3stings

onarms

,1ho

urSe

izure,

righ

the

miplegia

NR

Leftcerebral

infarctio

n(CTdo

ne14mon

thslater)

Barbiturates,

corticosteroids,

adrena

line

Partialright

hemiplegia,

oneseizure

Rigg

set

al5

38/M

Wasp:

multip

leon

left

face

andne

ck2da

ysRigh

the

miplegia,

denseglob

alap

hasia

NR

Ischae

mic

infarctio

nin

thedistribu

tion

oftheleftMCA;an

giog

ram:leftICA

occlusion

NR

NR

Rigg

set

al6

52/M

Wasp:

sing

le,locatio

nNR(previou

shistoryof

waspstingallergy)

Afew

hours,

with

worsening

24da

yslater

Ana

phylactic

shockwith

respiratoryarrest,slurredspeech

andlefthe

miparesisinitially,then

24da

yslater,acuteob

tund

ation

andqu

adripa

resis

NR

Initially,threesm

allfocal

ischae

mic

infarcts,twoin

therigh

tcentrum

semiovale

andon

ein

therigh

ttempo

rallob

e.Afterworsening

,diffu

sebilateralischa

emicwhite

matterlesion

san

dleftpa

rietal

andinsularcortical

infarctio

ns.MRA

andan

giog

ram:

completean

dne

arcompleteocclusions

oftherigh

tan

dleftICA,respectively

IVad

rena

line,

methylpredn

isolon

e,diph

enhydram

ine

NR

Spea

chet

al7

30/M

Bee:

sing

le,locatio

nNR

,1ho

urDecereb

rate

posturing,

extensor

plan

tarreflexes,

lefthe

miparesis,

hypo

reflexia;

aftercoma,

patient

hadmotor

apraxiaan

dleft

sensoryne

glect

NR

Normal

MRI

andCT

IVdiph

enhydram

ine,

steroids

andne

bulised

b2ag

onistan

dan

ticho

linergicmed

ications

Residu

alideo

motor

apraxia

SPEC

T:hype

rperfusion

oftheleft

dorsolateral

fron

talc

ortex,

butno

area

sof

hypo

perfusionor

othe

rab

norm

alities

Normal

VF

Crawleyet

al8

30/F

Wasp:

leftarm

,1ho

urFa

cial

andarm

swelling,

widesprea

durticaria,acute

pulmon

aryoe

dema,

visual

loss

Righ

tho

mon

ymou

ssupe

rior

quad

rantan

opia

Leftoccipitalischa

emic

infarct

SQad

rena

line,

IVge

lofusine

,IV

hydrocortison

e,IM

chlorphe

niramine,

IVfurosemide

Fullrecovery

from

quad

rantan

opia

Bhat

etal

935/M

Bee:

multip

le‘‘a

llover

thebo

dy’’

,1da

yMultip

lesw

ellings

allo

verthe

body

,vomiting

,dy

sarthria,

tinnitus,vertigoan

dsw

ayingga

it,hype

rten

sion

,bilateralcereb

ellar

sign

s,rhab

domyo

lysiswith

acute

rena

l(respiratory?)failure

Nopa

pilloed

ema

Bilateralcereb

ellarha

emorrhag

icinfarct

Dexam

etha

sone

,an

tihistamines,man

nitol,

insulin,ha

emod

ialysis

Decea

sed

Presen

trepo

rt57/F

Bee:

multip

leon

neck,

head

,Reye,

Rside

ofhe

rne

ck,face

andR

arm

2da

ysNau

sea,

vomiting

,vision

loss

BCVA

of20/1

5righ

teye,

20/2

5lefteye;

leftho

mon

ymou

she

miano

pia,

left

inferior

arcuatean

drigh

taltitud

inal

defect;Bilateral

oede

ma(right

eye.

lefteye)

w/

pallidha

emorrhag

icsw

elling

Hae

morrhag

icinfarct2da

yspo

st-

ischae

mic

stroke

IVan

tihistamines

and

antiemetics

Leftho

mon

ymou

she

miano

piawith

inferior

arcuate

defects;

central

vision

unaffected

righ

teyean

don

lymild

lyaffected

left

eye

NR=no

nerepo

rted

.

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J S Schiffman, R A Tang, E Ulysses,N Dorotheo, S S Singh, H M Bahrani

University of Texas Medical Branch, Galveston, TX,USA

Correspondence to: Rosa A Tang, MD, MPH, 2476Bolsover Street #635, Houston, TX 77005, USA;

[email protected]

doi: 10.1136/bjo.2004.042465

Accepted for publication 5 April 2004

References

1 Ewan PW. ABC of allergies: venom allergy. BMJ1998;316:1365–8.

2 Maltzman JS, Lee AG, Miller NR. Opticneuropathy occurring after bee and wasp sting.Ophthalmology 2000;107:193–5.

3 Day JM. Death due to cerebral infarction afterwasp stings. Arch Neurol 1962;7:184–6.

4 Starr JC, Brasher GW. Wasp sting anaphylaxiswith cerebral infarction. Ann Allergy1977;39:431–3.

5 Riggs JE, Ketonen LM, Bodensteiner JB, et al.Wasp sting-associated cerebral infarction: a rolefor cerebrovascular sympathetic innervation. ClinNeuropharmacol 1993;16:362–5.

6 Riggs JE, Ketonen LM, Wymer JP, et al. Acute anddelayed cerebral infarction after wasp stinganaphylaxis. Clin Neuropharmacol1994;17:384–8.

7 Speach DP, Wong TM, Cattarin JA, et al. Hypoxicbrain injury with motor apraxia following ananaphylactic reaction to hymenoptera venom.Brain Injury 1998;12:239–44.

8 Crawley F, Schon F, Brown MM. Cerebralinfarction: a rare complication of wasp sting.J Neurol Neurosurg Psychiatry 1999;66:550–1.

9 Bhat R, Bhat KR, Pais R, et al. Bilateralhaemorrhagic cerebellar infarction followinghoneybee sting. J Assoc Physicians India2002;50:721–2.

Cause of V pattern strabismus incraniosynostosis: a case reportStrabismus is a common association inpatients with craniosynostosis or craniofacialdysostosis (60–70%).1–3 V pattern exotropia isthe most common ocular motility problem.Various theories have been proposed to

explain the cause of the V pattern andsurgical attempts to correct it with weaken-ing procedures of the inferior oblique havebeen disappointing.2 3

This is a case report of one child with thisdisorder who underwent orbital computedtomography (CT) scans and had a markedimprovement of the V pattern followingstrabismus surgery based on the CT findings.

Case reportThis child with craniosynostosis had under-gone six previous cranial surgeries. She hadthree strabismus surgical procedures includ-ing anterior transpositions of the inferior

obliques in an attempt to correct a large Vpattern. She presented to us with a chin upposition, V pattern exotropia (60 prismdioptres), over-elevation in adduction, limita-tion of depression in adduction, and incomi-tant hypertropias in side gazes (fig 1).Objective fundus excyclotorsion was noted.Orbital imaging demonstrated that all

extraocular muscles in each eye were present,normal in size and shape but anatomicallydisplaced. The extraocular muscles in the lefteye were rotated clockwise and in the righteye were rotated counterclockwise (fig 2).Ineffectiveness of inferior oblique weakeningprocedures and the presence of muscleheterotopy led us to consider that the over-elevation in adduction was most likelyrelated to the anatomical displacement ofthe rectus muscles.Surgical exploration confirmed muscle

heterotopy. The lateral recti were foundslanting inferiorly (fig 3). Repositioning ofthe lateral recti superiorly to a more hor-izontal position and suturing the superiorborder of the muscle belly to the adjacentsclera about 18 mm from the limbus using a

Figure 1 Preoperative V pattern exotropia, over-elevation in adduction, under-depression inadduction in both eyes.

Figure 2 Preoperative coronal views on CTscan of both orbits showing evidence of rectusmuscle heterotopy. A vertical line joining thecentre of the belly of the vertical rectus musclesshows the relative temporal displacement of thesuperior rectus muscle compared to the nasaldisplacement of the inferior rectus muscle. Ahorizontal line joining the centre of the belly ofthe horizontal rectus muscles shows the relativeinferior displacement of the lateral rectusmuscle compared to superior displacement ofthe medial rectus muscle.

Figure 3 Intraoperative photograph. The lefteye is adducted with a muscle hook placedunder the lateral rectus muscle (LR). The lowerline highlights the downward slanting of the leftlateral rectus muscle. A curved ruler is used toshow the normal horizontal path of the lateralrectus muscle.

Figure 4 Postoperative clinical pictures showing improvement in V pattern exotropia withimprovement in versions in adduction.

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non-absorbable suture was the first surgicalprocedure performed by us on this patient.This led to some improvement of the Vpattern. This was followed by recession andnasal repositioning of the superior rectisuturing the nasal border of the muscle bellyto the adjacent sclera about 18 mm from thelimbus using a non-absorbable suture. Thisachieved good alignment in the primaryposition and eliminated the anomalous chinup position, markedly reduced the V pattern,eliminated the over-elevation in adduction,and improved depression in adduction (fig 4).

CommentV pattern strabismus in craniosynostosis maybe related to anatomical malposition of therectus muscles. This may be documented byorbital imaging, which could also aid inplanning the surgical approach. In thesecases the overelevation in adduction andunder depression in adduction may be dueto the anatomical displacement of the rectusmuscles.2

F G Velez, N Thacker, M T Britt, A L RosenbaumJules Stein Eye Institute, 100 Stein Plaza, UCLA Los

Angeles, CA, USA

Correspondence to: Dr Arthur L Rosenbaum, JulesStein Eye Institute, 100 Stein Plaza, UCLA Los Angeles,

CA 90095, USA; [email protected]

doi: 10.1136/bjo.2004.048413

Accepted for publication 13 April 2004

References

1 Coats DK, Paysse EA, Stager DR. Surgicalmanagement of V-pattern strabismus and obliquedysfunction in craniofacial dysostosis. JAAPOS2000;4:338–42.

2 Clark RA, Miller JM, Rosenbaum AL, et al.Heterotopic muscle pulleys or oblique muscledysfunction? JAAPOS 1998;2:17–25.

3 Limon de Brown, Monasterio FO, Feldman MS.Strabismus in plagiocephaly. J PediatrOphthalmol Strabismus 1998;25:180–90.

West Nile virus chorioretinitisWest Nile virus has been described in Africa,Europe, the Middle East, west and centralAsia, Oceania, and has emerged in recentyears in temperate regions of Europe andNorth America.1 West Nile virus was firstisolated from a febrile adult woman in theWest Nile District of Uganda in 1937 andbecame recognised as a cause of severehuman meningoencephalitis in elderlypatients during an outbreak in Israel in1957.2 In 1999, the plight of city birds and acollection of human encephalitis cases inNew York heralded the arrival of West Nilevirus on this side of the Atlantic. From 1999through 2001, there were 149 cases of humanWest Nile virus infection in the United States,including 18 deaths, but in 2002 alone morethan 3500 cases and 200 deaths werereported.3 In 2003, over 9000 cases werereported with more than 300 cases ofneuroinvasive disease.3

The Centers for Disease Control notes thatneuroinvasive disease includes those casesresulting in meningitis, encephalitis, ormeningoencephalitis.3 Cases with ocularinvolvement should probably be included inthis category as well. As our clinical experi-ence in such cases evolves so does ourunderstanding of the ophthalmic manifesta-tions of the disease. Here, we present a case

of ocular involvement with West Nile virus,highlighting the typical ocular findings.

Case reportAn 80 year old man convalescing in a nursinghome from neurological complications ofrecently acquired West Nile virus meningoen-cephalitis presented with bilateral visual lossof unspecified duration. The patient had beenhospitalised 4 months previously for serolo-gically confirmed West Nile virus encephali-tis. His infectious course was complicated byresidual right sided paresis, dysarthria, andgeneralised mental status changes withdementia. Over the following months as heregained his mental faculties he complainedto family members of decreased vision andcentral scotomas, worse in his left eye thanright. His best corrected visual acuity at thistime was 20/40 in the right eye and 20/60 inthe left eye. The patient’s ophthalmic andmedical histories were otherwise non-con-tributory. Biomicroscopic examinationrevealed normal anterior segments withoutinflammation and moderate nuclear scleroticand cortical changes involving both crystal-line lenses. Funduscopic examinationrevealed mild vitreous debris with moderatelylarge areas of retinal pigment epithelial andchoroidal atrophy in the posterior segment(fig 1A and B, right and left eyes, respec-tively) in addition to partially atrophic andpigmented chorioretinal foci throughout theretinal periphery (fig 2A and B, right and lefteyes, respectively).Over the next 3 months the patient devel-

oped problems with his activities of dailyliving at night and glare with lighting.Subsequent examination revealed progres-sion of the lenticular changes and the patientwas referred for cataract extraction. Hereturned 3 months later after uneventfullycataract surgery. He was not on any medica-tions at this time. Best corrected visual acuitymeasured 20/30 in the right eye and 20/40 in

the left eye. Normal anterior segments with-out inflammation and well placed posteriorchamber intraocular lenses were noted. Thevitreous debris persisted and his funduscopicexamination was without change bilaterally.Examination 6 months later and approxi-mately 16 months after initial West Nilevirus infection demonstrated stable ophthal-mic findings and visual acuity.

CommentAlthough ocular symptoms associated withWest Nile virus were first reported in 1956ocular findings in West Nile virus infectionwere first described in the medical literaturesoon after the West Nile virus epidemic inNorth America in 2002.4–8 Initial reportsdescribed analogous clinical findings consist-ing of mild anterior segment inflammation,vitritis, and discrete nummular outer retinal/choroidal lesions which were often linear indistribution and varied in appearance from‘‘creamy whitish-yellow’’ to atrophic withvarious degrees of pigmentation.5–7 9 Mildretinal haemorrhage was also occasionallypresent. Fluorescein angiography revealedthese ‘‘target’’ lesions to be hypofluorescentcentrally and hyperfluorescent peripherally.Leakage from the optic nerve is sometimespresent as optic neuritis and papilloedemamay be associated with contiguous centralnervous system involvement.8–10 Later reportsconfirmed these findings and suggested thatactive lesions associated with vitritis mayappear ‘‘creamy’’ in nature eventually pro-gressing to foci of well circumscribed chorio-retinal atrophy as the disease becomesinactive and subsequently becoming moreprominent with time.9 10 Occlusive vasculitiswithout chorioretinal findings has also beennoted in an isolated case.11

Various ocular inflammatory and infec-tious processes such as toxoplasmosis andjuvenile rheumatoid arthritis have beenassociated with periods of recurrence and

Figure 1 Moderately large areas of retinalpigment epithelial and choroidal atrophy withmild pigmentary disturbance of both foveae arepresent bilaterally, to a greater degree in theleft eye (B) compared to the right (A).

Figure 2 Partially atrophic and pigmentedchorioretinal foci analogous to those noted inprevious reports are distributed throughout theretinal periphery in the right (A) and left (B) eyesrespectively.

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exacerbation after intraocular surgery.12 Thishighlights an important issue with regard toWest Nile virus infection as the risk forneuroinvasive disease is higher for people50 years of age and older, many of whom arecurrently or soon will be candidates forcataract extraction. Our patient did well withroutine postoperative care and surveillanceafter uncomplicated cataract extraction in aneye previously affected by West Nile viruschorioretinitis. The eye remained quiescentwithout evidence of uveitis or reactivation ofpreviously affected fundus lesions. Althoughsurveillance would be recommended forthese patients, our findings suggest thatchorioretinitis associated with West Nilevirus appears to be an acute self limitedprocess without residual sequelae after sub-sequent intraocular surgery.

S ShaikhCentral Florida Retina, 44 Lake Beauty Drive Suite

200, Orlando, FL 32827, USA

M T TreseAssociated Retinal Consultants and William Beaumont

Eye Institute, 3535 W, 13 Mile Road #632, RoyalOak, MI 48073, USA

Correspondence to: Dr Saad Shaikh, Central FloridaRetina, 44 Lake Beauty Drive Suite 200, Orlando, FL

32827, USA; [email protected]

doi: 10.1136/bjo.2004.049460

Accepted for publication 3 May 2004

References

1 Solomon T, Ooi MH, Beasley DW, et al. WestNile encephalitis. BMJ 2003;326:865–9.

2 Smithburn KC, Hughes TP, Burke AW, et al. Aneurotropic virus isolated from the blood of anative of Uganda. Am J Trop Med1940;20:471–92.

3 US National Center for Infectious Diseases,Division of Vectorborne Infectious Diseases,Centers for Disease Control and Prevention, FortCollins, Colorado.

4 Goldblum N, Jasinka-Klingberg W,Klingberg MA, et al. The natural history of WestNile Fever. I. Clinical observations during anepidemic in Israel. Am J Hyg 1956;64:259–69.

5 Vandenbelt S, Shaikh S, Capone A Jr, et al.Multifocal choroiditis associated with West Nilevirus encephalitis. Retina 2003;23:97–9.

6 Adelman RA, Membreno JH, Afshari NA, et al.West Nile virus chorioretinitis. Retina2003;23:100–1.

7 Bains HS, Jampol LM, Caughron MC, et al. Vitritisand chorioretinitis in a patient with West Nilevirus infection. Arch Ophthalmol2003;121:205–7.

8 Vaispapir V, Blum A, Soboh S, et al. West Nilevirus meningoencephalitis with optic neuritis. ArchIntern Med 2002;162:606–7.

9 Hershberger VS, Augsburger JJ, Hutchins RK, etal. Chorioretinal lesions in nonfatal cases of WestNile virus infection. Ophthalmology2003;110:1732–6.

10 Anninger WV, Lomeo MD, Dingle J, et al. WestNile virus-associated optic neuritis and choriore-tinitis. Am J Ophthalmol 2003;136:1183–5.

11 Kaiser PK, Lee MS, Martin DA. Occlusivevasculitis in a patient with concomitant West Nilevirus infection. Am J Ophthalmol2003;136:928–30.

12 Bosch-Driessen LH, Plaisier MB, Stilma JS, et al.Reactivations of ocular toxoplasmosis aftercataract extraction. Ophthalmology2002;109:41–5.

Swimming goggles suckWe present a complication arising from theuse of swimming goggles in a patient withglaucoma drainage blebs.

Case reportA 73 year old white man with poorly con-trolled primary open angle glaucoma under-went routine trabeculectomy with adjunctive5-fluorouracil to the right eye, followed bythe same procedure to the left eye 6 weekslater. Preoperatively the intraocular pressureswere 28 mm Hg bilaterally and cup:discratios were 0.95 right, 0.8 left. Early post-operative intraocular pressure (IOP) in theright eye was low (5 mm Hg at weeks 2 and6), but uncomplicated. The recovery of theleft eye was uneventful, and at 3 months theIOPs were 10 mm Hg right eye, 12 mm Hgleft.However, at 4 months the patient pre-

sented with discomfort and redness in theright eye. A large extension of the bleb hadformed at the nasal limbus, with an asso-ciated corneal dellen (fig 1A and B). The IOPhad increased in the right eye, which wastreated with a needling procedure and 5-FUinjection, repeated 3 weeks later.Subsequently the bleb extension recededand the previously elevated right nasalconjunctiva was found to be firmly adherentto the underlying sclera (fig 1C).He re-presented 7 months after the initial

surgery with redness and swelling, this timein the left nasal conjunctiva (fig 1D). At thispoint the patient mentioned that he was akeen swimmer and inquired whether hisproblem could have been caused by the useof swimming goggles. He had resumedregular swimming 2 weeks before developingthe right eye complication, then stopped. Hehad resumed again 3 months before devel-oping the left.With this in mind, we set out to investigate

the pressure changes inside swimming gog-gles. With a pressure transducer fixed to oneeyepiece (fig1E), we recorded a comfortablerange of 21 to 25 mm Hg, discomfort over210 mm Hg and a maximum suction of244 mm Hg. Upon removing the goggle, atransient negative pressure spike was alsoproduced (fig 1F). Given these observationsand the timing of the clinical events, wesurmise that the patient’s bleb extensions

were plausibly consequent upon his aquaticactivities.

CommentPrevious reports of barotrauma sustainedwhile wearing overtight goggles includesuction petechiae1 and changes in the eyelidskin,2 but we are not aware of any informa-tion concerning the effects of swimminggoggles on glaucoma drainage blebs. Whengoggles are applied, firm pressure displaces asmall volume of air and creates a negative‘‘intragoggle’’ pressure, the basis by which aseal is maintained. In a person who hasundergone trabeculectomy, an increase in thetransconjunctival pressure gradient couldopen up a weakness in the perimeter of thebleb and cause it to extend in the direction ofleast resistance.Other experimental work has examined

the pressure changes occurring in the maskspace during scuba diving.3 This is a ratherdifferent system as the nose is included in themask, allowing the pressure to be equalisedby exhaling through the nose. The eye andperiocular structures can be subjected tosignificant negative pressures if this is notdone, but the duration is usually limited bythis pressure gradient acting across thetympanic membrane, causing pain andprompting the diver to ascend or equalise.Ocular barotrauma can result in subconjunc-tival haemorrhage and chemosis, and it hasbeen recommended that patients wait aminimum of 2 months after glaucoma filter-ing surgery before resuming scuba diving.4

We do not believe patients who haveundergone trabeculectomy need to ceaseswimming, but they should be aware thatgoggles may be able to produce excessivenegative pressure if they form a very tightseal.

L A Wakely, G Reeves, N Ashraff, A P WellsDepartment of Ophthalmology, Wellington Hospital,

Wellington, New Zealand

Correspondence to: Dr Laura Wakely, Department ofOphthalmology, Wellington Hospital, Wellington,

New Zealand; [email protected]

Figure 1 (A) (B) Right eye at 4 months postoperatively showing corneal dellen and nasal blebextension (A) and the adjoining isthmus (B) with arrows at each end. (C) Regression of the rightaccessory bleb after needling, 5-fluorouracil, and topical steroids. (D) Left eye at 7 monthspostoperatively with smaller and slightly inflamed nasal accessory bleb. (E) Pressure transducersetup measuring ‘‘intragoggle’’ pressure using AD Instruments Powerlab (www.adinstruments.com)and IOP transducer (gold disc). (F) Transducer recording showing several goggle applications(positive pressure, ‘‘a’’ labels) and the transient negative pressure spikes produced on removingthem (‘‘r’’ labels); In area 1 of the trace, the goggles were overtight and in area 2 they werecomfortable.

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doi: 10.1136/bjo.2004.048371

Accepted for publication 17 April 2004

References

1 Jowett NI, Jowett SG. Ocular purpura in aswimmer. Postgrad Med J 1997;73:819–20.

2 Ruban JM, Mallem M. The eyelid of thecompetitive swimmer [article in French: Lapaupiere du nageur de competition]. J FrOphtalmol 1995;18:426–34.

3 Senn P, Helfenstein U, Senn ML, et al. Ocularbarostress and barotrauma. A study of 15 scubadivers [in German]. Klin Monatsbl Augenheilkd2001;218:232–6; discussion 237–8.

4 Butler FK Jr. Diving and hyperbaricophthalmology. Surv Ophthalmol1995;39:347–66.

Immune recovery disease: a caseof interstitial keratitis and tonicpupil following bone marrowtransplantationImmune recovery disease results from animmunological response to circulating viralantigens in the host after bone marrowtransplant (BMT) mediated immune recon-stitution. It may also occur after successfulantiretroviral therapy in patients with HIVand AIDS. We report a case of a child withsevere combined immune deficiency (SCID)and disseminated varicella zoster virus (VZV)infection who developed interstitial keratitisand a tonic pupil after BMT.

Case reportAn 8 month old male infant was referred tothe ophthalmology clinic at Great OrmondStreet because of suspected congenital glau-coma. The past ophthalmic and familyhistory were unremarkable. The child wasborn with multiple congenital anomalies ofthe lower limbs which included bilateraltibial deficiencies, and an extreme talipesequinovarus of the right foot.The child had a known history of dissemi-

nated varicella infection caused by SCID(fig 1). On examination it was noted thathe had a generalised vesicular rash through-out his body extending to his eyelid margins.The eyes were white with clear corneas andhe was alert, fixing and following well withfull extraocular eye movements. Both pupilswere reactive to light with no afferentpupillary defect. The anterior chambers wereunremarkable and the intraocular pressurewith the Perkins tonometer was 16 mm Hgbilaterally. Examination of the fundus,including cup to disc ratio was normal. Hewas reviewed periodically over the next6 months while an inpatient undergoingtreatment for SCID. During this period, hewas persistently positive for VZV DNA in hisblood determined by polymerase chain reac-tion (PCR) analysis but was negative forEpstein-Barr virus (EBV), herpes simplexvirus (HSV), cytomegalovirus (CMV), andadenovirus DNA. He suffered a number ofexacerbations of the varicella infection andwas treated with systemic aciclovir, foscarnet,and cidofovir.At 12 months of age he underwent allo-

geneic BMT from a one antigen mismatchedunrelated donor following reduced intensityconditioning with fludarabine, melphalanand alemtuzumab. Engraftment was veryrapid with neutrophils appearing by day 10.Before BMT the CD3+ CD4+ count was

0.046109/l but 7 weeks after BMT the CD3+CD4+ count was 0.476109/l.Four weeks after BMT his mother noted

bilateral corneal haze which was moremarked on the left eye. He was reviewed inhis isolation cubicle with a hand held slitlamp and Perkins tonometer and was foundto have bilateral corneal stromal haze andcorneal vascularisation (fig 2). There was noconjunctival injection, and his intraocularpressure was 13 mm Hg in each eye. Bothpupils were reactive to light. The lymphocytecount had recovered at this point to morethan 1.06109/l.An examination under anaesthesia was

arranged and a diagnosis of interstitialkeratitis without epithelial involvement wasmade. He was treated with intensive topicalprednisolone acetate 1% (one drop every2 hours) and cyclopentolate 1% twice dailyto both eyes. Betaxolol 0.5% twice daily wasprescribed prophylactically to prevent raisedintraocular pressure which could exacerbatethe corneal haze. The child was reviewedregularly and the stromal vascularisation wasseen to regress. He was thus gradually weanedoff the steroid drops to one drop daily andthe cycloplegics were stopped. Threemonths after BMT his mother reported achange in pupillary size in the right eye.On examination the right pupil was mid-dilated and oval in shape, not reactive tolight and there was no evidence of poster-ior synechiae (fig 3). A diagnosis of a righttonic pupil was made.At the most recent review, 6 months

following BMT he had clear corneas centrallyin both eyes with some persistent peripheralstromal vessels, and a right tonic pupil.Unaided visual acuity was 0.60 logMAR withboth eyes using the Cardiff acuity test (KeelerLtd, Windsor, UK). There was a left fixationpreference and amblyopia therapy was com-menced with occlusive patches.Currently, the child has an ongoing mild

chronic graft versus host disease affecting theskin and intestine which is controlled withlow dose systemic steroids. His systemic

medications also include aciclovir 120 mgfour times daily.

CommentSevere combined immune deficiencies(SCID) are a rare heterogeneous group ofdisorders characterised by severe T cell and Bcell deficiency with low or absent antibodylevels. They usually manifest in the firstmonths of life with severe and recurringinfections leading to death often by the age of2 years.1

Since 1968 these diseases have beensuccessfully treated with haemopoietic stemcell transplantation.2 Varicella infection hasbeen associated with severe immune dys-function following BMT and it has beenshown that severe disseminated varicellainfection causes ocular disease that mimicsthe sequelae of herpes zoster ophthalmicus.3

In the adult population, the commonestcause of interstitial keratitis is HSV infectionwhereas varicella infection is considered arare cause.4 In children, although varicellainfection is extremely common, ocular com-plications of this disease are rare.5

If keratitis develops in association with achildhood viral exanthem it is important toconsider a number of possible infectiousagents such as HSV, EBV, mumps, syphilis,Lyme disease, or tuberculosis in the differ-ential diagnosis.4 In this setting, other docu-mented complications in association withSCID include bilateral viral endophthalmitis,6

CMV retinitis, and optic neuritis.7 In this casethe history and the physical findings werehighly suggestive of the diagnosis and wereconfirmed by PCR testing. As far as we areaware this is the first case of varicella

Figure 1 Photograph of the child with severesystemic varicella a few days after admission.

Figure 3 Photograph of the right eye showingthe tonic pupil and corneal stromalvascularisation.

Figure 2 Photograph of the left eye showingdeep and superficial corneal stromal vessels.

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associated interstitial keratitis and a tonicpupil occurring in a child with SCID follow-ing BMT.The signs of early interstitial keratitis and a

right tonic pupil were noted by the child’smother about 4 weeks after the allogeneicbone marrow transplant. We believe that animmunological response to pre-existing var-icella was responsible for the development ofthe eye signs. This signifies a positiveresponse from a nascent immune system inthe recipient—an example of immune recov-ery disease.There is experimental evidence to support

this as it has been shown that wholelymphocyte and splenocyte transfer leads toherpes simplex keratitis in SCID mice.8 Inother words SCID mice reconstituted with Tlymphocytes of the CD4 + phenotype devel-oped subsequent corneal lesions in relation toHSV infection. Conversely Mercadal et al haveshown that unreconstituted SCID miceremained lesion free when infected withHSV.8 This suggests that herpes simplexkeratitis is a T cell mediated immunopatho-logical reaction to virus in the cornea. In ourcase the corneal changes occurred followingbone marrow reconstitution. Before BMT theCD3+ CD4+ count was 0.046109/l but7 weeks following BMT the CD3+ CD4+count was 0.476109/l. The corneal changesbecome apparent at about 4 weeks afterBMT. We believe that our case illustrates asimilar mechanism in the human model inrelation to varicella infection.The tonic pupil developed as a consequence

of a post-viral ganglionitis affecting theciliary ganglion and the short posterior ciliarynerves, a rare but previously describedcomplication of varicella infection.9 Otherreported cases of ophthalmic immune recov-ery disease include a case of varicella zostervirus associated anterior stromal keratitis in apatient with AIDS10 and, in another case, inassociation with CMV retinitis.11

Considering BMT, varicella zoster virusassociated disease can be a frequent compli-cation following autologous and allogeneictransplantation.12 Other complications inrelation to BMT include pseudomembranousconjunctivitis,13 keratoconjunctivitis sicca,14

cataracts,15 and severe graft versus hostdisease.16

This child did suffer a graft versus hostdisease-like rash at the time of the develop-ment of the keratitis. While it is possible thatthe keratitis was purely Graft versus hostdisease this seems unlikely, given that therewas no conjunctival involvement and that thegraft versus host disease was extremely mild.Furthermore, in this case the disseminatedvaricella infection preceded the BMT andformed the basis for identifying a severeimmune deficiency in the child. It high-lights the importance of frequent ophthalmicfollow up in the immediate period followingBMT as there is an increased risk of oculardisease.

A S Ioannidis, M Forrest, K K NischalDepartment of Ophthalmology, Great Ormond Street

Hospital for Children, London, UK

M Forrest, K K NischalDepartment of Visual Sciences, Institute of Child

Health, London, UK

P Veys, E G DaviesDepartment of Bone Marrow Transplantation andImmunology, Great Ormond Street Hospital for

Children, London, UK

G WoodruffDepartment of Ophthalmology, Leicester Royal

Infirmary, Leicester, UK

Correspondence to: Mr K K Nischal, Department ofOphthalmology, Great Ormond Street Hospital, Great

Ormond Street, London WC1 3JH, UK;[email protected]

doi: 10.1136/bjo.2004.044057

Accepted for publication 26 April 2004

References

1 Rosen FS, Cooper MD, Wedgewood RJ. Theprimary immunodeficiencies. N Engl J Med1995;333:431–40.

2 Antoine C, Muller S, Cant, et al. Long-termsurvival and transplantation of haemopoietic stemcells for immunodeficiencies: report of theEuropean experience 1968–99. Lancet2003;361:553–9.

3 Walton RC, Reed KL. Herpes zoster ophthalmicusfollowing bone marrow transplantation inchildren. Bone Marrow Transplant1999;23:1317–20.

4 Schwartz GS, Harrison AR, Holland EJ. Etiologyof immune stromal (interstitial) keratitis. Cornea1998;17:278–81.

5 Ragozzino MW, Melton LJ, Kurland LT, et al.Population-based study of herpes zoster and itssequelae. Medicine 1982;61:310–16.

6 Borne MJ, Shields JA, Shields CL, et al. Bilateralviral endophthalmitis as the presenting sign ofsevere combined immunodeficiency. ArchOphthalmol 1994;112:1280–1.

7 Perren BA, Raisanen J, Good WV, et al.Cytomegalovirus retinitis and optic neuritis in achild with severe combined immunodeficiencysyndrome. Retina 1996;16:117–21.

8 Mercadal CM, Bouley DM, DeStephano D, et al.Herpetic stromal keratitis in the reconstituted SCIDmouse model. J Virol 1993;67:3404–8.

9 Heger T, Kolling GH, Dithmar S. Atypical tonicpupil as a complication of chickenpox infection.Ophthalmologe 2003;100:330–3.

10 Naseri A, Margolis TP. Varicella zoster virusassociated immune recovery stromal keratitis in apatient with AIDS. Br J Ophthalmol2001;85:1384.

11 Holland GN. Immune recovery uveitis. OcularImmunol Inflamm 1999;7:215–21.

12 Han CS, Miller W, Haake R, et al. Varicella zosterinfection after bone marrow transplantation:incidence, risk factors and complications. BoneMarrow Transplant 1994;13:277–83.

13 Jabs DA, Wingard J, Green R, et al. The eye inbone marrow transplantation. Arch Ophthalmol1989;107:1343–8.

14 Calinssendorff B, el Azazi, et al. Dry eyesyndrome in long- term follow-up of bone marrowtransplanted patients. Bone Marrow Transplant1989;4:675–8.

15 Dunn JP, Jabs DA, Wingard J, et al. Bonemarrow transplantation and cataractdevelopment. Arch Ophthalmol1993;111:1367–73.

16 Jack MK, Hicks JD. Ocular complications in high-dose chemoradiotherapy and marrowtransplantation. Ann Ophthalmol1981;6:709–11.

Occult macular dystrophy in an11 year old boyOccult macular dystrophy (OMD) is aninherited macular dystrophy characterisedby a progressive decline of visual acuitywithout visible fundus abnormalities.1–3 Inthese patients, the fluorescein angiogramsand conventional full field electroretinograms(ERGs) are normal, but the amplitudes of thefocal macular ERGs and multifocal ERGs aresignificantly reduced but only in the centralretina.1–4

The age at the onset of symptoms in OMDpatients is relatively old,1–3 and the first visitto the hospital is aged 20 years or more withthe youngest being 16 years in most cases.Here, we present an 11 year old boy who wasdiagnosed as having OMD because of theresults of electrophysiological and psycho-physical tests.

Case reportAn 11 year old boy was referred to ourhospital with a complaint of progressivedecline of vision in both eyes. His correctedvisual acuity was 20/25 in both eyes at 6 yearsof age, but had decreased to 20/33 at 10 yearsof age. Family history revealed no othermembers to have any eye diseases. At theinitial examination, his visual acuity was 20/40 right eye and 20/33 left eye with 23.0dioptres (D) in both eyes. The fundusexamination and fluorescein angiogramswere normal (fig 1). The peripheral visualfields were intact but a relative centralscotoma was detected with the I-2 targetwithin 10 degrees in both eyes. A moderatered-green defect was found on the Ishiharapseudoisochromatic plates, Hardy-Rand-Rittler pseudoisochromatic plates, andFarnsworth-Munsell 100 hue test.The amplitude of full field ERGs were

within the normal range for both rod andcone components (fig 2A). However, focalmacular ERGs with 5, 10, and 15 degreesstimulus spots3 were severely reduced andessentially absent (fig 2B). The multifocalERGs4 demonstrated a loss of local responsesin the central retina (fig 2C).Psychophysical rod and cone sensitivity

was performed on his right eye with 31 testpoints across the 60 degree horizontal mer-idian using a previously described method.5

The cone sensitivities were severely affectedin the central retina but fell within thenormal range in the periphery (fig 2D). Therod sensitivities were at the lower borderlineat almost all locations tested (not shown).At present (August 2003, 13 years old), his

acuity has decreased to 20/50 in both eyes, buthis fundi still remain normal in both eyes.

CommentThis boy had a progressive decrease of visualacuity in both eyes, and his fundus examina-tions and fluorescein angiograms were com-pletely normal. The amplitude of theconventional full field ERGs were also withinthe normal range for both rod and conecomponents. However, focal macular coneERGs and multifocal ERGs were severelyreduced in the central retina. Results ofpsychophysical perimetry showed a reductionof cone sensitivity but only in the centralretina. These findings are consistent with theclinical characteristics of OMD which wehave previously reported.1 3 4

OMD in children is very rare.1–3 In our 42consecutive OMD patients seen at the NagoyaUniversity Hospital from 1988 to 2003, theage at initial visit to the hospital ranged from16 to 74 years (mean 45.8 years), and 95.2%of patients visited the hospital at 20 year oldor more. To the best of our knowledge, thisboy is the youngest case with OMD reportedanywhere.We would like to emphasise that OMD can

be found even in children. Because thefundus examination and full field ERGs arenormal in these patients, these children areapt to be misdiagnosed as optic nerve dis-ease, central nerve disease, or psychological

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disorders. Focal or multifocal ERG techniquesare the only key to diagnose this rare type ofmacular dystrophy.

M Kondo, S Ueno, C-H Piao, Y Ito, H Terasaki,Y Miyake

Department of Ophthalmology, Nagoya UniversitySchool of Medicine, 65 Tsuruma-cho, Showa-ku,

Nagoya 466-8550, Japan

Correspondence to: Mineo Kondo, MD, Departmentof Ophthalmology, Nagoya University School of

Medicine, 65 Tsuruma-cho, Showa-ku, Nagoya 466-8550, Japan; [email protected]

doi: 10.1136/bjo.2004.047555

Accepted for publication 27 April 2004

References

1 Miyake Y, Ichikawa K, Shiose Y, et al. Hereditarymacular dystrophy without visible fundusabnormality. Am J Ophthalmol 1989;108:292–9.

2 Matthews GP, Sandberg MA, Berson EL. Fovealcone electroretinograms in patients with centralvisual loss of unexplained etiology. ArchOphthalmol 1992;110:1568–70.

3 Miyake Y, Horiguchi M, Tomita N, et al. Occultmacular dystrophy. Am J Ophthalmol1996;122:644–53.

4 Piao CH, Kondo M, Tanikawa A, et al. Multifocalelectroretinogram in occult macular dystrophy.Invest Ophthalmol Vis Sci 2000;41:513–17.

5 Jacobson SG, Voigt WJ, Parel JM, et al.Automated light- and dark-adapted perimetry forevaluating retinitis pigmentosa. Ophthalmology1986;93:1604–11.

Pseudomonas aeruginosamicrobial keratitis secondary tocosmetic coloured contact lenswearCosmetic coloured contact lenses are worn togive the appearance of a different or unusualeye colour and about 60 000 people in theUnited Kingdom obtain these types of contactlenses through eye care professionals.1 Asubset of these lenses—those with no opticalpower (‘‘plano’’ coloured lenses)—falls out-side legislation designed to restrict the sale ofcontact lenses to suitably qualified profes-sionals. We report a severe case of microbialkeratitis caused by Pseudomonas aeruginosawhich has resulted in lasting visual impair-ment in a patient obtaining cosmeticcoloured contact lenses from a fashion shoprather than through an eye care practitioner.

Case reportAn 18 year old south Asian male studentpresented in December 2003 with a 2 dayhistory of a foreign body sensation in his lefteye. One day before presentation the eye hadbecome slightly red. He had commenced theuse of Brolene eye drops which had beenpurchased from a large chain supermarket.The eye then became painful with eyelidswelling and he presented to the local districtgeneral hospital the following day. He wasdiagnosed with a corneal ulcer and referredto our institution.He reported a 12 month history of cosmetic

coloured plano contact lens wear, havingpurchased the lenses from a fashion shoprather than through an eye care professional.

30

20

10

100 ms

1 µV0

Retinal eccentricity (degree)

Cone sensitivity D

15°

ON

10°

5°

Multifocal ERG

Normal Patient Normal Patient

C

30 Hzflicker

Cone

Rod

Brightwhite

Focal macular cone ERGBFull field ERGA

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itivi

ty (d

B)

–30 30–20 –10 0 10 20

25 ms 25 ms25 µV 1 µV

25 ms

100 µV

25 ms

200 µV

50 ms

100 µV

ON

Figure 2 Results of conventional full field ERG, focal macular cone ERG, multifocal ERG, and coneperimetry in our patient. Full field ERGs, focal macular cone ERGs, multifocal ERG, and coneperimetry are recorded with previously reported methods.3–5

Grant support: Grant in aid 13307048 (YM),14370557 (HT), 14770952 (MK) from the Ministryof Education, Science, Sports and Culture, Japan.

Proprietary interest: None.

Figure 1 Fundus photographs (upper) and fluorescein angiograms (lower) of the 11 year old boy.

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No counselling was provided at the point ofpurchase regarding a hygiene routine, care oflenses, or possible complications associatedwith their use. He wore the lenses 12 hoursper day, 7 days per week without any over-night use. The lenses were designed to makethe eye appear grey or blue (patient’s naturaleye colour was brown). There was no pastmedical or ocular history of note includingamblyopia.On examination the unaided vision was 6/6

in the right eye and 6/36 in the left eye. Theleft eye demonstrated a mid-peripheral cor-neal infiltrate in the 4 o’clock position withoverlying 2.4 mm diameter ulcer, and sur-rounding stromal swelling (fig 1). There wasa 0.5 mm height hypopyon. The intraocularpressure was within the normal range. Theright cornea demonstrated a very smallperipheral infiltrate with no significant ante-rior chamber reaction. Both posterior seg-ments were unremarkable. A corneal scrapewas performed with the Gram stain demon-strating a small quantity of neutrophils andGram negative bacilli. Ofloxacin 0.3% dropswere commenced every hour to the left eye.The peripheral infiltrate resolved with thecorneal epithelial healed by day 10. Topicalprednisolone 0.5% was commenced on day 4.A more central mid stromal corneal infiltrateencroaching on the visual axis developed onday 1 after admission and has graduallybecome less prominent during follow up over3 months (fig 2) although the visual acuityremains reduced at 6/36. Pseudomonas aerugi-nosa was grown from the corneal scrape,sensitive to ciprofloxacin, ofloxacin, genta-micin, and ceftazadine. The right eye was notscraped, responded well to topical ciproflox-acin drops, and did not develop any scarring.The contact lenses and their cases were alsoinvestigated as there was a high degree ofsuspicion that clinically they would be con-taminated. All grew Pseudomonas aeruginosawith a sensitivity profile identical to thecorneal scrape specimen. Mixed coliformgrowth was also noted also in one of thecontact lens cleaning solutions.

CommentThe use of cosmetic coloured plano contactlenses, sourced via non-professional suppliersis becoming increasingly common and fash-ionable. Their use over the past 12 monthshas increased fourfold and stores havereportedly sold more than one million pairs.2

Their purchase is currently possible fromnon-eye care professional retailers withoutany ocular assessment, customised fitting, orverbal counselling regarding a hygiene rou-tine, care of the lenses, or possible complica-tions associated with their use. In addition,there is often no plan for follow up.

Potential complications are the same asthose for all contact lenses and have beendocumented in a recent case series in theUnited States.3 Pseudomonas aeruginosa micro-bial keratitis with vision loss requiringelective penetrating keratoplasty, presumedherpes simplex related corneal scarring caus-ing legal blindness, acute iridocyclitis, cornealhypoxia, microcystic oedema, punctuate ker-atopathy, corneal abrasions, and giant papil-lary conjunctivitis were all documented.In the United Kingdom, the Opticians Act

1989 states that a person who is not aregistered medical practitioner or registeredoptician shall not fit contact lenses. Plano (or‘‘afocal’’) contact lenses are not included inthis act because they have no optical power.The General Optical Council has receivedreports of these lenses being shared andexchanged between wearers and of sales staffdemonstrating fitting on themselves beforeoffering the lens to the purchaser.4 InNovember 2000 the General Optical Councilsubmitted recommendations to theDepartment of Health arguing that primarylegislation should be passed stipulating thatthe fitting and sale of plano contact lensesshould also fall within the terms of the act.On 28 October 2003 Mr John Robertson, MPfor Anniesland, Glasgow, moved a bill toamend the Opticians Act 1989 to includeplano contact lenses in the restrictionsalready placed on the sale of other contactlenses.5

This case report highlights the potentialcomplications of these lenses and supportslegislation restricting their sale.

B J Connell, A TulloManchester Royal Eye Hospital, Manchester, UK

P B MorganEurolens Research, The University of Manchester,

Manchester, UK

M ArmstrongManchester Royal Infirmary, Manchester, UK

Correspondence to: Benjamin J Connell, ManchesterRoyal Eye Hospital, Manchester, UK; connnellb@

netspace.net.au

doi: 10.1136/bjo.2004.049387

Accepted for publication 29 April 2004

References

1 Morgan PB. Healthcheck on the contact lensmarket. Optician 2003;226:32–33.

2 McKie R. ‘Alien’ lenses put young eyes at risk. TheObserver, 2003 November 9.

3 Steinemann TL, Pinninti U, Szczotka, et al.Ocular complications associated with the useof cosmetic contact lenses from unlicensed

vendors. Eye Contact Lenses 2003;29:196–200.

4 Cosmetic contact lenses draft explanatory notes.5 The United Kingdom Parliament. Bill moved by

Mr John Robertson (Glasgow, Anniesland): Non-Prescription Contact Lenses. 28 October 2003:Column 176, 1.21 pm.

Severe proliferative retinopathyin a patient with advancedmuscular dystrophyThe patient is a 25 year old white man withDuchenne muscular dystrophy (DMD), com-plicated by respiratory failure requiring ven-tilatory assistance and impaired cardiacfunction. His ocular complaints were ‘‘floa-ters’’ and decreased vision over the preceding6 weeks. He had no history of ocular diseaseor trauma. The patient’s level of alertness wasreported to routinely fluctuate but no newneurological findings were present. The bestcorrected visual acuity was count fingers inthe right eye and 20/70 in the left eye. Theintraocular pressures were 14 and 8 mm Hg.The anterior segment examination was unre-markable with no neovascularisation of theiris or angle. Biomicroscopy revealed bilateralvitreous haemorrhage. Indirect ophthalmo-scopy showed the retinal periphery to beattached in both eyes. The optic discs andmacula were partially obscured by haemor-rhage. Fluorescein angiography revealeddelayed filling and venous beading in botheyes, without central or branch, vascularocclusion. Hyperfluorescence, consistent withneovascularisation, was present along thetemporal vascular arcades and at the opticdiscs. Fundus photography corroborated theangiographic findings (see figs 1 and 2).Indirect laser with scleral depression

resulted in full treatment of retina outsideof the vascular arcades. Treatment appearedto have little effect on neovascular progres-sion. Overwhelming anaesthetic risk pre-vented intraocular procedures. Both eyesprogressed to subtotal traction retinal detach-ment and counting fingers vision.

CommentThe working diagnosis was retinal ischaemiasecondary to hypoperfusion or pan-micro-vascular occlusive disease. The cardiac ejec-tion fraction was 20% of predicted; the forcedvital capacity was 14% of predicted and theforced expiratory volume in 1 second was15% of predicted. We believe that cardio-pulmonary compromise was a primary

Figure 1 Large corneal infiltrate withoverlying area of ulceration on presentation.

Figure 2 Residual central corneal infiltrate at1 month after presentation.

Figure 1 Colour fundus photograph of theright eye depicting venous beading (arrow),neovascularisation of the disc (arrowheads),and vitreous haemorrhage.

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contributor to the development of retinalneovascularisation. Arterial blood gas analy-sis was not available. The patient was onCoumadin for cardiac indications. He was nota diabetic and finger stick blood sugars wereconsistently in the low to normal range.Additional normal evaluation included ery-throcyte sedimentation rate, anticardiolipin,C reactive protein, C3, C4, total complement,C1q complex, and a Raji assay. The presenta-tion, appearance, and course were not typicalfor Terson’s syndrome, Valsalva retinopathy,or Takayasu disease.Duchenne muscular dystrophy is the most

common X linked neuromuscular disorder. Ithas an incidence of one in 3500 male births.1–3

DMD results from a gene mutation thatleads to altered or absent dystrophin pro-duction.4 Dystrophin is normally expressed inthe retina and localises to photoreceptor term-inals and around retinal vessels. Deficiencyof dystrophin produces abnormal transmis-sion between photoreceptors and opticnerve bipolar cells and a diminishedelectroretinogram (ERG) signal.5 Mice lack-ing the Dp71 isoform of dystrophin suffergreater damage to the ganglion cell layerfollowing transient ischaemia than wild typemice.6 Therefore, dystrophin may be involvedin the regulation of ischaemic processes inthe retina. Cardiopulmonary assist is notroutinely associated with proliferative retino-pathy in adults. Retinal neovascularisation isnot prevalent in the Duchenne population,suggesting that absence of dystrophin is notsufficient to induce neovascularisation alone.In summary, rapidly progressive, bilateral

proliferative retinopathy may be associatedwith DMD in the presence of severe cardio-pulmonary compromise. Whether an absenceof dystrophin contributes directly or indir-ectly is unknown but consideration of thepossibility may lead to novel insights into thedevelopment of pathological retinal neovas-cularisaton. The visual prognosis with latepresentation in this setting is uncertaindespite full panretinal photocoagulation.Patients with advanced DMD may benefitfrom periodic fundus examination as it is notknown whether early treatment has thepotential to alter prognosis.

K LouieJohns Hopkins University School of Medicine,

Baltimore, MD, USA

R S ApteDepartment of Ophthalmology Washington University

School of Medicine, St Louis, MO, USA

K MoriDepartment of Ophthalmology, Saitama Medical

School, Iruma, Saitama, Japan

P GehlbachDepartments of Ophthalmology The Johns HopkinsUniversity School of Medicine, Baltimore, MD, USA

Correspondence to: Peter Gehlbach, MD, PhD, JohnsHopkins University School of Medicine, 600 N Wolfe

Street, Baltimore, MD 21287-9277, USA;[email protected]

doi: 10.1136/bjo.2004.046615

Accepted for publication 4 May 2004

References

1 Emery AEH. Duchene muscular dystrophy, 2nded. Oxford: Oxford University Press, 1993.

2 Engel AG, Franzini-Armstrong C. Myology. NewYork: McGraw-Hill, 1994.

3 Bogdanovich S, Perkins KJ, Krag TO, et al.Therapeutics for Duchenne muscular dystrophy:current approaches and future directions. J MolMed 2004;824:102–15.

4 Hoffman EP, Brown RH Jr, Kunkel LM. Dystrophin:the protein product of the Duchenne musculardystrophy locus. Cell 1987;515:919–28.

5 Pillers DA. Dystrophin and the retina. Mol GenetMetab 1999;68:304–9.

6 Dalloz C, Sarig R, Fort P, et al. Targetedinactivation of dystrophin gene product dp71:phenotypic impact in mouse retina. Hum MolGenet 2003;12:1543–54.

Bilateral decompressionretinopathy after orbitaldecompression surgeryDecompression retinopathy is defined asretinal haemorrhages that typically occurafter glaucoma filtration surgery.1 2

Orbital decompression is a common sur-gery performed to treat patients with thyroidrelated orbitopathy for functional or cosmeticindications.3 4 Many complications have beendescribed with the surgery, but this surgeryhas never been associated with retinal hae-morrhages.We describe a case of a 70 year old woman,

who developed bilateral retinal haemor-rhages after staged bilateral orbital decom-pression surgeries.

Case reportA 70 year old woman with the diagnosis ofeuthyroid Graves’ disease was referredbecause of severe proptosis. Past ophthalmichistory revealed two previous strabismussurgeries. Past medical history was unre-markable with no history of diabetes orcardiovascular disease, also she was nottaking aspirin or any other blood thinningmedications.Ophthalmic examination showed visual

acuity of 20/20 in each eye. Both orbits weremoderately firm to retropulsion. IOP waswithin normal limits in primary gaze (14,19 mm Hg) and slightly elevated in upgaze(17, 26 mm Hg). There were limitations inupgaze and lateral gaze in both eyes as wellas upper and lower lids retractions. There wasa mild degree of lagophthalmos with expo-sure keratopathy. Funduscopy was normaland did not show any evidence of micro-vascular disease or retinal haemorrhages.Hertel measurements were 22 mm on the

right and 23 mm on the left. Computedtomography scan showed enlargement ofthe extraocular muscles.She underwent balanced orbital decom-

pression surgery on the left side, includingdeep lateral and medial wall decompressionwith intraconal fat removal. Three days aftersurgery she noted spots in front of her lefteye. Visual acuity in that eye was 20/25.Funduscopic examination disclosed dot andblot haemorrhage with flame shaped hae-morrhages in the posterior pole of the left eye(fig 1).The patient was well informed of the

complication in the first eye and the chanceof developing retinal haemorrhages in theright eye after orbital decompression. Sheagreed to undergo surgery and 1 week latershe underwent balanced orbital decompres-sion on the right side. Three days later sheagain noted spots in front of her right eye.Best corrected visual acuity decreased to 20/160, and funduscopic examination revealedposterior pole retinal haemorrhages (fig 2).Three months postoperatively IOP in pri-

mary gaze decreased to 12 mm Hg in botheyes, and 14 and 16 mm Hg in upgaze.Exophthalmos decreased to 18 mm on eachside, and the lagophthalmos and exposurekeratopathy resolved. Fluorescein angiogra-phy showed evidence of blocked fluorescence,suggestive of retinal haemorrhage. There wasno evidence of neovascularisation, vasculo-pathy, or choroidal rupture. Visual acuitygradually improved over the course of3 months and returned to 20/20 in both eyes.

Figure 2 Fluorescein angiogram of the righteye showing venous beading (arrow), leakagefrom neovascularisation on the disc(arrowheads) and blocking vitreoushaemorrhage; the visible retina is attached.

Figure 1 Fundus photograph (upper image),left eye, 4 days after orbital decompressionsurgery on the left side showing scatteredretinal haemorrhages both in deep andsuperficial layers of the retina. VA 20/25.(Lower image) Fluorescein angiography, lefteye, late frame showing blocked fluorescencefrom retinal haemorrhages.

Supported in part by Research to Prevent Blindness,Juvenile Diabetes Foundation, Stewart Trust, NEI-KO8(PG).

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CommentDecompression retinopathy is a rare compli-cation that may occur after glaucoma filtra-tion surgery. It is associated with scatteredretinal haemorrhages concentrated in theposterior pole. It may be more common inpatients with marked elevated preoperativeintraocular pressure and after acute decreaseof IOP. The haemorrhages may be diffuse,both in deep and superficial layers of theretina, and may even show white centreswhen first observed.1 2

Retinal haemorrhages associated with ocu-lar decompression appear to be relativelybenign and usually resolve within weeks tomonths with no effect on visual acuity orintraocular pressure. A gradual decrease ofIOP preoperatively and intraoperatively isrecommended in order to avoid this compli-cation.1 2

Decompression retinopathy has not pre-viously been described as a complication oforbital decompression surgery. Our patienthad a relatively tight orbit with restrictivestrabismus and marked enlargement of theextraocular muscles. Significant force wasrequired to retract the globe to achieveexposure of the medial and deep lateralorbital walls. Retraction was frequentlyrelaxed to assure perfusion of the retina. Wehypothesise that the marked intraocularpressure fluctuation that occurs during thesesurgical manoeuvres may have contributed tothe retinal haemorrhages. It may also be thatrapid decrease in retrobulbar pressure hascaused ocular hypotony and retinal haemor-rhage.4

G J B Simon, R A Goldberg, J D McCannThe Jules Stein Eye Institute and Department of

Ophthalmology, David Geffen School of Medicine atUCLA, Los Angeles, CA, USA

Correspondence to: Guy J Ben Simon, MD, Jules SteinEye Institute, 100 Stein Plaza, Box 957006, Los

Angeles, CA 90095–7006, USA; [email protected]

doi: 10.1136/bjo.2004.049767

Accepted for publication 10 May 2004

References

1 Fechtner RD, Minckler D, Weinreb RN, et al.Complications of glaucoma surgery. Oculardecompression retinopathy. Arch Ophthalmol1992;110:965–8.

2 Dudley DF, Leen MM, Kinyoun JL, et al. Retinalhemorrhages associated with oculardecompression after glaucoma surgery.Ophthalmic Surg Lasers 1996;27:147–50.

3 Goldberg RA, Kim AJ, Kerivan KM. The lacrimalkeyhole, orbital door jamb, and basin of theinferior orbital fissure. Three areas of deep bonein the lateral orbit. Arch Ophthalmol1998;116:1618–24.

4 Otto AJ, Koornneef L, Mourits MP, et al.Retrobulbar pressures measured during surgicaldecompression of the orbit. Br J Ophthalmol1996;80:1042–5.

Retinal nerve fibre layer damageafter indocyanine green assistedvitrectomyRecently, indocyanine green (ICG) has beenused to stain and visualise the internallimiting membrane (ILM) during vitrect-omy.1 Some case series showed that visualfield defects on the nasal side can occur afterthe surgery through unknown cause.2 3 Here,we report a case in which nasal visual fielddefects occurred after ICG assisted ILMpeeling for epiretinal membrane (ERM).Detailed examination revealed that thesuperior and inferior retinal nerve fibre isseverely damaged in this case.

Case reportA 60 year old woman who received ICGassisted ILM peeling for ERM in her righteye was referred to our hospital. The pre-operative best corrected visual acuity (BCVA)was 20/60 in the right eye. According to thereferring ophthalmologist, 25 mg of ICG(Diagnogreen; Daiichi Pharmaceuticals) was

dissolved in 10 ml of distilled water, whichwas further diluted by a viscoelastic material(Healon; Pharmacia) to give 0.16% ICGsolution. To stain ILM, ICG was injected intoan air filled eye and the dye was washed2 minutes later. An air infusion cannula wasplaced at the temporal side. There was nocomplication during the surgery. Seventeendays after the operation, she noticed nasalvisual field loss, which got worse 22 daysafter the surgery. Sixty days after the surgery,she was referred to our hospital. At the initialvisit, the BCVA was 20/25 in the right eye.Goldmann perimetry revealed a nasal visualfield defect (fig 1A). In the right eye, arelative afferent pupillary defect was found.Ophthalmoscopic examination and fluores-cein angiography showed no abnormalities.The optic disc rim appeared to have lostcolour without being associated any cup orrim changes typically seen in glaucoma(fig 1B). Residual ICG was evident at theoptic disc and along the nerve fibre (fig 1C).The nerve fibre staining was most evident inthe superior and inferior quadrants. ICGangiography revealed ICG staining of theoptic disc and superior and inferior nervefibres, but no other abnormalities. Full fieldelectroretinogram (ERG) and multifocal ERG(VERIS science ver3.8, EDI) revealed noabnormalities. The results of visual evokedpotential testing were also non-remarkable.During our 8 month follow up period,

there was no significant change in the visualfield defect and the distribution of theresidual ICG. Scanning laser polarimetricanalysis (GDx VCC, Laser DiagnosticTechnologies, Inc, San Diego, CA, USA)performed 8 months after the surgeryshowed profound nerve fibre loss aroundthe disc, especially evident at superior andinferior quadrants (fig 2).

CommentIn this case, an air infusion cannula wasplaced at the temporal side and Goldmannperimetry showed nasal visual field defects.Thus, the dehydration injury to the retinaduring air-fluid exchange, which is observedat the opposite side of the cannula, is unlikelyto be the cause of this visual field defects.2–4

Optic neuropathy unrelated to the use of ICGmay be considered as a differential diagnosis.However, this visual field damage is nottypically seen in optic neuropathy and maybe rather associated with ICG, based on

Figure 2 Fundus photograph (upper image),right eye, 4 days after orbital decompressionsurgery on the right side showing scatteredposterior pole retinal haemorrhages. Note thehaemorrhage centred in the fovea. VA 20/160. (Lower image) Fluorescein angiography,right eye, late frame showing blockedfluorescence from retinal haemorrhages.

Figure 1 (A) Goldmann perimetry revealed a nasal visual field defect 4 months after ICG assistedpeeling of ILM for ERM. Visual field defect testing was also performed 8 months after the surgery,which demonstrated no remarkable change. (B) Fundus photograph taken 4 months after thesurgery shows that the optic disc rim appeared to have lost colour without being associated withany cup or rim changes typically seen in glaucoma. The appearance of the optic disc remainedunchanged during our 8 month follow up. (C) Infrared fundus photograph taken 8 months after thesurgery with an ICG filiter set. Residual ICG was evident at the optic disc and the nerve fibre. Thefluorescent intensity and distribution of residual ICG did not change remarkably during our 8 monthobservations.

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recent clinical findings.2 3 Retinal damagewas not evident by morphological, angio-graphic, and functional analysis. However, itwas evident that the nerve fibres aredamaged in this patient. Although the directcausal relation cannot be proved, it is highlylikely that the damage to the nerve fibre wascaused by the ICG because of the remarkablecorrespondence of the distribution pattern ofICG and the nerve fibre defects. This is alsosupported by our experimental findings thatICG showed neurotoxicity at concentrationslower than clinically employed.5 To ourknowledge, this is the first report of ICGinduced retinal nerve fibre damage assessedby scanning laser polarimetry.

A Iriyama, Y Yanagi, S Uchida, Y Tamaki,M Aihara, R Obata, Y Inoue

Department of Ophthalmology, University of TokyoSchool of Medicine, 7-3-1 Hongo, Bunkyo-ku, Tokyo

113-8655, Japan

Correspondence to: Yasuo Yanagi, Department ofOphthalmology, University of Tokyo School of

Medicine, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan; [email protected]

doi: 10.1136/bjo.2004.049999

Accepted for publication 13 June 2004

References

1 Kadonosono K, Itoh N, Uchio E, et al. Stainingof internal limiting membrane in macular

hole surgery. Arch Ophthalmol2000;118:1116–18.

2 Gass CA, Haritoglou C, Schaumberger M,et al. Functional outcome of macular hole surgerywith and without indocyanine green-assistedpeeling of the internal limiting membrane.Graefes Arch Clin Exp Ophthalmol2003;241:716–20.

3 Uemura A, Kanda S, Sakamoto Y, et al. Visualfield defects after uneventful vitrectomy forepiretinal membrane with indocyanine green-assisted internal limiting membrane peeling.Am J Ophthalmol 2003;136:252–7.

4 Hirata A, Yonemura N, Hasumura T, et al. Effectof infusion air pressure on visual field defects aftermacular hole surgery. Am J Ophthalmol2000;130:611–16.

5 Iriyama A, Uchida S, Yanagi Y, et al.Effects of indocyanine green on retinalganglion cells. Invest Ophthalmol Vis Sci2004;45:943–7.

Figure 2 The results obtained from the GDx-VCC from the right (top) and left (bottom) eyes taken8 months after the surgery. The left column shows the retardation image. The centre column showsthe results from analysis. The navy, blue, yellow, and red points represent significant loss of nervefibre layer, p,5%, 2%, 1%, and 0.5%, respectively. The right column shows that the general doublehump pattern is evident in the left eye but is lost in the right eye.

Competing interest: none.

NOTICES

Worldwide clinical trials for newtechnique for early detection of eyediseaseA unique new non-invasive technique forhigh resolution optical imaging of the eye isreceiving global acclaim. The technique,pioneered by the University of Kent, is

funded by the Toronto-based company,Ophthalmic Technology Inc (OTI). TheUniversity’s Applied Optics Group is currentlyworking with university hospitals in NewYork (USA), Osaka (Japan), Asahikawa(Japan), Amsterdam (Netherlands) andMilan (Italy) to carry out preliminary clinicaltrials. By combining two high-resolutionimaging technologies, the new techniqueprovides doctors with 3-D images of theretina, macula and the optic nerve. Suchhigh resolution images provide clinicianswith capabilities for early diagnosis andtreatment of common ocular diseases suchas glaucoma, diabetes and age-relatedmacula degeneration. OTI is planning in thenear future to extend the clinical research toother leading university medical centres inJapan, USA and Europe.Professor Adrian Podoleanu explained: ’At

Kent we created a very cost effective imagingsystem which simultaneously produces opti-cal coherence tomography (OCT) and scan-ning laser ophthalmoscope (SLO) images. Itsearly potential was immediately realised byOTI, who commissioned the assembly ofseveral prototypes to be tested in differentclinics worldwide before embarking oncommercial exploitation of the invention’.The clinical investigators together with the

Kent team have jointly published in interna-tional medical publications and presented atclinical and scientific conferences over 50publications and presentations related to thisresearch.For more information, contact the Media

Office on 01227 823581/823100 or [email protected] News releases canalso be found at: http://www.kent.ac.uk/news

Glaucoma Society Silver JubileeMeeting 2004The Silver Jubilee Meeting and Dinner for theGlaucoma Society will be held on 3 December2004 at the Royal College of Physicians inRegents Park, London. The meeting will takeplace between 8.30am and 5pm and thedinner will be held between 6.30pm and10pm. For further information, please con-tact: Janet Flowers, Administrator, 29 QuarryHill,Grays,Essex,RM175BT(tel:01375383172;e-mail: [email protected]).

Amsterdam Retina DebateThe Amsterdam Retina Debate will be heldon 10 December 2004 at the AcademicMedical Centre, Amsterdam, TheNetherlands. For further information, pleasecontact: Nicolaes Tulp Institute; tel: +31 20566 8585; fax: +31 20 696 3228; email:[email protected]

British Oculoplastic Surgery SocietyCall for papers for the 5th annual meeting ofthe BOPSS to be held on 15 and 16 May 2005at The Belfry, Birmingham. The abstractsubmission deadline is 4 February 2005,abstracts can be submitted online atwww.bopss.org.

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