case report medical and surgical treatment in pediatric orbital myositis...

Case ReportMedical and Surgical Treatment in Pediatric Orbital MyositisAssociated with Coxsackie Virus

Pedro Gil Joatildeo Gil Catarina Paiva Guilherme Castela and Rui Castela

Department of Ophthalmology Centro Hospitalar Universitario de Coimbra Praceta Professor Mota Pinto3000-075 Coimbra Portugal

Correspondence should be addressed to Pedro Gil pedroqgilgmailcom

Received 10 August 2015 Accepted 17 September 2015

Academic Editor Pradeep Venkatesh

Copyright copy 2015 Pedro Gil et alThis is an open access article distributed under theCreative CommonsAttribution License whichpermits unrestricted use distribution and reproduction in any medium provided the original work is properly cited

Purpose To report a case of orbital myositis associated with Coxsackie virus and its medical and surgical approach MethodsComplete ophthalmological examination and imaging and analytical investigation were performed Results A 6-year-old malepresented with subacute painless binocular horizontal diplopia Examination revealed bilateral best-corrected visual acuity (BCVA)of 2020 and right eye 45-prism-dioptre (PD) esotropia in near and distance fixations with no motility restrictions Serologicscreening was positive for Coxsackie virus acute infection and computerized tomography (CT) suggested right eye medial rectusorbital myositis An oral corticosteroid 10mgkgday regimenwas started A newCT after twomonths showed symmetrical lesionsin both medial rectus muscles Corticosteroids were increased to 15mgkgday After imagiological resolution on the 4th monthalternating 45 PD esotropia persisted Bilateral 7mm medial rectus recession was performed after 1 year without spontaneousrecovery At 1-year follow-up the patient is orthophoric with 20010158401015840 stereopsis and bilateral 2020 BCVA Conclusions To ourknowledge this is the first reported case of orbital myositis associated with Coxsackie virus This is also the first reported caseof isolated strabismus surgery after orbital myositis in pediatric age highlighting the favourable aesthetic and functional outcomeseven in cases of late ocular motility disorders

1 Introduction

Idiopathic orbital inflammatory syndrome (IOIS) formerlytermed orbital pseudotumor is a benign idiopathic inflam-matory disease that can affect any structure of the orbitPrevious authors have reported that it accounts for 50ndash63 of orbital disorders being the third most commonafter thyroid associated orbitopathy and lymphoproliferativedisease [1]

According to the current most consensual classificationorbital myositis represents a subgroup within the IOIS as aninflammatory process that primarily involves the extraocularmuscles [2]The relative incidence of this subtype to the IOISgroup is variable in different case series ranging from 29 to50 [3 4]

Some authors have identified two variants the first andmost common being idiopathic and the second consideredto be specific [2] The mechanisms for disease are not fullyunderstood although a significant role for the immunesystem response to some bacterial or viral antigens and

endotoxins has been proposed [5] In other patients orbitalmyositis can be related to a heterogeneous group of specificdiseases including bacterial or viral infections and systemicimmune-mediated diseases Therefore the classification ofmyositis as idiopathic is a diagnosis of exclusion and can onlybe assumed after a complete systemic investigation

We report an atypical presentation and successful surgicaloutcome for this rare disease describing for the first time anassociation with Coxsackie virus infection

2 Case Presentation

A 6-year-old male patient reported to the Emergency Roomof our tertiary care paediatric center complaining of binoc-ular horizontal diplopia with a subacute onset lasting forone week Complete ophthalmological examination revealeda best-corrected visual acuity (BCVA) of 2020 on botheyes and significant right eye (RE) esotropia measuring 45prism dioptres (PD) both in near and in distance fixation

Hindawi Publishing CorporationCase Reports in Ophthalmological MedicineVolume 2015 Article ID 917275 4 pageshttpdxdoiorg1011552015917275

2 Case Reports in Ophthalmological Medicine

Figure 1 Right eye esotropia in primary gaze position at presenta-tion

Figure 2 Brain computerized tomography at presentation showingthickening of the right eye medial rectus muscle associated withincreased spontaneous density

(Figure 1) No apparent restriction was present in terms ofocular motilityThe patient denied any orbital pain and therewas no conjunctival injection proptosis ptosis or any otherabnormality in anterior segment examination of both eyesFundoscopic examination was unremarkable

Complete blood count was normal PCR was negativeand the erythrocyte sedimentation rate was slightly increasedat a value of 18mmhour Complete serologic screeningwas performed for toxoplasmosis cytomegalovirus herpessimplex viruses 1 and 2 Epstein-Barr virus and Coxsackievirus All results were negative for acute infection except forCoxsackie virus which showed positive IgG 55900UmL andIgM 6890UmL counting compatible with recent infectionThe analysis was repeated 2weeks later when it showed a con-sistent positive result for acute infection IgG 148800UmLand IgM 9560UmL Thyroid hormone tests (TSH free T3and free T4) were normal Brain and orbital computerizedtomography (CT) showed discrete thickening of the REmedial rectus muscle associated with increased spontaneousdensity and diffuse enhancement after intravenous contrastadministration (Figure 2) No other abnormality was identi-fied and the remaining extraocular muscles were normal

The diagnosis of orbital myositis associated with Cox-sackie virus was suspected and the patient was initiated on anoral corticosteroid treatment with a dosage of 10mgkgdayHe was also started on a 3-hour daily regimen of patching ofthe left eye (LE)

The patient was reevaluated 2 and 4 weeks after the onsetof disease with no significant clinical improvement Twomonths later while the patient was still under the initial oralcorticosteroid regimen complete ophthalmological exami-nation was similar to the initial observation except for the

Figure 3 Brain computerized tomography at 2 months showingbilateral thickening of the medial rectus muscles associated withincreased spontaneous density

BCVA of the RE which had decreased to 2025 A newbrain and orbital CT was performed It showed overlappingcharacteristics comparing to the previous exam but this timesymmetrical both right and leftmedial rectus were thickenedcomparing to the other extraocular muscles associated withincreased spontaneous density and diffuse enhancement afterintravenous contrast administration (Figure 3) Comparingto the first performed CT there had been no significantreduction of the RE medial rectusrsquo thickness

Given the absence of a favourable response to the initialcorticosteroid regimen the daily dosage was increased to15mgkg

Fourmonths after the onset BCVA improved to 2020 onboth eyes while the remaining ophthalmological examina-tion still revealed 45 PD esotropia of the right eye both innear and in distance fixation with no stereopsis on the LangII test The patient underwent brain and orbital magneticresonance (MR) that showed a significant reduction of theright and left eyesmedial rectusrsquo thickness with no significantdifferences before and after contrast administration com-paredwith the other extraocularmuscles (Figure 4) No otherlesions were present

The patient still complained of binocular horizontaldiplopia Given the symmetrical BCVAs we opted for analternate occlusion strategy with good compliance

Eight months after the onset new MR showed no recur-rence of inflammatory lesions The patient denied binoculardiplopia and suppression of the RE was evident with Worthfour-dot testing Ophthalmological examination revealedalternating esotropia measuring 45 PD both in near and indistant fixation

Given the absence of spontaneous recovery of the ocularmotility disorder 1 year after the onset of disease we optedfor surgical treatment bilateral 7mm medial rectus reces-sion The 6-week postoperative visit showed an orthophoricpatient in all positions of gaze with no complaints of diplopiaand with a BCVA of 2020 on both eyes (Figure 5) Stereopsismeasurement was 20010158401015840 on Lang II test

With 1 year of postoperative follow-up making a total of2 years after the disease onset the patient is still orthophoricwith a BCVA of 2020 on both eyes and with no evidence ofrecurrence of disease both clinically and in imaging exams

Case Reports in Ophthalmological Medicine 3

Figure 4 Brain magnetic resonance at 4 months showing a signifi-cant reduction of the right and left eyes medial rectusrsquo thickness

Figure 5 Primary gaze position orthophoria at 6 months postoper-ative follow-up

3 Discussion

Since the first report of orbital myositis associated withsystemic diseases multiple case reports have been publishedwhere the causal relationship between orbital myositis and aspecific inflammatory or infectious disease is circumstantialThis is also true in our case It is difficult to ascertain a definitecausal relationship because the exact pathogenicmechanismsare still widely unknown Therefore the association is basedon the coexistence of a clinical diagnosis of orbital myositisand a systemic condition that can predispose to an inflam-matory process affecting the extraocular muscles

Not only is orbital myositis considered a rare disease perse but also the clinical case presented is atypical in manyways In terms of epidemiology children are not frequentlyaffected although when they are bilateral disease is com-mon as it was in our patient [6] Clinical presentation wasunspecific and the absence of orbital pain in eye movementsconjunctival injection ptosis or any other acute sign was aconfounding factor for the correct diagnosis The fact thatdiplopia was the only presenting sign obligates us to includeorbital myositis in the differential diagnosis of acute-onsetdiplopia

As for the complementary examination the findings onCT imaging were typical and alongside MR it proved usefulnot only for diagnosis but also for monitoring treatmentresponse

Previous reports have linked Coxsackie virus infection tomyositis [7] To our knowledge this is the first reported caseof orbital myositis associated with Coxsackie virus It is still

unclear whether viral or bacterial-associated orbital myositisdiffers from the idiopathic type in terms of clinical outcomeor treatment However it is important to recognize allpossible pathogens not only to guide systemic investigationbut also to expand our knowledge in terms of possibleetiopathogenic associations

According to current consensus the patient was startedon an oral corticosteroid daily regimen of 10mgkg Thisdosage proved insufficient and given the evidence of bilateralinvolvement we increased the dosage to 15mgkgday whichis the maximum proposed by most authors [2 8] It isnot clear whether this occurred due to some potential forlocal dissemination of the inflammatory process or whetherthe inflammatory response in the LE medial rectus wasdeferred by any defensive mechanism Asymmetric diseasehas been reported in the idiopathic type [9] but the reasonsbehind asymmetric and delayed onset disease in one eyeassuming a systemic infectious aetiology are unclear Likeprevious authors we suggest that an aggressive and earlydisease therapy might be beneficial [2] This could preventlocal dissemination to other extraocular muscles and orbitalstructures thus maybe reducing duration of disease and latestage sequelae

This case also highlights the importance of an integratedapproach to treatment Reducing inflammation is primordialbut accounting for all the comorbidities is also important Inour case the resulting strabismus was probably due to cica-tricial fibrotic contraction of medial rectus muscles leadingto esotropia Therefore prevention of strabismic amblyopiais of paramount importance Most authors reported promptclinical improvement and remission within days to weeksafter treatment in most patients [8] Our case is atypicalin the way that inflammation was adequately controlledwith the recommended corticosteroid regimen but the latestage sequelae of alternating strabismus posed a challengeGiven the rarity of this entity in children in the amblyopiaage interval and the favourable outcome in most patientsthere is scarce reference in the published literature regardingtreatment in cases of ocular motility disorders secondary toorbital myositis in paediatric age Bessant and Lee publisheda case series of 5 consecutive patients who had sufferedone or more episodes of orbital myositis resulting in apersistent ocular motility defect associated with significantdiplopia [10] Only one of these patients was in paediatricage (11 years) Botulinum toxin A (BTXA) was injectedin all patients after 3 months of evidence of absence ofimprovement in the ocular motility status According to theauthors BTXA not only is aimed at improving symptoms ofdiplopia but can also be used to determine whether a patienthas the potential to achieve binocular single vision after acorrective strabismus surgery Surgery was indeed performedin 2 of the 5 patients including the one in paediatric age

To our knowledge this is the first reported case of isolatedstrabismus surgery after orbital myositis in paediatric ageWe still consider as a valid approach the use of BTXA asa conservative first-line therapy prior to corrective surgeryHowever it is noteworthy to see that in our case medialrectus recession after 1 year of onset provided not only anexcellent aesthetic outcome but also a surprisingly good


functional outcome as the patient recovered a significantdegree of stereopsis after binocular alignment Given the lackof published reports there is no consensus regarding theoutcomes after BTXA or strabismus surgery and whetherthey are dependent on the duration of disease degree andtype of deviation number and type of affected extraocularmuscles laterality age and other factors According to theseclinical features for which published case reports can give asignificant contribution future guidelines might clarify therole for BTXA or surgery in these patients (with specialconsideration to those in the amblyopia age interval) and theadequate timing for each intervention

Conflict of Interests

No financial conflict of interests exists for any of the authors

References

[1] P A D Rubin and C S Foster ldquoEtiology and management ofidiopathic orbital inflammationrdquo American Journal of Ophthal-mology vol 138 no 6 pp 1041ndash1043 2004

[2] B G Schoser ldquoOcular myositis diagnostic assessment differ-ential diagnoses and therapy of a raremuscle diseasemdashfive newcases and reviewrdquo Clinical Ophthalmology vol 1 no 1 pp 37ndash42 2007

[3] B N Swamy P McCluskey A Nemet et al ldquoIdiopathicorbital inflammatory syndrome clinical features and treatmentoutcomesrdquo British Journal of Ophthalmology vol 91 no 12 pp1667ndash1670 2007

[4] S J A Yuen and P A D Rubin ldquoIdiopathic orbitalinflammationmdashdistribution clinical features and treatmentoutcomerdquo Archives of Ophthalmology vol 121 no 4 pp 491ndash499 2003

[5] G J Harris ldquoIdiopathic orbital inflammation a pathogeneticconstruct and treatment strategyrdquo Ophthalmic Plastic andReconstructive Surgery vol 22 no 2 pp 79ndash86 2006

[6] M K Jacob ldquoIdiopathic orbital inflammatory diseaserdquo OmanJournal of Ophthalmology vol 5 no 2 pp 124ndash125 2012

[7] N G Rodrıguez J I Ruan and M G Rodrıguez ldquoCoxsackievirus infection associated with myositis and polyarthritisrdquoAnales de Medicina Interna vol 25 no 2 pp 90ndash92 2008

[8] R M S Costa O M Dumitrascu and L K Gordon ldquoOrbitalmyositis diagnosis and managementrdquo Current Allergy andAsthma Reports vol 9 no 4 pp 316ndash323 2009

[9] A Ariatti G Galassi R Rovati and A Chiari ldquoCutting theedge of idiopathic recurrent orbital myositisrdquo Central EuropeanJournal of Medicine vol 8 no 6 pp 732ndash735 2013

[10] D A R Bessant and J P Lee ldquoManagement of strabismus dueto orbital myositisrdquo Eye vol 9 no 5 pp 558ndash563 1995

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Figure 1 Right eye esotropia in primary gaze position at presenta-tion

Figure 2 Brain computerized tomography at presentation showingthickening of the right eye medial rectus muscle associated withincreased spontaneous density

(Figure 1) No apparent restriction was present in terms ofocular motilityThe patient denied any orbital pain and therewas no conjunctival injection proptosis ptosis or any otherabnormality in anterior segment examination of both eyesFundoscopic examination was unremarkable

Complete blood count was normal PCR was negativeand the erythrocyte sedimentation rate was slightly increasedat a value of 18mmhour Complete serologic screeningwas performed for toxoplasmosis cytomegalovirus herpessimplex viruses 1 and 2 Epstein-Barr virus and Coxsackievirus All results were negative for acute infection except forCoxsackie virus which showed positive IgG 55900UmL andIgM 6890UmL counting compatible with recent infectionThe analysis was repeated 2weeks later when it showed a con-sistent positive result for acute infection IgG 148800UmLand IgM 9560UmL Thyroid hormone tests (TSH free T3and free T4) were normal Brain and orbital computerizedtomography (CT) showed discrete thickening of the REmedial rectus muscle associated with increased spontaneousdensity and diffuse enhancement after intravenous contrastadministration (Figure 2) No other abnormality was identi-fied and the remaining extraocular muscles were normal

The diagnosis of orbital myositis associated with Cox-sackie virus was suspected and the patient was initiated on anoral corticosteroid treatment with a dosage of 10mgkgdayHe was also started on a 3-hour daily regimen of patching ofthe left eye (LE)

The patient was reevaluated 2 and 4 weeks after the onsetof disease with no significant clinical improvement Twomonths later while the patient was still under the initial oralcorticosteroid regimen complete ophthalmological exami-nation was similar to the initial observation except for the

Figure 3 Brain computerized tomography at 2 months showingbilateral thickening of the medial rectus muscles associated withincreased spontaneous density

BCVA of the RE which had decreased to 2025 A newbrain and orbital CT was performed It showed overlappingcharacteristics comparing to the previous exam but this timesymmetrical both right and leftmedial rectus were thickenedcomparing to the other extraocular muscles associated withincreased spontaneous density and diffuse enhancement afterintravenous contrast administration (Figure 3) Comparingto the first performed CT there had been no significantreduction of the RE medial rectusrsquo thickness

Given the absence of a favourable response to the initialcorticosteroid regimen the daily dosage was increased to15mgkg

Fourmonths after the onset BCVA improved to 2020 onboth eyes while the remaining ophthalmological examina-tion still revealed 45 PD esotropia of the right eye both innear and in distance fixation with no stereopsis on the LangII test The patient underwent brain and orbital magneticresonance (MR) that showed a significant reduction of theright and left eyesmedial rectusrsquo thickness with no significantdifferences before and after contrast administration com-paredwith the other extraocularmuscles (Figure 4) No otherlesions were present

The patient still complained of binocular horizontaldiplopia Given the symmetrical BCVAs we opted for analternate occlusion strategy with good compliance

Eight months after the onset new MR showed no recur-rence of inflammatory lesions The patient denied binoculardiplopia and suppression of the RE was evident with Worthfour-dot testing Ophthalmological examination revealedalternating esotropia measuring 45 PD both in near and indistant fixation

Given the absence of spontaneous recovery of the ocularmotility disorder 1 year after the onset of disease we optedfor surgical treatment bilateral 7mm medial rectus reces-sion The 6-week postoperative visit showed an orthophoricpatient in all positions of gaze with no complaints of diplopiaand with a BCVA of 2020 on both eyes (Figure 5) Stereopsismeasurement was 20010158401015840 on Lang II test

With 1 year of postoperative follow-up making a total of2 years after the disease onset the patient is still orthophoricwith a BCVA of 2020 on both eyes and with no evidence ofrecurrence of disease both clinically and in imaging exams




3 Discussion













References
















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Disease Markers



OncologyJournal of





PPAR Research



Journal of

ObesityJournal of



















3 Discussion













References
















of






Disease Markers



OncologyJournal of





PPAR Research



Journal of

ObesityJournal of




















References
















of






Disease Markers



OncologyJournal of





PPAR Research



Journal of

ObesityJournal of





















of






Disease Markers



OncologyJournal of





PPAR Research



Journal of

ObesityJournal of
















case report medical and surgical treatment in pediatric orbital myositis...

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