Clinicopathological Report

Bilateral spontaneous idiopathic extraocular musclehaematomaSimon J Dean MBChB, Jonathan Ross MBChB and Ewan Kemp FRCOphthTennent Institute of Ophthalmology, Gartnavel Hospital, Glasgow, UK

ABSTRACT

The authors present a case of bilateral sequential extraocularmuscle haematoma, for which no apparent cause has beenidentified despite extensive investigation, and which resolvedwithout persisting morbidity. As far as the authors are aware,this is the first bilateral case to be reported.

Key words: bilateral, extraocular, haematoma, idiopathic,orbit.

INTRODUCTION

Spontaneous intramuscular extraocular muscle haematoma isa rarely reported entity. Often there is no obvious cause orsystemic disease related with the haemorrhage,1–3 with onlya few cases linked to existing comorbidity.4,5 Spontaneousresolution without intervention is the norm.1,3

CASE REPORT

A 76-year-old woman presented with sudden onset of right-sided globe displacement, with proptosis (5 mm) and hypo-globus (4 mm) (Fig. 1a). She was woken from sleep withnausea, described mild discomfort around the eye, andnoticed diplopia soon after. She had no history or familyhistory of bleeding diathesis, no recent trauma, and was nottaking or had ever taken anticoagulant medications. Heronly positive medical history was chronic stable hypothy-roidism of 20 years, for which she was on thyroxine replace-ment therapy. Her visual acuity was 6/6 in both eyes, with norelative afferent defect, colour defect, or subjective afferentdefect. A ‘mass’ was visible on fundoscopy in the superotem-poral quadrant, which proved to be extraocular on B-scanultrasonography. Consistent with her history of diplopia, dueto retrobulbar mass effect, she demonstrated moderate limi-tation of motility of the right eye in all positions of gaze. CTscanning revealed a superior orbital mass indistinguishablefrom superior rectus (Fig. 2a, arrow). She responded well to

oral steroid treatment with almost complete resolution of theright eye proptosis over a 2-week period. All blood testsincluding full blood count, thyroid function tests, coagulopa-thy screen, lupus anticoagulant, anti-cardiolipin antibodies,

� Correspondence: Mr Simon Dean, 134 Arlescote Road, Solihull, Birmingham B92 9HZ, UK. Email: simondean@xtra.co.nz

Received 5 September 2006; accepted 15 December 2006.

Figure 1. (a) Right orbital involvement (note loss of superiorsulcus). (b) Left orbital involvement (note resolving ecchymosisfrom right orbital biopsy). (c) Clinical resolution. In both (a) and(b) mydriasis is pharmacological.

Clinical and Experimental Ophthalmology 2007; 35: 369–371doi: 10.1111/j.1442-9071.2007.01487.x

© 2007 The AuthorsJournal compilation © 2007 Royal Australian and New Zealand College of Ophthalmologists

electrolytes, C-reactive protein and anti-neutrophil cytoplas-mic antibodies, were within normal limits. An orbital biopsywas performed, which returned connective tissue, blood clotand adipose tissue, with no inflammatory cell infiltration andno evidence of malignancy.

Second eye

Interestingly, 3 weeks following the initial event, and whenthe majority of clinical signs on the right side had resolved,the patient presented to the acute ophthalmology servicewith a sudden onset of left-sided proptosis and inferior dis-placement of the globe (Fig. 1b [note resolving right perior-bital ecchymoses from surgery]). An urgent CT scanconfirmed acute haemorrhage in the left superior rectusregion (Fig. 2b, arrow), and the slightly attenuated signal oforganizing haematoma in the area of the right superiorrectus. Magnetic resonance imaging confirmed bilateralintraconal masses inseparable from superior recti. Each dem-onstrated different intensity signal consistent with haemato-mas of different duration (Fig. 2c, double arrow). The patientcontinued on a tapering dose of oral steroid treatment andclinical improvement was rapid, with complete resolution8 weeks following the initial event (Fig. 1c). Her visualacuity and optic nerve function remained uncompromisedthroughout. Follow-up magnetic resonance imaging con-firmed resolution (Fig. 2d).

DISCUSSION

As far as the authors are aware, this is the first reportedidiopathic extraocular muscle haematoma to have a bilateralpresentation with no obvious aetiology. Perhaps this bilat-erality suggests a systemic causative factor, as yet uniden-tified despite thorough investigation. In this case it may berelated to chronic stable hypothyroidism, but it is obscureas to how this could cause bilateral haematoma within such

a close time frame. Both superior recti being involved isalso unusual. At the time of initial presentation, theworking differential diagnoses included dysthyroid eyedisease, lymphoma, idiopathic orbital inflammatory diseaseand subacute haemorrhage, all of which can give similarclinical signs. With spontaneous haemorrhage being rare, itwas considered necessary to exclude the potentially vision/life-threatening differential diagnoses. Initial difficulty inobtaining a CT scan meant the first eye had to be managedempirically, and explains the decision to start steroids andschedule an orbital biopsy. It was felt there was still suffi-cient need to proceed with the biopsy following the CT, asthe initial report was not definitive. The biopsy was helpfulin this case, and certainly once the second eye becameinvolved, allowed appropriate supportive management afterexcluding more serious differential diagnoses. If the radiol-ogy report had stated that haemorrhage was the most likelypossibility, the orbital biopsy would not have been neces-sary, as indeed is the case in most extraocular muscle hae-matomas. The use of steroids in this case was intended tobe partly therapeutic, and partly diagnostic, as a responseto steroid treatment can give clues as to the underlyingdisease process. Because the second event occurred whilethe patient was on a tapering dose of prednisone, it is ques-tionable whether they were of any benefit. Published casereports do not allow any firm conclusions to be drawn as tothe utility of steroid therapy in these cases, and certainly,patients managed without steroid intervention are reportedto experience rapid resolution of their symptoms.1,3 Con-sistent with previously reported unilateral cases, irrespec-tive of treatment modality, resolution in this instance wasrapid with full recovery.

ACKNOWLEDGEMENTS

The authors wish to thank Dr Fiona Roberts MRCPath forher valuable assistance with the histopathology.

Figure 2. (a) Axial CT demon-strating right orbital mass (arrow).(b) CT demonstrating bilateralorbital masses, recent onset left(arrow). (c) T2 weighted axial mag-netic resonance imaging illustratingdifferent intensities of haemorrhages(double arrow) with left morerecent. (d) Magnetic resonanceimaging demonstrating resolution.

370 Dean et al.

© 2007 The AuthorsJournal compilation © 2007 Royal Australian and New Zealand College of Ophthalmologists

REFERENCES

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2. Ben Simon GJ, McNab AA. Idiopathic orbital hemorrhagerelated to the inferior rectus muscle: a rare cause for acute-onsetdiplopia and unilateral proptosis. Ophthalmology 2005; 112:1838–43.

3. Hakin KN, McNab AA, Sullivan TJ. Spontaneous hemorrhagewithin the rectus muscle. Ophthalmology 1994; 101: 1631–4.

4. Thuente DD, Neely DE. Spontaneous medial rectus hemorrhagein a patient with acute myelogenous leukemia. J AAPOS 2002; 6:257–8.

5. Reifler DM, Leder D, Rexford T. Orbital hemorrhage and eyelidecchymosis in acute orbital myositis. Am J Ophthalmol 1989; 107:111–13.

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© 2007 The AuthorsJournal compilation © 2007 Royal Australian and New Zealand College of Ophthalmologists