Lyme Disease Associated Orbital Myositis: A Rare Cause of Orbital Swelling

Dr. Abdullah A. Cheema, MBBS BSc1

Ms. Raina Goyal1

1Ophthalmology Department

Royal Glamorgan Hospital

Cwm Taf Morgannwg University Health Board

Abstract

A 61-year-old man presented with a 1-day history of right eye pain, right periorbital swelling, and painful diplopia upon right gaze. He was triaged as having Orbital Cellulitis in the emergency room and was given intravenous metronidazole and ceftriaxone.

The patient has a medical history of Type 2 diabetes, right subclavian thrombosis, and Lyme disease, for which he was treated with Doxycycline and Rifampicin. He also has colour blindness and a raised immunoglobulin level, as well as a history of soft tissue thickening and acute inflammatory changes in the right periorbital region. Upon examination, the patient’s visual acuity was 6/9 in both eyes, and the right eye showed swelling and tenderness at the superior and inferior margins, chemosis, and restriction of left abduction and upgaze. Blood tests showed elevated CRP levels and a positive rheumatoid factor.

A CT scan revealed soft tissue thickening and fluid collection in the anterior compartment of the right eye, and a diagnosis of preseptal/impending orbital cellulitis was made. The patient was started on intravenous antibiotics, but showed no improvement after 3 days. A subsequent CT scan revealed thickening of the right lateral rectus muscle, and the patient was diagnosed with myositis (likely secondary to Lyme disease) and started on oral prednisolone, leading to marked improvement in symptoms.

The case highlights the need for targeted investigations and biopsy in patients with atypical features of extraocular muscle involvement, and the possibility of recurrence in systemic Lyme disease.

Introduction

The present medical case report details the diagnosis and treatment of a 61-year-old male with a 1-day history of right eye pain, right periorbital swelling, and painful diplopia on right gaze. The patient was triaged as orbital cellulitis in the Accident and Emergency department and was given intravenous (IV) Metronidazole and Ceftriaxone.

The patient has a past medical history (PMH) of Type 2 Diabetes Mellitus (T2DM), right subclavian thrombosis, and Lyme disease (IgM +ve and IgG -ve for Borrelia). The patient had previously been treated with Doxycycline/Rifampicin for Borrelia Burgdorferi 1 month prior to presenting and had soft tissue thickening and acute inflammatory changes in the right periorbital region. The patient’s past ocular history (POH) includes being colour blind (Ishihara 1/15 in both eyes).

On examination, the patient’s visual acuity was 6/9 in both eyes and there was no relative afferent pupillary defect (RAPD) in either eye. The right superior and inferior lids and lashes were swollen, tender, and solid to touch, and the right conjunctiva/sclera had 3+ chemosis predominant on the temporal side compared to the nasal side. The patient had 1+ restriction of left abduction and upgaze in the right eye, and the left eye movements were NAD. Fundoscopy was normal bilaterally, and the intraocular pressure (IOP) was 18 in both eyes.

The blood tests revealed a normal thyroid function, tests for CMV and EBV were negative, IgG serum was normal, ENA was negative, ANCA was negative, HIV test was negative, Antiphospholipids Ab were normal, ACE was negative, Rheumatoid Factor was positive, immunoglobulin was raised, ferritin was high, TTG was negative. Urea and electrolytes were NAD, CRP was elevated at 98, haemoglobin was 125, platelets were 370, white cell count was 4.5, and neutrophils were 3.1. Investigations conducted in a recent admission prior to presenting at the eye department included a normal ECHO, a normal CTTAP and a normal TTE.

A CT scan of the head showed soft tissue thickening and acute inflammatory changes in the right periorbital region, fluid collection in the anterior compartment laterally measuring approximately 6 mm x 15 mm, acute inflammatory changes extending into the lateral extraconal space, an intact right eye globe, and right preseptal cellulitis with fluid collection in the anterior compartment.

In view of the CT findings, blood tests, and the clinical picture, a working diagnosis of preseptal/impending orbital cellulitis was made. The patient was started on IV antibiotics (Metronidazole, Ceftriaxone, Flucloxacillin), however, after three days, there was no improvement. The swelling extended to the other lid and the diplopia worsened, and the eye movements remained painful. A review of the CT scan showed thickening of the single lateral rectus muscle on the right side, and a diagnosis of myositis (likely secondary to Lyme disease) was made. The patient was started on oral Prednisolone 40mg and showed a marked reduction in pain, redness, and diplopia after two days.

In conclusion, the extraocular muscle can be a target for an inflammatory response in various autoimmune, inflammatory, and infective conditions, although a large proportion remains “idiopathic.” Targeted investigations should be aimed at possible underlying or associated inflammatory or autoimmune conditions, and biopsy of the affected extraocular muscle may be considered if features are atypical. Patients should be warned of the possibility of future recurrent disease or the development of associated systemic medical conditions.

Further Reading

1. Seidenberg KB, Leib ML. Orbital myositis with Lyme disease. Am J Ophthalmol 1990;109:13–6.

2. Fatterpekar GM, Gottesman RI, Sacher M, et al. Orbital Lyme di- sease: MR imaging before and after treatment: case report. AJNR Am J Neuroradiol 2002;23:657–9.

3. Carvounis PE, Mehta AP, Geist CE. Orbital myositis asso- ciated with Borrelia burgdorferi (Lyme disease) infection. Ophthalmology 2004;111:1023–8.

4. Holak H, Holak N, Huzarska M, et al. Tick inoculation in an eyelid region: report on five cases with one complication of the orbital myositis associated with Lyme borreliosis. Klin Oczna 2006;108:220–4. 5. Tseng YH. Acute orbital myositis heralding herpes zoster ophthal- micus: report of a case. Acta Neurol Taiwan 2008;17:47–9

6. Fraser CL, Skalicky SE, Gurbaxani A, et al. Ocular myositis. Curr Allergy Asthma Rep 2013;13:315–21.

7. McNab AA. Orbital Myositis: A Comprehensive Review and Reclassification. Ophthalmic Plast Reconstr Surg. 2020 Mar/Apr;36(2):109-117. doi: 10.1097/IOP.0000000000001429. PMID: 31261163

8. Wheatcroft S, Elston J. Unilateral ptosis due to isolated involvement of the levator muscle in acute orbital myositis. Br J Ophthalmol 1999;83:631–2

9. Kim N, Yang HK, Kim JH, et al. IgG4-related ophthalmic disease involving extraocular muscles: case series. BMC Ophthalmol 2018;18:162

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