Non-traumatic extraocular muscle haemorrhage associated with anticoagulation use
- 1 Medicine, Swansea Bay University Health Board, Port Talbot, UK
- 2 Radiology, Swansea Bay University Health Board, Port Talbot, UK
- Correspondence to Dr Elizabeth Alexandra Davies; ElizabethAlexandra.Davies@wales.nhs.uk
Abstract
We present a case of a unilateral extraocular muscle haematoma in an adult female patient who was compliant with life-long oral anticoagulation for recurrent deep vein thrombosis. The patient presented with symptoms of sudden-onset left-sided headache radiating to the temporal region, which started 2 days prior. No obvious triggering factors were identified. Cranial and ocular examinations were within normal limits. Imaging revealed a haemorrhage related to the lateral rectus muscle of the left eye. Conservative management was employed with abstinence from anticoagulation for 2 weeks and a weaning regime of oral steroids. Under the clinical review of ophthalmology and interval radiological monitoring, symptoms were reduced with reduction of haemorrhage size. Anticoagulation was reinstated after 2 weeks. To our knowledge, this is the first case of a non-traumatic extraocular muscle haematoma to be reported in a patient on anticoagulation.
Background
Non-traumatic orbital haemorrhage (NTOH) is an uncommon event which has been associated with several underlying aetiologies including vascular and bleeding abnormalities, inflammation, infection and malignancy. Based on anatomical patterns, NTOH can be classified into five different categories (box 1).1
Non-traumatic orbital haemorrhage anatomical patterns
Non-traumatic orbital haemorrhage categories
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Diffuse intraorbital haemorrhage.
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Localised intraorbital haemorrhage (a very rare lesion, known also as haematic cyst).
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Subperiosteal orbital haemorrhage (almost always occurring in the orbital roof).
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Haemorrhage related to extraocular muscles.
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Haemorrhage related to orbital floor implants.
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Table data obtained from Chan et al. 1
NTOH in relation to extraocular muscles (EOMs) was described in a number of cases, in which the haemorrhage occurred mainly within the muscle sheath or the intermuscular septum. Fewer cases reported the haemorrhage to be located within the muscle belly.1 Chan et al concluded that vascular risk factors, namely, hyperlipidaemia, hypertension and antiplatelet use, could be considered as possible predisposing factors for non-traumatic EOM haemorrhage.1
Direct oral anticoagulants (DOACs) are increasingly prescribed for prevention and treatment of thrombotic vascular events.2 3 Rivaroxaban, a DOAC which works by inhibiting factor Xa, is favoured in some patients for its high compliance rate being prescribed as a single daily dose for venous thromboembolism prevention and treatment.2–4
Case presentation
A woman in her late 60s presented to the emergency department with a 2-day history of sudden-onset, pressure-like pain above her left eye. The pain radiated along the left temporal region and across the inner corner of her eye. The pain was aggravated by ocular movement, bright light, lying on the affected side and bending forward. Apart from increased stress, there were no acute events, specifically, no trauma, respiratory tract infection or forceful straining. The patient did not use spectacles or contact lenses and had never undergone ocular surgery or had orbital implants.
Following recurrent deep vein thrombosis, rivaroxaban 10 mg once daily was started as a life-long anticoagulation treatment, to which the patient was compliant. Other medical conditions included: osteoarthritis, dyspepsia, depression and asthma. In addition to anticoagulation, regular medications included fluoxetine, gabapentin and tramadol with topical ibuprofen as needed. There was no known history or family history of bleeding disorders. No documented COVID-19 infection was reported prior to the presentation, and she had received two doses of COVID-19 vaccination. The patient was not undergoing any other medical investigation.
Examination revealed only tenderness at the supraorbital foramen. The patient had normal eye movement, with exacerbation of pain on left lateral gaze. Visual fields appeared to be normal, and cranial nerves were intact with no peripheral focal neuropathy. A specialist ophthalmological examination confirmed normal colour vision and visual acuity, normal intraorbital pressure, and healthy optic discs and maculae.
Investigations
Initial non-contrast CT of the head identified a 21×12×12 mm intraconal, hyperdense soft tissue mass, slightly deviating the left optic nerve medially. The mass was thought either to rise from the left lateral rectus muscle or to be centred over it. While haematoma was considered as a possible diagnosis, malignancy with an intratumoural haemorrhage was also a concern. An MRI orbit with contrast revealed a well-defined oval lesion arising from the left lateral rectus muscle. The lesion was peripherally enhancing and mildly hyperintense on T1/T2. On susceptibility-weighted imaging (SWI) sequence, a small focus of low SWI signal, representing either haemorrhage or calcification, was noted. As the MRI scan was inconclusive and malignancy was still a concern, the patient had CT scan of the thorax, abdomen and pelvis, with no obvious malignancy identified.
Laboratory tests showed no coagulopathy (prothrombin time 10.5 s, activated partial thromboplastin time 24.6 s, Clauss fibrinogen 3.1 g/L) and haemoglobin was 143 g/L (normal 115–160 g/L).
Treatment
Rivaroxaban was withheld after discussing risks and benefits with the patient and her partner. The patient was also started on 60 mg of prednisolone once daily, with a plan of reducing the dose by 10 mg every 3 days. The pain was well controlled with oral analgesia, and the patient was discharged home with urgent follow-up.
Outcome and follow-up
The patient was readmitted the following week due to increased pain concerns, and further imaging was undertaken to rule out any complication. A repeat MRI concluded an appearance of resolving haematoma with significant size reduction of the previously described mass, which was now showing as high T1 signal. Afterwards, the patient was followed up by the medical team and the ophthalmology specialists. When reviewed 2 weeks after the initial presentation, the medical team decided to restart oral anticoagulation (rivaroxaban) and finish the last few days of the steroid course. At the 6-week follow-up appointment, the patient’s symptoms continued to improve, although she was still experiencing occasional shooting pain in the left frontal area. Ophthalmological examination remained unremarkable apart from a finding of mild nuclear cataract. A 4-month follow-up MRI demonstrated a further reduction in the haematoma and a slight reduction in the size of the belly of the left lateral rectus muscle, focal intramuscular T2 hyperintensity and resolving T1 hyperintensity with the surrounding retrobulbar fat returning to normal. MRI at 6 and 8 months showed that the left lateral rectus haematoma had continued to consistently reduce in size (figure 1).
Reduction of haematoma over serial MRIs. (A) Initial MRI: axial MRI showing a well-defined oval lesion arising from the left lateral rectus muscle. (B) MRI 7 days post-presentation: axial MRI demonstrating an appearance of resolving haematoma with significant size reduction. (C) MRI 8 months after presentation: axial MRI revealing significant resolution of the haematoma.
Discussion
NTOH related to the EOM is a rarely diagnosed clinical condition with characteristic radiological features.5 In a case series conducted by Chan et al, intra/juxta-oculomuscular haemorrhage was described to occur spontaneously in the absence of underlying vascular malformations. The study, which included 18 patients, reported that NTOH related to EOM mostly occurred in elderly women, and that hypertension, hyperlipidaemia and the use of antiplatelet were identified as the main risk factors. None of the patients suffered from systemic coagulopathy or were prescribed anticoagulant medications. Almost all patients woke up with sudden-onset unilateral pain (95%), diplopia (90%) and proptosis (95%). Visual disturbance was reported in 25% of episodes. On examination, moderate degree of axial proptosis was seen in the majority of patients, and intraocular pressure was often reported as normal.1 However, in our case, the diagnosis was more challenging as the patient presented with only acute periocular pain and, apart from tenderness on palpation, physical examination was unremarkable. In addition, none of the identified main risk factors were present in our patient.
EOM enlargement can result from a range of different pathologies, including inflammatory, infective, vascular and neoplastic conditions (table 1).5
Extraocular muscle enlargement aetiologies
| Inflammatory | Thyroid eye disease (most common cause)
Idiopathic orbital myositis IgG4-related disease inflammatory bowel disease-associated myositis Sarcoidosis Granulomatosis with polyangiitis |
| Infective | Cysticercosis Hydatid cyst Pyomyositis Orbital cellulitis |
| Neoplastic | Lymphoma (most common malignancy) Breast cancer Melanoma Neuroendocrine tumour Gastric adenocarcinoma |
| Vascular conditions | Carotid cavernous fistula Non-traumatic haemorrhage related to the extraocular muscle |
| Other | Amyloidosis |
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Various conditions could result in extraocular muscle enlargement including inflammation, infection, malignancy, amyloidosis and vascular conditions, with thyroid eye disease being the most common cause (table data obtained from Rana et al 5).
NTOH related to EOM is most commonly reported to occur in the inferior rectus muscle.1 Radiologically, it was often described as a well-defined mass with a characteristic anterior rounded border and a posterior tapering edge towards the orbital apex. Occasionally, fluid level may be present in the clot, of which presence supports a diagnosis of haematoma. Radiological findings on both CT and MRI scans vary depending on the haematoma age (table 2).5 However, since MRI can more accurately identify haemoglobin and its breakdown products, serial MRI scans can be more helpful in supporting the diagnosis of intramuscular haemorrhage.5 6
Radiological feature variation over time
| Timing of the bleed | Radiological features | |
| CT imaging | Acute haemorrhage | Hyperdense |
| Subacute haemorrhage | Hypoisodense | |
| MRI | Recent haemorrhage <2 days | Isointense T1 Hypointense T2W |
| 2 days–2 weeks (intracellular methaemoglobin) |
Hyperintense T1W Hypointense T2W |
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| 2 weeks–2 months (extracellular methaemoglobin) |
Hyperintense T2 |
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In both CT and MRI, radiological findings vary depending on when the imaging is done (table data obtained from Rana et al 5).
Initial imaging of our patient was concerning due to the unusual appearance of the lesion and its location. The first MRI described the lesion as a peripherally enhancing mass that was mildly hyperintense on T1 and T2. The report left the medical team with a wide differential that included inflammatory pathologies, haematoma and malignancies. Since the CT scan of the thorax, abdomen and pelvis showed no malignancy, steroids were started aiming to treat possible inflammatory aetiologies, and rivaroxaban was held as haematoma was also considered a possible differential. Subsequent MRI scans were more consistent with the appearance of haematoma as the mass demonstrated high T1 signal on the MRI performed 1 week post-discharge and this signal later resolved while a hyperintense T2 signal appeared.
Since their approval by the Food and Drug Administration in 2010, DOACs have been increasingly used for the prophylaxis and treatment of venous thromboembolism, in addition to their role in other thrombotic diseases.7 8 As with other anticoagulants, the most prominent complication of DOAC is bleeding.8 In their guidelines, the British Society of Gastroenterology recommended restarting DOAC 7 days after acute lower gastrointestinal bleeding.9 This was taken into account when restarting rivaroxaban for our patient, as there are no specific guidelines for EOM haemorrhage.
Patients with EOM-related NTOH often have an excellent outcome with complete resolution of symptoms. Surgical intervention has been required only in a minority of the published cases where the optic nerve was affected. By 6 months, most haematomas resolve on follow-up imaging.1 Our case has demonstrated a favourable outcome with conservative management. Despite restarting rivaroxaban, follow-up imaging reported continuous reduction in the haematoma size.
Learning points
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We believe that this is the first case to describe non-traumatic orbital haemorrhage in relation to extraocular muscle in a patient on anticoagulation.
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Extraocular muscle haemorrhage should be considered as a possible diagnosis for patients presenting with ocular symptoms and who are on anticoagulation.
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Serial MRI scans can be helpful to confirm the diagnosis.
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The patient’s haematoma continued to improve despite restarting rivaroxaban. As there are no current guidelines on restarting anticoagulation in similar cases, clinical judgement is required to decide an individualised management plan.
Ethics statements
Patient consent for publication
Acknowledgments
We would like to acknowledge the patient, who kindly has consented for her case to be published. We would also like to acknowledge Dr Bharati Vusirikala, from the Department of Ophthalmology, for his excellent clinical care of the patient and for supporting us while writing this case.
Footnotes
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Contributors DAR and NS have contributed as first authors. Both worked on the ward where the patient first presented and equally contributed to writing and finalising the case. RS has contributed by reporting the patient's scans for the purpose of writing the case. She has also contributed to the revision of the case and final approval before submission. EAD took the responsibility of diagnosing and managing the patient. She has also contributed to the revision of the case and final approval before submission.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.
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