Spontaneous postpartum lateral rectus haemorrhage

  1. Daire John Hurley ,
  2. Rory Murphy and
  3. Stephen Farrell
  1. Ophthalmology, Mater Misericordiae University Hospital, Dublin, Ireland
  1. Correspondence to Dr Daire John Hurley; dairehurley@gmail.com

Publication history

Accepted:11 Feb 2022
First published:03 Mar 2022
Online issue publication:03 Mar 2022

Case reports

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Abstract

A postpartum woman presented with sudden-onset left eyelid swelling and severe pain. Clinical examination revealed left exophthalmos and ophthalmoplegia with marked resistance to retropulsion of the left globe. The patient was not able to perceive light in the affected left eye and a relative afferent pupillary defect was present. CT orbits showed an enhancing lesion in the left retrobulbar space, suggestive of a lateral rectus haemorrhage. An emergency left lateral canthotomy and inferior cantholysis was performed. A day later, an MRI showed expansion of the left lateral rectus with significant mass effect on the globe. As the visual acuity remained reduced at counting fingers and there was a persistent relative afferent pupillary defect (RAPD), an exploratory orbitotomy and haematoma evacuation was performed. Three days postoperatively, the visual acuity had returned to 6/6. Eye movements normalised within 2 weeks and follow-up imaging revealed near complete resolution of the haematoma.

Background

Spontaneous orbital haemorrhage is a rare condition whereby haemorrhage develops within the orbit in the absence of preceding trauma. It may occur at any age and is commonly associated with vascular abnormalities or systemic predisposing factors.1 Typical presentation is with acute proptosis, ophthalmoplegia, pain, nausea and vomiting. Imaging is essential to determine the location of the haemorrhage. Urgent decompression is indicated in cases where there is potential optic nerve damage due to compressive ischaemia.

We present a case of severe, spontaneous lateral rectus haemorrhage in a woman 1 week postpartum requiring timely surgical intervention to restore vision.

Case presentation

A woman in her late 30s woke up with severe pain, sudden-onset left eyelid swelling, nausea and vomiting, prompting presentation to her local emergency department. She was 1 week postpartum following spontaneous vaginal delivery of a healthy baby at 40 weeks’ gestation. There was no history of recent trauma, surgery or infection. The patient was not on any regular medication and her medical history was otherwise unremarkable.

Clinical findings on presentation revealed left exophthalmos and ophthalmoplegia with marked resistance to retropulsion of the left globe. The patient was not able to perceive light (NPL) in the affected left eye and a relative afferent pupillary defect was present. The intraocular pressure (IOP) in the left eye was 40 mm Hg, and the left optic nerve and macula were grossly intact on initial examination.

There was no skin erythema or vesicles suggestive of bacterial or viral cellulitis. The patient was afebrile and normotensive with normal heart rate. Bloods, including full blood count, urea and electrolytes and coagulation studies, were within normal limits with the exception of a borderline reduced haemoglobin level of 93g/L and raised C reactive protein of 24.

Investigations

A CT of the orbits showed an enhancing lesion in the left orbit in the region of the lateral rectus which was compressing the globe and impinging on the optic nerve (figure 1). Clinical examination and imaging findings were consistent with an orbital compartment but no clear aetiology was determined at this stage.

Figure 1

CT orbits (first day of presentation): (A) axial and (B) coronal. This shows an enhancing lesion in the left orbit in the region of the lateral rectus causing compression of the globe and impingement on the optic nerve.

An emergency lateral canthotomy and inferior cantholysis was performed under local anaesthesia to relieve the orbital compartment syndrome. Within several minutes, the patient stated the pain had reduced from a 9/10 to 5/10. IOP reduced to 13 mm Hg and visual acuity improved to hand movements. The patient was given intravenous analgesia and started on oral amoxicillin/clavulanic acid (625 mg three times per day), acetazolamide (250 mg two times per day) and prednisolone (40 mg once daily).

An MRI orbit was performed the following day. This showed expansion of the left lateral rectus (3.7 × 2.0 × 2.4 cm) causing significant mass effect with associated proptosis (9 mm asymmetry vs the contralateral side) (figure 2). This displayed isotense to mildly hyperintense T1 signal with no postcontrast enhancement. These findings were consistent with haemorrhage. No other vascular abnormalities or solid tumours were identified. The other ocular muscles and the optic nerve were of normal appearance.

Figure 2

MRI orbits (second day of presentation): (A) axial and (B) coronal. This shows expansion of the left lateral rectus causing significant mass effect with associated proptosis. This was consistent with haemorrhage as it displayed isotense to mildly hyperintense T1 signal with no postcontrast enhancement.

Treatment

Despite immediate lateral canthotomy and inferior cantholysis being performed at presentation, the visual acuity remained reduced at counting fingers, and given the persistent nature of the RAPD and proptosis, an exploratory orbitotomy and haematoma evacuation was performed in the operation theatre under general anaesthesia. At the time of surgery, the anterior portion of the lateral rectus muscle appeared normal. Blunt dissection into the lateral rectus sheath at the posterior limit of the globe produced dark blood which was evacuated via suction. The conjunctiva was closed.

Outcome and follow-up

Day 1 postevacuation, visual acuity improved to 6/36. IOP remained high (26 mm Hg), but there was no evidence of disc swelling on examination. Day 2 postoperatively, the visual acuity had improved further to 6/9+1 and the IOP reduced to 18 mm Hg. The patient reported entire resolution of pain. The patient was discharged home the following day with a visual acuity of 6/6 and a short taper of oral prednisolone, in addition to oral acetazolamide 250 mg two times per day, and oral amoxicillin/clavulanic acid.

The patient was followed up weekly over the following month. Eye movements had normalised after 2 weeks. Colour vision testing using Ishihara was fast and full in the affected eye. The only positive finding on examination was subtle left-sided ptosis. A follow-up MRI orbits at 3 weeks revealed near complete resolution of the haematoma within the lateral rectus muscle with some mild persistent T2 fluid-attenuated inversion recovery (FLAIR) hyperintensity of the retrobulbar fat without residual mass lesion (figure 3).

Figure 3

MRI orbits (3 week follow-up): (A) axial and (B) coronal. This shows near complete resolution of the haematoma within the lateral rectus muscle with some mild persistent T2 FLAIR hyperintensity of the retrobulbar fat.

Discussion

Spontaneous orbital haemorrhage may be more accurately referred to as non-traumatic orbital haemorrhage. While the bleed may be truly spontaneous, often these occur in the setting of a vascular malformation or bleeding diathesis. A case series of 115 patients who presented to Moorfields Eye Hospital with spontaneous orbital haemorrhage found 90% of patients had associated vascular malformations, the majority of which were venous.1 Eleven per cent had additional or other predisposing factors, including childbirth (3.5%), prolonged headstands, hypertension or coagulopathy. There was no predisposing factor identified in only 5% of cases. Spontaneous haemorrhage into the lateral rectus was noted in one of these patients with no predisposing factors.

In our case report, the patient presented 1 week postvaginal delivery. Labour, and its associated Valsalva-like manoeuvre, has been previously described as a risk factor for the development of orbital haemorrhage.2 3 The forceful exhalation against a closed glottis of a Valsalva strain leads to increased venous pressure with a resultant surge in ocular circulation. This may manifest as haemorrhages in any orbital structure: periorbital skin, conjunctiva, retina or in the vitreous.4 Labour associated cases of Valsalva haemorrhagic retinopathy is a more recognised ophthalmic sequelae of labour in the literature.5 6 The only described spontaneous orbital haemorrhages in the literature are those within the Moorfields case series. They found three of the four patients who had spontaneous orbital haemorrhage associated with childbirth had complete resolution, while one lost total vision due to irreversible optic nerve compression. Our case is the first reported case of a postpartum orbital haemorrhage with a complete resolution of visual acuity from NPL to 6/6 following decompression and surgical evacuation.

Spontaneous orbital haemorrhages follow a bimodal distribution in terms of age of onset.1 In the younger population, less than 20 years of age, vascular anomalies are more likely and the haemorrhage tends to be localised. Whereas in the older population, structural and haemodynamic changes are more causative and the haemorrhage follows a more diffuse distribution. Visual loss is more likely to occur in patients over the age of 70.2 7 Bleeds may also recur at any interval, with 36% of patients in the case series having at least one further haemorrhage.1

Imaging is crucial in the work-up of these patients. CT allows quick detection and localisation of the likely haemorrhage source. However, the gold standard is MRI as this enables better assessment of related vascular anomalies and identifying the extent of any haemorrhagic cysts. Onami et al detailed the natural course of a spontaneous lateral rectus haemorrhage with MRI imaging.8 This was a case involving a 40-year-old woman, with no predisposing risk factors, that resolved with no intervention. They showed that resolution of the intramuscular haematoma correlated with an improvement of symptoms through a series of three MRI scans over 6 months.

A conservative approach may be adopted in cases with intact optic nerve function. A case series by Hakin et al examined three patients with spontaneous haemorrhage within rectus muscle with no apparent cause.9 All three completely resolved with no treatment. A case report by Zuercher et al looked at the outcome of a 77-year-old, hypertensive woman with a spontaneous lateral rectus haemorrhage.7 In this case, a conservative approach was adopted as the patient’s vision was preserved within the initial 12 hours of presentation. The Moorfields case series found that 93% of patients did not require surgical intervention, and of these, 62% had complete spontaneous resolution of the haemorrhage, 27% had partial resolution and 4% had no resolution. All case series referenced were in agreement that in patients with no visual disturbance and no orbital signs, careful observation with serial imaging is sufficient and a good visual prognosis can be expected.

While a large number of these cases may be self-limiting, prompt assessment of optic nerve function is essential to identify those with evidence of orbital compartment syndrome that may require emergent evacuation and decompression. Orbital compartment syndrome is a sight-threatening emergency whereby the sudden rise in intraorbital pressure exceeds the vascular perfusion of the ophthalmic artery resulting in optic nerve ischaemia. In patients who have diffuse bleeds causing compressive optic neuropathy, urgent lateral canthotomy and inferior cantholysis is required. If an encysted collection of blood is present, it may be more appropriate to drain the collection surgically as guided by orbital imaging studies.10 In our case, the patient required both the approaches to fully decompress such a significant haemorrhage. Appropriate and swift treatment may allow for excellent visual recovery as we noted here with close follow-up to monitor for recurrence.

Learning points

  • Spontaneous orbital haemorrhage must be considered in a postpartum patient with sudden onset of severe pain and eyelid swelling.

  • Urgent decompression is indicated in cases with significantly reduced visual acuity or in the presence of a relative afferent pupillary defect.

  • Lateral canthotomy and inferior cantholysis may be used in the emergency setting but formal evacuation in theatre may be required if orbital signs remain.

  • Imaging (CT or preferably MRI) is essential to determine the location of the haemorrhage.

Ethics statements

Patient consent for publication

Footnotes

  • Contributors DJH, RM and SF undertook background research on the topic; DJH wrote the initial draft of the manuscript; RM and SF reviewed and made edits to the manuscript. All authors have read and agreed to the published version of the manuscript.

  • 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.

  • 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.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

References

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