Ophthalmic Artery Pseudoaneurysm

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Article initiated by:

Yanhan Ren

All contributors:

Yanhan Ren, James B. Davis, MD, MBA, Grant A. Justin, MD, Michael T Yen, MD, Amanda D. Henderson, MD, Yanhan Ren

Assigned editor:

Amanda D. Henderson, MD, Michael T Yen, MD

Review:

Assigned status Update Pending

 by Amanda D. Henderson, MD on July 15, 2023.

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Contributing Editors:	add

Ophthalmic Artery Pseudoaneurysm (OAPA) is a rapidly onset, vision, and life-threatening condition that may occur in the context of trauma or post-surgical complications. Proper identification of the signs and symptoms of OAPA is crucial for determining management approaches to achieve optimal visual outcomes.

Disease Entity

Ophthalmic artery pseudoaneurysm is a rare condition characterized by the formation of a false aneurysm in the ophthalmic artery. The process of OAPA formation shares similarities with other large vessel pseudoaneurysms, wherein the shearing force resulting from trauma leads to the creation of a false lumen. Ophthalmic artery pseudoaneurysm is even more uncommon due to orbital walls protection and the cushioning provided by surrounding orbital adipose tissue. The risk of OAPA formation is significantly heightened only in the presence of direct trauma to the orbits. Additional risk factors identified in previous literature and clinical practice include the use of anticoagulation, high-energy impact, or penetration into the orbits. OAPA can also result from infection or surgical procedures.

Etiology

In contrast to a true aneurysm, a pseudoaneurysm is not contained by the vessel wall; instead, blood leaking through a defect in the arterial wall is contained by an out-pouching of adventitia or perivascular soft tissue. The primary cause of ophthalmic artery pseudoaneurysm (OAPA) is typically high-energy trauma, including blunt or penetrating injuries to the eye or head. However, it can also arise from inflammation, infection, or prior surgical procedures. Typically, the presentation is unilateral due to the nature of traumatic impact, but bilateral occurrence can occur if both orbits experience high-energy injuries.

Diagnosis

Patients with OAPA commonly arrive at the emergency room as trauma activations following a fall or motor vehicle accident. In the context of acute trauma, patients may have trouble to provide an accurate history. Diagnosing ophthalmic artery pseudoaneurysm (OAPA) typically necessitates a comprehensive approach, including clinical evaluation, detailed eye examinations, radiological imaging studies, and additional interventional procedures.

History

Patient who presents with ophthalmic artery pseudoaneurysm already suffered high energy impact injury to the head and will most likely present with mental status change and memory loss. If available, the history-taking portion should focus on the mechanism of facial and ocular injuries. High-energy blunt and penetrating injury can both contribute to these pseudoaneurysms formations. Some of the common types of blunt trauma injuries identified in past literature include fist fight, motor vehicle accidents (head strike, airbag deployment, whiplash injuries, etc.), fall. There have also been recordings of penetrating orbital projectile-induced OAPA formation, such as bullets and knives.

Other risk factors, such as prior history of ocular infection, cellulitis, arterial-venous fistulas, and any other significant orbital histories are also useful. Comorbidities such as diabetes and cardiovascular disease are also important as patients might be taking anti-platelets or antithrombotic medications which could greatly increase the risk of pseudoaneurysm malformation.

Pseudoaneurysm formation can be iatrogenic as well. Recent past surgical and procedural histories such as nasogastric tube placement, oculoplastic surgeries, ENT surgeries, or skull-based neurosurgeries are also helpful in identifying the source of the lesion.

Physical Examination

A comprehensive physical examination of the eye and its surrounding structures is essential. Examiner should focus on visual acuity, ocular motility, presence of chemosis, subconjunctival hemorrhage, proptosis, and pulsatile mass around the orbit. Drawing from clinical experience at UMass Memorial Medical Center, the appearance of a pulsatile mass around the orbit emerges as the most indicative sign of pseudoaneurysm formation.

Intraocular pressure can spike suddenly due to an increase in pseudoaneurysm volume or concomitant retrobulbar hemorrhage. Prompt reduction in IOP such as glaucoma eye drops, canthotomy, and cantholysis should be considered based on available IOP readings and physical examination findings. It is always helpful to collaborate with neurosurgery or neuro-critical care team to determine dilation status and obtain dilated fundus exam findings at the earliest convenience to identify potential retinal hemorrhages, ischemic injuries, or optic nerve compressions.

Diagnostic Procedures

This is the Initial CT Head Angiogram that captured OAPA (yellow arrow): Patient on eliquis initially presented to the ED after tripping and falling with positive head strike against cement pavement. Trauma team noted pulsatile hematoma and proptosis of the right orbit. OAPA started to appear 24 hours after initial resuscitation at the trauma bay. Radiology noted 2 small foci of contrast in the right superomedial orbit with possible active extravasation; a subdural hematoma with midline shift.

Previous studies have reported using various imaging techniques such as CT, CT-Angiogram, MRI, MRV, MRA, or digital subtraction angiography to aid in the diagnosis of pseudoaneurysm. CT and CT-Angiogram would be the ideal screening modalities, while digital subtraction angiography would be the most specific in diagnosis.

CT Head, CT angiogram

When patient first present to the emergency room, CT head without contrast and CT angiogram are extremely helpful in identifying intracranial bleeding and vascular lesions. CT angiograms of the head and the neck are also helpful in identifying vascular injuries. Based on past literature information and ED triaging experience, pseudoaneurysm may expand quickly in its early phase of development, which may mimic an expanding hematoma. However, the exact timing of OAPA progression may not occur until up to 24 hours after the initial injury. Follow-up imaging will be helpful in monitoring the size of progression and determining next step management. Unlike hematoma, pseudoaneurysms will stabilize in size without active contrast extravasation.

MRI, MRA, MRV, and Angiography

Patient with suspected OAPA typically requires Neuro-ICU level of care. Follow-up advanced imaging such as MRI, MRA, MRV are helpful in confirming the diagnosis and identifying the source of the lesion. Based on past literature research, MRI, MRA, and MRV are all reasonable diagnostic imaging studies for these types of scenarios. Digital subtraction angiography through Neuro-Intervention is the most confirmatory imaging yet most invasive. It will help to visualize the lesion in real-time with detailed information on size and hemodynamics. Angiography can be both diagnostic and curative to OAPA. However, detailed discussions on prognosis should be discussed in detail with patients as the location of these pseudoaneurysms is in proximity to the central retinal artery and embolization procedure may potentially lead to permanent vision loss.

Subsequent cerebral angiogram demonstrating the one of the two OAPA located at the middle right ophthalmic artery measuring about 7.17*5.28 mm without active extravasation or arterial-venous fistula, confirming the diagnosis of OAPA. Due to proximity to the right central retinal artery, endovascular embolization was deferred.

Management

Neuro-Intervention (endovascular occlusion)

Prompt endovascular occlusion can prevent complications such as enlargement, hemorrhage, and hematoma formation. This can be achieved by microcatheterization and coil embolization. In this procedure an endovascular wire is navigated close to the pseudoaneurysm, followed by a microcatheter which is then used to deliver the coil or glue for embolization. Angiography is then used to confirm complete coil embolization. These procedures were mostly successful in occluding OAPA and preventing vision loss (please refer to the final literature summary table).If the lesion site is near the origin site of the internal carotid artery, endovascular balloon embolization can be attempted, though this was mostly performed before the availability of coiling. If the proximal ophthalmic artery becomes occluded, branches of the external carotid artery can potentially provide collateral blood flow to the visual system through anastomoses with the extra-orbital branches of the ophthalmic artery. However, this anastomosis may also not occur and lead to permanent vision loss. Other novel materials were also used in the occlusion process. One study reported a case of OAPA presenting with persistent epistaxis after removal of a nasogastric tube. This patient was treated with 25% glue embolization (NBCA) injection to embolize the lesion.

Surgery, External Approach

Other than endovascular approach, there have also been recorded cases of external surgical approaches to surgically remove the pseudoaneurysms. It is a much more invasive technique and the final outcomes are worse compared to endovascular embolization. There have been only 2 recorded cases of external surgical approach completed in China and Japan . The surgery in China was performed on a patient who suffered blunt trauma (punch) to the orbit. The surgeons were able to successfully remove the pseudoaneurysm by entering through right cranio-orbital approach with orbital decompression. However, due to the degree of injury to the globe and optic nerve, visual acuity remained at NLP with total ophthalmoplegia . The case in Japan was also performed on a patient who sustained closed head injury from motor vehicle accident. The surgeons were successfully able to resect the pseudoaneurysm through extradural approach. However visual acuity remained at NLP after surgery.

Conservative Management

Final CT-Angiogram demonstrating the OAPA at the right mid-ophthalmic artery noted in in the initial CT-A and angiography, indicating the pseudoaneurysm has most likely self-embolized and no longer taking up any contrast.

At this time there has been one recorded conservative management case in approaching OAPA, which was done at UMass Memorial Medical Center. Through a multi-disciplinary care approach involving ophthalmology, neuro-intervention, neruo-critical care, the patient's pseudoaneurysm self-embolized 72 hours later as demonstrated by serial CT-Angiogram scans.

Prognosis

OAPAs are dangerous because they can enlarge over time and eventually rupture, resulting in intracranial hemorrhage or hematoma formation. Pseudoaneurysm of the dural portion of the ophthalmic artery can result in subarachnoid hemorrhage with a high risk of re-hemorrhage if left untreated.

Outcomes

Visual recovery in endovascular occlusion cases has been most likely successful with low rate of central retinal artery occlusion. Timely management of these pseudoaneurysms successfully reduced the rate of intracranial bleeding and mortalities. Current literature showed 0% of mortality rate among patients with OAPA who were managed surgically. However, should the initial trauma injury cause significant globe optic nerve injury, visual outcome may be limited and may eventually require evisceration or enucleation (please refer to the final literature summary table).

Complications

For patients who underwent successful endovascular embolization, some of the common complications recorded include ptosis and vasospasms. For patients who underwent interventions, but were unsuccessful in recovering beyond no light perceptions in the traumatic eyes, corneal edema, pain, and proptosis are the most common symptoms and these patients eventually required eviscerations or enucleations for comfort measures.

Cranial nerves are also susceptible to injuries even after pseudoaneurysm embolization or self-embolization, though this effect may be transient. For the one patient who underwent serial CT-Angiogram monitoring without monitoring at the University of Massachusetts Memorial Hospital, complete cranial nerve 3 palsy with persistent traumatic pupil of the injured eye was discovered on follow-up 1 month later. The exact etiologies for the palsy were unclear but may be related to transient compression after the traumatic injury. The CN3 palsy self-resolved about 3 months follow-up; however, the traumatic pupil persisted and visual acuity only corrected to 20/70 (the unaffected eye was 20/30). Given the patient’s age and cardiovascular risk factors, the decision was made to closely monitor without any more procedural intervention.

Literature Summary

Below is the table listing all past literatures discussing OAPA, the team’s treatment approaches, surgical outcomes, and complications. Due to limitation in journal access, some information might be missing.

Studies/Cases	Case size	Year	Types of injury	Location of OAPA	Management	Complications	Outcome
Czyz C, et al.	1	2017	Penetrating bullet intra-orbital	Right ophthalmic artery	Endovascular occlusion coiling	Corneal edema, hyphemia, hemorrhagic chemosis	NLP, evisceration
Chun H, et al.	1	2007	Motor vehicle accident, blunt head trauma	Extra-cranial, left ethmoid branch	Endovascular occlusion coiling	None	Intervention induced vasospasm, self-sealed
Balushi AA, et al.	1	2017	Penetrating bullet intra-orbital	Bilateral ophthalmic artery	Endovascular occlusion coiling	None	Clinically stable
Kelly ME, et al.	1	2009	Bicycle accident, blunt head trauma	Right ophthalmic artery	Endovascular occlusion coiling	Failed first attempt	Clinically stable
Rosssitti  S, et al.	1	2009	Non-trauma, progressive vision loss	Right ophthalmic artery	Endovascular occlusion coiling	None	Clinically stable
Li Y, et al.	1	2017	Fist fight, blunt head trauma	Right ophthalmic artery	External surgical resection	None	NLP, ophthalmoplegia
Dolati-Ardejani P, et al.	1	2012	Fall, blunt head trauma	Right ophthalmic artery	Endovascular embolization coiling	Failed procedure, occlusion of the proximal OphA	NLP
Liao Zb, et al.	1	2023	Motor vehicle accident, blunt head trauma	Right ophthalmic artery	Endovascular occlusion coiling	None	Clinically stable
Selcuk H, et al.	1	2005	Nasogastric tube placement	Left ophthalmic artery	Endovascular occlusion (NBCA-histoacryl (25%) injection)	None	Clinically stable
Kikkawa Y, et al.	1	2012	Closed head injury	Left ophthalmic artery	External surgical resection	None	NLP
Kanazawa R, et al.	1	2011	Fall, blunt head injury	Left ophthalmic artery	Endovascular occlusion coiling	Redo in 5 months	Clinically stable
Hopkins JK, et al.	1	2007	Unclear	Ophthalmic artery	Endovascular occlusion coiling	Unclear	Unclear
Shim YS	1	2021	Fall, blunt head injury	Right ophthalmic artery origin site	Endovascular occlusion coiling	None	Clinically stable
Deshmukh YR, et al.	1	2005	Iatrogenic, endovascular coil migration	Left ophthalmic artery near aneurysm rupture site	Surgical ligation	Vasospasm, cerebral edema	Neurologically intact without focal neuro deficit
Colewell L, Ren Y, et al. UMass Memorial Case (case not yet published)	1	2023	Fall, blunt trauma	Right mid-ophthalmic artery	Conservative observation	Ptosis, traumatic pupil	Clinically stable, visual acuity 20/70 in OD