British Journal of Neurosurgery, December 2014; 28(6): 787–790 © 2014 The Neurosurgical Foundation ISSN: 0268-8697 print / ISSN 1360-046X online DOI: 10.3109/02688697.2014.913776

SHORT REPORT

Intraorbital and intracanalicular ophthalmic artery aneurysms. Literature review and report of a case Giuseppe Maria Della Pepa*, Giovanni Sabatino*, Giuseppea La Rocca, Alba Scerrati, Giulio Maira, Alessio Albanese & Enrico Marchese Department of Vascular Neurosurgery, Institute of Neurosurgery, Catholic University of Rome, Rome, Italy

Brain magnetic resonance (MRI), followed by magnetic resonance angiography (MRA) and brain Computed Tomography Angiography (CTA), revealed an aneurysm close to the orbital apex, inferolaterally to the optic nerve, without signs of haemorrhage. Digital Subtraction Angiography (DSA) confirmed the aneurysm, 5 mm width, arising from the first intraorbital segment of the ophthalmic artery, partially thrombosed (Fig. 1). Pro-thrombotic risk factors were ruled out. The concomitant presence of visual loss and ophtalmoplegia (Apex Orbitae Syndrome) was likely caused by mass-effect over optic nerve in close proximity to third and sixth cranial nerve. Because of the acute symptoms onset, probably due to partial aneurismal thrombosis and in consideration of low haemorrhagic risk compared with high postoperative risks for both embolisation and surgery, a conservative medical therapy with dexamethasone and heparin was initiated. At one month follow-up visual acuity improved to 12/20, third cranial nerve palsy recovered completely, although partial sixth cranial nerve palsy still persists. MRI and DSA follow-up at three months displayed no substantial changes in aneurysm morphology.

Abstract This paper reviews literature about intraorbital ophthalmic artery aneurysms discussing presentation, aetiology and treatment options. In addition we report on a case of intraorbital ophthalmic artery aneurysm with acute onset of headache, visual loss and right eye ophthalmoplegia. Keywords: intraorbital aneurysm; intraorbital ophthalmic artery aneurysm; ophthalmic artery aneurysm; orbital apex syndrome

Introduction Intraorbital ophthalmic artery (OA) aneurysm is a rare entity compared with carotid-ophthalmic aneurysms arising from the wall of the internal carotid artery.1–2 Although aneurysms at the origin of the OA are common, there have been very few reports in the literature describing aneurysms arising from the artery itself.3 Even fewer have been described to occur within from the intraorbital portion of the artery. Generally these are associated with dural arterio-venous fistula (AVF), AVM or Moya Moya disease.3 Therapeutic options are still debated and management is still controversial.1–3 This paper reviews the literature about intraorbital OA aneurysms discussing presentation, aetiology and treatment options. In addition we report on a case of intraorbital OA aneurysm.

Literature review and case discussion The OA is the first major branch of the internal carotid artery after its emergence from the cavernous sinus and its entrance in subarachnoid space; it is composed of three segments: intracranial, intracanalicular and intraorbital.1 OA aneurysms are exceedingly rare findings: so far only 18 cases have been reported at the intraorbital segment, while only two cases at the intracanalicular segment. Cases of intraorbital and intracanalicular OA aneurysms reported in the literature with relevant treatment, and outcome is summarised in Table I.

Case report A 44-year-old man presented with acute onset of headache, nausea, vomiting, right eye visual loss and ophthalmoplegia. Right eye visual acuity was sensibly reduced to 5/20. Examination revealed sixth nerve palsy and incomplete third nerve palsy (with deficit in ocular elevation, adduction, mydriatic pupil with sluggish reaction to light).

*Authors equally contributed to the paper. Correspondence: Giuseppe Maria Della Pepa, MD, Department of Vascular Neurosurgery, Institute of Neurosurgery Catholic University of Rome, Largo A. Gemelli 8, 00168 Rome, Italy. Tel: ⫹ 39 0630154120. Fax: ⫹ 39 063051343. E-mail: [email protected] Received for publication 5 November 2013; accepted 6 April 2014

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Fig. 1. DSA-sagittal view (A) and 3D rotational DSA (B) displaying a 5-mm aneurysm arising from the first intraorbital segment of the ophthalmic artery, partly thrombosed. Axial (C) and coronal (D) T-2w MRI showing the aneurysm location at the orbital apex, underneath the optic nerve.

Intraorbital OA aneurysm appear to occur on the first or second part of intraorbital segments,4 especially where the ophthalmic artery crosses over the optic nerve. In the tight space of the optic canal the ophthalmic artery is in close proximity to the optic nerve, which explains the optic compression syndrome caused by an aneurysm in this localization. Our case displays a rather ‘strategical’ position inferolateral to the optic nerve, inferior to the third and medial to the sixth cranial nerve and provoked both the optic nerve conduction disorder and the oculomotor nerves disturbances.

Clinical presentation Most of intracranial OA aneurysms are asymptomatic if not haemorrhagic, while intracanicular/intraorbital ones are generally associated with visual loss and nerve palsies. From the literature review all intracanicular/ intraorbital cases show a clinical presentation due to visual symptoms and nerve palsies due to mass effect/ chronic pulsation.2,3,5,6 Distal intraorbital aneurysms show a rather benign course with slowly progressive decrease of visual acuity, whereas intracanalicular aneurysms, as our case, display a rapidly progressive loss of function of the optic nerve.1 Interestingly, only Meyerson and Lazar in 1971 have reported on an haemorrhagic presentation of an intraorbital intracranial aneurysm with an haemorrhagic presentation at onset, resulting in a sudden eye proptosis and blindness.

Aetiology Many authors described OA aneurysms on the first or second part of intraorbital segments, where the OA crosses over the optic nerve.2,4,6,7 Rengachary and Kishore explained the reasons of this frequency of congenital aneurysms on this segment of the OA. The regional vascular anatomy is the result of the formation of a complex arterial ring around the optic stalk by transient anastomoses between the primitive dorsal and ventral ophthalmic arteries and the stapedial artery. If one or more of these channels remains patent, an aneurysm attached to the parent vessel may form. This could explain the frequency of congenital aneurysms on this segment of the artery. A relevant number of aneurysms arising from the intraorbital portion of the OA are not ‘congenital’ but associated to other vascular malformations such as AVF/ AVM and thus the aneurismal formation may be explained by a haemodynamic stress that derived from the presence of the dural AVM: from the literature review 5 cases over 20 were associated with high flow malformations. Head trauma seems also a significant association (five cases) and these cases are generally considered post-traumatic pseudo-aneurysms.5–9

Treatment The best treatment option for these aneurysms remains unclear. Asymptomatic cases are generally treated conservatively1; in cases associated with other high flow malformations these are treated primarily.1

Kikuchi and Kowada (1994) Kawaguchi et al. (2001) Dehdashti et al. (2002) Ernemann et al. (2002) Kleinschmidt et al. (2004) Kleinschmidt et al. (2004)

Wang et al. (2007) Sabatino et al. (2009)

Pandey et al. (2010)

Li et al. (2012)

Pandey et al. (2013)

10 11 12 13 14 15

16 17

18

19

20

No No No No No Head kicked

No two accidents No Two concussions Head injury No No Missile injury No

Trauma history

57 years, F

25 years, F

57 years, F

No

Head injury

No

45 years, M No 52 years, M No

54 years, M 51 years, M 34 years, M 64 years, F 47 years, M 44 years, M

53 years, M 35 years, M 51 years, F 36 years, M 54 years, M 38 years, M 63 years, M 34 years, M 63 years, F

Age/Sex

Headache, ptosis

Blindness and right eye ophtalmoplegia

Headache and ptosis

Visual loss Asymptomatic

Disorientation and memory disturbances Frontal headaches Minimal visual field defect Periorbital pressure, visual loss Asymptomatic Chronic ache, visual loss

Painless, visual loss Progressive, visual loss Pulsating exophthalmos, visual loss Loss of central vision Haemorrhage, proptosis and blind Sudden blind Swelling, burning and visual loss Pulsating exopthalmos, blind Exophthalmos, visual loss

Clinical presentation

No

No

No

No No

No No No No No No

No No No No Yes No No No No

Haemorrage

IO

IO

IO

IO IO

IO IO IO IO IO IO

IC IC IO IO IO IO IO IO IO

Anesurysm location

Clipping and resection of pseudoaneurysm No

No

OA occluded OA occluded CCA ligation No OA obliteration No No OA clipping Trap and resection No No No OA sacrifice No OA and aneurysm embolisation Neck clipping No

Treatment for aneurysm

Multiple aneurysm

CM Multiple aneurysms Multiple aneurysms No

AVM AVF BA aneurysm No AVM AVM

No No No No No No AVF No No

Concomitant angiopathy

Outcome

Unchanged

Blindness, right eye ophtalmoplegia

Unchanged

Va preserved Unchanged

Unchanged Good Unchanged VA not recover Symptomless VA improved

VA improved VA improved Exophthalmos cured Unchanged Blind Blind Unchanged Blind VA recovered

M, male; F, female; IC, intracanalicular; IO, intraorbital; OA, ophthalmic artery; VA, visual acuity; CCA, common carotid artery; AVF, arteriovenous fistula; AVM, arteriovenous malformation; BA, basilar artery; CM, cavernous malformation; SAH, subarachnoid haemorrhage; ICA, internal carotid artery; rVA, right vertebral artery.

Piché et al. (2005) Choi et al. (2008) Mortada (1961) Rubinstein et al. (1968) Meyerson and Lazar (1971) Danziger and Bloch (1974) Rengachary and Kishore (1978) Rahmat et al. (1984) Ogawa et al. (1992)

Author/Year

1 2 3 4 5 6 7 8 9

Number

Table I. Concerning intraorbital and intracanalicular ophthalmic artery aneurysms.

Intraorbital/intracanalicular ophthalmic artery aneurysms 789

790

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Surgical clipping, parent vessel occlusion and trapping/ resection, while saving the central retinal artery, are all described as possibilities. Appropriate surgical techniques should be considered to preserve the normal ocular circulation. Because of the intimity of OA and the optic nerve, it was believed that neck clipping of the aneurysm is difficult. One other option is OA occlusion in the intraorbital segment (a rich anastomotic network between OA system and the ECA branches can still provide retinal blood supply and preserve patient vision)1–3; after OA occlusion, if collateral circle is not valid, acute retinal ischemia is still at risk in approximately 30% of cases.10,11 Seven OA aneurysm cases have been treated with sacrifice of the parent OA. Only two cases were associated with improvement of visual acuity after OA occlusion. However, it must be also noted that the majority of these cases in which visual acuity did not improve, and had an important visual compromise at admission. Fewer cases (3/20) describe direct intervention by aneurismal trap and resection or direct clipping of the aneurismal malformation and, despite technical difficulties described by authors (close proximity with II, III and VI cranial nerves and extremely limited space available), a clinical improvement was generally associated with the procedure (two cases: improvement, one case stabilization).8,11–13 Conservative treatment can be an option also in symptomatic cases (six cases reported in the literature) with general clinical improvement or stabilization. Prolonged heparin treatment is the most common choice as it reduces the risk of aneurismal thromboses and thus mass effect over optic nerves.2

Conclusions Intraorbital OA aneurysms are rare findings, associated with visual loss and nerve palsy. Haemorrhagic presentation is exceedingly rare. Most of cases are associated with high flow vascular malformations or have a post-traumatic aetiology. Treatment is still very debated, and options include direct aneurysm clipping, OA closure or conservative treatment.

Because haemorrhagic risk is low (only one case reported in literature) and symptoms generally depend on mass effect after aneurysm thrombosis, conservative heparin treatment is a feasible and less risky choice generally that yields to clinical improvement. Declaration of interest: The authors report no declarations of interest. The authors alone are responsible for the content and writing of the paper.

References 1. Qiao L, Wang H, Mao L, et al. Peripheral ophthalmic artery aneurysm. Neurosurg Rev 2011;34:29–38. 2. Sabatino G, Albanese A , Di Muro L, Marchese E. Bilateral intraorbital ophthalmic artery aneurysms. Acta Neurochir (Wien) 2009;151:831–2. 3. Dehdashti AR, Safran AB, Martin JB, Rüfenacht DA , de Tribolet N. Intraorbital ophthalmic artery aneurysm associated with basilar tip saccular aneurysm. Neuroradiology 2002;44:600–3. 4. Rengachary SS, Kishore PR. Intraorbital ophthalmic aneurysms and arteriovenous fistulae. Surg Neurol 1978;9:35–41. 5. Danziger J, Bloch S. An intra-orbital aneurysm of the ophthalmic artery. S Afr Med J 1974;48:2569–70. 6. Rahmat H, Abbassioun K, Amirjamshidi A . Pulsating unilateral exophthalmos due to traumatic aneurysm of the intraorbital ophthalmic artery. Case report. J Neurosurg 1984;60:630–2. 7. Rubinstein MK, Wilson G, Levin DC. Intraorbital aneurysms of the ophthalmic artery. Report of a unique case and review of the literature. Arch Ophthalmol 1968;80:42–4. 8. Li Y, Song WX, Zhang TM, et al. Intraorbital traumatic ophthalmic artery aneurysm: case report. Neurol India 2012; 60:657–60. 9. Kleinschmidt A , Sullivan TJ, Mitchell K . Intraorbital ophthalmic artery aneurysms. Clin Experiment Ophthalmol 2004;32:112–4. 10. Finnerty KN, Mancini R. Vision loss after maxillary artery embolization secondary to compressive optic neuropathy. Ophthal Plast Reconstr Surg 2013;29:e108–10. 11. Naqvi J, Laitt R, Leatherbarrow B, Herwadkar A . A case of a spontaneous intraorbital arteriovenous fistula: clinicoradiological findings and treatment by transvenous embolisation via the superior ophthalmic vein. Orbit 2013;32:124–6. 12. Ogawa A , Tominaga T, Yoshimoto T, Kiyosawa M. Intraorbital ophthalmic artery aneurysm: case report. Neurosurgery 1992;31:1102–4; discussion 4. 13. Wang YY, Thani NB, Han TF. Optic nerve penetration by a carotico-ophthalmic artery aneurysm. J Clin Neurosci 2010;17: 931–3.

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Intraorbital and intracanalicular ophthalmic artery aneurysms. Literature review and report of a case.

This paper reviews literature about intraorbital ophthalmic artery aneurysms discussing presentation, aetiology and treatment options. In addition we ...
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