Case Report
Orbital Compartment Syndrome Secondary to Direct Carotid Cavernous Fistula After
Carotid Cavernous Aneurysm Rupture: Case Report and Review of Literature
Mohammad Ghorbani1, Seyed Ebrahim Hejazian1, Alireza Dastmalchi1, Mehdi Chavoshinejad1, Sina Asaadi1,2
Key words
- Intracavernous aneurysm
- Orbital compartment syndrome
- Rupture
Abbreviations and Acronyms
CCA: Carotid cavernous aneurysm
CCF: Carotid cavernous fistula
ICA: Internal carotid artery
IOP: Intraocular pressure
OCS: Orbital compartment syndrome
From the 1Division of Vascular and Endovascular
Neurosurgery, Firoozgar Hospital, Tehran; and 2Neuroscience
Research Center, Shahid Beheshti University of Medical
Sciences, Tehran, Iran
To whom correspondence should be addressed:
Sina Asaadi, M.D.
[E-mail: s.asaadi90@gmail.com]
Citation: World Neurosurg. (2020) 133:409-412.
https://doi.org/10.1016/j.wneu.2019.08.037
Journal homepage: www.journals.elsevier.com/world-
neurosurgery
Available online: www.sciencedirect.com
1878-8750/$ - see front matter ª 2019 Elsevier Inc. All
rights reserved.
BACKGROUND
Carotid cavernous aneurysms (CCAs) that
arise from the cavernous segment of the
internal carotid artery (ICA) are often
asymptomatic and detected incidentally.
However, the mass effect of a giant CCA
can result in headache, diplopia, ptosis,
ophthalmoparesis, ophthalmoplegia, tri-
geminal neuropathy, and decreased vision
due to optic nerve compression.1,2
The risk of CCA rupture is low, but it
can result in carotid cavernous fistula
(CCF) due to the rupture of an aneurysm
into the cavernous sinus, along with
epistaxis due to erosion into the sphenoid
sinus, spontaneous thrombosis, and sub-
arachnoid hemorrhage.3-6
We report a case of a ruptured CCA
causing rapid progressive chemosis,
proptosis, and irreversible loss of vision.
CASE PRESENTATION
A 65-year-old woman with a history of poorly
controlled diabetes and hypertension over
WORLD NEUROSURGERY 133: 409-412, JANUARY 2020
- BACKGROUND: Carotid cavernous aneurysm (CCA) rupture is rare. However,
it can result in various complications such as carotid cavernous fistula (CCF),
epistaxis, spontaneous thrombosis, and subarachnoid hemorrhage.
- CASE DESCRIPTION: We report a 65-year-old woman with a history of un-
controlled diabetes who was referred with complaints of acute headache,
diplopia, proptosis, and chemosis. Ophthalmic examination revealed elevated
intraocular pressure in the right eye, optic disk edema, and retinal venous
congestion. Canthotomy was performed based on a diagnosis of orbital
compartment syndrome (OCS). Further imaging revealed a dilated superior
ophthalmic vein and cavernous sinus, as well as swelling of the extraocular
muscles in the right eye. Digital subtraction angiography revealed the fistulous
connection between the cavernous part of the internal carotid and cavernous
sinus (direct CCF) due to the large ruptured CCA, resulting in retrograde flow
through the superior and inferior ophthalmic veins. Successful endovascular
coiling of the aneurysm resulted in complete occlusion of the fistula. Post-
intervention ophthalmic examination demonstrated progressive improvement of
ophthalmic signs and symptoms; however, the patient’s right eye remained
sightless.
- CONCLUSIONS: In patients with clinical manifestation of OCS with no history
of any predisposing risk factors, diagnosis of ruptured cavernous sinus aneu-
rysm and resulting direct CCF should be considered. In such cases, emergent
imaging along with early endovascular intervention can resolve OCS and pre-
vent permanent ocular injury and vision loss.
the last 3 years presented with complaints of (OCS) due to infection, and endoscopic
acute headache, diplopia, mild proptosis, nasal biopsy was performed to rule out
and chemosis. The patient’s symptoms fungal infection (mucormycosis) while
began 10 days before receipt of empirical considering uncontrolled diabetes mellitus
topical antibiotic therapy with the probable as a predisposing factor, which revealed no
impression of acute right eye conjunctivitis positive findings.
in a primary healthcare center. After 2 days, A noncontrast computed tomography
the patient’s symptoms were aggravated, scan performed to diagnosis the underly-
with worsening of headache and right orbit ing cause of the elevated IOP revealed a
pain and severe chemosis, proptosis, oph- dilated superior ophthalmic vein of the
thalmoplegia, and acute loss of vision in the right eye and cavernous sinus (Figure 1A
right eye. and B). Magnetic resonance imaging of
Ophthalmic examination performed in the orbit confirmed dilatation of the
the Emergency Department revealed an right cavernous sinus and superior
intraocular pressure (IOP) of 39 mmHg in ophthalmic vein, as well as mild swelling
the right eye and 11.5 mmHg in the left of the extraocular muscles (Figure 1C).
eye. Slit-lamp examination revealed optic The patient was referred to our
disk edema and retinal venous congestion. institution with suspicion of vascular
Canthotomy was performed based on a malformation. Based on imaging findings,
diagnosis of orbital compartment syndrome emergency digital subtraction angiography
www.journals.elsevier.com/world-neurosurgery 409
 CASE REPORT
MOHAMMAD GHORBANI ET AL. ORBITAL COMPARTMENT SYNDROME DUE TO RUPTURED CAROTID CAVERNOUS ANEURYSM

Figure 1. (A and B) Axial brain computed tomography scans showing a
dilated right superior ophthalmic vein (arrow) and cavernous sinus
(arrowhead). (C) Magnetic resonance imaging showing right extraocular
muscle enlargement (arrowhead) and a dilated left superior ophthalmic
vein (arrow). (D) Digital subtraction angiography (DSA), lateral projection, of
the right internal carotid artery showing a direct carotid cavernous fistula
with high retrograde flow toward the superior and inferior ophthalmic veins
(arrows). (E) DSA after embolization of a carotid cavernous aneurysm
showing complete occlusion of the aneurysm (arrowhead). (F and G)
Ocular symptomatology before and 5 months after complete embolization.

was performed, which showed a fistulous
connection between the cavernous part of
the ICA and the cavernous sinus due to the
ruptured CCA large aneurysm, which had
resulted in retrograde flow through the
superior and inferior ophthalmic veins
(Figure 1D). The patient underwent
successful endovascular coiling of the
ruptured CCA, which resulted in
complete occlusion of the fistula
(Figure 1E).
Postintervention ophthalmic examina-
tions at 1 week and 5 months after the
embolization revealed progressive
improvement of all ophthalmic signs and
symptoms; however, the patient’s right
eye remained sightless (Figure 1F and G).
DISCUSSION
Although diplopia, proptosis, blurred
vision, and chemosis are well-known
ocular manifestations of CCF, they may
occur secondary to dural arteriovenous
fistula or CCA rupture.4,7 CCAs arise from
the cavernous segment of the ICA and
account for 2%e9% of intracranial
aneurysms.8 CCAs have a strong female
predominance and may be idiopathic,
iatrogenic, traumatic, or infectious.8 The
risk of CCA rupture is low, ranging from
0% in aneurysms smaller than 13 mm to
6.4% in those larger than 25 mm.9
We present a case of high-flow CCF due
to ruptured CCA resulting in progressive
proptosis, chemosis, and irreversible
vision loss despite endovascular
intervention at the appropriate time. CCF
is a well-known complication of ruptured
CCA, for which proper intervention can
lead to complete cure with no complica-
tions. Treatment strategies for direct CCF
include surgery and endovascular in-
terventions, including detached balloon,
coil embolization with or without of bal-
loons or liquid agents, and intracranial
covered stents. Surgical treatments for
CCF consist of various approaches,
including ligation of the CCA, surgical
trapping of the fistula, and surgical
transvenous packing. However, owing to
the high incidence of cranial nerve defi-
cits, ipsilateral ischemic stroke, and
residual fistulous communications, open
surgical procedures are rarely used except
in cases where endovascular therapy has
failed.10-12
For this patient, based on the etiology
of OCS, we chose an endovascular inter-
vention, which is the treatment of choice
in case of ruptured CCA. The aim of
endovascular interventions in treating CCF
is remove the fistula from the circulation,
preserve the parent artery, reduce the
length of hospital stay, and lower the rates
of morbidity and mortality.13-16
Occluding the orifice of the fistula with
a detachable balloon is a well-known
technique, but it has been abolished in
United States and some other countries
because of its disadvantages. These
include a failure rate of 10% in selective
occlusion of fistulas either in fistulas with
a smaller orifice size or in the carotid
sinus, difficulty in navigating to the fistula
in cases of segment c4 fistula owing to an
extremely acute angle, a risk of balloon
perforation during inflation due to bone
spicules, a risk of thromboembolic
complications caused by balloon manipu-
lation, and a risk of recurrence or residual

410 www.SCIENCEDIRECT.com WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2019.08.037


MOHAMMAD GHORBANI ET AL.
CCF in cases of incomplete occlusion of
the orifice.13,15,17,18
Endovascular coiling with or without
liquid agents has a higher rate of suc-
cessful fistula occlusion compared with
use of a detached balloon, owing to the
ease of access and availability of a wide
range of sizes of the embolic device.10
Nevertheless, there are some
disadvantages associated with both
transarterial and transvenous approaches,
including the possibility of liquid agent
reflux into the ICA and possible
occlusion of the ICA and ischemic
sequelae; protrusion of the coil into the
ICA, especially in large fistulas;
subarachnoid hemorrhage by lesions in
the petrosal sinuses or cortical vein from
catheter manipulation; hemodynamic
alteration from a change in venous
drainage pattern from the use of embolic
agents; and transient oculomotor
paralysis owing to a compressive effect
caused by the carotid sinus.11,19,20
Covered stents are simple and rapidly
inserted positioning devices that can offset
the mass effect and prevent coil herniation
and pseudoaneurysm formation, resulting
in a higher rate of fistula occlusion and
reduced risk of recanalization.21,22 How-
ever, despite these advantages, the use of
covered stents is associated with a risk of
late stent thrombosis, which can be
minimized by correct anticoagulant/anti-
platelet therapy but can restrict flow
diverter use in acute trauma.23 Other
disadvantages include the risk of cerebral
hemorrhage due to loss of self-regulation
with increased local flow associated with
edema in adjacent structures and less
flexibility, hindering placement in intra-
cranial vessels, which may be complicated
by periprocedural vasospasm.24,25
With the recent introduction of flow
diverters, treatment of CCF by a trans-
arterial approach offers new advantages
by preserving and remodeling the ICA,
as well as simplifying and reducing the
time and costs of CCF treatment.
Although the use of flow diverters alone
might not be effective in high-flow CCF,
placement of flow diverters in the ICA as
an adjunct to coil and balloon use can
minimize the risk of treatment failure
and subsequent complications by endo-
luminal reconstruction.26,27 However,
some disadvantages, including device
cost, need for dual antiplatelets, and
WORLD NEUROSURGERY 133: 409-412, JANUARY 2020
ORBITAL COMPARTMENT SYNDROME DUE TO RUPTURED CAROTID CAVERNOUS ANEURYSM
difficulty of microcatheter passage into
the ICA distal to the CCF due to signif-
icant injury to the vessel in some CCFs,
have limited the use of flow diverters.26
In our present case, high-flow CCF after
CCA rupture caused an acute increase in
IOP and the development of OCS via 2
mechanisms. The first mechanism is the
acute elevation of orbital venous pressure
through anterograde drainage of superior
and inferior veins, along with congestion
of orbital structures that compromise
orbital perfusion. This scenario is
responsible for hypoperfusion of the cen-
tral retinal artery and ciliary arteries,
causing anterior ischemic optic neuropa-
thy and extraocular muscle ischemia.28,29
The second is increased orbital pressure
due to retrobulbar hemorrhage, which
may directly damage the orbital compo-
nent of the optic nerve by compression or
by stretching and affecting the vasa
nervorum.30,31
In our case, despite prompt canthotomy
after suspicion of OCS, emergent imaging
was indicated owing to the failure of initial
decompression to decrease IOP.31,32
Computed tomography scan and mag-
netic resonance imaging followed by
digital subtraction angiography confirmed
the diagnosis of ruptured CCA as the eti-
ology of OCS. Unfortunately, despite
prompt endovascular coil embolization,
permanent vision loss occurred due to
optic nerve damage.
CONCLUSIONS
Given the critical importance of early
diagnosis and treatment of OCS to prevent
irreversible damage to orbital structures
and visual loss, we suggest that in cases of
suspected OCS in the absence of well-
known etiologies of OCS, undiagnosed
ruptured CCA should be considered as a
potential cause. In such cases, emergent
imaging plus early endovascular
intervention can resolve OCS and prevent
permanent ocular injury and vision loss.
REFERENCES
1. Stiebel-Kalish H, Kalish Y, Bar-On RH, et al.
Presentation, natural history, and management of
carotid cavernous aneurysms. Neurosurgery. 2005;
57:850-857 [discussion: 850-857].
2. Guan N, Mu S, Wang L, et al. Endovascular
treatment of 147 cases of cavernous carotid
www.journals.elsevier.com/world-neurosurgery
CASE REPORT
aneurysms: a single-center experience. J Stroke
Cerebrovasc Dis. 2016;25:1929-1935.
3. Hodes JE, Fletcher WA, Goodman DF, Hoyt WF.
Rupture of cavernous carotid artery aneurysm
causing subdural hematoma and death.
J Neurosurg. 1988;69:617-619.
4. van Rooij WJ, Sluzewski M, Beute GN. Ruptured
cavernous sinus aneurysms causing carotid
cavernous fistula: incidence, clinical presentation,
treatment, and outcome. AJNR Am J Neuroradiol.
2006;27:185-189.
5. Lee AG, Mawad ME, Baskin DS. Fatal subarach-
noid hemorrhage from the rupture of a totally
intracavernous carotid artery aneurysm: case
report. Neurosurgery. 1996;38:596-598 [discussion:
598-599].
6. Arai N, Nakamura A, Tabuse M, Miyazaki H. Late-
onset massive epistaxis due to a ruptured trau-
matic internal carotid artery aneurysm: a case
report. NMC Case Rep J. 2016;4:33-36.
7. Ghorbani M, Asaadi S, Wipplinger C,
Griessenauer CJ, Zangi-Abadi F, Mortazavi A.
Dural arteriovenous fistulas with venous drainage
patterns inducing ocular manifestations
mimicking a carotid cavernous fistula: report of 2
cases. World Neurosurg. 2019;127:216-219.
8. Ambekar S, Madhugiri V, Sharma M, Cuellar H,
Nanda A. Evolution of management strategies for
cavernous carotid aneurysms: a review. World
Neurosurg. 2014;82:1077-1085.
9. Wiebers DO, Whisnant JP, Huston J 3rd, et al.
Unruptured intracranial aneurysms: natural his-
tory, clinical outcome, and risks of surgical and
endovascular treatment. Lancet. 2003;362:103-110.
10. Dahn I, Lassen NA, Westling H. Blood flow in
human muscles during external pressure or
venous stasis. Clin Sci. 1967;32:467-473.
11. Ashton H. The effect of increased tissue pressure
on blood flow. Clin Orthop Relat Res. 1975:15-26.
12. Dalley RW, Robertson WD, Rootman J. Globe
tenting: a sign of increased orbital tension. AJNR
Am J Neuroradiol. 1989;10:181-186.
13. Lima V, Burt B, Leibovitch I, Prabhakaran V,
Goldberg RA, Selva D. Orbital compartment syn-
drome: the ophthalmic surgical emergency. Surv
Ophthalmol. 2009;54:441-449.
14. Cheung CA, Rogers-Martel M, Golas L,
Chepurny A, Martel JB, Martel JR. Hospital-based
ocular emergencies: epidemiology, treatment, and
visual outcomes. Am J Emerg Med. 2014;32:221-224.
15. Korkmazer B, Kocak B, Tureci E, Islak C, Kocer N,
Kizilkilic O. Endovascular treatment of carotid
cavernous sinus fistula: a systematic review. World
J Radiol. 2013;5:143-155.
16. de Aguiar GB, Jory M, Silva JM, Conti ML,
Veiga JC. Advances in the endovascular treatment
of direct carotid-cavernous fistulas. Rev Assoc Med
Bras (1992). 2016;62:78-84.
17. Ellis JA, Goldstein H, Connolly ES Jr, Meyers PM.
Carotid cavernous fistulas. Neurosurg Focus. 2012;
32:E9.
411

MOHAMMAD GHORBANI ET AL.
18. Eddleman CS, Hurley MC, Bendok BR, Batjer HH.
Cavernous carotid aneurysms: to treat or not to
treat? Neurosurg Focus. 2009;26:E4.
19. Taha MM, Nakahara I, Higashi T, et al. Endo-
vascular embolization vs surgical clipping in
treatment of cerebral aneurysms: morbidity and
mortality with short-term outcome. Surg Neurol.
2006;66:277-284 [discussion: 284].
20. Gemmete JJ, Ansari SA, Gandhi DM. Endovas-
cular techniques for treatment of carotid-
cavernous fistula. J Neuroophthalmol. 2009;29:62-71.
21. Malan J, Lefeuvre D, Mngomezulu V, Taylor A.
Angioarchitecture and treatment modalities in
posttraumatic carotid cavernous fistulae. Interv
Neuroradiol. 2012;18:178-186.
22. Tjoumakaris SI, Jabbour PM, Rosenwasser RH.
Neuroendovascular management of carotid
cavernous fistulae. Neurosurg Clin N Am. 2009;20:
447-452.
23. Morón FE, Klucznik RP, Mawad ME, Strother CM.
Endovascular treatment of high-flow carotid
cavernous fistulas by stent-assisted coil place-
ment. AJNR Am J Neuroradiol. 2005;26:1399-1404.
24. Mercado GB, Irie K, Negoro M, et al. Transvenous
embolization in spontaneous direct carotid-
cavernous fistula in childhood. Asian J Neurosurg.
2011;6:45-48.
412 www.SCIENCEDIRECT.com
ORBITAL COMPARTMENT SYNDROME DUE TO RUPTURED CAROTID CAVERNOUS ANEURYSM
25. Ringer AJ, Salud L, Tomsick TA. Carotid
cavernous fistulas: anatomy, classification, and
treatment. Neurosurg Clin N Am. 2005;16:279-295,
viii.
26. Wang YL, Ma J, Ding PX, Li YD, Han XW, Wu G.
Treatment of post-traumatic carotid-cavernous
fistulas with the Willis covered stent. A pre-
liminary prospective study. Interv Neuroradiol. 2012;
18:172-177.
27. Yin B, Sheng HS, Wei RL, Lin J, Zhou H,
Zhang N. Comparison of covered stents with
detachable balloons for treatment of post-
traumatic carotid-cavernous fistulas. J Clin Neurosci.
2013;20:367-372.
28. Kalyanpur TM, Narsinghpura K, Yadav M,
Mehta P, Paul K, Cherian M. Covered coronary
stent grafts as a treatment option for carotid-
cavernous fistulas: our initial experience. Neurol
India. 2011;59:895-898.
29. Gomez F, Escobar W, Gomez AM, Gomez JF,
Anaya CA. Treatment of carotid cavernous fistulas
using covered stents: midterm results in seven
patients. AJNR Am J Neuroradiol. 2007;28:1762-1768.
30. Cho KC, Seo DH, Choe IS, Park SC. Cerebral
hemorrhage after endovascular treatment of
bilateral traumatic carotid cavernous fistulae with
WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2019.08.037
CASE REPORT
covered stents. J Korean Neurosurg Soc. 2011;50:
126-129.
31. Ogilvy CS, Motiei-Langroudi R, Ghorbani M,
Griessenauer CJ, Alturki AY, Thomas AJ. Flow
diverters as useful adjunct to traditional endovas-
cular techniques in treatment of direct carotid-
cavernous fistulas. World Neurosurg. 2017;105:
812-817.
32. Wendl CM, Henkes H, Martinez Moreno R,
Ganslandt O, Bäzner H, Aguilar Pérez M. Direct
carotid cavernous sinus fistulae: vessel recon-
struction using flow-diverting implants. Clin Neu-
roradiol. 2017;27:493-501.
Conflict of interest statement: The authors declare that the
article content was composed in the absence of any
commercial or financial relationships that could be construed
as a potential conflict of interest.
Received 9 June 2019; accepted 6 August 2019
Citation: World Neurosurg. (2020) 133:409-412.
https://doi.org/10.1016/j.wneu.2019.08.037
Journal homepage: www.journals.elsevier.com/world-
neurosurgery
Available online: www.sciencedirect.com
1878-8750/$ - see front matter ª 2019 Elsevier Inc. All
rights reserved.