Key-words: Dissection - internal carotid artery - occlusion - self-enucleation - subarachnoid
hemorrhage
Introduction
Self-enucleation, also known as auto-enucleation or oedipism, is an uncommon type
of self-mutilation that is classified as a major self-injury.[[1 ]],[[2 ]] It is more common in men than in women[[3 ]],[[4 ]] and is overwhelmingly associated with acute psychosis due to untreated psychiatric
illnesses or drug use.[[1 ]],[[4 ]],[[5 ]],[[6 ]] Self-enucleation involves avulsion of the extraocular muscles, optic nerve, and
ophthalmic artery (OA), which may lead to devastating neurovascular complications.
These include contralateral visual field deficits due to optic chiasm injury, subarachnoid
hemorrhage (SAH), cerebrospinal fluid leak, infection, and internal carotid artery
(ICA) dissection.[[4 ]]
Only 10 cases of traumatic SAH due to OA avulsion have been reported in the literature,[[4 ]],[[7 ]],[[8 ]],[[9 ]] five of which were reported to be associated with diffuse hemorrhage in the basal
cisterns, mimicking the pattern seen in aneurysmal SAH. Given the risk of cerebral
vasospasm following SAH, which may lead to ischemic infarcts, these patients require
careful monitoring in an intensive care unit.[[5 ]] To the best of our knowledge, only one case of ICA dissection after self-enucleation
has previously been reported in the literature.[[4 ]] We present a unique case of self-enucleation with both SAH as well as ICA dissection
and occlusion.
Case Report
The patient is a 53-year-old male with a history of bipolar disorder, schizophrenia,
and multiple suicidal attempts who electively stopped his psychiatric medications
at 1 month prior to presentation. During an acute psychotic episode, the patient self-enucleated
his right eye. He arrived unconscious at the emergency department (ED), and he was
intubated. The empty right orbit was washed out and packed with iodoform gauze in
the ED, and the patient was then transferred to our facility. Neurological examination
revealed a Glasgow Coma Score (GCS) of 6, withdrawal in all extremities except left
upper extremity, and positive left corneal, cough, and gag reflexes. Computed tomography
(CT) images showed extensive SAH (Fisher grade 4), as well as intraventricular hemorrhage
and acute hydrocephalus [[Figure 1 ]]. CT angiography and digital subtraction angiography (DSA) revealed occlusion of
the right ICA [[Figure 2 ]]. The gauze in the right orbit was gently removed with irrigation and no bleeding
was encountered. A plastic conformer was placed inside the eyelids to maintain the
socket space and allow the socket to granulate in, and antibiotic eye ointment was
applied to prevent infection.
Figure 1: (a) Axial computed tomography head image showing acute diffuse subarachnoid hemorrhage
in the frontotemporoparietal and basilar cisterns. (b) Axial computed tomography head
images more superiorly showing intraventricular hemorrhage in the 3
Figure 2: (a) Coronal computed tomography angiography image demonstrating occlusion of the
right internal carotid artery just distal to the bifurcation of the right common carotid
artery. (b) Coronal three-dimensional reconstruction of the computed tomography angiography
image in Figure 2a. (c) Lateral digital subtraction angiography with injection into
right common carotid artery demonstrating occlusion of the right internal carotid
artery near the bifurcation of the right carotid bifurcation
Per discussion with the family, the patient was initially managed conservatively in
the neurointensive care unit. His GCS, however, improved, and he was extubated on
posttrauma day (PTD) 2, with a GCS of 13. Mental status then declined overnight. An
external ventricular drain (EVD) was placed, revealing an elevated opening pressure
of 30 cmH2O, and resulted in immediate improvement. Neurological examination showed
a reactive left pupil and full motor strength in all extremities, except for weakness
in the left distal lower extremity that persisted throughout the hospital stay. Magnetic
resonance imaging (MRI) demonstrated acute ischemia in the right distal anterior cerebral
artery (ACA) and middle cerebral artery (MCA) territories [[Figure 3 ]]. Repeat cerebral angiogram confirmed complete occlusion of the right cervical ICA
(C1–C7 segments) with robust cross-filling of the right MCA from the anterior communicating
and posterior communicating arteries [[Figure 4 ]]. Daily transcranial Doppler (TCD) readings showed elevated flow velocities in bilateral
ACAs and MCAs. The patient failed multiple EVD clamping trials and had persistent
hydrocephalus, necessitating placement of a ventriculoperitoneal shunt. Head CT on
PTD 28 showed a hypodensity within the right supplementary motor area [[Figure 5 ]], consistent with subacute ischemia seen on prior MRI studies and likely the cause
of persistent left lower extremity weakness. During a psychiatric evaluation, the
patient attributed his self-enucleation act to command auditory hallucinations and
was transferred to an inpatient psychiatric unit for continuing care. At discharge,
the patient was alert but confused with a GCS of 14. Motor strength was 4+/5 in the
right hemibody, 4/5 in the left upper extremity, and 2/5 proximally, and 0/5 distally
in the left lower extremity. The patient was given instructions to follow-up for orbital
reconstruction with dermis fat graft.
Figure 3: Axial diffusion-weighted imaging image showing a hyperdensity in the right supplementary
motor area, indicating an ischemic infarct
Figure 4: Coronal digital subtraction angiography image with injection into the left internal
carotid artery demonstrating filling of the right anterior cerebral and middle cerebral
arteries through the anterior communicating artery
Figure 5: Repeat axial computed tomography head image on posttrauma day 28 showing a hypodensity
in the right supplementary motor area, consistent with a subacute infarct
Discussion
We present a unique case of self-enucleation, leading to both SAH and ICA dissection/thrombosis
and subsequent coma (GCS 6), followed by a dramatic initial recovery with conservative
management only. We also conducted a comprehensive review of the English-language
literature for previous cases of self-enucleation that resulted in intracranial complications
[[Table 1 ]].
Table 1: Summary of literature reporting cases of self-enucleation associated with intracranial
complications
We hypothesize that the ICA dissection associated with self-enucleation is a consequence
of the traumatic OA avulsion at its take-off from the ICA.[[8 ]],[[9 ]] It leads to hemorrhage in the basilar cisterns mimicking a pattern seen in aneurysmal
rupture. Subsequent ICA occlusion results from disruption of the arterial wall, facilitating
an acute thrombotic event. Gauger et al. postulated that the dissection may result
from direct force transmission to the ICA during enucleation.[[4 ]] ICA dissection with subsequent occlusion may constitute a life-threatening event
in patients without adequate collateral blood flow.[[22 ]],[[23 ]]
Our patient suffered from an ischemic infarct in the right supplementary motor area,
which was the likely cause of the left lower extremity weakness. Ischemic infarcts
presenting within 2 weeks following SAH are concerning for vasospasm, as in Kotlus
and Lo case,[[5 ]] which involved bilateral vasospastic strokes following self-enucleation. To assess
for vasospasm in our patient, TCD measurements were performed daily and showed persistently
elevated flow velocities in multiple arteries, but a subsequent DSA showed no evidence
of vasospasm. While TCD is a convenient noninvasive test to screen for cerebral vasospasm,[[24 ]],[[25 ]] DSA remains the gold standard and has a higher diagnostic accuracy.[[26 ]] We believe that our patient's infarct was likely not due to vasospasm but rather
due to an embolic event precipitated by ICA dissection and occlusion.[[27 ]]
A complication commonly reported in previous cases of self-nucleation but not seen
in our patient is contralateral temporal field deficit (CTFC).[[6 ]],[[12 ]],[[15 ]],[[21 ]],[[28 ]] CTFC indicates chiasmal injury from avulsion of the optic nerve at its intracranial
segment or more than 4 cm proximal to the optic globe and may improve with steroids.[[28 ]],[[29 ]]
Conclusion
Self-enucleation is a dramatic and unfortunate complication of acute psychosis that
presents as a neurologic, ophthalmologic, and psychiatric emergency. We reported a
unique case of self-enucleation resulting in both SAH and ICA dissection and occlusion
that improved initially with conservative management.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms.
In the form the patients have given their consent for their images and other clinical
information to be reported in the journal. The patients understand that their names
and initials will not be published and due efforts will be made to conceal their identity,
but anonymity cannot be guaranteed.
