Traumatic Globe Luxation

From EyeWiki


Disease Entity

Globe luxation is a rare condition  that can present spontaneously or following trauma, where there is complete prolapse of the globe from the orbit. Varying degrees of globe prolapse may be encountered in clinical practice with varying clinical/functional and structural outcomes.[1][2][3]

Classification

Several classifications may be employed in relation to globe luxation.

It may be classified as partial or incomplete (prolapse of the globe alone with a structurally intact optic nerve and extraocular muscles) or Complete (prolapse along with severed optic nerve and extraocular muscles).[1][2][4]

Alternatively it may be classified in to luxation alone (avulsion incomplete), dislocation( globe prolapse into the paranasal sinuses or intracranial cavity) or complete avulsion.[5][6][7]

Etiology

Based on the etiology globe luxation can be classified as spontaneous, voluntary and post-traumatic. Spontaneous luxation may occur without conscious effort, with or without predisposition factors. Voluntary globe luxation is the ability to protrude the globe from the patient, most often by simple conscious eyelid manipulation and   traumatic luxation occurs following a trauma. Trauma seems to be the most common etiology for the luxation of the globe, the spectrum of which include road traffic accidents, surfboard injury and from other forms of extreme sports, human and animal bites, domestic blunt or penetrating injury. Road traffic accidents appear to be the common cause.[4][8][9][10][11]

There are also 56 reported cases (71 globes) of self enucleation/auto enucleation (Oedipism Rex)[12] since 1933-2015 with a significant proportion either from underlying mental illness (mostly schizophrenia) or those under influence of recreational drugs.[13][14][15][16]

Gouging was a brutal fight in which a combatant was successful if he would press the adversary’s eyeball out with his thumb by sudden severe torsion on the globe. This was a common cause of globe luxation many years ago. Surprisingly no serious or permanent damage was reported to occur in these eyes.[17]  It was also reported in obstructed labour as a problem due to faulty application of forceps during delivery. [18]

In general, globe luxation can occur spontaneously, following trauma, or can be associated with other systemic diseases like thyroid eye disease, shallow orbits (Crouzon syndrome), chronic obstructive pulmonary diseases, floppy eye lid syndrome, high orbital fat contents, or lax extraocular muscles.[19][20][21] Mechanical maneuvers like Valsava, eyelid manipulation, general anesthesia, contact lens insertion and removal and trauma have also has been reported as the cause of globe luxation.[20][22]

Figure 1. Globe luxation in Crouzon syndrome. Note the shallow orbit on the CT-scan

Epidemiology

Traumatic luxation of the globe is a rare condition with only 106 cases reported until 2018.9 Amaral et al reported that its more common in males 4.7:1 with a mean age of 29.5 years (5 - 74 years).[23][24] Traumatic globe luxation into the paranasal sinuses is even more rare and until 2016 there were only 24 cases reported with male female ratio of 6:1, 42% of which were caused by traffic accidents. The maxillary sinus is the most common involved (87.5%) following with ethmoidal sinus (12.5%). [24][25]

Figure 2. CT-scan of the globe luxation: (left) coronal view, into the maxillary sinus; and (right) sagittal view

Pathophysiology

Figure 3. Globe luxation from fall on an iron fence.[27]
Isolated anterior luxation of the globe alone, without optic nerve or extraocular muscle avulsion, occurs when a blunt object is insinuated in the superomedial orbit, between the globe and the superomedial orbit. This not only induces a globe prolapse but is also followed by a secondary severe reflex spasm of the orbicularis oculi locking the globe in place. While the eyelid normally play an important preventive role in globe prolapse, blunt or sharp objects encountered in daily life such as bike handles, gift wrapping tubes, door handles, fences, etc may induce a globe prolapse from secondary raised intraorbital pressure whilst pushing the upper eyelid backwards.[3][26][27][28][29][30]

A second mechanism is the coup-contre coup injury, commonly associated with craniofacial trauma. 

A third mechanism is direct result of sudden reduction of the orbital cavity volume following blow-in fractures, typically of the orbital roof.[3][31] Paradoxically, large displaced orbital wall blow out fractures may precipitate a globe prolapse into one of the paranasal sinuses – most commonly the maxillary sinus and less commonly the ethmoidal sinus and even more rarely the intracranial cavity.[3][25][32][33][34]

An avulsion of the optic nerve and/or extraocular muscle(s) following the globe luxation may be from sharp objects, or a bony fragment that may transect that the optic nerve, aggravated  by extreme forward displacement of the globe. The latter movement may cause a complete disruption of optic nerve fibers at the lamina cribrosa, which lack a myelin sheath. Extraocular muscle involvement is also common following severe maxillofacial trauma e.g Le Fort II or III fractures, with almost 90% of midfacial fractures associated with ocular trauma presenting with significant proptosis or some degree of globe luxation.[4][35]

Morris et al had proposed three hypotheses for globe luxation. The first is an elongated object entering the medial orbit may act as a fulcrum propelling the globe forward. The second is a wedge-shaped object entering the orbit medially and displacing the globe anteriorly. The third is a direct transection of the optic nerve by a penetrating sharp object.[36]
Figure 4. Globe luxation with superior orbital rim fracture29

Clinical Features

Figure 5. Complete globe avulsion36

Presenting clinical symptoms may vary from a completely asymptomatic patient (underlying psychiatric conditions – ‘la belle indifference’) to one with severe pain and visual loss. Acute presentation include a restless patient with profuse bleeding, periorbital ecchymosis, apparent proptosis with immobile globe with secondary blepharospasm. Rarely, the cut end of the optic nerve may also be visualized.[37]

Apart from visual loss persistent globe luxation can also lead to other complications such as corneal abrasion, exposure keratopathy, secondary blepharospasm and pthisis bulbi in late stages from ocular ischemia and/or corneal perforation. Life threatening complications such as meningitis, intracranial hemorrhage and cerebrospinal fluid leakage have also been reported.[4] In auto-enucleation cases, the injury and complications may be more severe and even life threatening. These include visual field defects not only in the ipsilateral eye but also contralateral eye from chiasmal damage causing temporal field defect and also neurovascular sequale such as subarachnoid hemorrhage.[16][37][38][39]

Loss of extraocular muscle(s)’ is reported when one or more muscles are avulsed or transected. The most common muscle involved is the medial rectus followed by the inferior rectus, superior rectus, lateral rectus and oblique muscles respectively in that order.[4][35][40]

In typical globe luxation the globe is visualized, outside the orbit, in rare situations, the patient may present with an ‘empty socket’. In such situations, the globe may be prolapsed into one of the paranasal sinuses (maxillary sinus followed by the ethmoid sinus).[1][34][36][41] Displacement of the globe into paranasal sinus can be easily explained by large displaced blow out fractures. Careful examination should be performed when the trauma involves the ethmoid sinus due to the proximity of the skull base with potential CSF leak (‘liquor fistula’).[42]

Even more rarely, globe luxation into the into the anterior cranial fossa may occur  following by extensive and displaced frontal fracture of orbital roof, with a combination of high intraorbital pressure and dehiscence of the orbital roof as causative factors.[7][43][44]

Management

Management of the globe luxation is an ophthalmic and medical emergency,  as most cases end up with poor visual prognosis despite best and early management. There are two established maneuvers that have been described to repositioned the luxated eye ball. Both require some form of cooperation by the patient and if possible with some sedation and/or anesthesia with an orbicularis or facial nerve block. The first maneuver is performed while asking the patient to look down. The upper eyelid is gently and firmly pulled upward while the globe is simultaneously depressed with the index finger of the other hand to repositioning the globe. The second method is using Desmares retractor which is introduced between the upper lid and the globe. Once the tip is under the eyelid, digital manipulation is performed to depress and reposition the globe into the orbit.[45][46]
Figure 6. Illustration the technique of manually reposition the globe luxation. (a) Ask the patient to look downward while we pull the upper eyelid upward and push with index finger of the other hand down and backward as shown with arrow. (b) Same procedure but using desmares to help pull the upper eyelid.
In cases where it associated with optic nerve or extraocular muscle damage, appropriate restoration must be performed as early as possible to restore full anatomical restoration and hopefully functional recovery of the globe. Secondary procedures that may be considered include performing a tarsohaphy, upper eyelid retraction repair, orbital fracture repair, and orbital decompression in severe cases.[4][31] High dose intravenous corticosteroid and neuroprotective agent may also be considered to reduce posttraumatic inflammation and edema. Panje et all recommended 1 mg dexamethasone/kg body weight initially continue with 0,5 mg/kg every six hours for next 24 hour, and 1 mg/kg per day for one or two days for the optic nerve injury.  After successful globe reduction, the patient should have immediate relief of pain and return of vision and extraocular muscle function if there is  no optic nerve and extraocular muscle damage. Such functional recovery has been reported up to a week post luxation.[20][29][46][47][48]

Management for globe luxation into the paranasal sinus is similar to blow out fracture repairs but much more delicately performed with least induced iatrogenic trauma and undue delay. 

There are two techniques to reposition the globe luxated into the paranasal sinuses. The first is direct traction on the globe with instrumental help. The  second is manual repositioning of the globe via trans maxillary  or trans nasal approach.[5][25][32][49] In these cases, close collaboration with a craniomaxillofacial surgery team is essential for good exposure with least trauma t to the vital orbital structures including  the globe and the optic nerve.[50] Orbital reconstruction may then be performed either with autologous bone or more commonly one of the alloplastic implants - titanium plates,  porous poplyethylene or bioresorbable implants.[25][32][49]
Figure 7. (a) Patient pre op; (b) Patient post op with tarsoraphy 3 weeks, (c,d) Patient after 4 weeks, note the full eye movement and achievement of 20/20 visual acuity27 
Table 1. Step by step management for globe luxation
Timeline Approach Note
At Emergency Department Global assessment, note emergency situation

a. life threatening

b. vision threatening

Ophthalmic assessment

CT-scan

Decide if the reposition of the globe can be done directly, if there is no optic nor extraocular muscle avulsion

Head injury

Retrobulbar haemorrhage, globe luxation

Optic nerve avulsion

Extraocular muscles avulsion

Orbital fracture

Give analgesic (iv, per oral, topical)

Try to reposition the globe using manual eye repositioning

Intraoperative Explore and reattach the extraocular muscle, tarsoraphy, upper eyelid retraction repair, orbital fracture repair, and orbital decompression in severe cases High dose intravenous corticosteroid and neuroprotective agents may also be considered to reduce post-traumatic inflammation and edema
Figure 8. Globe luxation in orbital fracture; (a) presentation of the globe luxation; (b) note the superior orbital rim fracture; (c) fracture reconstruction with globe reposition; and (d) one week post op.29 

In patients who present with autoenucleation, management should include psychiatric consultation, suicide precautions and securing family support. Rapid parenteral tranquilization is often needed as a prophylaxis against suicide and further self mutilation.[14][15][51]

The sequence of management thus is initial globe repositioning, followed by exploration and repair of avulsed extraocular muscles when present preferably as soon as possible. It should be remembered the longer the globe and orbital structures are prolapsed, the poorer the structural and thus functional and even esthetic prognosis owing to ocular ischemia, exposure keratopathy, extraocular muscle retraction within the orbit. Preservation of the globe even in patients with  visual loss is important in the view of aesthetic and psychological considerations of the patient and family.[1][3][52][53]

In exceptional cases of either several and completely avulsed globe along with optic nerve and extraocular muscles or in patients presenting late with severe exposure , corneal perforation or phthisis, enucleation may be performed  (5,9% ).[35][54] Enucleation therefore is the last option in managing this condition, and only should be done when the integrity of the globe can’t be repositioned and reinserted after all possible efforts, and also in such case of complete optic nerve and extra ocular muscle avulsion.[52][53] In such patients the socket should be rehabilitated either as a delayed primary procedure or secondarily with an orbital implant and customized prosthesis to restore normal appearance thus aiding psychological rehabilitation.

Summary

The globe luxation is one of the emergency case in opthalmology as it threatening the vision of the patient. Early diagnosis and proper management by repositioning the globe and try to reattached all the extra ocular muscle will lead to possibility of regaining visual recovery, better cosmetic, physiological result, and improves psychological status of the patient. 

Submitted in Recognition of and on behalf of the Asia Pacific Ophthalmic Trauma Society (APOTS)

References

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