British Journal ofOphthalmology, 1992,76,349-352

Management of traumatic optic neuropathy - a studyof 23 patients

Joseph A Mauriello, Joseph DeLuca, Abbott Krieger, Michael Schulder, Larry Frohman

AbstractTwenty three patients with traumatic opticneuropathy were managed by medical andsurgical treatment as follows. High dose intra-venous steroids were initiated in all patients. Ifvisions did not improve significantly after 24 to48 hours decompression of an optic nervesheath haematoma by medial orbitotomy andneurosurgical decompression of the opticcanal were considered based on computedtomographic scan findings. Nine of 16 patientswho received steroids only showed significantimprovement. One of three showed improve-ment on optic nerve decompression aftersteroid failure; three or four showed improve-ment with combined optic nerve sheathdecompression by the medial orbitotomy anddecompression of the optic canal by frontalcraniotomy. A lucid interval of vision afterinjury and an enlarged optic nerve sheath wereassociated with an improved prognosis. Five ofthe 23 patients had a lucid interval and all fivehad a final improved vision, while only five of18 patients without a lucid interval improved.Similarly seven of the nine with an enlargedoptic nerve sheath showed improvement whileonly three of 10 patients (three bilateral cases)who presented with no light perceptionimproved with medical and surgical treatment.While a prospective controlled study of themanagement of traumatic optic neuropathy isnecessary this preliminary study suggests thattreatment of traumatic optic nerve sheathhaematoma by optic nerve sheathdecompression should be considered inselected patients.

Although the natural history of traumatic optic

Department ofOphthalmology,University of Medicineand Dentistry ofNewJersey, New JerseyMedical School, EyeInstitute of New Jersey,Newark, New Jersey07107, USAJ A MaurielloJ DeLucaA KriegerM SchulderL FrohmanCorrespondence to:J A Mauriello, MD.Accepted for publication24 October 1991

neuropathy is unknown recent studies suggestthat high dose steroids, and even surgicaldecompression of the optic canal, may restorevision in selected patients. 1-7 While visualrecovery after decompression of an enlargedoptic nerve sheath due to traumatic haemorrhagehas been reported this treatment has receivedlittle attention in the literature.'-" The results of23 patients who received high dose steroids andunderwent decompression of the optic nervesheath and the optic canal are presented.

Patients and methodsAll patients in this series had loss of vision aftertrauma wth an afferent pupillary defect in theinvolved eye and were treated within the first 48hours of injury. According to the protocol atUMD-NJ Medical School, a regional traumacentre., all patients received a complete ophthal-mic examination by one of us. Patients withpenetrating ocular injuries or optic nerve avulsionwere excluded from the study. All patientsreceived an initial 1 g loading dose of SoluMedrol (methylprednisolone) and then 250 mgintravenously every 6 hours for 72 hours aftermedical clearance by the trauma team. Patientswith increased intraorbital pressure due tohaemorrhage were treated by emergency lateralcanthotomy with drainage of subperiosteal orintraorbital haemorrhage. High resolution brainand orbital computed tomography (CT) scanswere obtained while treatment was instituted.Thin 1-5 cm sections through the intraorbitaland intracanalicular optic nerve were obtained todefine 'surgically treatable pathology'; enlargedoptic nerve sheath with presumed intrasheathhaemorrhage; or narrowing of the optic canal ororbital apex by bone spicules. If vision improved

m * a-,Figure 1 (Case 1jEnlarged optic nerve sheath (arrow) shown of axial (left) and coronal (right) C7 scan. Note 'ring' ofincreased soft tissue density corresponding to blood in the subarachnoid space that surrounds the relatively lucent optic nerve.Patient had return ofvision to 20125 with intravenous steroids and no surgery.

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Figure 2 (Case 23) Lateralwall impinges on orbitalapex (curved arrow). Theretrobulbar portion ofthenerve appears thickened(straight arrows). Patientunderwent elevation ofthelateral wall of the orbit andcraniotomy withdecompression ofthe root ofthe optic canal. The opticnerve was markedlyshrunken at the level ofthecanal; the sheath contained apaste-like substance. Thepatient had no return ofvision.

Figure 3 (Case 19) AxialCT scan shows enlargednerve (arrows) due to sheathhaematoma behind globe.

over the first 24 to 72 hours steroids werecontinued for a total of 5 to 7 days and were thentapered. If vision did not improve after 24 to 72hours of steroids decompression of an opticnerve sheath by medial orbitotomy anddecompression of the optic canal with removal ofapical orbital bone fragments by frontal cranio-tomy were considered ifon CT scan: (1) the opticnerve sheath appeared enlarged by presumedintrasheath haemorrhage; or (2) the optic canalor optic nerve at the orbital apex appearedcompressed by bone spicules (Figs 1-3). If visiondid not improve and no 'surgically treatablepathology' was present on CT scan steroids weregradually tapered after 3-4 days. Patients withmedical problems and, early in the study, thosewith unilateral involvement were not alwaysconsidered surgical candidates.

SURGICAL TECHNIQUEIn all patients the decompression of the opticnerve sheath was performed by a conjunctivalapproach under general anaesthesia similar tothat described for pseudotumour cerebri.'213 A270 degree conjunctival peritomy was performed.The superior and inferior rectus muscles wereisolated on 4-0 silk traction sutures. The medialrectus muscle at its insertion was sutured with adouble armed 5-0 vicryl suture that was locked

at each edge of the muscle; the muscle was thencut at its insertion and detached from the globe asif a standard muscle recession were to be per-formed. The two free arms of the vicryl suturewere taped without tension with a 1/4 inch steri-strip to the patient's brow and cheek on theopposite side. Care was taken to avoid tractionand damage to the medial rectus muscle.Multiple running bites were taken through thestump of the muscle with a 4-0 silk tractionsuture. A Schepens retractor or thin ribbonretractor was placed in the medial orbit betweenthe globe and the medial rectus muscle.Cottonoids, cotton tip applicators, and suctionfacilitated exposure of the optic nerve sheaththrough the operating microscope. A no 11 bladewas used to make three 2-3 mm longitudinalincisions into the nerve sheath away from super-ficial blood vessels. The medial rectus musclewas resutured into position after the tractionsutures were removed and the conjunctivalperitomy closed.The neurosurgical procedure was then

performed through a coronal flap by the neuro-surgeons (AK, MS). The optic canal wasunroofed and any bone spicules that impinged onthe nerve were removed. All patients continuedto receive steroids for 72 hours after surgery. Forthose with improvement in vision after surgerythe steroids were continued for a total of 5 to 7days and then tapered.

Results

SURGICAL FINDINGSAt surgery a swollen haemorrhagic nerve sheathwas identified in three of the seven patients and aclear yellowish fluid was recovered after theincision into the nerve sheath. In patient no 17,no blood or fluid was recovered at the time of theoptic nerve sheath decompression yet the visionreturned to 20/25.

Case no 23 presented with NLP vision; theentire lateral wall of the orbit was displacedposteromedially against the optic nerve (Fig 2).At the time of neurosurgical decompression ofthe optic nerve at the optic canal the nerve sheathwas filled with a whitish pink paste.

VISUAL RECOVERYNine of 16 patients showed significantimprovement on steroids alone; seven (case nos1-6, 10) of the nine who showed improvementhad an enlarged optic nerve sheath (Table 1).Case nos 12 and 16 had no significant pathologyon CT scan. Three of the nine patients requiredimmediate bedside canthotomy prior to treat-ment with steroids for increased intraorbitalpressure. Of the seven patients (case nos 7, 8, 9,11, 13-15) who had no improvement on steroidssix presented with no light perception (NLP)and one with LP (case no 9) in the involved eye.Three patients (case nos 7-9) with optic nervesheath haematoma and two patients (case nos 13,15) with optic canal fractures did not havesurgery for a variety of reasons including medicalcontraindications.One of three patients who did not respond to

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Management oftraumatic optic neuropathy - a study of23 patients

Table I Patients with traumatic optic neuropathy treated with steroids alone

Case nolagelsex Type ofinjury Initial/final vision CTfinding

1. 26, M Blunt trauma, bat CF 2 ft/20/25 Optic nerve sheath haematoma2. 44, M Bluftit trauma 20/70-2/20/20-2 Optic nerve sheath haematoma3. 15, M Fall from bicycle CF 1 ft/20/400 Optic nerve sheath haematoma4. 27, M Blunt trauma 20/200/20/40 Optic nerve sheath haematoma5. 18, M Motor vehicle accident CF 2ft/20/30 Optic nerve sheath haematoma6. 58, M Sinus endoscopy, surgery CF 3 ft/20/25 Optic nerve sheath haematoma7. 42, M Blunt trauma, fist NLP/NLP Possible optic nerve sheath

haematoma8. 13, M Knife to orbit NLP/NLP Optic nerve sheath haematoma9. 90, M Motor vehicle accident LP/LP Optic nerve sheath haematoma

Possible optic nerve avulsion10. 54, M Blunt trauma CF 3 ft/20/20 Optic nerve sheath haematoma,

left tripod fracture11. 27, M Gunshot right orbit NLP/NLP No significant pathology*12. 53, M Motor vehicle accident 20/400/20/25 No significant pathology13. 36, M Fist NLP/NLP Apical medial optic canal fracture14. 27, M Gunshot left orbit NLP/NLP No significant pathology15. ?, M Motor vehicle accident NLP/NLP Optic canal fracture16. ?, M Blunt trauma 20/800/20/40 No significant pathology

*Central retinal artery occlusion.CF=counting fingers, NLP=no light perception, LP=light perception.

Table 2 Characteristics ofpatients with traumatic optic neuropathy treated with steroids andoptic nerve sheath decompression

Case nolagelsex Type ofinjury Initiallfinal vision CTfinding

17. 28, M Knife to orbit CF 1 ft/20/25 Optic nerve sheath haematoma18. 18, M Gunshot to orbits Bilateral NLP/NLP Right optic nerve sheath

haematoma*19. 27, M Motor vehicle accident NLP/NLP Right optic nerve sheath

haematoma

* Left optic nerve avulsed and not treated; right optic nerve sheath haematoma decompressed.

Table 3 Characteristics ofpatients with traumatic optic neuropathy treated with steroids, opticnerve sheath decompression, and neurosurgical decompression ofthe optic canal*

Case nolagelsex Type ofinjury Initiallfinal vision CTfinding

20. 12, M Gunshot to orbits NLP both eyes/NLP Right optic nerve avulsion, leftOD 20/200 OS optic nerve sheath haematoma

with bone spicules at orbitalapex

21. 32, M Gunshot to orbits NLP both eyes/NLP Right optic nerve avulsion, leftOD HM OS optic nerve sheath haematoma

with bone spicules at orbitalapex

22. 34, F Motor vehicle accident NLP both eyes/ Left optic nerve sheathimproved haematoma, bilateral optic canalpupillary reaction fractureboth eyest

23. 28, M Blunt trauma NLP/NLP Optic nerve sheath haematoma,apical bone directly impingingon optic nerve

* In each case finding on CT was treated surgically. Patients with presumed avulsion were not treated.t Unable to assess exact vision due to mental status after injury.HM=hand movements.

steroid treatment showed improvement on opticnerve sheath decompression by medialorbitotomy of an enlarged sheath (Table 2).Three patients who did not respond to steroids

had optic nerve sheath decompression; one of thepatients improved to 20/25 (case no 17) (Table2). Four patients who did not respond to steroidshad combined optic nerve sheath decompressionand neurosurgical decompression of the opticcanal; three showed improved vision (Table 3).

All five patients with a lucid interval of visionafter the injury had a final improved vision whileonly five of 16 patients without a lucid intervalimproved. Seven of 10 patients (three bilateralcases) who presented with NLP had no improve-ment with any treatment. Three (four eyes) ofthe 10 with improvement had combined opticnerve sheath decompression and neurosurgicaldecompression of the optic canal.No medical or surgical complications occurred

in this series of 23 patients.

DiscussionThe present data suggest the efficacy of steroidsand possibly surgery in selected patients withtraumatic optic neuropathy. All 23 patientsinitially received high dose steroids. Nine of 16patients showed significant improvement onsteroids without surgery. In patients withoutvisual improvement after 24 to 48 hours ofintravenous steroids surgery was considered if:(1) an enlarged optic nerve sheath with presumedintrasheath haemorrhage decompression of theretrobulbar portion of the optic nerve sheath, or(2) a narrowed optic canal or orbital apex by bonespicules were present on CT scan. To date therole of optic nerve fenestration in the treatmentof traumatic optic neuropathy has not beenclearly elucidated.8-'0One of three patients showed visual improve-

ment after optic nerve sheath decompression.Three of four showed improvement withcombined optic nerve sheath decompression andneurosurgical decompression of the optic canalby neurosurgical frontal craniotomy. One patientdespite such combined surgery showed noimprovement in vision. Decompression of theoptic canal may alternatively be performed bymedial orbitotomy with a skin incision or by atransantral approach.2's'6 The latter approachmay have less morbidity than a craniotomy;however there were no complications in thisseries and frontal craniotomy allows completedecompression of the optic canal and removal ofapical orbital bone fragments.

Optic nerve sheath haematomas are relativelyrare and are difficult to demonstrate radio-graphically. For these reasons there are only afew reported cases of decompression of anenlarged optic nerve sheath.8" In this seriesfluid was not always recovered at the time ofoptic nerve sheath decompression and in onesuch patient (case no 17) the final vision was20/25. The findings may be due to (1) oedemathat involves the entire nerve and not just thesubarachnoid space, or (2) the collection of bloodmay be focal away from the surgical site.Magnetic resonance imaging may be helpful inlocalising blood.The natural history of traumatic optic neuro-

pathy is unknown, and even the exact mechanismby which corticosteroids reduce optic nerveinjury is unclear. 1-7 The rationale for treatment isas follows. Steroids probably decrease the intra-neural or extraneural oedema and relievecompression of the nerve fibres.4 By reducingvasospasm steroids may also limit contusionnecrosis of the nerve.'The effect of steroids on the resorption of an

optic nerve sheath haematoma is also unclear.Surgical evacuation of non-resorbing nervesheath haematoma should be considered wheninitial treatment with steroids fails to improvevision.8"' Timely surgical optic nerve sheathdecompression is initiated in order to avoidirreversible damage to the axons of the opticnerve compressed by the subarachnoid bloodwithin the swollen nerve sheath.The data show that a favourable prognosis is

associated with (1) a lucid interval, and (2) anenlarged optic nerve sheath. All five patientswith a lucid interval of vision after the injury had

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a final improved vision. Seven of the nine whoshowed improvement with steroids had anenlarged optic nerve sheath. Poor prognosis isassociated with (1) initial NLP vision, (2) nolucid interval, and (3) displacement of largebone fragments against the optic nerve (caseno 23). Seven of 10 patients (three bilateralcases) who presented with NLP had noimprovement with any treatment. Only five of 15patients who did not have a lucid intervalultimately improved.

Patients who present without a lucid intervaland with NLP vision should be evaluated andtreated. In the three patients (case nos 20-22)who presented with NLP and subsequentlyimproved, the final vision was 20/200, handmotions, and indeterminable in the third patientdue to intracranial injuries. The latter patienthad bilaterally improved pupillary responses.

In summary optic nerve sheath decompressionalone or combined with decompression of theoptic canal may be indicated in selected patientswho fail to respond to high dose intravenoussteroids. The definitive role of surgery in themanagement of traumatic optic neuropathyremains unclear until a protocol is adopted on amulticentre level. Initiation of steroids for 24 to48 hours affords sufficient time for consultations,radiological studies, and conference with thepatient and family prior to any surgicalintervention.

Presented in part at the Annual Meetings of the American Societyof Ophthalmic, Plastic and Reconstructive Surgery, New Orleans,

Louisiana, 28 October 1989 and Anaheim, California, 12 October1991. The work was supported in part by an unrestrictedophthalmology departmental grant from Research to PreventBlindness, New York, New York.

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