Bimodal Approach: A Key to Manage a Case of Traumatic Superior Orbital Fissure Syndrome.

An unusual complication associated with maxillofacial trauma is the superior orbital fissure syndrome (SOFS). Trauma-related SOFS often presents within 48 h of injury, but presentation can be delayed by several days. This article sums up the particulars of the syndrome and treatments done in the literature and discusses our experience of managing this complex case. Copyright:

INTRODUCTION

Rochon–Duvigneaud syndrome referred to as the superior orbital fissure syndrome (SOFS) is a rare complication of craniomaxillofacial traumas. It was first described by Hirschfield in 1858.[1]

The incidence of traumatic SOFS is 1 (0.8%) in every 130 cases of facial fractures according to Zachariades.[2]

Lakke further defined the complete SOFS, which consisted of paresis of the ocular muscle, sensory disturbance in the distribution of the first branch of the trigeminal nerve, and retro-orbital pain.[3] The optic nerve could be also involved, designating the orbital apex syndrome, as described by Kjaer in 1945.[4]

It may be the result of fractures of the Le Fort II and III types as well as fractures of the zygomaticomaxillary complex (ZMC), neoplasm of the retrobulbar space, hematoma in the orbital muscle cone and retrobulbar hematoma, hematoma and infection of the cavernous sinus, infection of the meninges and the central nervous system as well as infection of the retrobulbar space.[3]

Direct bony compression of the contents of the superior orbital fissure (SOF) and/or a compression hematoma may cause the signs and symptoms of the syndrome which are either complete or partial depending upon the degree of compression of its related anatomical structure.[4]

The SOF has two basic morphological types, which differ according to size parameters as well as the location of the structures within the fissure.[6]

According to Govsa et al. (1999), the following structures lie in the greatest proximity to each other: the superior branch of the oculomotor nerve and the medial rim of the fissure, the trochlear nerve and the superior rim, the abducens nerve, and the inferior edge.[7]

The commonly encountered clinical features are as follows: (a) ptosis of the upper eyelid; (b) complete immobility of the eye and slight exophthalmos; (c) paralysis of the pupillary and ciliary muscles resulting in dilated fixed pupil and accommodation paralysis; and (d) anesthesia in the area of distribution of all branches of the first division of the trigeminus nerve supplying the cornea, upper eyelid, bridge of the nose, and forehead.[8]

The purpose of this paper is to share our quandary of a case which showed signs of an orbital apex syndrome but was finally diagnosed as a SOFS and managed surgically along with megadose steroid therapy.

CASE REPORT

A 23-year-old male was brought to our hospital following a head injury concomitant with facial trauma, reported as a road traffic accident. He had a history of loss of consciousness and amnesia. Advanced Trauma Life Support protocol was carried out. The Department of Neurosurgery was consulted for the extradural hemorrhage in the left frontal and anterior temporal region along with mild pneumocephalus. The Department of Oral and Maxillofacial Surgery was consulted to evaluate and manage multiple facial fractures, and the Department of Ophthalmology was consulted for visual assessment due to periorbital injuries. On preliminary head-and-neck examination, the patient showed left periorbital edema and ecchymosis, marked subconjunctival hemorrhage in the left eye, complete left eyelid ptosis, a step deformity palpable at the left infraorbital rim, and a reduced left facial projection. The ophthalmologic examination revealed a dilated pupil only reactive to light along with acute loss in visual acuity in the left eye; however, the intraocular pressure was not elevated bilaterally. Evidence of slight paresthesia over the left eyelid, complete restriction of range of motion of the left globe in all fields of gaze was noted. A forced duction test had a negative result for entrapment of extraocular muscles. A fundoscopic examination confirmed no optic nerve damage bilaterally.

A basal craniomaxillofacial computed tomography scan revealed a left frontotemporal skull fracture with ipsilateral involvement of the supraorbital and infraorbital rims, left lateral wall of orbit fracture, a fracture of the posterolateral and anterior walls of the right maxillary sinus, and a fracture of the left zygomatic body and arch, all of which had subtle displacement.

Magnetic resonance image (MRI) scan with contrast of the orbit confirmed that both globes were sound with no noticeable muscle entrapment, although obstruction of the left SOF was seen.

Thus, the diagnosis made was of a left ZMC fracture with accompanying SOFS due to constriction of the fissure, its contents, and the surrounding structures by bony fragments.

Following primary assessment, the patient was kept under observation in the intensive care unit where injectables levetiracetam 500 mg and methylprednisolone 1 g were administered twice a day for 1 week, after which he was given fitness for undergoing an open reduction with internal fixation of the left ZMC fracture [Figure 1]. The procedure was performed under general anesthesia using a left maxillary vestibular incision in order to lessen the bony obstruction of the SOF. The patient also received a perioperative dose of intravenous dexamethasone 8 mg which was later tapered over a period of 3 days. A postoperative posteroanterior Waters’ view was done to judge the reduction and reassess the SOF. The patient was followed up monthly by the Oral and Maxillofacial Surgery Department. There was complete resolution of ptosis and partial improvement in ophthalmoplegia after 12 weeks [Figures 2-4].

Figure 1

Open reduction and internal fixation done for the zygomaticomaxillary complex fracture

Figure 2

(Left) Restricted upward gaze after 1 month; (Right) Improved upward gaze after 3 months

Figure 4

(Left) A 23 year old male with superior orbital fissure syndrome 1 week post trauma; (Middle) 1 month post treatment; (Right) 3 months post treatment

(Left) Restricted medial gaze after 1 month; (Right) Improved medial gaze after 3 months

DISCUSSION

The SOF serves as a pathway that allows communication between the orbit and the middle cranial fossa. It lies at the apex of the orbit, bounded medially by the lesser wing of the sphenoid, inferiorly and laterally by the greater wing of the sphenoid, and superiorly by the frontal bone. It transmits the oculomotor, trochlear, and abducens nerves (cranial nerves III, IV, and VI), as well as the first three branches of the trigeminal nerve: the frontal, lacrimal, and nasociliary nerves.[9]

In a review of 11,284 patients of craniofacial fractures, Chen and Chen found 33 cases of SOFS (0.3%).[3]

Reymond et al. in their cadaveric study on 100 skulls concluded that SOF had mainly two morphological variants: Type “a” with characteristic narrowing of the fissure and Type “b” that lacks such narrowing but was significantly shorter.[6] According to Morard et al.'s (1994) measurements, the size of the SOF is on average 3 mm × 22 mm in length.[10] Our case shows the Type “a” fissure on the unaffected side, and the affected side was obstructed with the fracture fragments.

Studies consider the narrow SOFS as a risk factor for SOFS[11] which was seen in our patient.

One study stated that the rationale behind the treatment of SOFS of traumatic origin lies primarily in minimizing further irreparable damage to the neuronal structure. A general consensus lies toward initial observation period of 10–14 days before any surgical manipulation of fracture segments is done;[5] we managed our case the same way.

In general, exploration of the SOF is indicated if after 4 months no improvement is seen in sensory and motor functions or in cases of acute and total ophthalmoplegia accompanied by progressive exophthalmos;[8] however, we only performed surgery to reduce the zygomatic complex fracture and recovery was seen.

Since Anderson et al. reported the benefits of corticosteroids in traumatic optic neuropathy in the early 1980s, application of high-dose corticosteroid treatment in optic nerve injury has increased considerably. Visual improvement was seen in 42.8% of patients treated by surgery and 58.8% of patients with medical treatment, which consisted of a combined use of prednisolone, mannitol, urokinase, and Vitamin B12. The megadose corticosteroid treatment includes intravenous methylprednisolone 30 mg/kg over 15 min followed by an infusion of 5.4 mg/kg/h for 48 h from the 1st day of trauma.[12] For our patient, the regimen followed was intravenous methylprednisolone 1 g twice a day for a week followed by perioperative intravenous dexamethasone 8 mg which was tapered postoperatively over 3 days.

Even though our patient presented with initial visual loss and put us in a perplexed situation whether to treat it as an orbital apex or a SOFS, confirmatory diagnosis was made with the help of fundoscopy and an MRI scan. The rationale for preliminary diminished visual acuity was thought to be due to compression of the optic nerve by the fracture leading to neuropraxia or axonotmesis which gradually showed 80% resolution after 3 months.

We concluded that prompt surgical intervention along with megadose corticosteroid therapy is essential in treating cases of complex facial fractures leading to SOFS.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.

Research quality and ethics statement

The authors followed applicable EQUATOR Network (http://www.equator-network.org/) guidelines, notably the CARE guideline, during the conduct of this report.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.