YIJOM-3286; No of Pages 10 Int. J. Oral Maxillofac. Surg. 2015; xxx: xxx–xxx http://dx.doi.org/10.1016/j.ijom.2015.10.014, available online at http://www.sciencedirect.com Case Reports Trauma R. E. Warburton, C. C. D. Brookes, B. A. Golden, T. A. Turvey Orbital apex disorders: a case Department of Oral and Maxillofacial Surgery, School of Dentistry, University of North series Carolina at Chapel Hill, Chapel Hill, NC, USA R. E. Warburton, C. C. D. Brookes, B. A. Golden, T. A. Turvey: Orbital apex disorders: a case series. Int. J. Oral Maxillofac. Surg. 2015; xxx: xxx–xxx. # 2015 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Abstract. Orbital apex syndrome is an uncommon disorder characterized by ophthalmoplegia, proptosis, ptosis, hypoesthesia of the forehead, and vision loss. It may be classified as part of a group of orbital apex disorders that includes superior orbital fissure syndrome and cavernous sinus syndrome. Superior orbital fissure syndrome presents similarly to orbital apex syndrome without optic nerve impairment. Cavernous sinus syndrome includes hypoesthesia of the cheek and lower eyelid in addition to the signs seen in orbital apex syndrome. While historically described separately, these three disorders share similar causes, diagnostic course, and management strategies. The purpose of this study was to report three cases of orbital apex disorders treated recently and to review the Key words: orbital apex syndrome; superior literature related to these conditions. Inflammatory and vascular disorders, orbital fissure syndrome; cavernous sinus neoplasm, infection, and trauma are potential causes of orbital apex disorders. syndrome; orbital compartment syndrome; pto- Management is directed at the causative process. The cases described represent a sis; proptosis; ophthalmoplegia; optic neuropa- rare but important group of conditions seen by the maxillofacial surgeon. A review thy; maxillofacial surgery. of the clinical presentation, etiology, and management of these conditions may prompt timely recognition and treatment. Accepted for publication 16 October 2015 1 Orbital apex syndrome (OAS) is an uncom- oculosympathetic paresis, and impairment SOFS developing into OAS or CSS. For mon disorder characterized by impairment of the ophthalmic and maxillary branches the purpose of discussion OAS, SOFS, and 1 of cranial nerves III, IV, VI, and the oph- of cranial nerve V. CSS presents with CSS can be grouped together as a single thalmic branch of cranial nerve V, and optic ophthalmoplegia, ptosis, proptosis, de- condition, differentiated chiefly by the neuropathy. Patients present with ophthal- creased vision, and loss of sensation in anatomical position of the causative pa- moplegia, proptosis, ptosis, visual im- the ipsilateral forehead, eyelids, cornea, thology. 4,5 1 pairment, a fixed dilated pupil, and and cheek. CSS can present bilaterally. Three new cases of orbital apex disor- hypoesthesia of the ipsilateral forehead, Cranial nerve involvement may be com- ders are reported to illustrate the three 1 upper eyelid, and cornea. Superior orbital plete or incomplete in all three syndromes. syndromes and highlight several key caus- fissure syndrome (SOFS) presents similarly While frequently described separately ative factors. The relevant anatomy, etiol- to OAS, without the accompanying optic in the literature, these three orbital apex ogy, and available diagnostic and 2,3 nerve impairment. Cavernous sinus syn- disorders share a similar etiology, diag- treatment modalities are also described. 1,6 drome (CSS) involves palsy of cranial nostic course, and treatment. These syn- This study was reviewed by the Institu- nerves III, IV, and VI, optic neuropathy, dromes can be progressive in nature, with tional Review Board and deemed exempt. 0901-5027/000001+010 # 2015 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Please cite this article in press as: Warburton RE, et al. Orbital apex disorders: a case series, Int J Oral Maxillofac Surg (2015), http:// dx.doi.org/10.1016/j.ijom.2015.10.014 YIJOM-3286; No of Pages 10 2 Warburton et al. Case series clinic with a 4-day history of right mid- facial pain and swelling without orbital Case 1 involvement. He was admitted to the hos- A 16-year-old Caucasian male with pital with a presumed facial infection of marked midfacial hypoplasia, retrogenia, unknown cause for further work-up and and right-sided hemihypoplasia presented intravenous antibiotics. Shortly after ad- for a Le Fort III osteotomy modified to mission, the patient developed rapid onset maintain nasal position, right split thick- of ophthalmoplegia, proptosis, ptosis, and ness parietal bone harvest with grafting to complete vision loss OD. The right pupil the bilateral supraorbital rims and mid- was found to be fixed and dilated. Oph- face, and genioplasty. Preoperative oph- thalmology was consulted and the patient thalmic evaluation revealed normal vision was found to have an intraocular pressure (20/20 in the right eye (OD) and 20/25 in of 57 mmHg OD by handheld tonometry. the left eye (OS)) with no afferent pupil- The patient was diagnosed with an OAS lary defect or anisocoria. secondary to an orbital compartment syn- The patient was administered 125 mg of drome, and an emergency lateral canthot- methylprednisolone and 1 g of cefazolin omy with inferior cantholysis was intravenously at the start of the procedure performed bedside. Intraocular pressure and every 4 h subsequently. The midface improved to 38 mmHg OD after the pro- was mobilized and advanced without dif- cedure. Vision loss and ophthalmoplegia ficulty. The inner cortex remained intact were unchanged. Brimonidine and dorzo- during the parietal bone harvest. The or- lamide/timolol were administered topical- bital osteotomies were located within ly and an emergency CT of the face was 1 cm of the orbital rim and carried into obtained. Imaging revealed a large cystic the inferior orbital fissure. No fracture or mass in the right maxillary sinus with Fig. 1. Intraoperative CT scan revealing no extension of the osteotomies to the orbital erosion of the posterior maxillary sinus fracture or hematoma involving the right or- apex was observed. wall and right orbital cellulitis with poste- bital apex or optic canal. 7 Upon completion of the procedure, the rior globe tenting (Fig. 2). right pupil was noted to be fixed and The patient was taken as an emergency dilated with a 4-mm discrepancy when der of his neurological examination was to the operating room for drainage of the compared to the left pupil. Bilateral pupils benign. Magnetic resonance imaging right maxillary sinus via a Caldwell–Luc were non-reactive, which was attributed to (MRI) of the brain and orbits was performed, approach and further orbital decompres- the depth of anesthesia. The coronal and which revealed no signs of orbital apex sion. Otolaryngology was contacted to intraoral incisions were reopened and the compression or intracranial pathology. determine the benefit of concomitant en- right orbit was explored, with no hemato- The patient was diagnosed with a pre- doscopic sinus surgery, but the decision ma or pulsatile bleeding noted. Ophthal- sumed SOFS. Intravenous methylprednis- was made to defer additional sinus inter- mology was consulted to help evaluate for olone (125 mg) was continued every 4 h vention at that time. After drainage of the an intraorbital cause of the patient’s ani- throughout his hospitalization. A formal maxillary sinus, the right intraocular pres- socoria. Intraocular pressures were found ophthalmic evaluation at less than 24 h sure improved to 16 mmHg by handheld to be normal at 15 mmHg OD and after surgery found visual acuity to be tonometry and no additional orbital de- 20 mmHg OS by handheld tonometry. 20/40 OD, 20/20 OS. The patient under- compression was pursued. Cultures were The neurosurgery service was consulted went serial ophthalmic examinations, obtained and the maxillary sinus contents to rule out any intracranial pathology. An which showed slight improvement in pto- were sent for histopathological evaluation. emergency computed tomography (CT) of sis and continued ophthalmoplegia. The Postoperative visual acuity improved to the head and face was obtained, which patient was discharged on postoperative 20/200 OD. The right pupil was sluggishly revealed no intracranial hemorrhage, mid- day 2 with a 7-day prednisone taper. At the reactive. Ophthalmoplegia was unchanged. line shift, or brainstem compression. No time of discharge, the patient’s vision was The patient was started on vancomycin and fracture or hematoma involving the super- 20/30 OD, 20/20 OS with a 2-mm discrep- piperacillin/tazobactam. Maxillary sinus ior orbital fissure or orbital apex was noted ancy in pupillary size and minimal reac- cultures revealed no growth. The intraop- (Fig. 1). The patient’s surgical wounds tivity to light OD. erative pathology results returned as sino- were then closed and he was transferred Three weeks postoperatively, the patient nasal mucosa with features consistent with to the pediatric intensive care unit for showed improved extraocular movements acute on chronic rhinosinusitis. Serial oph- close neurological monitoring. and ptosis OD. The binocular diplopia and thalmic examinations revealed improved The patient’s postoperative ophthalmic pupillary dilation remained. Five weeks visual acuity and pupillary reactivity, al- examination revealed complete