Orbital apex syndrome - Semantic Scholar...Orbital apex syndrome Steven Yeh and Rod Foroozan Purpose of review Visual loss from optic neuropathy and ophthalmoplegia involving multiple
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Orbital apex syndrome
Steven Yeh and Rod Foroozan
Purpose of review
Visual loss from optic neuropathy and ophthalmoplegiainvolving multiple cranial nerves are the hallmarks of an orbitalapex syndrome. Historically, the terms superior orbital fissure,orbital apex, and cavernous sinus have been used to define theanatomic locations of a disease process. However, thediagnostic evaluation and management is similar for each ofthese entities. The authors reviewed the literature on thediagnosis and evaluation of disorders involving the orbital apex.Recent findings
High-resolution MRI is the preferred modality for evaluatingmost lesions involving the orbital apex. CT is a useful tool inthe setting of trauma, to evaluate bone involvement, or whenMRI is contraindicated. Although laboratory studies may beuseful adjuncts in the diagnostic evaluation of lesions involvingthe orbital apex, surgical biopsy is often required for definitivediagnosis.Summary
Orbital apex syndromes may result from a variety ofinflammatory, infectious, neoplastic, iatrogenic/traumatic, andvascular conditions. A detailed history with review of systemsis important in narrowing the differential diagnosis.Management is directed at the underlying cause and may beguided by surgical biopsy. Corticosteroids may be useful if aninflammatory etiology is suspected, but should be used withcaution.
Keywords
orbital apex, superior orbital fissure, cavernous sinus,Tolosa–Hunt syndrome, magnetic resonance imaging
(A) Block section illustration of the cavernous sinus and it contents. (B) The duralwall of the cavernous sinus has been removed and the trigeminal nerve has beenreflected forward to reveal the venous spaces of the cavernous sinus. (C) Thecavernous sinus has been completely removed to show the intracavernoussegment of the carotid artery. The location of the cavernous sinus is indicated bythe white dotted line. ICA, internal carotid artery. (Reprinted with permission fromElsevier [1].)
Orbital apex syndrome Yeh and Foroozan 491
Neoplastic
1. Head and neck tumors: nasopharyngeal carcinoma,
enhancement within the orbital apex (larger arrow) and
cavernous sinus (smaller arrow) in a 77-year-old woman who
developed a left sixth nerve palsy and optic neuropathy
Total ophthalmoplegia developed, prompting a craniotomy and cavernous sinusbiopsy, which revealed nongranulomatous inflammatory changes. Despitecorticosteroids and the addition of other immunomodulatory agents, the patientlost vision to no light perception, and repeat neuroimaging revealed progressionof the inflammatory process (right).
Figure 4. Axial CT (left) showing opacification within the
ethmoid (larger arrow) and sphenoid (smaller arrow) sinuses
Coronal CT (right) confirms opacification within the ethmoid sinus in a43-year-old man who developed fevers, visual loss, and a sixth nerve palsy.Endoscopy revealed mucosal inflammation within the paranasal sinuses and puswithin the orbital apex. His symptoms improved after surgical drainage andtreatment with intravenous antibiotics.
Orbital apex syndrome Yeh and Foroozan 493
negative bacilli, and anaerobes [24•,25,26]. Bilateral cav-
ernous sinus involvement has also been reported in as-
sociation with central nervous system Actinomyces israelii[27,28]. A mixed infection with S. aureus and Pseudomo-nas aeruginosa has also been observed to cause an OASwith cavernous sinus thrombosis [29].
Neoplastic
The possibility of a neoplasm should be considered in
the differential diagnosis of an OAS, especially in any
patient with a known history of cancer. Primary ocular or
orbital tumors, neoplasms of the paranasal sinuses, or
central nervous system tumors may invade the orbital
apex. Metastatic disease may also involve the cavernous
sinus. Tumors that most commonly cause a CSS include
2 Kline LB: The Tolosa–Hunt syndrome. Surv Ophthalmol 1982, 27:79–95.
••3 Lin CC, Tsai JJ: Relationship between the number of involved cranial nerves
and the percentage of lesions located in the cavernous sinus. Eur Neurol2003, 49:98–102.
This retrospective analysis investigated the relationship between the number ofcranial nerves affected and percentage of lesions located within the cavernoussinus. The authors collected 68 total patients with cranial nerve palsies and deter-mined the percentage of patients with cavernous sinus lesions. In increasing orderof cranial nerve involvement (one to four cranial nerves), 17.7%, 44.4%, 56.3%,and 77.8% of patients were found to have cavernous sinus lesions.
5 Lenzi GL, Fieschi C: Superior orbital fissure syndrome. Review of 130 cases.Eur Neurol 1977, 16:23–30.
6 Thajeb P, Tsai J-J: Cerebral and oculorhinal manifestations of a limited form ofWegener’s granulomatosis with c-ANCA-associated vasculitis. J Neuroimag-ing 2001, 11:59–63.
7 Zarei M, Anderson JR, Higgins JN, et al.: Cavernous sinus syndrome as theonly manifestation of sarcoidosis. J Postgrad Med 2002, 48:119–121.
8 Calistri V, Mostardini C, Pantano P, et al.: Tolosa–Hunt syndrome in a patientwith systemic lupus erythematosus. Eur Radiol 2002, 12:341–344.
9 Tokumaru AM, Obata T, Kohyama S, et al.: Intracranial meningeal involvementin Churg–Strauss syndrome. AJNR Am J Neuroradiol 2002, 23:221–224.
•10 Islam N, Asaria R, Plant GT, et al.: Giant cell arteritis mimicking idiopathic
orbital inflammatory disease. Eur J Ophthalmol 2003, 13:392–394.This is a case report of a 72-year-old woman who presented with a dull right eyeache, proptosis, and right-sided ophthalmoplegia. An elevated erythrocyte sedi-mentation rate and fluorescein angiogram showing almost complete choroidal non-perfusion suggested giant cell arteritis. Temporal artery biopsy confirmed the di-agnosis.
11 Hunt WE: Tolosa–Hunt syndrome: one cause of painful ophthalmoplegia. JNeurosurg 1976, 44:544–549.
12 Hunt WE, Meagher JN, LeFever HE, et al.: Painful ophthalmoplegia. Its rela-tion to indolent inflammation of the cavernous sinus. Neurology 1961,11:56–62.
••13 International Headache Society: The international classification of headache
disorders. Cephalalgia 2004, 24(suppl 1):1–151.A description of THS and diagnostic criteria are given. THS is defined as episodicorbital pain associated with paralysis of one or more of the third, fourth, and/or sixthcranial nerves, which usually resolves spontaneously but tends to relapse and re-mit. Diagnostic criteria include one or more episodes of unilateral orbital pain per-sisting for weeks if untreated; paresis of one or more of the third, fourth, and/or sixthcranial nerves; and/or granuloma demonstrated by MRI or biopsy; paresis coincid-ing with pain or following it within 2 weeks; resolution of pain and paresis within 72
496 Neuro-ophthalmology
hours when treated with corticosteroids; and exclusion of other causes of painfulophthalmoplegia.
15 Forderreuther S, Straube A: The criteria of the International Headache Soci-ety for Tolosa–Hunt syndrome need to be revised. J Neurol 1999, 246:371–377.
16 Yousem DM, Atlas SW, Grossman RI, et al.: MR imaging of Tolosa–Huntsyndrome. AJNR Am J Neuroradiol 1989, 10:1181–1184.
17 Bray WH, Giangiacomo J, Ide CH: Orbital apex syndrome. Surv Ophthalmol1987, 32:136–140.
18 Balch K, Phillips PH, Newman NJ: Painless orbital apex syndrome from Mu-cormycosis. J Neuroophthalmol 1997, 17:178–182.
19 Fernandes YB, Ramina R, Borges G, et al.: Orbital apex syndrome due toAspergillosis: case report. Arq Neuropsiquiatr 2001, 59:806–808.
20 Petrick M, Honegger J, Daschner F, et al.: Fungal granuloma of the sphenoidsinus and clivus in a patient presenting with cranial nerve III paresis: casereport and review of the literature. Neurosurgery 2003, 52:955–959.
21 Bafna S, Lee AG: Presumed tuberculosis presenting as a cavernous sinussyndrome. J Neuroophthalmol 1997, 17:207–208.
22 Hui AC, Wong WS, Wong KS: Cavernous sinus syndrome secondary totuberculous meningitis. Eur Neurol 2002, 47:125–126.
23 Rebai R, Boudawara MZ, Bahloul K, et al.: Cavernous sinus tuberculoma:diagnostic difficulties in a personal case. Surg Neurol 2001, 55:372–375.
•24 Cannon ML, Antonio BL, McCloskey JJ, et al.: Cavernous sinus thrombosis
complicating sinusitis. Pediatr Crit Care Med 2004, 5:86–88.This is a case report and literature review of cavernous sinus thrombosis in thesetting of acute sinusitis. A 12-year-old girl presented in critically ill condition after1 week of productive cough, progressive dyspnea, and left eye swelling. Spiral CTof the chest revealed septic emboli and lung abscesses. Head CT with contrastshowed thromboses of the cavernous sinuses, superior ophthalmic veins, and fa-cial veins. The patient recovered after parenteral antibiotics, but a right-sided opticneuropathy persisted.
25 Ebright JR, Pace MT, Niazi AF: Septic thrombosis of the cavernous sinuses.Arch Intern Med 2001, 161:2671–2676.
26 Southwick FS, Richardson EP Jr, Swartz MD: Septic thrombosis of the duralvenous sinuses. Medicine 1986, 65:82–106.
27 Holland NR: CNS Actinomyces presenting with bilateral cavernous sinus syn-drome. J Neurol Neurosurg Psychiatry 1998, 64:4.
29 Colson AE, Daily JP: Orbital apex syndrome and cavernous sinus thrombosisdue to infection with Staphylococcus aureus and Pseudomonas aeruginosa.Clin Infect Dis 1999, 29:701–702.
30 Currie JN, Coppeto JR, Lessell S: Chronic syphilitic meningitis resulting insuperior orbital fissure syndrome and posterior fossa gumma. A report of twocases followed for 20 years. J Clin Neuroophthalmol 1988, 8:145–159.
31 Fairley C, Sullivan TJ, Bartley P, et al.: Survival after rhino-orbital-cerebral mu-cormycosis in an immunocompetent patient. Ophthalmology 2000,107:555–558.
•32 Lee AG, Quick SJ, Liu GT, et al.: A childhood cavernous conundrum. Surv
Ophthalmol 2004, 49:231–236.This is a case report and literature review of Burkitt lymphoma presenting withbilateral cavernous sinus lesions. A 9-year-old boy presented with left-sided tooth-ache, headache, and, subsequently, vertical diplopia. Examination revealed a thirdnerve palsy, and cranial MRI showed bilateral cavernous sinus lesions. Blood smearand bone marrow biopsy revealed Burkitt lymphoma, which was treated with che-motherapy.
33 Kalina P, Black K, Woldenberg R: Burkitt’s lymphoma of the skull base pre-senting as cavernous sinus syndrome in early childhood. Pediatr Radiol 1996,26:416–417.
34 Rubin MM, Sanfilippo RJ: Lymphoma of the paranasal sinuses presenting ascavernous sinus syndrome. J Oral Maxillofac Surg 1992, 50:749–751.
35 Julien J, Ferrer X, Drouillard J, et al.: Cavernous sinus syndrome due to lym-phoma. J Neurol Neurosurg Psychiatry 1984, 47:558–560.
•36 Schick U, Bleyen J, Hassler W: Treatment of orbital schwannomas and neu-
rofibromas. Br J Neurosurg 2003, 17:541–545.The authors discuss their treatment of five orbital peripheral nerve tumors. Twocases, both schwannomas, involved the orbital apex and superior orbital fissure.Their surgical management is outlined.
•37 Vassilikos C, Pepe P, Christopoulos C: Ciliary neurinoma: a very rare intraor-
This is a case report of a 75-year-old woman who presented with a 1-month historyof decreased vision, diplopia, retroorbital pain, and exophthalmos. CT and MRIrevealed a 12 × 16-mm mass involving the orbital apex. The mass was surgicallyremoved and pathology revealed a benign ciliary neurinoma. The patient’s painresolved and visual acuity was partially restored.
38 Srinivasan S, Fern AI, Wilson K: Orbital apex syndrome as a presenting signof maxillary sinus carcinoma. Eye 2001, 15:343–345.
39 McDonald HR, Char DH: Adenoid cystic carcinoma presenting as an orbitalapex syndrome. Ann Ophthalmol 1985, 17:757–759.
40 Gore HL, Corin SM, Klussmann KG, et al.: Mucoepidermoid carcinoma pre-senting as an orbital apex syndrome. Ophthalmic Surg 1992, 23:59–61.
41 Veness MJ, Biankin S: Perineural spread leading to orbital invasion from skincancer. Australas Radiol 2000, 44:296–302.
42 Ryan MW, Rassekh CH, Chaljub G: Metastatic breast carcinoma presentingas cavernous sinus syndrome. Ann Otol Rhinol Laryngol 1996, 105:666–668.
•45 Harkness KA, Manford MR: Metastatic malignant melanoma presenting as a
cavernous sinus syndrome. J Neurol 2004, 251:224–225.This is a case report of a 21-year-old fair-skinned man with a 10-day history ofprogressive bifrontal headache and diplopia. Partial third, fourth, and sixth nervepalsies were observed initially, and the patient’s pupillary reaction was normal. CTof the brain and orbits was normal. Symptoms progressed to complete ptosis andophthalmoplegia with a fixed, dilated pupil. Routine blood tests and MRI of the brainand orbits with gadolinium contrast were normal at this time. Excisional biopsy oftwo skin nevi showed dysplastic nevi but no malignant features. Diagnosis of ma-lignant melanoma was made with a core biopsy of a 4.1-cm axillary lymph node.Despite whole-brain radiotherapy, the patient died 4 months after initial presenta-tion after developing multiple metastases.
46 Pless M, Rizzo JF 3rd, Shang J: Orbital apex syndrome: a rare presentation ofextramedullary hematopoiesis: case report and review of literature. J Neu-rooncol 2002, 57:37–40.
•47 Yeh S, Yen MT, Foroozan R: Orbital apex syndrome after ethmoidal artery
ligation for recurrent epistaxis. Ophthal Plast Reconstr Surg 2004, 20:392–394.
This is a case report of a 34-year-old man with severe, recurrent epistaxis whounderwent external anterior and posterior ethmoidal artery ligation on the right side.Severe visual loss from optic neuropathy and complete ophthalmoplegia devel-oped after surgery. CT revealed surgical clips within the right orbital apex. Emer-gent removal of the surgical clips and medial wall decompression were performed.Despite prompt recognition and treatment, severe visual loss and ophthalmoplegiapersisted.
48 Vassallo P, Tranfa F, Forte R, et al.: Ophthalmic complications after surgeryfor nasal and sinus polyposis. Eur J Ophthalmol 2001, 11:218–222.
49 Jaison SG, Bhatty SM, Chopra SK, et al.: Orbital apex syndrome: a rare com-plication of septorhinoplasty. Indian J Ophthalmol 1994, 42:213–214.
50 Rene C, Rose GE, Lenthall R, et al.: Major orbital complications of endo-scopic sinus surgery. Br J Ophthalmol 2001, 85:598–603.
51 Anderson RL, Panje WR, Gross CE: Optic nerve blindness following bluntforehead trauma. Ophthalmology 1982, 89:445–455.
52 Jarrahy R, Cha ST, Shahinian HK: Retained foreign body in the orbit andcavernous sinus with delayed presentation of superior orbital fissure syn-drome: case report. J Craniofac Surg 2001, 12:82–86.
•53 Atri A, Sheen V: Cavernous sinus syndrome and headache due to bilateral
carotid artery aneurysms. Arch Neurol 2003, 60:1327–1328.This is a case report of an 89-year-old woman with a history of hypertension andPaget disease who developed right-sided headache. Two years earlier she hadfallen, resulting in a fracture of her arm; weeks later, she developed pain and swell-ing in the right eye followed by numbness of the right forehead and blurred vision.Neurologic examination revealed right-sided ptosis, esotropia, complete ophthal-moplegia, and decreased sensation in the V1 and V2 dermatomes. MRI and MRangiography showed bilateral cavernous carotid aneurysms with thrombi. At-tempted endovascular occlusion of the aneurysm on the right side was unsuccess-ful.
54 Lach B, Nair SG, Russell NA, et al.: Spontaneous carotid–cavernous fistulaand multiple arterial dissections in type IV Ehlers–Danlos syndrome. J Neu-rosurg 1987, 66:462–467.
Orbital apex syndrome Yeh and Foroozan 497
••55 de Keizer R: Carotid–cavernous and orbital arteriovenous fistulas: ocular fea-
tures, diagnostic and hemodynamic considerations in relation to visual impair-ment and morbidity. Orbit 2003, 22:121–142.
This is a retrospective review of 101 cases of direct dural carotid–cavernous andorbital arteriovenous fistulas. The diagnostic triad of arterialized loops, exophthal-mos, and glaucoma are discussed. Diagnostic procedures such as ultrasound,color Doppler of the orbit and carotid systems, and MRI and MR angiography arereviewed. Management strategies including conservative therapy, balloon emboli-zation, and direct or indirect surgery are discussed. Of the 10 orbital arteriovenousshunts with signs of dural fistulas, findings spontaneously resolved in eight pa-tients, one patient required direct surgery (which was successful), and one pa-tient’s nonprogressive orbital findings persisted.
56 Tveteras K, Kristensen S, Dommerby H: Septic cavernous and lateral sinusthrombosis: modern diagnostic and therapeutic principles. J Laryngol Otol1988, 102:877–882.
57 DiNubile MJ: Septic thrombosis of the cavernous sinuses. Arch Neurol 1988,45:567–572.
58 Oliven A, Harel D, Rosenfeld T, et al.: Hypopituitarism after aseptic cavernoussinus thrombosis. Neurology 1980, 30:897–899.
59 Schuknecht B, Simmen D, Yuksel C, et al.: Tributary venosinus occlusion andseptic cavernous sinus thrombosis: CT and MR findings. AJNR Am J Neuro-radiol 1998, 19:617–626.
••60 Wolfsberger S, Ba-Ssalamah A, Pinker K, et al.: Application of three-tesla
magnetic resonance imaging for diagnosis and surgery of sellar lesions. JNeurosurg 2004, 100:278–286.
This is a study to determine the value of high-field MRI for diagnosis and surgery ofsellar lesions. High-field MR images were obtained with 3-T MRI, with emphasis on
sellar and parasellar structures in 21 patients. Three-tesla MR images were com-pared with standard 1- to 1.5-T MR images already obtained with intraoperativefindings with attention to the medial border of the cavernous sinus to assess forpossible invasion of a sellar tumor. Three-tesla MRI was superior to standard MRIfor predicting tumor invasion through the medial cavernous sinus border. Betterdelineation of the lateral sinus compartment was also observed with 3-T MRI. Iden-tification of the cavernous sinus segments of the third, fourth, fifth (V1 and V2), andsixth cranial nerves was also improved with 3-T MRI. Three-tesla MRI was found tobe superior to standard MRI for delineation of parasellar anatomy and tumor infil-tration of the cavernous sinus, and may be valuable for intraoperative navigation.
61 Unger JM: Orbital apex fractures: the contribution of computed tomography.Radiology 1984, 150:713–717.
••62 Schick U, Hassler W: Neurosurgical management of orbital inflammations
and infections. Acta Neurochir (Wien) 2004, 146:571–580.This is a retrospective review of the treatment and clinical outcomes of 22 orbitalinflammations and infections, with a subgroup involving the orbital apex. The sur-gical approach in each of these cases was determined by the anatomic location ofthe disease process within the orbit. A transantral approach was used in one pa-tient with a mucocele involving the orbital apex and maxillary sinus. A pterionalextradural approach was useful in two patients with lesions of the orbital apex. Apterional intradural operation was performed in five patients with inflammation ofthe optic canal extending into the intracranial space.
63 Smith JR, Rosenbaum JT: A role for methotrexate in the management of non-infectious orbital inflammatory disease. Br J Ophthalmol 2001, 85:1220–1224.