Orbital apex syndrome is a clinical entity in which numerous disorders (neoplastic, traumatic, inflammatory, infectious, and several other) lead to damage of the optic nerve and compromise blood flow into the orbit, resulting in symptoms such as blurred vision, proptosis, diminished corneal reflex, and palsies of cranial nerves responsible for eye movement. A thorough clinical workup is mandatory in order to raise clinical suspicion, whereas imaging studies are necessary to confirm the underlying etiology.
Orbital apex syndrome (OAS) is a rarely described disorder that may develop as a complication of various conditions, including trauma of the craniomaxillofacial area, malignant disorders of the orbit (rhabdomyosarcoma and lymphoma being the two most common neoplasias), infections (such as herpes zoster ophthalmicus or fungal invasion), and inflammatory changes, either isolated or systemic (eg. Wegener's granulomatosis, sarcoidosis, and polyarteritis nodosa)     . This ailment can be seen in any decade of life, with some studies establishing a mean age of 45-50 years . The typical signs stem from pathological effects of the conditions on the structures that pass through the superior orbital fissure and the orbital apex - cranial nerves supplying the extraocular muscles (trochlear, oculomotor, and abducens nerves) and the surrounding anatomical landmarks (numerous divisions of the trigeminal nerve), as well as the optic nerve and blood vessels  . According to certain reports, blurred vision arising from reduced visual acuity and a fixed dilated pupil is the most frequent symptom in OAS  . In addition, proptosis (eye protrusion), ptosis, reduced sensation on the forehead (hypoesthesia), hyposecretion from the lacrimal apparatus, and diplopia or even vision loss are other notable features  .
- Unilateral Proptosis
Patients with orbital aspergillosis commonly present with unilateral proptosis and associated sinus disease. [ncbi.nlm.nih.gov]
- Unilateral Red Eye
A 16-year-old girl presented with a unilateral red eye, progressive visual loss and diplopia. A detailed clinical assessment with appropriate investigations led to a diagnosis of Orbital Apex Syndrome (OAS) secondary to Tuberculosis (TB). [ncbi.nlm.nih.gov]
The diagnosis of orbital apex syndrome can be made early on with a detailed history taking and a proper physical examination. Firstly, the physician should assess the onset of complaints and their progression, whereas a detailed inspection of the orbit and a thorough neurological examination may provide solid evidence to make a presumptive diagnosis. Typical findings encountered during the exam are ophthalmoplegia, decreased corneal reflex (a sign of trigeminal nerve palsy) and a variable degree of visual impairment  . Imaging studies must be employed whenever these symptoms are present. Plain radiography, apart from its beneficiary role in delineating skeletal trauma and fractures, possesses a limited capacity to discriminate between different etiologies, which is why computed tomography (CT), particularly helical CT (sometimes with iodine as a contrast agent) is recommended for assessing signs pointing to orbital apex syndrome . Gadolinium-enhanced magnetic resonance imaging (MRI), however, is a superior technique that allows a more detailed evaluation of the soft tissues and the vascular structures that may be further examined through MR or CT angiography  . In the absence of a clear underlying cause, a thorough laboratory workup must be carried out, including a complete blood count (CBC), serum electrolytes and inflammatory parameters (C-reactive protein and erythrocyte sedimentation rate), antinuclear and extractable nuclear antibodies (ANA and ENA, respectively), antineutrophilic antibodies (ANCAs), angiotensin-converting enzyme (ACE), and serum protein electrophoresis .
- Treponema Pallidum
pallidum Viruses: Herpes Zoster Neoplastic Head and neck tumors: nasopharyngeal carcinoma with extension into the orbit, primary orbital adenoid cystic carcinoma with extension to the orbital apex Neural tumors: neurofibroma, meningioma, ciliary neurinoma [eyewiki.aao.org]
Spirochetes: Treponema pallidum 4. Viruses: Herpes zoster Traumatic 1. Penetrating injury 2. Non penetrating injury 3. Orbital apex fracture 4. Retained foreign body 25. Iatrogenic 1. Sinonasal surgery 2. Orbital/facial surgery Neoplastic 1. [slideshare.net]
Here we present an extremely rare and serious case of orbital apex syndrome secondary to fungal nasal septal abscess caused by Scedosporium apiospermum in a patient with uncontrolled diabetes. [ncbi.nlm.nih.gov]
Assessing micafungin/triazole combinations for the treatment of invasive scedosporiosis due to Scedosporium apiospermum and Scedosporium boydii. J Antimicrob Chemother. 2014;69:3027–32. [bmcinfectdis.biomedcentral.com]
- Gnathostoma Spinigerum
Her serum was positive for Gnathostoma spinigerum using an immunoblotting technique. Parasites removed from the skin lesions revealed the typical head bulbs with 4 circumferential rows of hooklets and fine cuticular spines on their surface. [ncbi.nlm.nih.gov]
- Actinomyces Israelii
Bilateral cavernous sinus involvement has also been reported in association with central nervous system Actinomyces israelii. Management comprises appropriate antibiotic treatment supplemented by anticoagulation to prevent septic emboli, steroids to [slideshare.net]
In addition to our case, we review the clinical presentation, imaging findings, treatment options, and prognosis of 14 other reported cases. [ncbi.nlm.nih.gov]
The treatment for traumatic OAS is controversial, but endoscopic optic nerve and orbital apex decompression could be considered in patients who fail to respond to corticosteroids. [ijo.in]
Patients with ocular motor deficits in orbital apex syndromes caused by extension of nasopharyngeal rhabdomyosarcoma have an excellent prognosis for recovery after treatment of the tumor. The long-term prognosis for visual recovery, however, is poor. [ncbi.nlm.nih.gov]
We discuss rhino-orbital mucormycosis in detail, because it has a grave prognosis if it is not diagnosed and treated in a timely fashion. Access to the complete content on Oxford Medicine Online requires a subscription or purchase. [oxfordmedicine.com]
Epidemiology It is a rare condition and most of the literature is case reports rather than series [ 2, 3 ]. The estimated incidence of Tolosa Hunt syndrome (THS) is about one case per million per year [ 4 ]. [patient.info]
Dufour X, Kauffmann-Lacroix C, Ferrie JC, Goujon JM, Rodier MH, et al. (2006) Paranasal sinus fungal ball epidemiology, clinical features and diagnosis. A retrospective analysis of 173 cases from a single center in France, 1989-2002. [omicsonline.org]
Epidemiological profile of 277 patients with facial fractures treated at the emergency room at the Ent 248 5 Department of Hospital do Trabalhador in Curitiba/PR, in Int Arch Otorhinolaryngol. 2012; 16(4): Gabikian P, Chowdhary AM, Kott B, Lazar DA, Britz [docplayer.es]
RegelmaМГige Kontrollen des Schrittmachers sind acyclovir interactions medications. 446 Epidemiology. Brezincch. Renal vein thrombosis after martial arts trauma. Anz. Ast. [masa42.ru]
Raeder’s syndrome, also known as paratrigeminal oculosympathetic syndrome, is a rare disorder with very few epidemiological and case series studies available.  Table 1: Differentials for common misdiagnoses of superior orbital fissure syndrome The [eyewiki.aao.org]
The variable pathophysiology of acute orbital compartment syndrome has not been elucidated fully. [emedicine.medscape.com]
[…] from high impact forces.  Iatrogenic: sinonasal surgery, orbital/facial surgery Traumatic: penetrating, nonpenetrating, orbital apex fracture Vascular Carotid cavernous aneurysm, carotid cavernous fistula, cavernous sinus thrombosis Other: Mucocele Pathophysiology [eyewiki.aao.org]
Prompt diagnosis and treatment in cases of orbital apex syndrome, especially in immunocompromised patients, is important to prevent visual and life-threatening complications. [ncbi.nlm.nih.gov]
Our aim is to emphasize the importance of good glycemic control during any invasive procedure in a diabetic patient, to prevent any dreadful complications. References Kim IK, Kim JR, Jang KS, Moon YS, Park SW. [djo.org.in]
Supported in part by Research to Prevent Blindness, Inc, New York, NY; Fund for the New Jersey Blind, Newark, NJ; Lions Eye Research Foundation of New Jersey, Newark, NJ; The Eye Institute of New Jersey, Newark, NJ; and the Gene C. [healio.com]
V. 1 Without undergoing screening or preventive action, D, Doebbeling, B. [skoncept.ru]
- Bone I, Hadley D. Syndromes of the orbital fissure, cavernous sinus, cerebello-pontine angle, and skull base. J Neurol Neurosurg Psychiatry. 2005;76(Suppl 3):iii29-iii38.
- Aryasit O, Preechawai P, Aui-Aree N. Clinical presentation, aetiology and prognosis of orbital apex syndrome. Orbit. 2013;32(2):91-94.
- Sugamata A. Orbital apex syndrome associated with fractures of the inferomedial orbital wall. Clin Ophthalmol. 2013;7:475-478.
- Kurimoto T, Tonari M, Ishizaki N, et al. Orbital apex syndrome associated with herpes zoster ophthalmicus. Clin Ophthalmol. 2011;5:1603-1608.
- Imaizumi A, Ishida K, Ishikawa Y, Nakayoshi I. Successful Treatment of the Traumatic Orbital Apex Syndrome due to Direct Bone Compression. Craniomaxillofac Trauma Reconstr. 2014;7(4):318-322.
- Warburton RE, Brookes CC, Golden BA, Turvey TA. Orbital apex disorders: a case series. Int J Oral Maxillofac Surg. 2016;45(4):497-506.
- Gupta R, Khan YA. Traumatic orbital apex syndrome. Can J Ophthalmol. 2015;50(1):e8-e11.