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Orbital Apex Syndrome

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.


Presentation

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) [1] [2] [3] [4] [5]. This ailment can be seen in any decade of life, with some studies establishing a mean age of 45-50 years [2]. 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 [1] [5]. According to certain reports, blurred vision arising from reduced visual acuity and a fixed dilated pupil is the most frequent symptom in OAS [2] [3]. 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 [6] [7].

Fishing
  • Gnathostomiasis should be suspected in patients with an orbital apex syndrome who live or travel in an endemic area, have eaten raw fish, and develop a migratory skin rash.[ncbi.nlm.nih.gov]
Varicella-Zoster Virus Infection
  • Careful and long-term follow-up of patients with ophthalmic zona is required for possible ophthalmic complications of varicella zoster virus infections. A pericardial patch graft might be placed due to the development of necrotizing scleritis.[ncbi.nlm.nih.gov]
Trismus
  • A 65-year-old man became aware of pain around the right temporomandibular joint and mild trismus, and palsies of the cranial nerves III, IV, V, and VI were observed.[ncbi.nlm.nih.gov]
Unilateral Proptosis
  • Abstract Patients with orbital aspergillosis commonly present with unilateral proptosis and associated sinus disease.[ncbi.nlm.nih.gov]

Workup

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 [1] [7]. 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 [1]. 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 [1] [7]. 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 [1].

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]
Pseudomonas
  • Pseudomonas is an important mimicker to consider in sinusitis causing orbital apex syndrome, and it may occur following functional endoscopic sinus surgery.[ncbi.nlm.nih.gov]
Ischemic Changes
  • Ophthalmic examination showed right eye fixation in all directions and ischemic change of retina. Blood testing showed elevated muscle enzyme associated with muscle destruction.[ncbi.nlm.nih.gov]

Treatment

  • 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]
  • RESULTS: After treatment, we observed a substantial reduction in the size of the mass, but side effects of treatment developed. The visual acuity of the left eye was no light perception.[ncbi.nlm.nih.gov]
  • Patients had a good outlook, after treatment directed at the cause, with a modest improvement in vision and exophthalmos in more than half of patients.[ncbi.nlm.nih.gov]
  • In this review, differential diagnosis of the orbital apex syndrome (loss of cranial nerves II, III, IV, ophthalmic division of V, and VI) is outlined, and features, diagnosis, and treatment of mucormycosis are discussed.[ncbi.nlm.nih.gov]
  • Severe visual loss and ophthalmoplegia may persist despite prompt recognition and treatment.[ncbi.nlm.nih.gov]

Prognosis

  • The long-term prognosis for visual recovery, however, is poor.[ncbi.nlm.nih.gov]
  • CONCLUSIONS: The current study reinforces evidence that OAS indicates poor prognosis and highlights the importance of early diagnosis.[ncbi.nlm.nih.gov]
  • 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]
  • To achieve better prognosis and survival, clinicians should be aware of this distinct manifestation.[ncbi.nlm.nih.gov]
  • OBJECTIVES: To present the clinical features, determine the causes and evaluate the prognosis of orbital apex syndrome in patients of Songklanagarind Hospital.[ncbi.nlm.nih.gov]

Etiology

  • A thorough clinical workup is mandatory in order to raise clinical suspicion, whereas imaging studies are necessary to confirm the underlying etiology.[symptoma.com]
  • Orbital apex syndrome is symptomatically related to superior orbital fissure syndrome and cavernous sinus syndrome with similar etiologies.[eyewiki.aao.org]
  • Corticosteroids may be useful if an inflammatory etiology is suspected, but should be used with caution.[ncbi.nlm.nih.gov]
  • Treat as per disease etiology 23. Swamy BN, McCluskey P, Nemet A, Crouch R, Martin P, Benger R, Ghabriel R, Wakefield D. Idiopathic orbital inflammatory syndrome: Clinical features and treatment outcomes.[slideshare.net]
  • […] cell arteritis Thyroid disease Traumatic causes [ edit ] Iatrogenic (following surgery) Orbital apex fracture Penetrating injury Vascular causes [ edit ] Carotid aneurysm Diagnosis [ edit ] Diagnostic methods vary, and are based on specific possible etiologies[en.wikipedia.org]

Epidemiology

  • The discussion covers the phenotype spectrum, epidemiology, mode of inheritance, pathogenesis, and clinical profile of each condition, all of which is accompanied by a wealth of illustrations.[books.google.com]
  • 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]
  • RegelmaМГige Kontrollen des Schrittmachers sind acyclovir interactions medications. 446 Epidemiology. Brezincch. Renal vein thrombosis after martial arts trauma. Anz. Ast.[masa42.ru]
  • 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]
Sex distribution
Age distribution

Pathophysiology

  • The variable pathophysiology of acute orbital compartment syndrome has not been elucidated fully.[emedicine.medscape.com]
  • […] from high impact forces. [6] 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]
  • Fifty-nine years after the description of this condition, the pathophysiological mechanisms and etiopathogenesis of the disease still remain controversial and obscure.[bjorl.org]
  • Although the pathophysiology of cranial nerve involvement is not yet clear, it is suggested to be due to either direct viral invasion or a combination of inflammation and occlusive vasculitis. 11-13 Treatment of HZO is mainly antivirals.[reviewofophthalmology.com]
  • , ischemia and microinfarction of axons due to damage to pial microvasculature, direct bony impingement with a canal fracture, and continued edema and hemorrhage within the closed space of the optic canal all have been proposed to play a role in the pathophysiology[emedicine.medscape.com]

Prevention

  • Prompt diagnosis and treatment in cases of orbital apex syndrome, especially in immunocompromised patients, is important to prevent visual and life-threatening complications. 2012 The Authors.[ncbi.nlm.nih.gov]
  • 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]
  • A vaccine to prevent herpes zoster and postherpetic neuralgia in older adults. N Engl J Med, 2005; 352 :2271–2284. [ PubMed ] [ Google Scholar ] 7. Arda H, Mirza E, Gümüş K, Oner A, Karakucuk S, Sırakaya E.[ncbi.nlm.nih.gov]
  • V. 1 Without undergoing screening or preventive action, D, Doebbeling, B.[skoncept.ru]
  • When treating a nasal septal abscess, serious complications could be prevented by prompt, effective surgery [ 14 ].[bmcinfectdis.biomedcentral.com]

References

Article

  1. 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.
  2. Aryasit O, Preechawai P, Aui-Aree N. Clinical presentation, aetiology and prognosis of orbital apex syndrome. Orbit. 2013;32(2):91-94.
  3. Sugamata A. Orbital apex syndrome associated with fractures of the inferomedial orbital wall. Clin Ophthalmol. 2013;7:475-478.
  4. Kurimoto T, Tonari M, Ishizaki N, et al. Orbital apex syndrome associated with herpes zoster ophthalmicus. Clin Ophthalmol. 2011;5:1603-1608.
  5. 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.
  6. Warburton RE, Brookes CC, Golden BA, Turvey TA. Orbital apex disorders: a case series. Int J Oral Maxillofac Surg. 2016;45(4):497-506.
  7. Gupta R, Khan YA. Traumatic orbital apex syndrome. Can J Ophthalmol. 2015;50(1):e8-e11.

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Last updated: 2018-06-22 01:47