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ORBITAL APEX SYNDROME
Dr. Reshma Peter
APEX OF ORBIT
• Posterior most end of pyramid shaped orbit
• 4 orbital walls converge here at craniofacial junction
• Comp...
SOF
• bony cleft at orbital apex
•Lies b/w lateral wall and the roof of the orbit with optic strut at its
superomedial mar...
•pear-shaped with a broad base
•long axis extends upward at an angle of 45° from the base
medially to the apex directed su...
• Annulus of zinn encircles the optic foramen and central part of
SOF,dividing it into upper,middle and lower part
• The s...
The middle part
confined within the tendinous ring
more susceptible to shearing injury during craniofacial trauma
contain...
Lower part:
• Inferior ophthalmic vein
The inferior venous compartment is given by the confluence
of the SOV and IOV which...
Radiographic enlargement of the superior orbital fissure
may accompany pathologic processes such as
Aneurysms, Meningioma,...
•Located within the lesser wing of sphenoid
• It connects the orbit to the middle cranial fossa
•From an anterior view, th...
• 2 bony roots that connect the lesser wing of the sphenoid
with the body of the sphenoid form the optic canal.
• The infe...
Optic canal
Optic foramen:
Vertically : 6-6.5mm
horizontally: 4.5-5mm
>7mm : Abnormal
(enlarges in optic nerve
gliomas, Me...
• Each optic canal passes posteromedially at an angle of
approximately 35° to the sagittal
• opens posteriorly into the ch...
•Throughout its intraorbital and intracanalicular course, the optic
nerve is surrounded by pia mater, arachnoid, and dura ...
Inferior orbital fissure
 20-mm bony defect
 Lies between lateral wall and floor of the orbit
 bounded by the :
• sphen...
Structures passing through it:
Zygomatic nerve (V2)
 Infraorbital nerve (V2)
Infraorbital artery
 Infraorbital vein
 ...
Superior orbital fissure syndrome applies to lesions located
immediately anterior to the orbital apex, including the struc...
Syndrome Definition
Orbital apex syndrome involves damage to
oculomotor nerve (III)
trochlear nerve (IV)
abducens nerve...
Cavernous sinus syndrome
 (involvement of cranial nerves III, IV, V1, V2, VI, and periarterial
sympathetic plexus)
•sensor...
• Traumatic optic neuropathy (involvement of cranial
nerve II): The intracanalicular optic nerve may be
damaged by sphenoi...
• The superior orbital fissure, orbital apex, and cavernous
sinus are all contiguous, and although these terms
define the ...
Anterior Syndromes: Total Ophthalmoplegia
Posterior Syndromes: Trigeminal Involvement
Inflammatory (OID)
1. Thyroid orbitopathy
2. Sarcoidosis
3. Wegeners granulomatosis
4. Giant cell arteritis
5. Orbital inf...
Infectious
1. Fungi: Aspergillosis, Mucormycosis
2. Bacteria: Streptococcus spp., Staphylococcus spp.,
Actinomyces spp., G...
Iatrogenic
1. Sinonasal surgery
2. Orbital/facial surgery
Neoplastic
1. Head and neck tumors: nasopharyngeal carcinoma, ad...
Clinical Presentation
• Vision Loss
• Uniocular Diplopia
• Ophthalmoplegia
• Periorbital/Facial Pain
• Axial Proptosis
• P...
History taking
• h/o blunt orbital trauma
• h/o visual loss- whether at the time of injury or subsequently.
Progressive de...
Examination
• Initial management in facial injuries assessing the airway
security,hemodynamic stability, and cervical spin...
• Visual acuity :including pinhole vision and colour vision of
Each eye must be recorded.
• Confrontation visual fields ma...
• Assessment of ocular motility:
• Volitional movements are examined at the bedside
• forced ductions and force generation...
Orbital inspection, palpation, and assessment of globe position
•Periocular ecchymosis, edema, and proptosis in trauma
•Or...
• subcutaneous or intraorbital emphysema.-due to Disruption of
the mucosal integrity of the maxillary or ethmoidal sinus
•...
• Anterior segment trauma including corneal injury, hyphema,
iridodialysis, lens dislocation, and posterior segment trauma...
CT scanning
• In facial trauma and suspected fractures, noncontrast CT
scans - most appropriate initial imaging technique....
• Coexistent cervical injury may preclude direct coronal
projections.
• Reconstructed coronal views may be needed in patie...
Plain radiography
The orbital apex may be visualized with 2 radiographic projections
AP view for the superior orbital fiss...
MRI
• The poor resolution of bone on MRI significantly limits its
role in general orbital trauma.
• However, better soft t...
• The best resolution of orbital structures is presently obtained by MRI
using standard T1w or T2w TSE pulse sequences.
• ...
Newer functional MRI (fMRI) with blood-oxygenation-level-
dependent (BOLD) techniques and fNMR MRI can evaluate retinal
ph...
Angiography
Angiography may be considered in patients with
 orbital apex fractures
 with clinical features consistent wi...
Visual field assessment
• Automated static threshold perimetry (eg, Humphrey
Visual Field analysis) or
• Kinetic perimetry...
Formal color testing
• Dyschromatopsia is expected in optic neuropathy
• It may be formally documented with use of the
Far...
Visual-evoked potentials
(VEP)
• may assess the integrity of the visual pathway
• Able to compare pathways from each eye.
...
Beta-2 transferrin
a definitive test for CSF rhinorrhea
Infections/DM/Tuberculosis/Syphilis/TAO/NSOI/GCA
Vasculitis including SLE, RA, Wegners Granulomatosis,
Sarcoidosis, Anemia...
Flowchart for OAS Assessment
Management of
OAS
Observation
Medical
Management
Surgical
Management
• Infectious:
 Antibiotics
 Antifungals
 Antiviral...
THANK YOU
Orbital apex syndrome
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Orbital apex syndrome Slide 1 Orbital apex syndrome Slide 2 Orbital apex syndrome Slide 3 Orbital apex syndrome Slide 4 Orbital apex syndrome Slide 5 Orbital apex syndrome Slide 6 Orbital apex syndrome Slide 7 Orbital apex syndrome Slide 8 Orbital apex syndrome Slide 9 Orbital apex syndrome Slide 10 Orbital apex syndrome Slide 11 Orbital apex syndrome Slide 12 Orbital apex syndrome Slide 13 Orbital apex syndrome Slide 14 Orbital apex syndrome Slide 15 Orbital apex syndrome Slide 16 Orbital apex syndrome Slide 17 Orbital apex syndrome Slide 18 Orbital apex syndrome Slide 19 Orbital apex syndrome Slide 20 Orbital apex syndrome Slide 21 Orbital apex syndrome Slide 22 Orbital apex syndrome Slide 23 Orbital apex syndrome Slide 24 Orbital apex syndrome Slide 25 Orbital apex syndrome Slide 26 Orbital apex syndrome Slide 27 Orbital apex syndrome Slide 28 Orbital apex syndrome Slide 29 Orbital apex syndrome Slide 30 Orbital apex syndrome Slide 31 Orbital apex syndrome Slide 32 Orbital apex syndrome Slide 33 Orbital apex syndrome Slide 34 Orbital apex syndrome Slide 35 Orbital apex syndrome Slide 36 Orbital apex syndrome Slide 37 Orbital apex syndrome Slide 38 Orbital apex syndrome Slide 39 Orbital apex syndrome Slide 40 Orbital apex syndrome Slide 41 Orbital apex syndrome Slide 42 Orbital apex syndrome Slide 43 Orbital apex syndrome Slide 44 Orbital apex syndrome Slide 45 Orbital apex syndrome Slide 46 Orbital apex syndrome Slide 47 Orbital apex syndrome Slide 48 Orbital apex syndrome Slide 49
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Orbital apex syndrome

  1. 1. ORBITAL APEX SYNDROME Dr. Reshma Peter
  2. 2. APEX OF ORBIT • Posterior most end of pyramid shaped orbit • 4 orbital walls converge here at craniofacial junction • Complex association b/w bony, neural, and vascular elements • Has 2 orifices situated in the sphenoid bone  Optic foramen  Superior orbital fissure
  3. 3. SOF • bony cleft at orbital apex •Lies b/w lateral wall and the roof of the orbit with optic strut at its superomedial margin •bounded by greater and lesser wing of sphenoid •the largest communication between orbit and middle cranial cavity •It is situated lateral to optic foramen
  4. 4. •pear-shaped with a broad base •long axis extends upward at an angle of 45° from the base medially to the apex directed superotemporally. •SOF is divided at the spina recti lateralis by the annulus of Zinn, the common tendinous origin of the recti muscles. DIMENSIONS Length: around 22 mm Width : 2-3 mm at the apex 7-8 mm at the base
  5. 5. • Annulus of zinn encircles the optic foramen and central part of SOF,dividing it into upper,middle and lower part • The superior part contains Trochlear nerve (IV) Frontal and lacrimal branches Of the ophthalmic division Of the trigeminal nerve (V1) Superior branch of ophthalmic vein Recurrent branch of the lacrimal artery(occasionally)
  6. 6. The middle part confined within the tendinous ring more susceptible to shearing injury during craniofacial trauma contains  Superior and inferior branches of the oculomotor nerve (III)  Nasociliary nerves (V1)  Abducens nerve (VI)  Inferior branch of ophthalmic vein  Fibers from the internal carotid sympathetic plexus
  7. 7. Lower part: • Inferior ophthalmic vein The inferior venous compartment is given by the confluence of the SOV and IOV which drain into the cavernous sinus
  8. 8. Radiographic enlargement of the superior orbital fissure may accompany pathologic processes such as Aneurysms, Meningioma, Choroidoma,pituitary adenoma and Tumors of the orbital apex
  9. 9. •Located within the lesser wing of sphenoid • It connects the orbit to the middle cranial fossa •From an anterior view, the entrance to the optic canal is the most superior and medial structure in the apex. • Attains adult dimensions by age 3 and is symmetric in most persons Optic Foramen
  10. 10. • 2 bony roots that connect the lesser wing of the sphenoid with the body of the sphenoid form the optic canal. • The inferior root separates the optic canal from SOF and also is referred to as the optic strut. • The superior root forms the roof of the optic canal and separates it from the anterior cranial fossa. • The body of the sphenoid forms the medial wall of the canal.
  11. 11. Optic canal Optic foramen: Vertically : 6-6.5mm horizontally: 4.5-5mm >7mm : Abnormal (enlarges in optic nerve gliomas, Meningiomas) Optic canal: length : 8 to 10 mm width : 5 to 7 mm lateral wall is shortest medial wall is longest Structures passing through it •Optic nerve and its meningeal covering •Ophthalmic artery •Sympathetic nerves
  12. 12. • Each optic canal passes posteromedially at an angle of approximately 35° to the sagittal • opens posteriorly into the chiasmatic groove (which terminates posteriorly at the tuberculum sellae). • The canal has an intimate relationship to the sphenoid sinus, and with extensive sinus pneumatization, the optic canal may become completely surrounded by a posterior ethmoidal Onodi air cell, the sphenoid sinus, or an aerated anterior clinoid process.
  13. 13. •Throughout its intraorbital and intracanalicular course, the optic nerve is surrounded by pia mater, arachnoid, and dura mater, giving the nerve a sheath. •Thus, optic nerve is a white matter tract of the brain and carries with it meningeal coverings. •Within the orbit, the optic nerve is quite mobile however, within the canal, the optic nerve sheath remains adherent to the sphenoid periosteum and thus is fixed. •Optic nerve glioma or Meningioma may lead to unilateral enlargement of Optic canal, seen on Xrays
  14. 14. Inferior orbital fissure  20-mm bony defect  Lies between lateral wall and floor of the orbit  bounded by the : • sphenoid • zygomatic • maxillary • palatine bones  Communicates orbit with inf temporal fossa and pterygopalatine fossa
  15. 15. Structures passing through it: Zygomatic nerve (V2)  Infraorbital nerve (V2) Infraorbital artery  Infraorbital vein  br from Inferior ophthalmic vein leading to pterygoid plexus Max division of trigeminal Nerve Parasympathetics to lacrimal gland Orbital Br from pterygopalatine ganglion
  16. 16. Superior orbital fissure syndrome applies to lesions located immediately anterior to the orbital apex, including the structures exiting the annulus of Zinn and often those external to the annulus as multiple cranial nerve palsies may be seen in the absence of optic nerve pathology. Features • ophthalmoplegia, • upper eyelid ptosis • nonreactive dilated pupil • anesthesia over the ipsilateral forehead, • loss of corneal sensation (and hence loss of corneal reflex • orbital pain • Axial proptosis. • neurotrophic keratopathy]
  17. 17. Syndrome Definition Orbital apex syndrome involves damage to oculomotor nerve (III) trochlear nerve (IV) abducens nerve (VI) ophthalmic branch of the trigeminal nerve (V1) with optic nerve (II) dysfunction The orbital apex syndrome is a SOF syndrome with loss of vision. Cavernous sinus syndrome (CSS) may include the features of an OAS with added involvement of the maxillary branch of the trigeminal nerve (V2) oculo-sympathetic fibers more commonly bilateral
  18. 18. Cavernous sinus syndrome  (involvement of cranial nerves III, IV, V1, V2, VI, and periarterial sympathetic plexus) •sensory deficits in the maxillary branch of the trigeminal nerve orbital sympathetic innervation involvement •Traumatic carotid-cavernous fistula may be present. •vascular congestion • proptosis •Chemosis •Ophthalmoplegia •elevated intraocular pressure (IOP) •vascular bruit CSF rhinorrhea  in fracture involving the sphenoid sinus, fovea ethmoidalis, or cribriform plate.
  19. 19. • Traumatic optic neuropathy (involvement of cranial nerve II): The intracanalicular optic nerve may be damaged by sphenoid fractures • The firm attachment of the dural sheath to the optic nerve may make the intracanalicular nerve particularly susceptible to acceleration or deceleration injuries.
  20. 20. • The superior orbital fissure, orbital apex, and cavernous sinus are all contiguous, and although these terms define the precise anatomic locations of a disease process, the etiologies of these syndromes are similar. • In some instances, patients who have features of a SOFS may subsequently develop orbital apex and cavernous sinus pathology.
  21. 21. Anterior Syndromes: Total Ophthalmoplegia
  22. 22. Posterior Syndromes: Trigeminal Involvement
  23. 23. Inflammatory (OID) 1. Thyroid orbitopathy 2. Sarcoidosis 3. Wegeners granulomatosis 4. Giant cell arteritis 5. Orbital inflammatory pseudo tumor 6. Tolosa hunt syndrome Vascular 1. Carotid cavernous aneurysm 2. Carotid cavernous fistula 3. Cavernous sinus thrombosis 4. Sickle cell anemia Etiology of Orbital Apex Syndrome
  24. 24. Infectious 1. Fungi: Aspergillosis, Mucormycosis 2. Bacteria: Streptococcus spp., Staphylococcus spp., Actinomyces spp., Gram-negative bacilli, anaerobes, Mycobacterium tuberculosis 3. Spirochetes: Treponema pallidum 4. Viruses: Herpes zoster Traumatic 1. Penetrating injury 2. Non penetrating injury 3. Orbital apex fracture 4. Retained foreign body
  25. 25. Iatrogenic 1. Sinonasal surgery 2. Orbital/facial surgery Neoplastic 1. Head and neck tumors: nasopharyngeal carcinoma, adenoid cystic carcinoma, squamous cell carcinoma 2. Neural tumors: neurofibroma, meningioma, ciliary neurinoma, schwannoma, gliomas 3. Metastatic lesions: lung, breast, renal cell, malignant melanoma 4. Hematologic: Burkitt lymphoma, non-Hodgkin lymphoma, leukemia 5. Perineural invasion of cutaneous malignancy Etiology of Orbital Apex Syndrome
  26. 26. Clinical Presentation • Vision Loss • Uniocular Diplopia • Ophthalmoplegia • Periorbital/Facial Pain • Axial Proptosis • Ptosis • Ocular Deviation • Headache • Loss of sensations over the face
  27. 27. History taking • h/o blunt orbital trauma • h/o visual loss- whether at the time of injury or subsequently. Progressive decrease in vision suggests optic neuropathy due to hemorrhage into the optic nerve sheath, retrobulbar hematoma, compression by a bony fragment, or possibly arachnoiditis at the site of fracture. • h/o diplopia  binocular misalignment. Diplopia will be worse in the field of gaze of the paretic muscle. • h/o ptosis • Past ophthalmic history- antecedent spectacles, decreased vision, amblyopia, strabismus, and previous ocular surgery. • h/o Sensory disturbances in the distribution of V1 and V2
  28. 28. Examination • Initial management in facial injuries assessing the airway security,hemodynamic stability, and cervical spine integrity. • An assessment of neurologic status must be made, and head injuries must be excluded. • Additional soft tissue and bony injuries of the head and neck must be sought. • In patients with suspected orbital apex fractures, the examination should focus on an assessment for the presence of following that may demand acute intervention  an optic neuropathy  an evolving orbital compartment syndrome, or  ruptured globe
  29. 29. • Visual acuity :including pinhole vision and colour vision of Each eye must be recorded. • Confrontation visual fields may be performed at the bedside prior to more formal perimetric assessment. • Assessment of pupil responses: The direct and consensual light responses An absolute or relative afferent pupil defect or an efferent pupil defect (as seen in third nerve palsy, ciliary ganglion injury, and traumatic mydriasis) is recorded.
  30. 30. • Assessment of ocular motility: • Volitional movements are examined at the bedside • forced ductions and force generation examinations are undertaken with appropriate topical anesthesia and patient cooperation. These assessments help differentiate between ocular motility disturbance caused by entrapped muscles, intramuscular hematoma, and nerve damage. • Assessment of integrity of cranial nerve V: Sensory disturbances should be sought in the territories of branches of V1 and V2.
  31. 31. Orbital inspection, palpation, and assessment of globe position •Periocular ecchymosis, edema, and proptosis in trauma •Orbital hematoma, intraorbital emphysema, and orbital volume changes with orbital wall fractures all alter the globe position. •Axial displacement of the globe should be assessed by exophthalmometry. •Increased resistance to globe retropulsion is seen with orbital hemorrhage.
  32. 32. • subcutaneous or intraorbital emphysema.-due to Disruption of the mucosal integrity of the maxillary or ethmoidal sinus • Orbital rim fractures • Traumatic telecanthus is seen in naso-orbito-ethmoid (NOE) fractures and lateral canthal dystopia is seen in displaced zygomaxillary complex (ZMC) fractures. • exclude a coexistent globe rupture or injury. • IOP is recorded.
  33. 33. • Anterior segment trauma including corneal injury, hyphema, iridodialysis, lens dislocation, and posterior segment trauma including retinal commotio, retinal detachment, choroidal rupture, and scleral rupture. • Pharmacologic pupil dilation for fundus examination to assess optic nerve head perfusion, disc swelling, and peripapillary hemorrhages. • In patients with head injuries, pharmacological pupil dilation should only be undertaken after neurosurgical consultation
  34. 34. CT scanning • In facial trauma and suspected fractures, noncontrast CT scans - most appropriate initial imaging technique.  • Associated intracranial injury, associated facial fractures, and intraorbital hematoma may be assessed. • Axial and coronal views 3-mm cuts review the orbit, and 1-mm axial cuts may be used to assess the optic canal.
  35. 35. • Coexistent cervical injury may preclude direct coronal projections. • Reconstructed coronal views may be needed in patients with neck injury. • Best images of relationship between the bone and soft tissues • Metallic orbital foreign bodies
  36. 36. Plain radiography The orbital apex may be visualized with 2 radiographic projections AP view for the superior orbital fissure oblique view for the optic foramen
  37. 37. MRI • The poor resolution of bone on MRI significantly limits its role in general orbital trauma. • However, better soft tissue differentiation may be obtained. • MRI reveals the abnormal signal indicative of recent hemorrhage in optic nerve sheath hematoma.  • Associated neurological damage • Wooden foreign bodies
  38. 38. • The best resolution of orbital structures is presently obtained by MRI using standard T1w or T2w TSE pulse sequences. • Fat appears hyperintense (bright) on T1w and T2w images, and other structures, such as vessels, nerves, and muscles, appear darker (hypointense) than orbital fat. • Gd-DTPA enhances vascular structures, such as cavernous sinus or the venous plexus surrounding Meckel’s cave and the hypophysis. • Fat suppression techniques like STIR with or without contrast enhancement are especially useful for the diagnosis of retrobulbar optic neuritis and intraorbital meningiomas. Townsend ,Clinical application of MRI in ophthalmology., NMR Biomed
  39. 39. Newer functional MRI (fMRI) with blood-oxygenation-level- dependent (BOLD) techniques and fNMR MRI can evaluate retinal physiology and oxygenation. PET, SPECT, MRS with NAA, DSA and MRA/MVA with MOTSA can aid in diagnosis. Although MRI and magnetic resonance angiography may be helpful in diagnosing intracranial aneurysms or shunts at the cavernous sinus, the “gold standard” for intracranial vascular disease is catheter angiography and super selective vessel exploration.
  40. 40. Angiography Angiography may be considered in patients with  orbital apex fractures  with clinical features consistent with a carotid artery injury, revealing  carotid artery dissection  carotid artery spasm  carotid-cavernous fistula
  41. 41. Visual field assessment • Automated static threshold perimetry (eg, Humphrey Visual Field analysis) or • Kinetic perimetry (eg, Goldmann perimetry) • used in patients with adequate cooperation and fixation to document visual field disturbance with optic neuropathy. • No specific visual field loss pattern is pathognomonic for traumatic optic neuropathy.
  42. 42. Formal color testing • Dyschromatopsia is expected in optic neuropathy • It may be formally documented with use of the Farnsworth-Munsell 100 hue test or the Farnsworth panel D-15. • These tests require patient cooperation and may not be appropriate in the acute setting.
  43. 43. Visual-evoked potentials (VEP) • may assess the integrity of the visual pathway • Able to compare pathways from each eye. • They are a consideration in patients with altered level of consciousness or in whom bilateral optic neuropathy is suspected
  44. 44. Beta-2 transferrin a definitive test for CSF rhinorrhea
  45. 45. Infections/DM/Tuberculosis/Syphilis/TAO/NSOI/GCA Vasculitis including SLE, RA, Wegners Granulomatosis, Sarcoidosis, Anemia Evaluation Infections, Tuberculosis, HSV, Sarcoidosis, LGBS, Invasive Aspergillosis, Lymphoproliferative disorders Granulomas, Mass Lesions, Fractures Confirmation of Diagnosis
  46. 46. Flowchart for OAS Assessment
  47. 47. Management of OAS Observation Medical Management Surgical Management • Infectious:  Antibiotics  Antifungals  Antivirals • Non Infectious:  Corticosteroids  Immunosuppressant • Spontaneous resolution under 4 weeks e.g.: OID, TAO etc. • Orbitotomies • Endoscopic decompression • Tumor removal/ debulking surgery
  48. 48. THANK YOU
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