causes of painful opthalmoplegia

1. PAINFUL
OPHTHALMOPLEGIA
Dr Jahnara J
DNB Resident

2. • Implies absence of ocular movements-
indicates paralysis, weakness or restriction of
extraocular muscles
• Various classifications
internal/external
total/partial
pupil involving/sparing
painful/ painless

3. Painful ophthalmolegia is
•periorbital or hemicranial pain plus any
combination of
•ipsilateral ocular motor palsies &/
•oculo-sympathetic palsy &/
•sensory loss in distribution of ophthalmic and
maxillary division of trigeminal nerve.”

4. SUPERIOR ORBITAL FISSURE

7. Complete & Incomplete SOFS
Complete SOFS : when all the cranial nerves
entering through the SOF are involved.
Incomplete SOFS : when any of the cranial
Nerves ( generally CN VI) is spared
Optic nerve involvement is absent

8. Orbital apex syndrome
Lesion is more posterior
Involvement of III, IV, VI, ophthalmic Br of V with
optic nerve dysfunction

9. CAVERNOUS SINUS SYNDROME

10.  Anterior - extends into medial end of superior orbital fissure.
 Posterior - upto apex of petrous temporal bone.
 Medial – Pitutary above and sphenoid below
 Lateral – temporal lobe and uncus
 Superior – optic chiasma
 Inferior - endosteal

11. VASCULAR
INFLAMMATORY INFECTIOUS
NEOPLASTIC
MISCELLANEOUS

12.  Ischemic Neuralgia
 Intracavernous carotid artery aneurysm
 Posterior communicating artery aneurysm
 Carotid cavernous fistula
 Cavernous sinus thrombosis
 Carotid dissection

13.  Orbital pseudotumour
 Tolosa-Hunt syndrome
 SOFS & Orbital apex Syndrome
 Giant cell arteritis
 Thyroid ophthalmopathy
 Wegeners granulomatosis
 Sarcoidosis
 Idiopathic hypertrophic pachymeningitis

14.  Fungal (mucormycosis, actinomycosis)
 Mycobacterial (tuberculosis)
 Bacterial
 Viral: Herpes Zoster

15. • PRIMARY CRANIAL TUMORS;
pituitary adenoma, meningioma, sarcoma,
gasserian ganglion neuroma
• LOCAL METASTASIS
nasopharyngeal carcinoma, cylindroma,
chordoma
• DISTANT METASTASIS
lymphoma, neuroblastoma, multiple
myeloma

16.  Severe pain in eye and forehead may precede
3rd N palsy in diabetes, hypertension and GCA
 In ophthalmic div area
 Due to ischemic occlusive changes in the dural
vessels supplying the nerves in cavernous
sinus

17. Presence of pupil sparing/ involvement is used to
distinguish patients requiring neuroradialogical
investigations Vs investigations for a vascular
disease

18. SITE
• ICA- 85%
• Post comm A :25-40%
• Ant comm A: 25-30%
• MCA: 13-30%
• Basilar A :3-9 %

20. • Pain is due to compression of trigeminal nerve,
• stretching of vessel wall or
• leaking in to subarachnoid space
90% Of cases, pain precedes ophthalmoplegia

21. VI NERVE PALSY OTHER FEATURES
 Direct effect of
aneurysm/
 False localising sign of
SAH
•Basilar artery aneurysm
present with pupil paresis
along with no invt of EOM

22.  Unique; grow to large size without rupturing
 2-6 % of intracranial aneurysms
 Rupture into cavernous sinus: CCF
 symptoms may result from compression of
structures within the cavernous sinus…. ocular
motor nerves, the oculosympathetic pathway,
and the first and second divisions ofthe
trigeminal nerve

23. • Symptoms.. diplopia (89%), retrobulbar
pain (61%), headache (19%), blurred vision
(14%)

24.  involve the abducent nerve early.
 The pupil sparing third nerve palsy

25. • Transfemoral cerebral angiography “gold
standard”
about site, size, relationship with the parent
vessel ,perforators..
• CT scan :SAH
• MRI scan : aneurysms that are larger than 5
mm. degree of intramural thrombus in giant
aneurysms.

26. Abnormal communications between the carotid
arterial system and the venous cavernous sinus.
MECHANISMS
•Trauma (75%): High flow basal skull fracture
• Spontaneous causes (25%): rupture of intracavernous
aneurysms, collagen vascular diseases,
neurofibromatosis

27. compression and ischemia related to increased
venous pressure and reduced arterial pressure.
The direction of blood flow through a direct CCF
may be
Posterior: into the superior and inferior petrosal
sinuses, or
anterior: into the orbital veins (severe features)

28. PROPTOSIS
Develops rapidly
pulsating
CONJUCTIVAL CHEMOSIS
Limited to interpalpebral bulbar, inferior
palpebral conjuctiva
Arterialisation of conjuntival/ episcleral veins:
HALLMARK

29. • DIPLOPIA
III Nerve palsy :compression by intra
cavernous aneursym prior to rupture,
traumatic nerve palsy in direct CCF,
compression by fistula/ reduced flow through
vaso vasorum of oculomotor nerve
• VI Nerve palsy
Most common, due to it location near ICA
• Mechanical restriction due to orbital edema

30. • Pulsating exophthalmos
• Exposure keratopathy
• Ocular pulsations and bruits
• Glaucoma
• CRVO
• Choroidal effusion/ detachment

32.  Arteriography : gold standard
 CECT and MRI : Dilated cavernous sinus and
superior ophthalmic vein
 MRA

33.  placement of intravascular coils,
 carotid artery ligation or finally
 surgical clipping

36. Cavernous Sinus
“Communications & Sources of infection”
• Face, nose PNS, orbit
Anterior foci –
Ophthalmic veins
• Middle ear and mastoid, lateral sinus phlebitis
Posterior foci –
Petrosal sinuses
• Meningitis, cerebral abscesses
Superior foci –
Cerebral veins
• Peritonsillar abscess, maxillary diseases
Inferior foci –
Pterygoid plexus
• From one side to other
Medial foci –
Intercavernous sinuses
• Sphenoidal sinus, nasal septum, turbinates
Internal foci – ethmoidal
veins/emissary veins

37. Etiology of CST
Septic CST
• Infectious
Aseptic CST
Trauma
Postsurgery
• Rhinoplasty
• Basal skull (including maxillary)
• Tooth extraction
Hematologic
• Polycythemia rubra vera
• Acute lymphocytic leukemia
Malignancy
• Nasopharyngeal tumor
Other
• Ulcerative colitis
• Dehydration
• Heroin

38. Ocular manifestation of cavernous sinus thrombosis
SIGN INVOLVED STRUCTURES
Ptosis Edema of upper eye lid
Sympathetic plexus
III cranial nerve
Chemosis Thrombosis of superior and inferior
ophthalamic vein
Proptosis Venous engorgement
Sensory loss/ Periorbital pain V cranial nerve
Corneal ulcers Corneal exposure due to proptosis
Complete ophthalmoplegia CN III, IV, VI
Decreased visual acuity or blindness Central retinal artery/ vein occlusion
secondary to ICA arteritis, septic emboli,
ischemic optic neuropathy

39.  The mainstay of therapy is early and aggressive
antibiotic administration.
 Although S aureus is the usual cause, broad-spectrum
coverage for gram-positive, gram-negative,
and anaerobic organisms should be instituted pending
the outcome of cultures.
 The indication of anticoagulation is still
debated because of possible bleeding

40.  Hemorrhagic infarction of a pituitary
adenoma/tumor.
 Considered a neurosurgical emergency.
 Precipitated by pregnacy, radiation and trauma
Presentation:
 Variable onset of severe headache
 Nausea and vomiting
 Meningismus
 Unilateral/Bilateral ophthalmoplegia due to mass
effect
 Drowsiness,coma, subarachnoid hemorhage

41.  Diagnose with CT/MRI
 Differentiate from leaking aneurysm
 Treatment:
 Surgical - Transsphenoid decompression
 Visual defects and altered consciousness
 Medical therapy – if symptoms are mild
 Corticosteroids

42.  IDIOPATHIC INFLAMMATORY DISORDERS
1. Tolosa-hunt syndrome
2. Orbital pseudotumor and
3. idiopathic hypertrophic pachymeningitis
No systemic or constitutional features
Diagnosis of exclusion

43.  Non specific granulomatous inflammation
ofcavernous sinus or superior orbital fissure.
 Diplopia with ipsilateral periorbital or
hemicranial pain, ( steady, boring)
 3rd, 4th , 6th , & 1st division of trigeminal
nerve are involved
 Horner’s syndrome may be present
 Characterised by remission,relapses,high ESR&
response to steroid

44.  INVESTIGATIONS
CBC, CSF: inconclusive
CAROTID ANGIOGRAPHY: Abnormal
configuration of intracavernous carotid artery
ORBITAL VENOGRAPHY: Superior
ophthalmic vein occlusion. Partial/ absent
filling of cavernous sinus
TREATMENT
Corticosteroids

45.  NSOI is a benign inflammatory process of the
orbit characterized by a polymorphous
lymphoid infiltrate with varying degrees of
fibrosis, without a known local or systemic
cause.
 etiology :unknown. ?infectious/immune
 Ass with Crohn's disease, systemic lupus
erythematous, rheumatoid
arthritis, myasthenia gravis, and ankylosing
spondylitis

46. Presentation
 Unilateral presentation
 Pain followed by diplopia
 Proptosis/ erythema/swelling
 Vision loss occassionally
Work up
CBC, Metabolic panel, thyroid function
tests,ANCA, RA Factor
 Imaging: CT scan

48.  Enlargement of the EO muscles
 Unilateral single muscle inflammation with
tendon involvement is most common.
 MR> SR> LR> IR
 Enlargement of muscle belly and tendon
 There may be infiltrates throughout the orbital
fat bordering the muscle, blurring the margin of
the muscle

49.  NSAIDs, such as ibuprofen, have been used in
mild cases of NSOI
 SYSTEMIC CORTICOSTEROIDS mainstay
therapy for NSO
 RADIATION THERAPY: when NSOI is found
to be resistant to or intolerant to corticosteroid
 Other options: Calcineurin inhibitors, MABS

50.  Pediatric NSOI differs from the adult
presentation and is more commonly
characterized by bilateral manifestation,
uveitis, disc edema

51.  Diffuse thickening of dura with inflammation
 Headache and cranial nerve palsies
 Diagnosis of exclusion
 Serological & CSF evaluation , gadolinium
enhanced MRI and biopsy of duramater or
orbital tissue.

52.  Idiopathic granulomatous inflammation of
multiple organs
 young adults 20–40
 Ocular invt 25-50% case, but orbital
inflammation <1%
 Symptoms similar to pseudotumor
 The lacrimal gland is the most commonly
affected orbital tissue

53.  Isolated orbital involvement is rare
 DIAGNOSIS: clinical, lab, and radiographic
CXR: hilar lymphadenopathy/ fibrosis
ACE: increased
Gallium Scan: Abnormal uptake in lacrimal
gland/pulmonary hila
Biopsy
 TREATMENT: Systemic steroids

54. Wegener’s granulomatosis
giant cell arteritis
polyarteritis nodosa,
hypersensitivity vasculitis
Orbital
manifestations

55.  necrotizing granulomatous inflammation and
vasculitis primarily affecting the entire
respiratory tract and kidneys.
 Ocular involvement 50% of patients
 Most common: conjunctivitis, marginal
ulcerative keratitis, episcleritis, scleritis, uveitis,
retinal vasculitis,and optic neuropathy

56.  Orbital involvement in 50% with ocular
manifestations
 proptosis, pain, redness,orbital congestion, and
ophthalmoparesis
 EO muscle: direct vasculitis/cranialneuropathy
 Orbital involvement
Extension from
PNS
Orbital apex
syndrome

57.  DIAGNOSIS
Clinical:history of concomitant or prior sinus,
respiratory illness, bilaterality
anemia, leukocytosis, thrombocytosis, ESR,CRP
ANCA: positivity 60-96% in disseminated
60-70% in localised form
Biopsy
 TREATMENT
systemic corticosteroids in combination with
cyclophosphamide.

58.  systemic vasculitis characterized by focal nonnecrotizing
granulomatous inflammation of small to medium-sized
arteries, particularly the cranial arteries arising from the
aortic arch
 average age of 70 years
PRESENTATION,
 GENERAL:headache, jaw claudication,polymyalgia, fever,
anorexia, scal tenderness,weight loss, tender temporal artery
 MC: sudden onset vision loss
( causes: AION, PION, CRVO/CRAO, choroidal ischemia,
and lesions of the chiasm or retrochiasmal visual pathways)

59.  Diplopia 15% : ocular motor nerve, brain stem, or
EOmuscle ischemia.
 Orbital ischemia :occlusion of both the ophthalmic artery
and
the collateral vascular anastomoses supplying the orbit.
 anterior segment ischemia:
conjunctival injection, corneal edema, aqueous cell and
flare,
rubeosis iridis, progressive cataract, and hypotony
 posterior segment involvement:
Venous stasis retinopathy and choroidal ischemia
 Generalized orbital ischemia may produce a clinical
picture simulating orbital inflammation with pain,
chemosis, proptosis, ophthalmoplegia,and visual loss

60.  DIAGNOSIS
Clinical
ESR increased/ CRP
Temporal artery biopsy
 TREATMENT
emergency, high dose steroid titrated according to ESR

61. Female 4x
Smokers 7x
3rd-4th decade of life
Associated with:
90% Graves hyperthyroidism
6% Euthyroidism
3% Hashimoto thyroiditis
1% Primary hypothyroidism
THYROID OPHTHALMOPATHY

62. •5 MAIN MANIFESTATIONS
Soft tissue involvement
lid retraction
proptosis
optic neuropathy
restrictive myopathy
CLINICAL MANIFESTATIONS OF
TRO

63. 30-50% of patients with TED
Initial limitation by inflammatory edema, later by fibrosis
Frequency: Elevation deficit> abduction> depression> adduction
Surgery is indicated if diplopia in primary gaze provided disease
is quiescent and angle of deviation is stable fr 6 mo
OPHTHALMOPLEGIA IN TRO

64. Congestive phase
exposure keratopathy
PAIN IN TRO

65.  Infections :preseptal (periorbital)
postseptal (orbital),
the septum acting as a natural barrier to the passage of
microorganisms
 Bacteria: staphylococci / streptococci most common
 Ethmoid sinusitis: the commonest cause of orbital cellulitis
at all ages
 Postseptal (orbital) infection is divided into five stages, each
with increasing risk to sight and life: (1) inflammatory
edema,(2) orbital cellulitis, (3) subperiosteal abscess, (4)
orbital abscess, and (5) cavernous sinus thrombosis

66. PRESENTATION
pain, heat, redness, and swelling in the periorbital region
A history of fever, upper respiratory tract infection,
lacrimal outflow obstruction, sinusitis,or trauma
The presence of a demarcation line corresponding to the
arcus marginalis, conjunctival chemosis, proptosis,
ophthalmoplegia, or loss of vision are: features of orbital
(postseptal) infection,
Orbital apex syndrome or cavernous sinus thrombosis must
be considered in more severe cases
. Finally, signs of meningitis –such as opisthotonos or
lethargy : intracranial spread of an orbital infection

67. DIAGNOSIS
History/ clinical examination
CBC, CRP, Blood culture/ pus culture
CT scan: sinus disease/ sub periosteal abcess
CT with contrast: differentiate an abscess from
inflammatory phlegmon.
MRI: CST/ intracranial spread/ non radioopaque FBs
TREATMENT
urgent intravenous antibiotics, systemic rehydration,
and treatment of anyunderlying systemic disease (e.g.,
diabetes, renal failure

69.  RARE: to be considered in immuno compromised
 MC: Mucor & Aspergillus
 Spread from PNS
 Rhino-orbital-cerebral disease occurs in up to half of all
patients
PRESENTATION
 sinusitis or facial pain, pharyngitis, and a foulsmelling
seropurulent nasal discharge
 decreased VA, RAPD, trigeminal insensitivity,
ophthalmoplegia, and proptosis. ( orbital apex invt)
 Characteristic black eschar : ischaemic necrosis

70. DIAGNOSIS
ESR, CBC ( Neg Blood culture)
Biopsy : Gomori’s methamine silver (GMS) stain,
potassium hydroxide (KOH)preparation and H&E stain,
and Sabouraud’s agar without inhibitors is appropriate for
culture.
CT or MR: extent of disease.
CT : mucosal thickening, bony destruction, and venous
filling defects suggestive of thrombosis
TREATMENT
Debridement of devitalised
tissue, Liposomal amphotericin B

71.  GRADENIGO’S SYNDROME : 6th nerve palsy
with trigeminal pain secondary to suppurative
process of otitis media.
 Involvement of petrous part of temporal bone
 Pain is due to Gasserian ganglion involvement

72.  CAVERNOUS SINUS MENINGIOMA

73.  MENINGIOMA OF
CLIVUS

75.  Incidence: III> IV> VI
 Diplopia due to dysfunction of the nerve from
which it originates
 may compresses, and produces dysfunction of
an adjacent nerve.
 Pain due to trigeminal dysfunction

76.  situated within Meckel’s cave on the
anterosuperior surface of the petrous portion of
the temporal bone
 trigeminal schwannoma can be distinguished
from trigeminal neuralgia by the relatively
longer duration of painful episodes, absence of
trigger zones and associated neurologic deficits

77.  50% of patients with gasserian ganglion schwannomas
experience ocular symptoms, including diplopia from
compression of the ocular motor nerves, loss of vision fro
compression of the optic nerve,

78.  Granulocytic sarcoma/ chloroma
 Ocular adnexal lymphomas
 Multiple myeloma

79.  Otorhinologic
symptoms
(50%):loss of
hearing,
discharge
from the ear, nasal
obstruction,
nasal bleeding
and discharge
 Lymphatic
spread

82.  Visual manifestations: invasion of orbits or from
extension of the tumor intracranially
 Maxillary & Ant Ethmoid: invade orbit
Posterior Ethmoid and Sphenoid : intracranially
 Ocular : proptosis, facial pain, eye pain, loss of vision,
epiphora, displacement of the globe limitation of eye
movements, diplopia, chemosis of the conjunctiva, and
swelling of the optic disc

83.  Site of origin: major and minor salivary glands, the
lacrimal gland, the mucous glands of the lip, cheek,
floor of the mouth, tongue,pharynx, tonsil, nasal
mucous membrane, paranasal sinuses,larynx
 symptoms and signs are identical with those produced
by other malignancies that arise in these regions
 Perineural invasion

84.  2-3 % of all cancer patients develop orbital mets

85.  Clinical manifestations : abrupt onset of orbital
swelling or orbital mass, blurred vision, double
vision and pain

86.  Begins in childhood and may continue into adulthood.
 least two attacks of a cranial nerve palsy and unilateral
migrainous head pain
 3rd nerve: Most common
 The headache general resolves over days but the
cranial neuropathy may persist for weeks
 Enhancement of the third nerve on MRI suggesting
that this condition is inflammatory in nature.
 Indeed, the course may be shortened in some cases
using corticosteroid treatment.
 Complete recovery is the rule

87.  Common in middle aged men
 Unilateral pain along 1st , 2nd division of 5th
nerve associated with ocular sympathetic
paralysis
 Type 1 – multiple C.N [3rd ,4th ,5th & 6th ]
involvement with headache & Horner’s
 Type 2 – hemicranial pain with ipsilateral
ocular sympathetic paralysis

88. CONCLUSION
1. It is misleading, however, to think of ‘painful
ophthalmoplegia’ as a separate clinical entity with specific
etiologic significance
2. At best, there is some localizing value of this symptom
complex to the cavernous sinus–parasellar–superior orbital
fissure region, although orbital, meningeal, and even posterior
fossa disease may present in a similar fashion
3. With regard to etiology, inflammatory, infiltrative, neoplastic,
and vascular diseases can all result in nonspecific painful
ophthalmoplegia