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Approach to Pain ophthalmoplegia.

This document discusses painful ophthalmoplegia, which presents as periorbital or hemicranial pain, ipsilateral ocular motor palsies, and sensory loss in the trigeminal nerve distribution. Causes include aneurysms, carotid cavernous fistulas, cavernous sinus thrombosis, tumors, and infections. Evaluation involves imaging like MRI/CT/angiography. Management depends on the underlying cause but may include antibiotics, anticoagulation, surgery, or steroids. Prognosis depends on early diagnosis and treatment, with potential for residual neurological deficits.

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PAINFUL OPHTHALMOPLEGIA MODERATOR , DR PAVANA ACHARYA PRESENTED BY, DR TINTU SUSAN JOY
PAINFUL OPHTHALMOPLEGIA  Periorbital or hemicranial pain  Ipsilateral ocular motor palsies &/  Oculo-sympathetic palsy &/  Sensory loss in distribution of ophthalmic and maxillary division of trigeminal nerve.”
CAVERNOUS SINUS
CAVERNOUS SINUS RELATIONS 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
STRUCTURES PASSING THROUGHM SOF
CAUSES OF PAINFUL OPHTHALMOPLEGIA  TRAUMA  ANEURYSM  Intracavernous carotid artery  Posterior communicating artery  Basilar artery  CAROTID CAVERNOUS FISTULA  CAVERNOUS SINUS THROMBOSIS  ISCHEMIC  Diabetes  Hypertension
 TUMORS  Primary intracranial  Local or distant metastasis  Pituitary apoplexy  Meningeal carcinomatosis or lymphomatosis o INFECTION  Mucormycosis or other fungal infection  Herpes zoster  Tuberculosis  Bacterial sinusitis, mucocele, periostitis  Syphilis
 INFLAMMATION  Sarcoid  Wegener's granulomatosis  Tolosa-Hunt syndrome  Orbital pseudotumor  Giant cell arteritis  OPHTHALMOPLEGIC MIGRAINE
ANEURYSMS VASCULAR CAUSES- 1
INTRA CAVERNOUS CAROTID ARTERY ANEURYSM  Enlarge gradually in the cavernous sinus (ruptureCCF)  Anterior expansion, May erode optic foramen and superior orbital fissure   compressive optic neuropathy,  ocular motor nerve paresis and  proptosis  Erosion medially may lead to hypopituitarism
UNRUPTURED ANEURYSM  Sixth cranial nerve- most commonly involved  Oculomotor nerve palsy  Trigeminal nerve involvement leads to facial pain  ( referred pain from adjacent tendorium)  ( constant and severe , but can be episodic)  90% Of cases, pain precedes ophthalmoplegia  Apparent pupillary sparing  oculo sympathetic ans parasympathetic fibres affected.  Visual loss not characteristic as in ophthalmic artery aneurysm.
POSTERIOR COMMUNICATING ARTERY ANEURYSM  Young women  Manifest as  sudden apoplectic event due to SAH  Third nerve palsy due to slow enlargement without rupture  Painful third nerve palsy with pupil involvement .
OPHTHALMIC ARTERY ANEURYSM  Compression of optic nerve and chiasma  vision loss and inferior field defect( optic nerve compressed superiorly against superior dural shelf of optic canal.)
MANAGEMENT  Pupil invovement mandates immediate efforts to rule out aneurysm by MRI( > 4mm), MRA, CTA or digital subtraction angiography( gold standard).  Unruptured aneurysm treatment depends on its size and position, age and general health of patient.  Direct ( metal clips) or indirect ( endovascular) approach.  Recovery is most likely in incomplete paresis if surgery done within 1-2 weeks of onset.  Incomplete recovery after several months leads to aberrant regeneration.
CAROTID CAVERNOUS SINUS FISTULA  Abnormal communications between the cavernous sinus and carotid artery.  Direct fistula trauma(75%) , usually high flow  spontaneous rupture of intracavernous carotid artery(low flow)  Indirect fistula( dural shunts) Atherosclerosis, collagen vascular diseases, congenital malformation VASCULAR CAUSE 2
OCULAR MANIFESTATIONS – DIRECT TYPE  Due to venous congestion and decreased arterial flow.  Exophthalmos( within hrs or several days in rapid flow fistulas )  PULSATING EXOPHTHALMOS UNCOMMON(orbit tight from haemorrhage and edema, if present abolished by compression of I/L carotid in neck)  Frozen orbit ( no ocular motor function)  diminished arterial flow to cranial nerves,  6 th nerve affected MC,  mechanical restriction due to venous congestion and orbital oedema.
 Conjuctival chemosis and arterialization of episceral vein( hallmark of c-c fistula).  Bruit over superior orbital vein or over temple( with bell of steth, not pathognomonic).  Vision loss (optic nerve ischemia from apical orbital compression)  High episcleral venous pressure and IOP .  Fundus venous engorgement , dot blot haemorrhages, rarely CRVO.
A) Severe epibulbar injection (B) haemorrhagic chemosis;
 Low flow dural fistulas  between cavernous sinus or one or more meningeal branches of internal carotid artery.  Often misdiagnosed as chronic conjuctivitis, Orbital pseudo tumour, thyroid eye disease, orbital cellulitis.
Corkscrew (arterialized) vessels caused by low-flow (carotid- cavernous sinus)
INVESTIGATIONS  Orbital ultrasonography, CT, , MRI HOCKEY STICK SIGN of engorged superior ophthalmic vein. Contrast-enhanced axial CT showing enlarged cavernous sinus, dilated orbital fissure with hypertrophy of extra- ocular muscles and varices at orbital apex on the left side.
PROGNOSIS AND TREATMENT  Prognosis of C-C fistula varies but severe vision loss is often permanent esp if frozen orbit encountered.  Detachable flow quided balloons used to close the fistulas.
ISCHEMIC NEURALGIA  Severe pain in eye and forehead may precede 3rd N palsy in diabetes, hypertension and GCA.  Due to ischemic occlusive changes in the dural vessels supplying the nerves in cavernous sinus.  Pupil sparing third nerve palsy. VASCULAR CAUSE 3
SINUSES OF BRAIN. CAVERNOUS SINUS THROMBOSIS INFECTIVE CAUSE 1
CAVERNOUS SINUS COMMUNICATION
• 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
DANGEROUS AREA OF FACE
ETIOLOGY OF CAVERNOUS SINUS THROMBOSIS SEPTIC • Sphenoid or ethmoidal sinus • Dental infections • Facial cellulitis • Otitis ASEPTIC • Polycythemia • Sicke cell disease • Trauma • Neurosurgery • Pregnancy • OCP.
SIGNS AND SYMPTOMS  Febrile, Nausea , vomiting  U/L Periorbital pain  Adnexal edema  Ptosis  Chemosis and severe congestion.  Proptosis  Opthalmoplegia( 3, 4, 6 CN)  Decreased visual acuity or blindness (Central retinal artery/ vein occlusion secondary to ICA arteritis, septic emboli, ischemic optic neuropathy)  Initially unilateral  Bilateral
INVESTIGATION  CT without contrast and MRI with fat suppression will localise the lesion.  Sinus disease nearly always present.  Contrast enhanced scans ordered with axial and coronal view.
TREATMENT  Septic thrombosis   antibiotics and anticoagulation.  Corticosteroids reduce inflammation  If sinus disease – debridement of sinuses.  If suspicious of mucor- Amphotericin B  Aseptic thrombosis  manage the underlying condition.
ORBITAL APEX SYNDROME  Less than 1% orbital cellulitis patients develop orbital apex syndrome  > 50% Diabetic  In DM, rhinocerebral mucormycosis most common cause.  Ketoacidosis important risk factor. INFECTIVE CAUSE -2
CLINICAL FEATURES  Vision loss early feature(optic nerve involvement)  Complete ophthalmoplegia(3, 4 ,6 th CN)  Ptosis( 3rd CN)  Decreased corneal sensation( 5 th CN)  Adnexal edema  Conjuctival chemosis and congestion  Proptosis  Eschars( Infarction superimposed on septic necrosis)
B) Axial view from orbital MRI demonstrating mucormycosis of the ethmoid sinuses with extension into orbit. Patient with mucormycosis of the right orbit, resulting in an orbital apex syndrome.
INVESTIGATIONS  Orbital CT without contrast and MRI with fat suppression will localise the lesion
MANAGEMENT  AMPHOTERICIN B/antibiotics.  Adjuctive hyperbaric O2 helpful.  Sinus as well as orbital exenteration.  High morbidity and mortality.  Permanent neurological deficits common.
ORBITAL CELLULITIS  Serious infection of soft tissues behind orbital septum.  Common in children.  The major causes of orbital cellulitis are  sinusitis (58%), MC- ethmoid  lid or face infection (28%),  foreign body (11%), and  hematogenous (4%).  Staphylococcus, Haemophilus influenzae and Streptococcus are the most common causative organisms. INFECTIVE CAUSE 3
SYMPTOMS  Pain exacerbated by eye movement,  Oedema of lids  Double vision  Visual impairment  Recent H/O sinus infection or respiratory symptoms
SIGNS  VA reduced, colour vision impaired  Tender firm erythematous warm eyelids  Conjunctival chemosis and SCH,  axial proptosis or dystopia( if abscess present)  Painful ophthalmoplegia  intraocular pressure may be elevated.  A rapid loss of vision (optic nerve compression, optic neuritis, or vasculitis).  With posterior extension, cavernous sinus thrombosis, subdural empyema, and intracranial abscess may develop.  Systemic symptoms may include malaise and fever.
(A) Right orbital cellulitis with ophthalmoplegia; B) axial CT shows preseptal and orbital opacification
MANAGEMENT  TC, DC  Blood cultures  Culture of nasal discharge  High resolution CT of orbit, sinuses and brain  Hospital admission  IV antibiotics  Monitoring of optic nerve function (any deterioration surgical decompression)  Drainage of orbital abscess.  Drainage of infected sinus if lack of response to antibiotics.
GRADENIGO SYNDROME  Caused by mastoiditis or acute petrositis  6th nerve damage at petrous tip.  Later accompanied by facial weakness , pain and hearing difficulties.  Pain is due to Gasserian ganglion involvement INFECTIVE CAUSE 4
SUPERIOR ORBITAL FISSURE SYNDROME  CAUSES  Trauma Craniofacial fractures  Syphilis  Hematoma of retrobulbar space or cavernous sinus,  Infection  Neoplasm
SIGNS  Ptosis  Proptosis  Ophthalmoplegia  Fixed dilated pupil  Anaesthesia of upper eyelid and forehead.
TREATMENT  Exploration – neoplasm, retrobulbar haemorrhage, physical impingement, infection.  Following trauma Prognosis is poor if nerves are severed.  Dexamethasone 1mg/kg( loading dose) followed by 0.5 mg postoperatively after reduction of fracture.  OR  I V methyl prednisolone 30 mg/kg followed by 5.4 mg/kg/hr for 23 hrs.(Acute spinal cord injury study).
PITUITARY APOPLEXY  Hemorrhagic infarction of a pituitary adenoma/tumor.  Life threatening condition  Precipitated by pregnacy, radiation and trauma INTRA CRANIAL TUMOURS
PRESENTATION:  Variable onset of severe headache  Nausea and vomiting  Meningismus  Drowsiness, coma, subarachnoid hemorrhage  Unilateral/Bilateral painful ophthalmoplegia.  Vision loss(damage to visual pathway at level of chiasma.)
A) Axial MRI view of enlarged apoplectic pituitary gland involving chiasm. (B) Sagittal MRI view from the same patient MRI GOLD STANDARD FOR NEURO IMAGING, delineate tumour and hemorrhage.
TREATMENT  Decompression of sella trans sphenoidally.  Supplementation of pituitary hormones.( high dose corticosteroids.)
 Cavernous sinus meningioma  Schwannomas of ocular motor nerves( 3rd CN>4th CN> 6th CN)  Gasserian ganglion schwannomas  situated within Meckel’s cave (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. INTRA CRANIAL TUMOURS
GASSERIAN GANGLION SCHWANNOMAS  50% of patients have ocular symptoms,  diplopia from compression of the ocular motor nerves,  loss of vision from compression of the optic nerve
RAISED INTRACRANIAL PRESSURE SECONDARY TO TUMOURS  Cause downward displacement of brainstem  May stretch one or both 6 th cranial nerves over petrous tip 6th nerve paresis  FALSE LOCALISING SIGN
NASOPHARYNGEAL CARCINOMA  Tumours arising from roof of nasopharynx infilterate parapharyngeal space , invade base skull, enter orbit through superior orbital fissure.  Diplopia earliest feature.  3rd , 4th , 6th CN and ophthalmic division of 5th CN commonly involvrd( facial pain).  50 % -loss of hearing, sense of fullness in ear.  9th, 10th, 11th CN may be involved.  Horners syndrome  Late visual loss  Neurotrophic keratitis  Proptosis(6%)( extension of tumour into orbit) LOCAL METASTASIS
DISTANT METASTASIS  Lymphoma  Multiple myeloma  Carcinomatous metastasis DISTANT METASTASIS abrupt onset of orbital swelling or orbital mass, blurred vision, double vision and pain
GIANT CELL ARTERITIS  Granulomatous necrotising arteritis affecting large and medium size arteries.  Smoking, low BMI, early menopause are risk factors  Rare < 50 yrs  Female: male- 4:1 INFLAMMATORY
SYMPTOMS  Scalp tenderness  Headache  Jaw claudication( pathognomonic)  Weight loss, fever, night sweats  Double vision  SIGNS  Thickened tender inflamed nodular non pulsatile arteries  ocular motor palsies including pupil involving third nerve palsy  Arteritic anterior ischemic optic neuritis  Scalp gangrene rare
INVESTIGATIONS  ESR- high(>60 mm/hr)  CRP – high  Thrombocytosis, normocytic normochromic anaemia  Temporal artery biopsy  Treatment  Oral prednisolone 1mg/kg/day  CRP helps to monitor disease activity.
WEGENER GRANULOMATOSIS  Necrotizing granulomatous inflammation and vasculitis primarily affecting the entire respiratory tract and kidneys.  Ocular involvement 50% of patients  Most common: marginal ulcerative keratitis, episcleritis, scleritis, uveitis, retinal vasculitis, and optic neuropathy
 Orbital involvement in 50% with ocular manifestations  Proptosis, pain, redness, orbital congestion, and ophthalmo paresis  EO muscle: direct vasculitis/cranial neuropathy  Orbital involvement Extension from PNS Orbital apex syndrome
 DIAGNOSIS  Clinical : history of concomitant or prior sinus, respiratory illness, bilaterality  anemia, leukocytosis, thrombocytosis  cANCA +  TREATMENT  systemic corticosteroids in combination with cyclophosphamide.  resistant cases cyclosporin, azathiprine  Surgical decompression in severe orbital involvement.
THYROID ORBITOPATHY  Immunological disorder that affects the orbital muscles and fat.  Middle-aged adults (30–50years)  Women :men3-4:1  Bilateral process but is often asymmetrical  multiple muscles are involved simultaneously, most commonly the inferior and medial rectus
 Symptoms and signs include  dry eyes,  conjunctival injection,  lid retraction,  exophthalmos,  diplopia,  corneal exposure, and  rarely optic nerve compression.  Graves’ disease usually runs a progressive course for 3–5years and then stabilizes.
ORBITAL IMAGING  Increased fat lucency is seen, as well as extraocular muscle enlargement confined to the bellies, but with sparing of the insertions and origins. Echography  Thickened muscles with medium to high internal reflectivity and an irregular acoustic structure are seen on echography.
PATHOLOGY  The enlarged, rubbery muscles show variable amounts of edema and infiltration with inflammatory round cells .  An increased amount of acid mucopolysaccharides infiltrates the orbital tissue.
enormously thickened extraocular muscle. (C) Both fluid and inflammatory cells separating the muscle bundle may be seen. The inflammatory cells are predominantly lymphocytes, plus plasma cells.
TREATMENT  Systemic corticosteroids or radiotherapy may be indicated for acute orbital inflammation and congestion.
ORBITAL PSEUDO TUMOUR  Nongranulomatous acute to subacute inflammatory disease with no systemic manifestations that may affect teenagers to the elderly.  Space occupying orbital infilteration.  Symptoms include  Acute or sub acute pain,  conjunctival injection,  chemosis,  lid edema,  Proptosis and  Mild to moderate ophthalmoplegia.  A palpable mass is detected in 50% of cases
(A) Left idiopathic orbital inflammatory disease. (B) CT axial view shows ill-defined orbital opacification; (C)(C) coronal view
COURSE  Spontaneous remission after few weeks  Intermittent episodes of activity  Severe prolonged inflammation fibrosis of orbital tissue frozen orbit( ophthalmoplegia, ptosis, visual impairment)
IVESTIGATIONS  Posterior Tenon’s capsule shows thickening and enhancement.  A shaggy orbital infiltrate or discrete mass is present, which may mold to the globe or optic nerve sheath.  The lacrimal gland may be enlarged.
PATHOLOGY  The pseudotumor is a gray rubbery mass composed of a polymorphic infiltrate of lymphocytes, eosinophils, plasma cells, and polymorphonuclear leukocytes.  In the sclerosing type, the dominant feature is scarification and collagen deposition.
TREATMENT  Systemic corticosteroids typically result in a dramatic improvement.  Rarely, some lesions may require cytotoxic agents.  The sclerosing type shows little or no response to treatment.  Prognosis generally is excellent, with complete resolution of disease.
MYOSITIS  An acute to subacute idiopathic inflammation of the extraocular muscles, myositis may affect teenagers to the elderly.  Typically, the disease is unilateral and involves only one muscle, most commonly the superior or lateral rectus.  Symptoms include  pain,  motility restriction,  exophthalmos, and  displacement of the globe.
vascular injection over the insertion of the right medial rectus coronal CT shows enlargement of the right medial rectus
ORBITAL IMAGING  Enlargement of an extraocular muscle is seen, with involvement of the entire muscle from origin to insertion.  Echography  Echography shows a thickened muscle with low internal reflectivity and a regular acoustic structure.
Treatment and Prognosis  Systemic corticosteroids generally result in prompt resolution. The prognosis is excellent.
MULTIPLE SCLEROSIS  Idiopathic demyelinating disease  More common in women  Presentation typically in third or fourth decade with relapsing / remitting demyelination  Ophthalmic features  Optic neuritis( retrobulbar )  Internuclear ophthalmoplegia  Nystagmus  Rarely- ocular motor nerve palsies, skew deviation, hemianopia. DEMYELINATING
INTERNUCLEAR OPHTHALMOPLEGIA UNILATERAL INO  Lesion in Medial longitudnal fasciculus(MLF)  Other causes stroke, tumours  Straight eyes in primary position  Defective adduction in side of lesion  Nystagmus of contralateral eye on abduction.  Gaze to side of lesion normal.  Convergence intact.
BILATERAL INO  Limitation of right adduction and nystagmus of left eye on left gaze  Limitation of left adduction and nystagmus of right eye on right gaze. WEBINO( wall eyed bilateral INO)  Rostral midbrain lesion  Convergence deficit  Bilateral exotropia and abducting nystagmus.
TOLOSA HUNT SYNDROME  Non specific granulomatous inflammation of cavernous sinus or superior orbital fissure or orbital apex.  Diagnosis of exclusion  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.
RAEDERS PARATRIGEMINAL SYNDROME  Common in middle aged men  Type 1 – multiple C.N [3rd ,4th ,5th & 6th ] involvement with headache & Horner’s  Type 2 – hemicranial pain with ipsilateral ocular sympathetic paralysis  A complete neuro radiological investigations required to rule out pituitary adenoma, chondroma, meningioma, aneurysm of internal carotid artery.
OPHTHALMOPLEGIC MIGRAINE  Mobius disease or trigeminal ophthalmoplegic syndrome.  Begins in childhood and may continue into adulthood.  Ophthalmoplegia starts at height of attack of headache, or after its cessation.  Transient attack 1-4 weeks  After repeated episodes permanent paralysis  3rd nerve: Most common  Intense vascular spasm
DIAGNOSTIC CRITERIA  Onset at first decade  h/o multiple episodes  I/L ophthalmoplegia  Normal CT and angiography.
REFERENCES  Yanoff And Duker Ophthalmology  Jack J Kanski, Clinical Ophthalmology  L C Dutta Modern Ophthalmology  Walsh And Hoyt Neuro Ophthalmology.
Approach to Pain ophthalmoplegia.