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DUKE ELDER TUTORIAL NEURO-OPHTHALMOLOGY, STRABISMUS, PAEDIATRICS Deep Sarode ST3 NEURO-OPHTHALMOLOGY Nerve disorders Pupillary disorders • Optic neuritis • Ischaemic optic neuropathy Visual field defects • Papilloedema • Congenital disc anomalies Miscellaneous conditions • Nerve palsies OPTIC NEURITIS Symptoms Signs • Blurred vision • ReducedVA • Retro-orbital pressure/pain • RAPD • Pain on eye movements • Reduced colour vision • (systemic neurology) • Enlarged blind spot • Pain on eye movements • In 1/3 cases,optic disc swelling + haemorrhages OPTIC NEURITIS – MANAGEMENT • Explanation of condition,recovery (weeks) and associated with MS • MRI brain and spine if patient agreeable • Pulsed IV methylprednisolone in certain (uncommon) situations • Oral steroids? • Optic NeuritisTreatment Trial (ONTT) showed no treatment effect and higher risk of recurrence OPTIC NEURITIS SYNDROMES Devic’s disease (neuromyelitis optica) • Bilateral optic neuritis with transverse myelitis • Aquaporin-4 antibody positivty Anti-MOG optic neuropathy Leber’s hereditary optic neuritis • Mitochondrial mutations • Bilateral sequential optic neuropathy in young adulthood ANTERIOR ISCHAEMIC OPTIC NEUROPATHY Symptoms • Sudden onset visual loss • Painless • Headache,jaw claudication,scalp tenderness Signs • ReducedVA • RAPD • Altitudinal field defect • Disc swelling with haemorrhages and cotton wool spots • Temporal artery:tender,thickened,weak pulse ANTERIOR ISCHAEMIC OPTIC NEUROPATHY Investigations • Bloods (FBC, U+Es, LFTs,lipids, glucose,CRP,ESR) Management (arteriticAION) • Pulsed IV methylprednisolone • PPI • Temporal artery biopsy ANTERIOR ISCHAEMIC OPTIC NEUROPATHY Management (non-arteriticAION) • Consider commencing secondary prevention therapy (aspirin, statin) • Smoking cessation advice • BP checkBILATERAL DISC SWELLING Causes • Idiopathic intracranial hypertension • Cerebral venous sinus thrombosis • Space-occupying lesion • Malignant hypertension BILATERAL DISC SWELLING Signs • Blurred disc margin • Flame haemorrhages • Cotton wool spots • Obscuration of blood vessels • Absence of spontaneous venous pulsation Investigations • (US to assess for drusen) • Urgent LP,CTB,CTV as inpatient IDIOPATHIC INTRACRANIAL HYPERTENSION Risk factors? • Raised BMI Diagnosis? • Female • Full ophth exam:swollen disc + enlargement of blindspot • retinoids)s (OCP,acne drugs:tetracycl• CT/MRI +/- lumbar puncture Presentation? Management? • Features of raised ICP (headache,N+V,etc.) • Weight loss • Brief obscurations of vision • Acetazolamide/shunting/fenestrationif chronic uncontrolled vision loss • 6th nerve palsy TOBACCO-ALCOHOL AMBLYOPIA/NUTRITIONAL OPTIC NEUROPATHY • Chronic progressive bilateral optic neuropathy • May be associated with B12/folate deficiency • Treat with replacement of vitamin deficiencies CONGENITAL DISC ANOMALIES • Optic atrophy • Optic disc hypoplasia • Optic disc pit • Optic disc coloboma • Optic disc aplasia RD 3 NERVE PALSY Signs • Ptosis • Limited abduction • Limited elevation • Unreactive dilated pupil (loss of parasympathetic supply) Management • Urgent CTA to rule our PCommA • Bloods (FBC,CRP,ESR,U+E,LFT,glucose,lipids,anti-AChRAbs) • Control of diplopia and review in 6 weeks to assess recovery TH 4 NERVE PALSY Signs • Hypertropia • Positive Bielschowsky test Management • Bloods (FBC, CRP, ESR,U+E,LFT, glucose,lipids, anti-AChRAbs) • MRI head as outpatient • Control of diplopia and review in 6 weeks to assess recovery 6TH NERVE PALSY Signs • Loss of abduction Management • Urgent neuro-imaging if neurological red flags present • Bloods (FBC, CRP, ESR,U+E,LFT, glucose,lipids, anti-AChRAbs) • Control of diplopia and review in 6 weeks to assess recovery HORNER’S SYNDROME Signs • Miosis • Partial ptosis and reverse ptosis • Anhydrosis • Apparent enophthalmos Investigations • Topical apraclonidine test (Horner’s pupil dilates) • Bloods (FBC,CRP,ESR,U+E,LFT,glucose,lipids,anti-AChRAbs) • If neck trauma or neck pain,urgent CTA to rule out carotid dissection • If not acute,MRI head + neck + thorax +/- angiography PUPILLARY DISORDERS Pupil disease Relative size? Reactive to light? Reactive to accommodation? 3 nerve palsy Holmes Adie Argyll-Robertson Marcus Gunn Parinaud’s syndrome Horner’s Tropicamide drops PUPILLARY DISORDERS Pupil disease Relative size? Reactive to light? Reactive to accommodation? 3 nerve palsy Large No No Holmes Adie Argyll-Robertson Marcus Gunn Parinaud’s syndrome Horner’s Tropicamide drops PUPILLARY DISORDERS Pupil disease Relative size? Reactive to light? Reactive to accommodation? 3 nerve palsy Large No No Holmes Adie Large initially Very slow Slow Argyll-Robertson Marcus Gunn Parinaud’s syndrome Horner’s Tropicamide drops PUPILLARY DISORDERS Pupil disease Relative size? Reactive to light? Reactive to accommodation? 3 nerve palsy Large No No Holmes Adie Large initially Very slow Slow Argyll-Robertson Small No Yes Marcus Gunn Parinaud’s syndrome Horner’s Tropicamide drops PUPILLARY DISORDERS Pupil disease Relative size? Reactive to light? Reactive to accommodation? 3 nerve palsy Large No No Holmes Adie Large initially Very slow Slow Argyll-Robertson Small No Yes Marcus Gunn Normal Less than other pupil Yes (RAPD) Parinaud’s syndrome Horner’s Tropicamide drops PUPILLARY DISORDERS Pupil disease Relative size? Reactive to light? Reactive to accommodation? 3 nerve palsy Large No No Holmes Adie Large initially Very slow Slow Argyll-Robertson Small No Yes Marcus Gunn Normal Less than other pupil Yes (RAPD) Parinaud’s syndrome Normal/large No Yes Horner’s Tropicamide drops PUPILLARY DISORDERS Pupil disease Relative size? Reactive to light? Reactive to accommodation? 3 nerve palsy Large No No Holmes Adie Large initially Very slow Slow Argyll-Robertson Small No Yes Marcus Gunn Normal Less than other pupil Yes (RAPD) Parinaud’s syndrome Normal/large No Yes Horner’s Small Yes Yes Tropicamide drops PUPILLARY DISORDERS Pupil disease Relative size? Reactive to light? Reactive to accommodation? 3 nerve palsy Large No No Holmes Adie Large initially Very slow Slow Argyll-Robertson Small No Yes Marcus Gunn Normal Less than other pupil Yes (RAPD) Parinaud’s syndrome Normal/large No Yes Horner’s Small Yes Yes Tropicamide drops Large No No BINOCULAR VISUAL FIELD DEFECTS Bitemporal superior hemianopia Pituitary mass Bitemporal hemianopia Chiasmal lesion Bitemporal inferior hemianopia Craniopharyngioma BINOCULAR VISUAL FIELD DEFECTS Left inferior quadrantanopia Right parietal lobe lesion PITS Left superior quadrantanopia Right temporal lobe lesion Left homonymous hemianopia Right occipital lobe lesion Left homonymous incongruous hemianopia Right posterior optic radiations lesion MONOCULAR VF DEFECTS Optic nerve Optic nerve • Anterior ischemicoptic neuropathy(AION) • Optic neuritis • Glaucoma • Optic nerve trauma • Orbital mass Retina Retina • Hemi-RVO/RAO • CRVO/CRAO • Superiorretinal detachment • Completeretinal detachment MYASTHENIA GRAVIS Symptoms Signs • Variable diplopia • Ptosis • Variable ptosis • Diplopia • Variable limb weakness • Fatiguability • PositiveTensilon test • Positive icepack test MYASTHENIA GRAVIS Investigations • Anti-AChRAbs, anti-MuSKAbs • CT thorax Management • Anticholinesterase (pyridostigmine) • Steroids/immunosuppression PARINAUD’S DORSAL MIDBRAIN SYNDROME • Light-near dissociation • Upgaze palsy • Convergence retraction nystagmus • Dilated pupils • Bilateral upper lid retraction (Collier’s sign) NEUROFIBROMATOSIS TYPE 1 Ophthalmic features Systemic features • Optic nerve glioma • Cafe-au-lait spots • Lisch nodules • Axillary freckling • Choroidal naevi • Neurofibromas • Retinal astrocytoma • Sphenoid dysplasia • Long bone cortical thinning/dysplasia NEUROFIBROMATOSIS TYPE 2 Ophthalmic features Systemic features • Cataract • Acoustic neuroma • Combined hamartoma of RPE and • Meningioma retina • Glioma MYOTONIC DYSTROPHY Ophthalmic features Systemic features • Cataract • Myotonia/weakness • Chronic progressive external • Diabetes mellitus ophthalmoplegia • Frontal balding • Pigmentary retinopathy • Cardiac conduction abnormalities • Hypogonadism STRABISMUS • Duane’s retraction syndrome • Brown’s syndrome • V pattern • A pattern • Investigations DUANE’S RETRACTION SYNDROME • Congenital syndrome due to dysgenesis of CN6 • Type 1:eso,limited abduction • Type 2:exo,limited adduction • Type 3:eso,limited abduction and adduction BROWN’S SYNDROME • Tight superior oblique tendon • Primary may be due to dysgenesis of superior oblique tendon • Secondary due to surgery,inflammation,trauma etc ALPHABET PATTERNS V pattern A pattern • Inferior oblique overaction • Superior oblique overaction • Superior rectus underaction • Inferior rectus underaction • Superior oblique underaction • Inferior oblique underaction • Brown’s syndrome • Craniofacial abnormalities PAEDIATRICS • Amblyopia • Retinopathy of prematurity • Congenital cataract • Congenital glaucoma • Periorbital cellulitis • Miscellaneous conditions AMBLYOPIA • Reduced vision in one or both eyeGeneral diagnosis and due to abnormal development of management vision in childhood • Full ophthalmic exam • Refractive correction Causes • Occlusion therapy • Strabismus • Orthoptic correction • Refractive • Correction of ocular pathology • Stimulus-deprivation RETINOPATHY OF PREMATURITY Pathophysiology Presentation • Retinal blood supply develops from• Screening optic disc outwards • Leukocoria due to retinal detachment • Usually fully developed at 36 weeks • Premature delivery results in Management underdeveloped vasculature and therefore ischaemic retina • Diode laser to ischaemic retina if appropriate stage • Aggravated by oxygen therapy CONGENITAL CATARACT Presentation Diagnosis • Leukocoria • Ophthalmoscopy • PoorVA • Squint Management? • Cataract extraction Causes • (Depending on age,lens implantation delayed • Genetic/family history until eye is fully grown) • Metabolic • Maternal infections (TORCH) Complications? • Congenital glaucoma – especially if surgery • Idiopathic before 1 year CONGENITAL GLAUCOMA Presentation • Watery eye without red eye,usually within 1st year • Enlargement of the cornea (buophthalmos) • Corneal clouding Diagnosis • Full clinical examination + tonometry Management? • Surgery (goniotomy,trabeculotomy,etc.) PERIORBITAL CELLULITIS – SYMPTOMS AND SIGNS Preseptal cellulitis Orbital cellulitis • Swollen/erythematous lid • Pain,worse on eye movement • Pain • Reduced vision • Diplopia • White eye • Ocular and orbital examination norma• Pyrexia • ReducedVA • RAPD • Reduced colour vision • Restriction of EOM • Proptosis • Lid swelling,injection,chemosis OTHER PAEDIATRIC CONDITIONS • Coat’s disease: retinal telangiectasia leading to exudation • Retinoblastoma: malignant tumour of retinoblasts • Rhabdomyosarcoma: malignant tumour of mesenchymal cells • Toxocara:infectious retinal granuloma/endophthalmitis • Juvenile idiopathic arthritis: chronic arthralgia,associated with anterior uveitis • Kawasaki’s disease: conjunctivitis,rash,adenopathy,strawberry tongue, hands/feet rash,pyrexia