Incomitant esotropia

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  • Special forms… duane + mobius
  • Bridle n leash phenomenon
  • Incomitant esotropia

    1. 1. INCOMITANT ESOTROPIA DR. YOUSAF JAMAL FCPS RESIDENT OPHTHALMOLOGY UNIT 29/08/2009
    2. 2. CONTENTS Introduction Important tests Etiology and management Take home message Mcqs Word for the day
    3. 3. INTRODUCTION When esotropia varies in horizontal gaze Mechanism  neurological  mechanical
    4. 4. Some tests Forced duction test Active force generation test Hess chart
    5. 5. Forced duction test
    6. 6. Hess chart What is it When to do How to do Interpretation  Eye involved  Mechanical vs. neurogenic  Evolution over time
    7. 7. CAUSES OF INCOMITANT ESOTROPIA Sixth Nerve palsy Medical rectus restriction Special forms
    8. 8. Sixth nerve Abducent nerve Purely motor Supplies lateral recti Pathway  Mid pons fasiculus pontomedullay- junction intracavernous intraorbital LR
    9. 9. CAUSES OF 6TH NERVE PALSY (adults) Idiopathic  Increased ICP Vasculopathic (most  Cavernous sinus common)  Thrombosis  Diabetes  Meningioma  Hypertension  Aneurysm  Atherosclerosis  Metastasis Trauma  Multiple sclerosis  basal skull fracture  Sarcoidosis
    10. 10. …Contd… Vasculitis  Invasion thru skull Stroke base  Nasopharyngeal ca Acoustic neuroma  Chordoma Meningitis  Chondrosarcoma Metabolic  Infectious  Vit. B12  Lyme disease  W-k syndrome  Syphilis
    11. 11. Children Idiopathic Birth trauma Viral infections Vaccination Increased ICP  Hydrocephalus Gradenigo syndrome Brainstem glioma**Harley RD. Paralytic strabismus in children. Etiologic incidence and management of the third, fourth, and sixth nerve palsies.Ophthalmology. 1980 Jan;87(1):24-43.
    12. 12. PresentationSymptoms Horizontal diplopia Worse for distance Pronounced in the lateral gaze
    13. 13. Signs Esotropia in primary position Worse for distance Limited abduction Normal adduction Binocular diplopia Face turn
    14. 14. Differential Diagnosis Myasthenia gravis Restrictive thyroid myopathy Duane syndrome Medial orbital wall blowout fracture Convergence spasm Myositis Divergence paralysis
    15. 15. Work Up History:  Age of onset  Prior therapy e.g.. Glasses, patching  Symptoms fluctuation  HTN, DM, thyroid, trauma, other causes Examination:  Neurological:  MS, increased ICP, Gradenigo syndrome, stroke, acoustic neuroma
    16. 16. Ophthalmic Examination Optic nerve functions  VA+ BCVA  Visual fields Motility test  Restricted movements Ophthalmoscopy  Papilledema
    17. 17. …Contd… Hess chart Forced duction test
    18. 18. Investigations BP FBS HBA1c Serology  Lyme  syphilis
    19. 19. …Contd… CT MRI Brain  <45 years (if –ve then LP)  45-55 years with no hx of vasculopathy  VI th nerve palsy + severe pain or neurological signs  Any Hx of Ca  Bilateral VI th Nerve palsy  Papilledema
    20. 20. In children Emphasis on  Trauma  Recent illness  Ear infections Otoscopic examination MRI brain for all children
    21. 21. Treatment Tx underlying cause Orthoptic TX  Base out prism  Patching or fogging Botulinum toxin in ipsilateral MR Surgery
    22. 22. Surgery If persists for > 6 months Recession/resection Transposition of SR/IR insertions  Jansen procedure  Hummelsceim procedure
    23. 23. Medial Rectus Restriction Causes Thyroid myopathy Medial orbital wall fracture Excessive resection of MR
    24. 24. THYROID MYOPATHY Subset of Thyroid eye disease i.e. also called*  Graves eye disease  Thyroid ophthalmopathy  Thyroid related ophthalmopathy  Thyroid orbitopathy  Thyroid related immune orbitopathy  Thyroid eye disease *american academy of ophthalmology. 2008-2009,Section 6
    25. 25. Pathogenesis Autoimmune Infiltration of  Lymphocytes  Plasma cells  Mast cells Deposition of mucopolysaccharides especially hyaluronic acid Leads to edema and later fibrosis that cause restriction
    26. 26. …Contd… Muscles may increase up to 6-8 times of normal size Non-tendinuous part involved Frequency*  Inferior rectus (60-70%)  Medial rectus (25%)  Then superior and lateral rectus*Char DH, Norman D. The use of computed tomography and ultrasonography in the evaluation of orbital masses. Surv Ophthalmol 1982;27:29.
    27. 27. PresentationSymptoms: Decreased vision  Compressive optic neuropathy Double vision  Vertical  Horizontal
    28. 28. Signs (for myopathy) Often bilateral & asymmetric Restricted movements Hypotropia Esotropia Abnormal head position
    29. 29. Work-up (for myopathy)History Duration, pain, vision, known thyroid disease,  smokerOcular examination Visual acuity IOP measurement  Increased on attempted gaze
    30. 30. …Contd… Forced duction test  Positive Diplopia measurement  Prism  Cover/uncover & alternate cover test  Hess chart
    31. 31. …Contd… TFTs EMG & tensilon tests show no abnormality Orbital ultrasound CT  Axial/coronal views MRI
    32. 32. Treatment General  Smoking cessation  Medical internist or endocrinologist opinion  Prisms temporarily used for diplopia in primary positions
    33. 33. Surgery Indications  Diplopia in primary or reading positions  Abnormal head position When to do??*  Angle of deviation stable for > 6 months  In chronic & inactive cases  After orbital decompression surgery*Scot WF, Thalaker JA. Diagnosis an treatment of thyroid myopathy, Ophthalmology 1974;73:437.
    34. 34. …Contd… Goal  To achieve BSV in primary & reading position Technique  Recession is preferred Tx bcz resections worsen the restriction  Adjustable & non-absorbable sutures used  Initial under correction is desirable
    35. 35. Medical Chemodenervation  Botulinum toxin A in affected muscle  1.5-5 units  Onset of action…1-3 days  Duration…3 months
    36. 36. Special forms DUANE SYNDROME MÖBIUS SYNDROME
    37. 37. DUANE SYNDROME Characteristics  Failure of innervation of LR by 6th nerve  Innervation of LR by 3rd nerve Imaging studies  Hypoplasia / aplasia of 6th N. Nucleus
    38. 38. ….Contd.. Mostly sporadic Autosomal dominant (5-10%) Females > Males (3:2) Left eye > right Systemic associations  Goldenhar syndrome  Klippel-feil syndrome  Wilderwanck syndrome
    39. 39. History First described by  Sinclair in 1895  Bahr in 1896  Stilling in 1887  Wolff in 1900 Duane described in 1905*  54 cases and offered theories*Duane A. Congenital deficiency of abduction, associated with impairment of adduction, retraction movements, contraction of the palpebral fissure and oblique movements of the eye. 1905. Arch Ophthalmol. Oct 1996;114(10):1255-6; discussion 1257
    40. 40. Clinical Features BSV intact in primary position Limited horizontal movements  Restricted abduction  Restricted adduction  Both Upshoot or downshoot Retraction of the globe
    41. 41. ClassificationTwo types Brown* Huber***Brown HW., (1950) Congenital structural muscle anomalies in: Allen JH ed. Strabismus Ophthalmic Symposium. St Louis, Mosby, pp 205-36**Huber A., (1974) Electrophysiology of the retraction syndrome. British journal of ophthalmology 58, 293-300
    42. 42. Brown’s ClassificationBased on clinical observations Type A  Limited abduction and less limited adduction Type B  Limited abduction but normal adduction Type C  Limited adduction > limited abduction
    43. 43. Huber’s ClassificationType 1 (70%-80%): Inability or limited abduction Normal or minimal defect in adduction Esotropia with head straight Globe retraction & palpebral-fissure narrowing on adduction Usual face turn to affected side
    44. 44.  Type 1 must be differentiated from 6th nerve palsy  Globe retraction  Mild Esotropia  Fissure changes  Upshoot and downshoot
    45. 45. Type 2 (about 7%) Limited adduction Normal or minimal defect in abduction Exotropia of the affected eye Globe retraction and palpebral-fissure narrowing on adduction Face turn to normal side
    46. 46. Type 3 (about 15%) Limited abduction and adduction Globe retraction and palpebral-fissure narrowing on attempted adduction Possible upshoot and downshoot on adduction Straight or nearly straight head position
    47. 47. Left type I (left)Type III (left)
    48. 48. Management General measures  Prisms: up to 25 error  Special seating arrangement for children in schools  Vision therapy for secondary convergence insufficiency  Special rear mirrors while driving
    49. 49. Surgery Standard management Indications  Unacceptable face turn  Significant misalignment  Severe retraction  Upshoot & downshoot
    50. 50. ProceduresType 1 Recession of MR Recommended for > 20 deviation LR resection not favorable Partial or full transposition of vertical recti
    51. 51. Type 2 Recession of involved LR for small deviations Recession of both LR in large deviations Resection of MR not favorable
    52. 52. Type 3 For Severe globe retraction  Recession of both MR & LR
    53. 53. MÖBIUS SYNDROME Very rare Paul julius Möbius, a German neurologist, in 1888 and 1892  Both congenital facial diplegia and bilateral Abducent nerve palsies In 1939, henderson  Congenital unilateral facial palsy
    54. 54. Pathology Involvement of cranial nerves  Facial nerve in all cases  Abducent nerve (75%)  Hypoglossal nerve… usual  Glossopharyngeal, vagus & accessory nerves… uncommon  Occulomotor & trochlear nerves… rare
    55. 55.  Other systems involved  Limbs  Chest  Orofacial defects
    56. 56. Presentation Ocular  6th nerve palsy  Bilateral tight MR restriction  Esotropia or straight eyes  Both abduction limited  Adduction is better with convergence
    57. 57.  Systemic  Mask like facies  Defective lid closure  Tongue atrophy  Limb anomalies  Low IQ
    58. 58. Treatment MR recession
    59. 59. Take Home Message Complete Hx Thorough Ophthalmic examination Tests interpretation Enough knowledge Physician/endocrinologist/neurologist opinion
    60. 60. MCQs1. 9 month girl has abnormal movement of Rt eye which started shortly after birth but stable over time. Good VA, left face turn, with face turn eyes are straight, Rt eye moves normally but Lt fails to abduct past midline. Esotropia = 20 PD, cycloplegic refraction +1.00 sphere. Next step in management should be:a. Neurological evaluation with neuro imagingb. Prescription of full cyclolegic refractionc. Observation onlyd. Strabismus surgery for deviation in primary positionAns. C… case of duane syndrome with little face turn
    61. 61. 2. A 30 year-old man developed a right sixth nerve palsy and facial pain. CT scan revealed opacity of the mastoid air cells. Diagnosis is…a. Wallenbergs syndromeb. Millard-Gublers syndromec. Gradenigos syndromed. Möebius syndromeAns: c
    62. 62. 3. A 6 year-old girl had bilateral Esotropia and absent facial expression. There are also punctate corneal staining due to exposure keratopathy. Corneal sensation appears normal. Diagnosis..a. Duanes syndromeb. Möbius syndromec. accommodative Esotropiad. intermittent divergent squintAns: b
    63. 63. 4. All of the following would be expected to show restriction during forced duction testing except:a. Thyroid associated orbitopathyb. Internuclear ophthalmoplegiac. Orbital fracture with IR entrapmentd. Congenital fibrosis of extra ocular musclesAns. b
    64. 64. a. mechanical/neurological strabismusAns. mechanicalb. DiagnosisAns. Duane syndrome
    65. 65. a. mechanical/neurological strabismusAns. Neurologicalb. Under acting muscleAns. Right LRc. DiagnosisAns. Right 6th nerve palsy
    66. 66. a. mechanical/neurological strabismusAns. mechanicalb. restricted gaze?Ans. Left up and down gazec. DiagnosisAns. Left orbital floor fracture with IR restriction
    67. 67. a. What is the primary position of the affected eye?Ans. Left Hypotropia b. In which direction is the eye movement affected?Ans. Left up gaze and abductionc. Type of strabismusAns. Mechanicald. DiagnosisAns. Thyroid eye disease
    68. 68. WORD FOR THE DAY
    69. 69. Next lectureDecreased vision (transient) by Dr mushtaq & Journal club By Dr Maria

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