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Grand Rounds Conference Jinghua Chen, MD, PhD University of Louisville Department of Ophthalmology and Visual Sciences May 1, 2015 History CC: Double vision for two days. HPI: 12 year old boy presented with diplopia for 2 days. About 2 days ago he noticed double vision and he had problem looking to his right with his right eye. He denied any vision change. Past Medical History POH: PMH: Allergy: FH: Eye Medication: Myopia (-3.50 D OD and -2.25 D OS) Asthma Chocolate None contributory None Exam OD OS BCVA: 20/20 20/20 Pupils: 43 43 No RAPD OU IOP: 13 11 EOM Limited abduction OD CVF: Full OU Anterior segment: Normal OU DFE: Normal OU Eye Movement Exam in ED Lab Workup in ED Hematology: Normal ICP: 26 (<25 mm Hg) CSF: Appearance: clear Color: colorless RBC: 10 (0/ µL) WBC: 138, 94% lymph (0-8/ µL) Glucose: 40 (50-80 mg/dL) Total protein: 47 (15–45 mg/dL) MRI without and with Contrast Normal MRI T2 weighted images Assessment 12 year old boy presented with diplopia for 2 days. LP shows elevated ICP, mild increased WBC, normal MRI Other history: One week ago patient developed high fever with some neck stiffness. He also complained eye pain and headache. Impression: Differential Right 6th nerve palsy due to Meningitis Diagnosis: Infection, viral illness Brain tumor: lesion of cerebellopontine angle Nucleus aplasia: Duane’s syndrome Ischemic mononeuropathy: most common in adults Trauma Inflammation: petrous bone, facial pain, Gradenigo syndrome Migraine headache Elevated pressure inside the brain Management Patient received one time dose of iv Rocephin inside hospital. Follow up office visit 10 days later. Patient states doing better. No diplopia, crossing or drifting. No blurred vision. The sixth nerve has the longest subarachnoid course of all cranial nerves Ophthalmology 2nd . 2004: 1324 Pediatric Sixth Nerve Palsies The Rochester Epidemiology Project - Olmsted County residents The annual combined incidence of third, fourth, and sixth nerve palsies was 7.6 per 100,000 (95% confidence interval, 5.1 to 10.1). The fourth (36%), followed by the sixth (33%), the third (22%), and multiple nerve palsies (9%). Am J Ophthalmol. 1999 Apr;127(4):388-92. American Academy of Ophthalmology; 2006:118 Etiologies of Acquired 6th Nerve Palsy *6% to 30% attributed to a miscellaneous group of causes that includes leukemia, migraine, pseudotumor cerebri, multiple sclerosis; the miscellaneous group of etiologies reflects the poor localizing value of sixth nerve paresis. **6% to 29%, etiology undetermined, reflecting vulnerability of the nerve to conditions which are transient, benign and unrecognizable. J Ophthalmic Vis Res. 2013 Apr; 8(2): 160–171. Isolated Sixth Nerve Palsy The 6th cranial nerve is the most frequently affected nerve in an isolated ocular motor palsy. Diabetes mellitus, hypertension or history of a recent viral infection. Syndromes of the Sixth Nerve PalsyLocalizing Signs 1. Brainstem 2. Subarachnoid space 3. Petrous apex 4. Cavernous sinus/superior orbital fissure 5. Orbit Brainstem Syndrome A lesion in the posterior fossa may be compressive, ischemic, inflammatory (multiple sclerosis in young adults) or degenerative and may involve the fifth, seventh and eighth cranial nerves J Ophthalmic Vis Res. 2013 Apr; 8(2): 160–171. Subarachnoid Space - Elevated Intracranial Pressure Syndrome Downward displacement of the brainstem causing stretching of the sixth nerve as its exits the pons and inside Dorello’s canal. 30% of patients with pseudotumor cerebri have sixth nerve paresis as the only neurologic deficit Other pathologies in the subarachnoid space include hemorrhage, meningeal infections (viral, bacterial, fungal), inflammation (sarcoidosis) or infiltrations (lymphoma, leukemia, carcinoma). J Ophthalmic Vis Res. 2013 Apr; 8(2): 160–171. Petrous Apex Syndrome Gradenigo's Syndrome 1904 the syndrome was introduced by Giuseppe Gradenigo A complication of otitis media and mastoiditis Triad of diplopia, facial pain and otorrhea http://pedemmorsels.com/gradenigos-syndrome-and-otitis-media/ Cavernous Sinus Syndrome Third, fourth, fifth, sixth and sympaththetic fibers. Internal carotid artery aneurysm J Ophthalmic Vis Res. 2013 Apr; 8(2): 160–171. Orbital Syndrome Proptosis Congestion of conjunctival vessels and chemosis Optic atrophy or papilledema. Horner syndrome may be present Trigeminal signs Tumors of the orbit, orbital pseudotumor, thyroid eye disease, orbital cellulitis or myositis. The Six Mimickers of Sixth Nerve Palsy Thyroid eye diseases Myasthenia gravis Duane’s syndrome Spasm of the near reflex Delayed break in fusion Old blowout fracture of the orbit Management of Pediatric Patients Identify and treat the cause of the condition, and to relieve the symptoms. Neoplasms, especially of the posterior fossa account for 39%. (Robertson DM, Arch Ophthalmol 1970;83:574-579.) Trauma accounts for 54.4%. (Abbas Bagheri, J Ophthalmic Vis Res 2010;5:32-37.) Maintain binocular vision: Fresnel prisms Injection of botulinum toxin into the ipsilateral medial rectus. Development of an abducens nerve palsy following minimal head trauma should raise the suspicion of a compressive lesion such as a tumor. Spontaneous recovery of an abducens nerve palsy may occur even with skull base tumors or leukemia, perhaps from axonal regeneration, resorption of hemorrhage in tumors or immune response to the tumor. Surgery: Vertical muscle transposition procedures such as Jensen's, Hummelheim's or whole muscle transposition. Operation on both the lateral and medial rectii of the affected eye. References BSCS 2014-2015 Book 5, Neuro-Ophthalmology: 220-221 Azarmina M, Azarmina H. The Six Syndromes of the Sixth Cranial Nerve J Ophthalmic Vis Res. 2013 Apr; 8(2): 160–171. Shrader EC, Schlezinger NS. Neuro- ophthalmologic evaluation of abducens nerve paralysis. Arch Ophthalmol. 1960;63:84–91. Rucker CW. The causes of paralysis of the third, forth, and sixth cranial nerves. Am J Ophthalmol. 1966;61:1293–1298. Johnston AC. Etiology and treatment of abducens paralysis. Trans Pac Coast Otoophthalmol Soc Annu Meet. 1968;49:259–277. Robertson DM, Hines JD, Rucker CW. Acquired sixth-nerve paresis in children. Arch Ophthalmol. 1970;83:574–579. Rush JA, Younge BR. Paralysis of cranial nerves III, IV, and VI. Cause and prognosis in 1000 cases. Arch Ophthalmol. 1981;99:76– 79. Patel SV, Mutyala S, Leske DA, Hodge DO, Holmes JM. Incidence, associations, and evaluation of sixth nerve palsy using a population-based method. Ophthalmology. 2004;111:369–375. Bagheri A, Babsharif B, Abrishami M, Salour H, Aletaha M. Outcomes of surgical and non-surgical treatment for sixth nerve palsy. J Ophthalmic Vis Res. 2010;5:32–37. Quah BL, Ling YL, Cheong PY, et al. A review of 5 years' experience in the use of botulinium toxin A in the treatment of sixth cranial nerve palsy at the Singapore National Eye Centre. Singapore Med J.1999 Jun;40(6):405-9. Boger WP 3rd, Puliafito CA, Magoon EH. Recurrent isolated sixth nerve palsy in children. Ann Ophthalmol. 1984 Mar;16(3):237-8, 240-4. Holmes JM, Mutyala S, Maus TL. Pediatric third, fourth, and sixth nerve palsies: a population-based study. Am J Ophthalmol. 1999 Apr;127(4):388-92. Repka MX, Lam GC, Morrison NA. The efficacy of botulinum neurotoxin A for the treatment of complete and partially recovered chronic sixth nerve palsy. J Pediatr Ophthalmol Strabismus 1994;31:79-83.