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Paediatric Neuro-Ophthalmology


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This seminar gives an overview of this complex yet diagnostically rewarding sub specialty. Kinldy download to view the presentation properly.

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Paediatric Neuro-Ophthalmology

  2. 2. THE PAEDIATRIC LOW VISION PATIENT 1 in 1000 children has low vision 75 % learning in early years due to vision Vision impairment: Long term consequences Vital to diagnose: Scope of Vision Restoration and Rehabilitation Centers for Disease Control. NECH P No. 99-0444. National Center for Environmental Health, Centers for Disease Control.
  3. 3. 200,000 to 300,000 children with severe visual impairment. Only 15,000 in blind school. Neuro-ophthalmological disease in around 35% of these. Vision 2020 document, Government of India.
  5. 5. CORTICAL VISUAL IMPAIRMENT CVI is defined as visual impairment caused by damage to the central nervous system. Most common cause of bilateral visual impairment in the developed world Associated with: Prematurity and ROP Causes: Hypoxia/Ischemia Perinatal strokes, defined as occurring between 28 weeks of gestation and 7 days of age, have an estimated incidence of up to 1 in 4,000 live births. Most common cause of CVI. Congenital Brain Malformation CNS Infection Ventriculoperitoneal Shunt Block Head Injury Child Abuse Metabolic Irregularity
  6. 6. PRESENTAT ION Brodsky MC, Fray KJ, Glasier CM. Perinatal cortical and subcortical visual loss: mechanisms of injury and associated ophthalmologic signs. Ophthalmology. 2002;109:85–94.
  7. 7. CVI DIAGNOSIS/PROGNOSIS/MANAGEM ENT Consider the four A’s of visual loss: Acuity, Assimilation, Attention, and Apraxia. Poor Visual Function, Lack of Eye Contact Associated Neurological/Systemic Abnormality Extensive Role of Neuroimaging (MRI, PET) Poor Prognosis: depends on initiation of treatment Periventricular Leukomalacia (Sub-cortical) is indicator of damage to optic radiation: Poor Prognosis, Tonic Down gaze PERIVENTRICULAR LEUKOMALACIA
  8. 8. DELAYED VISUAL MATURATION When a child fails to show the expected visual function for his age but does so spontaneously after a period of time. These infants often have straight eyes, no nystagmus, and brisk pupillary responses, but seem to “look straight through” the examiner. TYPES: Type 1: Isolated DVM Type 2: DVM with associated systemic disease, mental retardation, or other neurodevelopmental disorders Type 3: DVM with ocular disease, such as bilateral cataracts, severe corneal opacities, colobomas, retinal dystrophy, optic nerve hypoplasia, or albinism Uemura Y, Oguchi Y, Katsumi O. Visual developmental delay. Ophthalmic Paediatr
  9. 9. MANAGEM ENT Early diagnosis of CVI is vital Management is multifocal and requires team work Often is patient specific rather than protocol based Often the prognosis is poor and visual outcome is low, 50% will never see, associated IQ defects, learning disabilityBrodsky MC. Pediatric Neuro-Ophthalmology. Third Edition. Springer. 2016.
  10. 10. CONGENITAL OPTIC NERVE ABNORMALITIES Optic Nerve Hypoplasia Excavated Optic Disc Anomalies Optic Disc Coloboma Morning Glory Disc Anomaly Peripapillary Staphyloma Optic Pit Congenital Tilted Disc Syndrome Congenital Disc Pigmentation Bilateral optic disc anomalies: poor vision and nystagmus in infancy Unilateral optic disc anomalies: preschool presentation with sensory esotropia CNS malformations are common in patients with malformed optic discs, need imaging Color Vision Normal Any unilateral structural abnormality that reduces visual acuity in infancy may lead to superimposed amblyopia
  11. 11. OPTIC NERVE HYPOPLASIA Most common optic disc anomaly Clinical Features: Abnormally small discs, double ring sign, vessel tortuosity Confirmed on MRI Systemic Associations Amblyopia ManagementZekki et al, BJO,
  12. 12. SYSTEMIC ASSOCIATION: • 13-34%: pituitary abnormalities on imaging like empty sella, ectopic posterior pituitary, nonvisualized infundibulum and posterior pituitary. • Hypopituitarism occurs in 75% of optic nerve hypoplasia patients, although the majority of the patients have no pituitary abnormalities on neuroimaging. • Additionally growth hormone deficiency (70%), hypothyroidism (43%); DIAGNOSIS: • Clinical, Imaging • VER low amplitude, ERG: WNL PROGNOSIS • Developmental delay often occurs in affected children. • Developmental delay in 32% and cerebral palsy in 13% • Patients with bilateral ONH and abnormal gray or white matter development had a higher rate of developmental delay (65%) than those without abnormalities (35%). Kaur, Savleen et al. “Optic Nerve Hypoplasia.” Oman Journal of Ophthalmology 6.2 (2013): 77–82. PMC. Web. 11 Feb. 2018.
  14. 14. PHACE (posterior fossa malformations, large facial hemangiomas, arterial anomalies, cardiac anomalies and aortic coarctation, and eye anomalies) syndrome. Frieden IJ, Reese V, Cohen D. PHACE syndrome. Arch Dermatol. 1996;132:307– 11.
  15. 15. SWOLLEN OPTIC DISC Pseudo Papilledema Papilledema IIH Secondary Intracranial Hypertension Disc Edema in Systemic Disease Optic Neuritis
  16. 16. PSEUDO PAPILLEDEMA Optic Disc Drusen Anterior to the lamina cribrosa, Lipofuscin Buried drusen may present as pseudo disc edema Visual field defects have been detected in 71%–87% of eyes with visible disc drusen and in 21%–39% of eyes with pseudopapilledema but no visible drusen Vascular occlusions have been reported in patients with disc drusen
  17. 17. Other causes of Pseudo papilledema: Persistent Fetal Vasculature Bermeister’s papilla Hypermetropic/Nano ophthalmic eyes Juxtapapillary Myelination Hyaloid Traction
  18. 18. PAPILLEDEM A ICSOL first possibility of raised ICP Most common tumors associated with childhood papilledema are midbrain and cerebellar glioma, medulloblastoma, and ependymoma. Clinical Presentation: Headaches, transient visual
  19. 19. IIH/ PSEUDOTUMOU R CEREBRI IIH is a condition characterized by signs and symptoms of increased intracranial pressure without evidence of a mass lesion or hydrocephalus Dandy Criteria for Primary IIH: Smith JL. Whence pseudotumor cerebri? J Clin Neuroophthalmol.1985;5:55–6. Johnston I, Hawke S, Halmagyi M, Teo C. The pseudotumor syndrome: disorders of cerebrospinal fl uid circulation causing intracranial hypertension without ventriculomegaly. Arch Neurol.1991;48:740–7.
  20. 20. IIH(PEDIATRICVSADULT) No gender predisposition Better response to steroids (80% remission) Surgery is indicated in progressive visual loss (20%) Brodsky MC. Pediatric Neuro-Ophthalmology. Third Edition. Springer. 2016.
  21. 21. DISC EDEMA IN SYSTEMIC DISEASE Malnutrition Severe Anaemia Addison's Disease Organ Transplantation Down Syndrome SLE Diabetes Malignant Hypertension Sarcoidosis DIABETIC PAPILLOPATHY SARCOIDOSIS
  22. 22. OPTIC NEURITIS Paediatric Optic Neuritis: Bilateral Optic Disc Swelling Often Post Infectious rather than MS Pediatric MS accounts for only 2%–5% of all MS cases Neurodiagnostic criteria for pediatric MS include: • Two or more non-encephalopathic clinical CNS events that have presumed inflammatory cause, separated by more than 30 days and involving two or more areas of the CNS. • One non-encephalopathic episode typical of MS, associatedwith MR findings demonstrating dissemination in space and dissemination in time. • One prior episode of ADEM followed by a nonencephalopathic attack, equal or greater than three months after the initial symptom onset of ADEM, associated with new MR lesions and showing Krupp LB, Tardieu M, Amato MP, et al. International Pediatric Multiple Sclerosis Study Group pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders: revisions to the 2007 definitions. Mult Scler. 2013;19:1261–7.
  23. 23. DIAGNOSTIC EVALUATION Battery of investigations to rule out other causes. Neuroimaging is essential. (MRI of head and spine) Cecocentral scotomas and largeBrodsky MC. Pediatric Neuro-Ophthalmology. Third Edition. Springer. 2016.
  24. 24. TREATME NT No clear cut guidelines ONTT is used currently Steroids are the mainstay of treatment after excluding infectious causes. Immunomodulators like azathioprine andBrodsky MC. Pediatric Neuro-Ophthalmology. Third Edition. Springer. 2016.
  25. 25. BRAIN TUMOUR RELATED VISUAL LOSS Primary brain tumors are the most common solid neoplasms in children and are second only to leukemia in overall frequency during childhood. Pediatric intracranial tumors in general • Headache (33%) • Nausea and vomiting (32%) • Poor coordination or abnormal gait (27%) • Papilledema (13%) Pediatric intracranial tumors (age <4 years) • Macrocephaly (41%) • Nausea and vomiting (30%) • Irritability (24%) • Lethargy (21%) Wilne S, Collier J, Kennedy C, et al. Presentation of childhood CNS tumors: a systematic review and meta-analysis. Lancet Oncol. 2007;8:685–95.
  26. 26. VISUAL PATHWAY LESIONS 50% intracranial tumours can present with ocular findings Associated Features: nystagmus, ocular motor dysfunction, reduced visual acuity, visual field deficits, dyschromatopsia, an afferent pupillary defect, anisocoria, optic atrophy, or papilledema.Wilne S, Collier J, Kennedy C, et al. Presentation of childhood CNS tumors: a systematic review and meta-analysis. Lancet Oncol. 2007;8:685–95.
  27. 27. Suprasellar: Suprasellar tumors in children include optic pathway gliomas, craniopharyngiomas,germinomas, pituitary adenomas. Supratentorial tumours: Astrocytomas are the most common supratentorial tumors in childhood, constituting approximately 30% of such tumors. Posterior Fossa Tumours: Medulloblastoma : most common malignant brain tumor of childhood. This embryonal tumors of the posterior fossa comprises 40% of posterior fossa tumors.
  28. 28. Brain Stem Tumours: Brainstem gliomas represent about 15% of pediatric CNS tumors. The mean age at diagnosis is 7–9 years, with no gender predilection. Most common seen are diffuse gliomas (58-75%). Brainstem tumors are suggested by the triad of long tract signs, cranial neuropathies, and ataxia.
  29. 29. MANAGEMENT Unlike adult tumors, extracranial tumors rarely metastasize to the intracranial compartment in children. However, seeding of certain brain tumors in children occurs often along the CSF pathways, causing invasion of the leptomeninges. Due to delay in diagnosis, brain tumors in children may be long standing, leading to more severe and less reversible visual loss Need extensive imaging (MRI, Angiography, PET, MR Spectroscopy)  Long-term survivors of childhood cancer have a ten times greater incidence of cerebrovascular disease.  Treatment is multimodal:  Chemotherapy  Surgery  Radiotherapy,  Long term follow-up  Post Radiation:  SMART: stroke-like migraine attacks after radiation therapy  Moyamoya Disease: hazy like a puff of cigarette smoke drifting in the air,” is a descriptive name applied to the angiographic finding of an abnormal network of collateral vessels at the base of the brain in the region of theBrodsky MC. Pediatric Neuro- Ophthalmology. Third Edition. Springer.
  30. 30. Moyamoya Disease
  31. 31. OPTIC ATROPHY Optic atrophy is a morphologic sequel to a multitude of anterior visual pathway insults that culminate in the loss of retinal ganglion cell axons. Leading cause of severe visual impairment among 2,527 Nordic children, followed by retinopathy of prematurity and amblyopia. Etiology: Tumours (29%), post inflammatory (meningitis, optic neuritis) (17%), trauma (11%), undetermined (11%), hereditary (9%), perinatal disease (9%), hydrocephalus (6%), neurodegenerative disease (5%) Repka MX, Miller NR. Optic atrophy in children. Am J Ophthalmol. 1988;106:191–3.
  33. 33. PUPILLARY REACTIONS Extremely Vital RAPD assessment Lesion Localisation
  34. 34. ANISOCORIA
  35. 35. Depending of Localisation:  Relevant Imaging  CT, MRI, MRA  Ipsilateral Horner syndrome (first order) and contralateral superior oblique palsy (fourth nerve palsy) suggests a lesion of the trochlear nucleus or its fascicle in the brainstem  Ipsilateral Horner syndrome (third order) and an abducens paresis (sixth nerve palsy) suggests a lesion in the cavernous sinus  Acute Painful Horner Syndrome should be presumed related to a dissection of the Ipsilateral internal carotid artery
  37. 37. OCULAR MOTOR NERVE PALSIES Oculomotor Nerve Trochlear Nerve Abducens Nerve Multiple Nerves •Is there history of trauma? •Is there diurnal variation? •Is there history of headache? •Are there neurological deficits? •Is there history of previous episode? •Is there a head
  38. 38. 3RD NERVE LESIONS Causes: Congenital palsy (43%), trauma (20%), infection and inflammation(13%), tumor (10%), aneurysm (7%), and ophthalmoplegic migraine (7%) Miller NR. Solitary Oculomotor nerve palsy in childhood. Am J Ophthalmol. 1977;83:106–11.
  40. 40. MANAGEMENT OF 3RD NERVE LESIONS Treat the underlying cause Look out for aneurysm of the PCA Neuroimaging is vital Goal is: Binocular alignment, Ptosis correction, Amblyopia management
  41. 41. 4TH NERVE LESION S 71.2% have head tilt opposite to side of palsy  most common isolated cranial nerve palsy and the most common cause of acquired vertical
  42. 42. Etiology: Congenital in 56%, craniofacial anomalies12%, head trauma in 5%, Idiopathic in 14%. Tarczy-Hornoch K, Repka MX. Superior oblique palsy in pediatric patients. J AAPOS. 2004;8:133–40.
  43. 43. (1) Is there a right or left hypertropia in primary position? (2) Does the deviation increase in right gaze or left gaze? (3) Does it increase with head tilt to the
  44. 44. MANAGEMENT OF 4TH NERVE LESIONS The trochlear nerves are particularly vulnerable to closed head trauma when there may be contre coup of the tectum of the midbrain against the edge of the tentorium. Management: Surgical First choice of procedure: ipsilateral inferior oblique muscle weakening Second procedure: deviation is greater than 15 prism diopters is either ipsilateral superior rectus recession, when the vertical deviation is worse in up gaze, or contralateral inferior rectus recession, when the deviation is worse in down gaze.
  45. 45. 6TH NERVE LESIONS Clinical Features:  head turn toward the side of the lesion  horizontally incomitant esotropia that increases in gaze toward the affected eye and decreases or disappears in gaze away from the
  46. 46. Etiology: neoplasm (39%), trauma (20%), inflammation (17%), and idiopathic conditions (9%) Robertson DM, Hines JD, Rucke CW. Acquired sixth nerve paresis in children. Arch Ophthalmol. 1970;83:574–9.
  47. 47. MANAGEMENT OF 6TH NERVE LESIONS Newborns: Observation 4 to 10 weeks Older Infants/ Children: If after period of fever/immunisation: Observation for 3 months Extensive imaging, neurological and otolaryngologic evaluation The highest rates of spontaneous recovery have been reported in idiopathic (67%), infectious (50%), inflammatory (90%), and traumatic (33%–50%) cases. Aroichane M, Repka MX. Outcome of sixth nerve palsy or paresis in young children. J Pediatr Ophthalmol Strabismus 1995;32(3):152–156.
  48. 48. MULTIPLE CRANIAL NERVE PALSIES  Difficult to manage  Prognosis depends on presentation, degree of nerve involvement and time of intervention  Imaging is essential in every case.
  50. 50. SACCADE INITIATION FAILURE OR OCULAR MOTOR APRAXIA The term saccade initiation failure or ocular motor apraxia is used to specify impaired voluntary saccades and variable deficit of fast-phase saccades during vestibular or optokinetic nystagmus. Clinical Features: poor head control, head thrusting fixation using VOR, hypotonia, motor delay Evaluation: VEP, Neuroimaging
  51. 51. CHILDHOOD DORSAL MIDBRAIN SYNDROME Etiology: Pretectal lesions Clinical Features:The constellation of deficits are (1) vertical gaze palsy, (2) light–near dissociation of the pupils, (3) eyelid retraction (Collier’s sign), (4) disturbance of vergence, (5) fixation instability, and (6) skew deviation. Evaluation: Neuroimaging Management: Treat the cause
  52. 52. MYASTHENIA GRAVIS 9.1 per 1 million total population 4.2% were between 0 and 9 years, 9.5% were between 9 and 19 years Transient Neonatal Familial Infantile Congenital Myasthenia Syndromes Autoimmune Myasthenia The prognosis for survival, improvement, and remission in a child with myasthenia gravis is better than that in an adult. Brodsky MC. Pediatric Neuro-Ophthalmology. Third Edition. Springer. 2016.
  53. 53. Diagnosis: EMG/NCS Blood Tests Genetic Tests Treatment:  Breathing Support  Nutritional Support  Medications  Thymectomy  IVIG  Plasmapheresis • 2 years, 57 cases • AChR antibodies: positive titers were found in 67% • Tensilon test: 47% of generalized cases and in 39% of ocular cases and results were abnormal in 88% and 100%, respectively. • Nerve conduction studies: abnormal results found in 65% • Pyridostigmine: all demonstrated improvement. • 18 patients steroids, improvement 94%. • 21 patients intravenous immunoglobulin (IVIG), improvement in 81%. • Ocular presentations, all were started on pyridostigmine; improvement occurred in 88% patients.
  54. 54. NYSTAGMUS Record type of movement, frequency, amplitude, direction
  55. 55. NYSTAGMUS
  57. 57. PHACOMATOSIS Neurofibromatosis Sturge-Weber syndrome •seizures/ glaucoma •cerebral atrophy, calvarial thickening, and cortical calcifications on CT Klippel–Trenaunay–Weber syndrome Von Hippel Lindau •retinal angiomatosis, central nucleus of the amygdala (CNA) hemangioblastoma (50%), renal cell carcinoma (22%), pheochromocytoma (14%) Tuberous Sclerosis
  58. 58. NF1: NEUROFIBROMATOSIS Clinical Features: Plexiform Neurofibroma of the Lid, Lisch Nodules, Café au Lait spots, Neural Hamartomas Imaging: MRI: Unidentified Bright Objects, Optic Nerve Tract Gliomas, Intracranial
  59. 59. Prognosis: Cognitive disability is another common problem in children with NF1 Patients with NF1 have a life expectancy of about 15 fewer years less when compared with the general population, with malignancies (brain tumors and malignant peripheral nerve sheath tumors) and cerebrovascular disease contributing disproportionately to mortality. Treatment: Carboplatin and Vincristine used for glioma reduction. Poor Prognosis: 32% of patients showed improvement, 28% worsen, 40 % no change Recently Avastin has been shown to decrease tumor size. Radiotherapy not useful, leads to secondary malignanciesFisher MJ, Loguidice M, Gutmann DH, et al. Visual outcomes in children with neurofi bromatosis type 1-associated optic pathway glioma following chemotherapy: a multicenter retrospective analysis. Neuro Oncol. 2012;14:790–7.
  60. 60. NF2: NEUROFIBROMATO SIS Clinical Features: Bilateral Vestibular Schwannoma, cutaneous schwannomas, spinal schwannomas, lack of Lisch nodules (with rare exceptions), fewer café au lait spots, and the presence of juvenile-onset cataracts, CHRPE, Collision tumours Imaging: MRI: Vestibular Schwannoma, ON sheath
  61. 61. TRANSIENT, UNEXPLAINED AND PSYCHOGENIC VISUAL LOSS Some children have visual disturbances that occur in the absence of, or are out of proportion to, their objective ophthalmological findings. The most common cause of episodic visual loss disturbances in childhood is migraine. May occur at some point in up to 15% of the population. Lipton RB, Stewart WF, Diamond S, Diamond ML, Reed M. Prevalence and burden of migraine in the United States: data from the American Migraine Study II. Headache. 2001;41:
  62. 62. MIGRAI NE Clinical features: Blurred vision, foggy vision, flickering lights, colored lights, zigzag lines, and a heat-wave sensation. Descriptions provided by children tend to be more picturesque: “a star breaking into a million pieces,” “heat waves,” “water coming down a window,” “lines coming down from the sky like Investigation s: •MRI •EEG •PET Treatment: •For abortive phase: Sumatriptan, Ketorlac, Prochlorperazine •For prophylaxis: Amitriptyline, Verapamil, Topiramate •Biofeedback, Relaxation,
  63. 63. Vital to differentiate and refer to clinician. Other diseases like coagulopathy, cardiac disease, infectious mononucleosis, mental diseases like schizophrenia can also cause transient visual disturbances.
  64. 64. UNEXPLAINEDVISUALLOSS PSYCOGENIC VISUAL LOSSNon organic loss of vision, diagnosis of exclusion Good prognosis with psychiatric help. Brodsky MC. Pediatric Neuro-Ophthalmology. Third Edition. Springer. 2016.
  65. 65. MEDICAL MANAGEMENT • Appropriate refractive correction. • Surgical intervention whenever required • Appropriate glare control glasses. • Prescription and training to use a low vision device • Binocular vision therapy. • Patching therapy in cases of amblyopia • Appropriate referral for other disability • Prognosis for improvement should be explained to the individual.
  66. 66. FUNCTIONAL MANAGEMENT • Emphasis on size, color, contrast, distance and illumination of targets • Choose toys /games keeping in mind the developmental levels. • Eye tracking exercise • Peripheral awareness therapy • Searching and scanning strategies • Central or eccentric viewing strategies • Visual reach activities. • Office based vision stimulation therapy • Support from special educators. • Orientation and mobility instruction
  67. 67. EDUCATIONAL MANAGEMENT • Levels of lightening and sensitivity to glare • Positioning of the individual • Size of material • Placement of material in optimal visual field • Appropriate colors • Brightness • Need for clutter reduction • Spacing of visual information • Orientation and mobility in the environment
  68. 68. LOW VISION AIDS Principles of magnification at distance point Magnification is calculated by the formula M(t)=BVA/TA, BVA =Best Visual Acuity, TA= Targeted Acuity Distance vision =6/60 and Goal distance acuity= 6/15 (as this acuity is required for reading street signs, bus number and watching television so 60/15 = 4x The power ,size and contrast as more is the power of telescope lesser will be the field of
  69. 69. Principles of magnification at near point:  Relative distance magnification: Moving the object closer to the eye (shortening the distance) causing a proportional enlargement of the retinal image. eg. Sitting in front of the television, holding a book close while reading (most children can easily do this) etc.  Relative size magnification: Enlarging the physical size of an object, such as large print rather than standard print. eg. Large print books, writing large letters.  Angular magnification: Using an optical system such as telescopes, magnifiers.  Electronic magnification: Enlargement of Christine Dickinson. Low Vision Principles and Practices. Manchester: Butterworth Heinemann; 2001. Magnification; pp. 69-118
  70. 70. LogMAR visual acuity prior to the introduction of low vision devices (LVDs) was 0.90 ± 0.05 for distance and for near it was 0.61 ± 0.05. After the intervention, the acuities improved significantly for distance (0.2 ± 0.27; P < 0.0001) and near (0.42 ± 0.17; P = 0.001). The most common reported difficulties were related to their academic activities like copying from the blackboard (80%), reading textbook at arm's length (77.2%), and writing along a straight line (77.2%).  Absolute raw score of disability pre-LVD was 15.05 which improved to 7.58 post-LVD. An improvement in functional vision post visual rehabilitation was especially found in those activities related to their studying lifestyle
  71. 71. SUMMARY
  72. 72.  Beyond Retinal Implants  Second Sight’s Orion 1 Cortical Implant  Chances of vision even in optic nerve, tract, chiasmal