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Screening and diagnosis of optic pathway gliomas in children with neurofibromatosis type 1 by using sweep visual evoked potentials. Chang BC, Mirabella G, Yagev R, Banh M, et al. Invest Ophthalmol Vis Sci 2007 Jun; 48(6):2895-2902 NF-1 is an autosomal dominant phakomatosis with a prevalence of 1/2000 to 1/5000. Up to 24% have optic pathway gliomas (OPGs). In addition to visual acuity, visual field testing, neuroimaging, conventional pattern visual evoked potentials (VEPs) have been used but may be challenging and inconsistent secondary to the prolonged testing and unpredictable visual attentiveness of the child. The authors utilized sweep visual evoked potentials (SVEPs) in 16 patients with OPGs, 14 children with NF-1 without OPGs, and 16 age matched controls. All children had best corrected VA of 6/9 or better. Comparison between groups showed significant reduction of mean log contrast sensitivity in the OPG group compared with the non OPG and control group. Log contrast sensitivity was moderately sensitive in identifying patients with OPG and highly specific in screening out patients with no OPG. The children with no OPGs displayed no difference in visual functioning compared with control subjects. The authors believe that the SVEP can be a useful noninvasive screening test for presymptomatic OPGs in patients with NF-1 and normal visual acuity. Ocular pathologic findings of neurofibromatosis type 2. McLaughlin ME, Pepin SM, Maccollin M, Choopong P, Lessell S. Arch Ophthalmol 2007 Mar; 125(3):389-94. This is a case report of the ocular pathologic findings of a 34 year old woman who died from complications of NF 2. They identified 3 NF2 associated lesions: juvenile posterior subcapsular cataracts, epiretinal membranes, and an intrascleral schwannoma. The juvenile PSC demonstrated individual displaced lens cells just anterior to the posterior lens capsule and in the posterior lens cortex. Their exam of the ERM showed a mixture of cuboidal and spindled cells, most consistent with Muller cells and astrocytes. The NF2 gene product merlin has 2 important functions: 1) stabilization of adherens junctions at sites of cell-cell contact and 2) down regulations of the Rac signaling pathway. Defects in cell-cell adhesion and cell morphology secondary to abnormal adherens junctions may underlie the developmental or hamartomatous and neoplastic lesions of NF2. 56
Visual outcome of a cohort of children with neurofibromatosis type 1 and optic pathway glioma followed by a pediatric neuro-oncology program. Dalla Via P, Opocher E, Pinello ML, Calderone M et al. Neuro Oncol 2007 Oct; 9(4):430-7. Epub 2007 Aug 17. They evaluated the visual outcome of 20 children with NF-1 and optic pathway glioma (OPG). The mean age at time of diagnosis of OPG was 40 months (range 16 month to 9.5 years). At time of referral, the visual acuity in 6 children was
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What’s New and Important in Pedia
Page 3 and 4:
ROP Retinopathy of Prematurity: the
Page 5 and 6: same diagnosis in 4 of 34 images (1
Page 7 and 8: PREMATURITY Perinatal care in the t
Page 9 and 10: STRABISMUS Causes and outcomes for
Page 11 and 12: METHODS: The medical records of all
Page 13 and 14: initial positive response to medica
Page 15 and 16: deviation as an intermittent exodev
Page 17 and 18: STRABISMUS SURGERY Unilateral reces
Page 19 and 20: The effect of topical tetracaine ey
Page 21 and 22: +/-0.08 and 0.34 +/-0.07 mm, for th
Page 23 and 24: THYROID Cigarette smoking and thyro
Page 25 and 26: excluding from the cataract group t
Page 27 and 28: Long-Term Results of Lensectomy in
Page 29 and 30: CATARACT SURGERY Pediatric anterior
Page 31 and 32: Suture-related complications after
Page 33 and 34: included vitrectomy, capsulotomy, c
Page 35 and 36: medication. At the last follow-up,
Page 37 and 38: REFRACTIVE ERROR / REFRACTIVE SURGE
Page 39 and 40: demonstrated a positive effect on t
Page 41 and 42: ANTERIOR SEGMENT Conjunctival Melan
Page 43 and 44: RESULTS: Directly after birth, corn
Page 45 and 46: and photophobia (80%). The children
Page 47 and 48: Validity and reliability of the Chi
Page 49 and 50: outcomes achieved at cessation of t
Page 51 and 52: METHODS: One hundred and thirteen c
Page 53 and 54: Verisyse intraocular lens implantat
Page 55: GENETICS Marfan syndrome: from mole
Page 59 and 60: primarily affect the photoreceptors
Page 61 and 62: single morphogenetic anomaly in dev
Page 63 and 64: Three genome-wide association studi
Page 65 and 66: • Cafe-au-lait macules • Freckl
Page 67 and 68: Laryngeal and tracheal anomalies Po
Page 69 and 70: Treatment of retinoblastoma: Curren
Page 71 and 72: Secondary acute myeloid leukemia af
Page 73 and 74: one. Patients treated with radiatio
Page 75 and 76: Neuro-ophthalmological management o
Page 77 and 78: CONCLUSION: Canalicular involvement
Page 79 and 80: peripheral retinal vasculitis. The
Page 81 and 82: This article describes a 7-month-ol
Page 83 and 84: eference eyes. Photopic flash ERGs
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Page 87 and 88: ORBIT Porous orbital implants, wrap
Page 89 and 90: PLASTICS Conjunctival epithelial ch
Page 91 and 92: The authors conclude that lash ptos
Page 93 and 94: .03). SBP and DBP were significantl
Page 95 and 96: CONGENITAL INFECTION Congenital lym
Page 97 and 98: PEDIATRICS Visual Manifestations of
Page 99 and 100: controversial, ranging from one to
Page 101 and 102: SYSTEMIC Visual function and ocular
Page 103 and 104: Toxocara infection. Toxocara GWC an
Page 105 and 106: congenital cataracts. The retina wa
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Childhood blindness: a UK epidemiol
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safe and efficient procedure to cor
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