Dotto, Patrícia de Freitas; Berezovsky, Adriana; Cappellano, Andrea Maria; Silva, Nasjla Saba da; Sacai, Paula Yuri; Rocha, Daniel Martins; Andrade, Erica Pinheiro de; Silva, Frederico Adolfo B.; Salomão, Solange Rios

doi:10.5935/0004-2749.20210022

Patrícia de Freitas Dotto Corresponding author: Patrícia de Freitas Dotto Quaresma. E-mail: patdotto@gmail.com

Laboratório de Eletrofisiologia Visual Clínica, Departamento de Oftalmologia e Ciências Visuais, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil.

http://orcid.org/0000-0003-4231-4801

Adriana Berezovsky

Laboratório de Eletrofisiologia Visual Clínica, Departamento de Oftalmologia e Ciências Visuais, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil.

http://orcid.org/0000-0001-6350-8221

Andrea Maria Cappellano

Programa de Neuro-Oncologia, Instituto de Oncologia Pediátrica, Grupo de Apoio ao Adolescente e à Criança com Câncer, Universidade Federal de São Paulo, São Paulo, SP, Brazil.

Nasjla Saba da Silva

Programa de Neuro-Oncologia, Instituto de Oncologia Pediátrica, Grupo de Apoio ao Adolescente e à Criança com Câncer, Universidade Federal de São Paulo, São Paulo, SP, Brazil.

Paula Yuri Sacai

Laboratório de Eletrofisiologia Visual Clínica, Departamento de Oftalmologia e Ciências Visuais, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil.

http://orcid.org/0000-0001-9224-857X

Daniel Martins Rocha

Laboratório de Eletrofisiologia Visual Clínica, Departamento de Oftalmologia e Ciências Visuais, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil.

Erica Pinheiro de Andrade

Laboratório de Eletrofisiologia Visual Clínica, Departamento de Oftalmologia e Ciências Visuais, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil.

http://orcid.org/0000-0002-3331-786X

Frederico Adolfo B. Silva

Programa de Neuro-Oncologia, Instituto de Oncologia Pediátrica, Grupo de Apoio ao Adolescente e à Criança com Câncer, Universidade Federal de São Paulo, São Paulo, SP, Brazil.

Solange Rios Salomão

Laboratório de Eletrofisiologia Visual Clínica, Departamento de Oftalmologia e Ciências Visuais, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil.

Corresponding author: Patrícia de Freitas Dotto Quaresma. E-mail: patdotto@gmail.com

Disclosure of potential conflicts of interest: None of the authors have any potential conflicts of interest to disclose.

Figuras (3)
Tabelas (4)

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Figure 1
Representative sweep visually evoked potential (SVEP) response from the better-seeing eye (BSE) and the worse-seeing eye (WSE) (right panel) and orbital magnetic resonance imaging (MRI) (left panel) from subject S01. Right panel: SVEP from channel 1 (O1-Cz). Dots represent noise registered for each of 10 linearly spaced spatial frequencies presented (from 1.00 to 15.10 cycles/degree presented at 80 cm for the right eye and from 0.1 to 3.01 cycles/degree presented at 30 cm for the left eye); grating acuity was estimated by linear fit and extrapolation to zero amplitude, i.e., the value at which the regression line touches the axis of spatial frequencies; the final acuity scores were thresholds of 10.71 cycles/degree, equal to 0.44 logMAR (or 20/55 Snellen fraction) for the BSE and 1.18 cycles/degree, equal to 1.40 logMAR (or 20/510). The signal-to-noise ratio was 4.22 for the right eye and 3.56 for the left eye. Left panel: Enhanced axial T1-weighted MRI (960/20 [repetition time msec/echo time msec]) shows high signal intensity around the left optic nerve from its intraorbital portion to the chiasm, whereas the intraorbital portion of the right optic nerve is normal. The reduced grating visual acuity (GVA) in the right eye (BSE) is probably due to effects on the optic nerve at the chiasm level. Abbreviations: Ch= channel; 2F1s= harmonic of the stimulus frequency; Spat Freq= spatial frequency; Thrsh= grating acuity threshold (cycles/degree); SNR= signal-to-noise ratio; Sc SNR= maximum SNR within the cursors (dotted lines) that define the data used to estimate threshold; Pk SNR= maximum SNR at peak mean amplitude in the record.

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Figure 2
Binocular grating visual acuity scores (in logMAR units) from 25 children with optic pathway tumors compared with age norms from our own laboratory. Boys are represented by black triangles.

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Figure 3
Distribution of 25 children with optic pathway tumors considering World Health Organization classification(19), age at sweep visually evoked potential (SVEP) evaluation (in months), and binocular grating acuity scores (in logMAR units).

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Table 1
Demographics, recognition (Snellen optotypes), and grating visual acuity (GVA) measured by the sweep visually evoked potentials (SVEP) technique, both expressed in the logarithm of the minimum angle of resolution (logMAR), and the spherical equivalent of the refractive error, in diopters (D), from the better-seeing eyes (BSE) and the worse-seeing eyes (WSE) of 10 healthy children

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Table 2
Demographics, tumor type, tumor location, suspected mechanism of effect on optic pathway, and treatment of 25 children with optic pathway tumors according to visual impairment category

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Table 3
Demographics, clinical features, grating visual acuity (GVA) scores, and visual deficits in 25 children with optic pathway tumors according to visual impairment category

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Table 4
Summary of studies on visual abnormalities in children with brain tumors, including findings from the current study

Individual	Sex/age (mo)	BSE		WSE
Individual	Sex/age (mo)	Snellen optotypes (logMAR)	GVA (logMAR)	Snellen optotypes (logMAR)	GVA (logMAR)
C01	F/52	0.00	0.03	0.00	0.04
C02	F/55	0.00	0.02	0.00	0.06
C03	M/60	0.00	0.04	0.00	0.05
C04	M/70	0.00	0.01	0.00	0.01
C05	F/72	0.00	0.05	0.00	0.06
C06	F/80	0.00	0.01	0.00	0.03
C07	F/85	0.00	0.01	0.00	0.01
C08	M/88	0.00	0.06	0.00	0.08
C09	F/93	0.00	0.06	0.00	0.07
C10	M/95	0.00	0.07	0.00	0.09

Patient	Sex	Age (mo)	Tumor onset (mo)	Tumor type	Tumor location	Tumor effect on visual system	Treatment
Mild visual impairment (n=10)
S01	M	12	3	Astrocytoma	Hypothalamic/chiasmatic	Inward growth	Chemotherapy
S02	M	13	0	Pilocytic astrocytoma	Hypothalamic/chiasmatic	Mass effect	Chemotherapy + resection
S03	F	15	3	Pilocytic astrocytoma	Optic tract (left)	Inward growth	Observation
S04	F	32	0	Glioblastoma	Occipital lobe	Inward growth	Chemotherapy + resection
S05	M	43	31	Astrocytoma	Hypothalamic/chiasmatic	Inward growth	Observation
S06	F	50	36	Low-grade glioma	Hypothalamic/chiasmatic	Inward growth + raised ICP	Chemotherapy + VPS
S07	F	65	12	Pilocytic astrocytoma	Hypothalamic/chiasmatic	Inward growth + raised ICP	Chemotherapy + VPS
S08	M	79	24	Pilocytic astrocytoma	Hypothalamic/chiasmatic	Mass effect + raised ICP	Resection + VPS
S09	M	94	4	Astrocytoma	Hypothalamic/chiasmatic	Mass effect + raised ICP	Chemotherapy + resection + VPS
S10	M	95	36	Low-grade glioma	Hypothalamic/chiasmatic	Inward growth	Observation
Moderate visual impairment (n=8)
S11	M	3	1	Teratoid/rhabdoid	Hypothalamic/chiasmatic	Mass effect	Resection
S12	F	8	5	Pilocytic astrocytoma	Hypothalamic/chiasmatic	Inward growth + raised ICP	Chemotherapy + VPS
S13	F	9	0	Low-grade glioma	Hypothalamic/chiasmatic	Inward growth	Observation
S14	F	10	6	Pilocytic astrocytoma	Hypothalamic/chiasmatic	Inward growth	Chemotherapy
S15	F	17	6	Astrocytoma	Hypothalamic/chiasmatic	Mass effect + raised ICP	Chemotherapy + resection
S16	F	36	6	Low-grade glioma	Hypothalamic/chiasmatic	Inward growth	Chemotherapy
S17	F	40	5	Astrocytoma	Hypothalamic/chiasmatic	Inward growth	Chemotherapy + EVP
S18	M	54	21	Pilocytic astrocytoma	Hypothalamic/chiasmatic	Mass effect + raised ICP	Chemotherapy + resection + VPS
Severe visual impairment (n=7)
S19	F	18	6	Low-grade glioma	Hypothalamic/chiasmatic	Inward growth + raised ICP	Chemotherapy + VPS
S20	M	20	14	Pilocytic astrocytoma	Hypothalamic/chiasmatic	Mass effect	Chemotherapy + resection
S21	M	26	6	Desmoplastic infantile ganglioglioma	Occipital lobe	Mass effect	Resection
S22	F	26	22	Pilocytic astrocytoma	Hypothalamic/chiasmatic	Inward growth + raised ICP	Chemotherapy + VPS
S23	M	33	6	Low-grade glioma	Hypothalamic/chiasmatic	Inward growth	Chemotherapy
S24	M	37	18	Astrocytoma	Hypothalamic/chiasmatic	Mass effect + raised ICP	Chemotherapy + resection + VPS
S25	M	42	0	Pilomyxoid astrocytoma	Lateral geniculate body (right)	Inward growth	Resection

Patient	Sex/age (mo)	Clinical presentation	Seizures	Ocular motility and nystagmus	Fundus	GVA OU (logMAR)	GVA deficit (logMAR)	GVA BSE (logMAR)	GVA WSE (logMAR)	IAD (logMAR)
Mild visual impairment (n=10)
S01	M/12	Proptosis	No	XT LE	Cupping WSE	0.44	0.11	0.44	1.40	0.96
S02	M /13	NF1 and shaking eyes	No	ORTHO/Nys	NA	0.63	0.30	0.61	0.63	0.02
S03	F/15	Ptosis	No	ORTHO	Normal OU	0.71	0.38	0.71	3.00	2.29
S04	F/32	Bulging fontanelle	Yes	XT LE	Normal OU	0.40	0.29	0.39	0.53	0.14
S05	M /43	NF1 and shaking eyes	No	ORTHO	Normal OU	0.35	0.30	0.35	0.40	0.05
S06	F/50	Vomiting and shaking eyes	No	ORTHO	Pallor OU	0.17	0.12	0.16	1.45	1.29
S07	F/65	Weight loss	No	XT LE	Normal OU	0.22	0.17	0.22	0.98	0.76
S08	M /79	Eye misalignment	No	XT RE	Pallor WSE	0.43	0.38	0.43	3.00	2.57
S09	M /94	NF1 and shaking eyes	Yes	XT OD/Nys	Pallor OU	0.39	0.34	0.47	0.55	0.08
S10	M /95	Vomiting and ptosis	No	ORTHO	Normal OU	0.24	0.19	0.26	0.66	0.40
Moderate visual impairment (n=8)
S11	M/3	Vomiting and eye misalignment	No	XT	Normal OU	1.21	0.57	1.19	1.20	0.01
S12	F/8	Shaking eyes	Yes	ORTHO/Nys	Normal OU	0.93	0.56	0.93	3.00	2.07
S13	F/9	Shaking eyes	No	ORTHO/Nys	Cupping OU	0.91	0.54	0.44	0.95	0.51
S14	F /10	Shaking eyes	Yes	ORTHO/Nys	Cupping OU	0.79	0.42	0.93	0.93	0.00
S15	F/17	Shaking eyes	No	ORTHO/Nys	Pallor OU	0.84	0.66	1.09	1.25	0.14
S16	F /36	Shaking eyes	No	ORTHO/Nys	Normal OU	0.70	0.65	0.71	0.75	0.04
S17	F /40	Shaking eyes	No	ORTHO/Nys	NA	0.80	0.75	0.80	3.00	2.80
S18	M /54	Vomiting	No	XT RE	Cupping OU	0.65	0.60	0.65	1.15	0.50
Severe visual impairment (n=7)
S19	F /18	Weight loss and eye misalignment	Yes	XT RE	Cupping OU	1.30	1.12	1.42	3.00	1.58
S20	M /20	Shaking eyes	No	ORTHO/Nys	Pallor OU	1.00	0.82	1.03	1.04	0.01
S21	M /26	Hypotonia	No	XT	Normal OU	1.31	1.17	1.31	1.37	0.06
S22	F /26	Eye misalignment	Yes	ET/Nys	Pallor OU	1.16	1.02	1.17	1.27	0.10
S23	M /33	Shaking eyes	No	XT LE/Nys	Pallor OU	1.04	0.93	1.04	3.00	1.96
S24	M /37	Shaking eyes	Yes	ET/Nys	Pallor OU	1.14	1.09	1.14	3.00	1.86
S25	M /42	Vomiting	No	ORTHO/Nys	Normal OU	1.50	1.45	1.50	3.00	1.50

Author, year, and location	N	Age range (years)	Tumor location	Visual abnormalities at diagnosis
Wisoff et al., 1990, USA⁽²³23 Wisoff JH, Abbott R, Epstein F. Surgical management of exophytic chiasmatic-hypothalamic tumors of childhood. J Neurosurg. 1990; 73(5):661-7.⁾	16	0.2-21.0	Supratentorial, midline, and infratentorial	LVA (n=11) VF loss (n=6) Strabismus/nystagmus (n=4)
Suharwardy & Elston, 1997, UK⁽²²22 Suharwardy J, Elston J. The clinical presentation of children with tumours affecting the anterior visual pathways. Eye (Lond). 1997; 11(Pt 6):838-44.⁾	17 (12 boys)	1.0-13.0	Supratentorial, midline, and infratentorial	LVA (n=16)) VF loss (n=9) Optic disc atrophy (n=7) Papilledema (n=4) Swollen disc (n=3) Disc pallor (n=2)) Afferent pupillary defect (n=9) Proptosis (n=3)
Grabenbauer et al., 2000, Germany⁽²⁰20 Grabenbauer GG, Schuchardt U, Buchfelder M, Rödel CM, Gusek G, Marx M, et al. Radiation therapy of optico-hypothalamic gliomas (OHG) - radiographic response, vision and late toxicity. Radiother Oncol. 2000;54(3):239-45.⁾	25 (14 boys)	1.5-16.0	Optic pathway	Visual disturbances (n=17) Strabismus (n=5) VF deficits (n=20)
Baroncini et al., 2007, France⁽²¹21 Baroncini M, Vinchon M, Minéo JF, Pichon F, Francke JP, Dhellemmes P. Surgical resection of thalamic tumors in children: approaches and clinical results. Childs Nerv Syst. 2007;23(7):753-60.⁾	16 (9 girls)	2.4-14.9	Thalamic	Visual dysfunction (n=7)
Crawford et al., 2007, USA⁽²⁴24 Crawford JR, Santi MR, Vezina G, Myseros JS, Keating RF, LaFond DA, et al. CNS germ cell tumor (CNSGCT) of childhood: presentation and delayed diagnosis. Neurology. 2007;68(20):1668-73.⁾	30 (21 boys)	6.0-17.0	Supratentorial, midline, and infratentorial	LVA (n=13) Suprasellar: bitemporal hemianopsia (n=4) Pineal: 1 upgaze paralysis (Parinaud syndrome) Disseminated: mixed VF defects (n=1)
Santamaría, 2008, Spain⁽²⁵25 Santamaría A, Martínez R, Astigarraga I, Etxebarría J, Sánchez M. [Ophthalmological findings in pediatric brain neoplasms: 58 cases]. Arch Soc Esp Oftalmol. 2008;83(8):471-7. Spanish.⁾	58	<14.0	Supratentorial, midline, and infratentorial	Papilledema (n=17) Optic atrophy (n=14) Nystagmus VF lossPupillary alterations (n=18) Dyschromatopsia (n=4) Amaurosis (n=3); Legal blindness (n=3)
Wilne et al., 2011, UK⁽¹1 Wilne S, Collier J, Kennedy C, Jenkins A, Grout J, Mackie S, et al. Progression from first symptom to diagnosis in childhood brain tumours. Eur J Pediatr. 2012;171(1):87-93.⁾	139 (82 boys)	0.8-16.7	Supratentorial, midline, and infratentorial	Papilledema (n=50) Nystagmus (n=25) Reduced visual acuity (n=20) Squint (n=18) Diplopia (n=18)
Pillai et al., 2012, Canada⁽⁷7 Pillai S, Metrie M, Dunham C, Sargent M, Hukin J, Steinbok P. Intracranial tumors in infants: long-term functional outcome, survival, and its predictors. Childs Nerv Syst. 2012;28(4):547-55.⁾	35 (18 boys)	0.0-1.0	Supratentorial, infratentorial	Sunset eyes (n=21)Nystagmus (n=3)
Ghodsi et al., 2015, Iran⁽²⁶26 Ghodsi SM, Habibi Z, Hanaei S, Moradi E, Nejat F. Brain tumors in infants. J Pediatr Neurosci. 2015;10(4):335-40.⁾	(20 boys)	-1.0	Supratentorial (n=11) Infratentorial (n=6)	Supratentorial: sunset eyes (n=2), nystagmus (n=2), visual loss/no pursuit (n=2), ptosis (n=1) Infratentorial: sunset eyes (n=5), abducens nerve palsy (n=3)
Hoffmann et al., 2015, Germany⁽²⁷27 Hoffmann A, Boekhoff S, Gebhardt U, Sterkenburg AS, Daubenbüchel AM, Eveslage M, et al. History before diagnosis in childhood craniopharyngioma: associations with initial presentation and long-term prognosis. Eur J Endocrinol. 2015;173(6):853-62.⁾	411	0.0-9.0	Sellar/suprasellar	Visual impairment (n=161) Papilledema Optic atrophy
Alswaina et al., 2015, Saudi Arabia⁽²2 Alswaina N, Elkhamary SM, Shammari MA, Khan AO. Ophthalmic features of outpatient children diagnosed with intracranial space-occupying lesions by ophthalmologists. Middle East Afr J Ophthalmol. 2015;22(3):327-30.⁾	26 (14 boys)	0.1-17.0	Supratentorial, midline, and infratentorial	LVA with disc pallor (n=10) LVA with disc swelling (n=3) Acquired strabismus with disc pallor or swelling (n=4) Acquired esotropia with diplopia (n=3) Acquired exotropia (n=4) Nystagmus (n=3)
Sánchez-Sánchez et al., 2016, Mexico⁽²⁸28 Sánchez-Sánchez LM, Vázquez-Moreno J, Heredia-Delgado JA, Sevilla-Castillo R. Presentación clínica de tumores intracraneales supratentoriales (ST) e infratentoriales (IT) en pacientes pediátricos. Gac Med Mex. 2016;152(2):158-62.⁾	51 (28 boys)	0.0-15.0	Supratentorial (n=19) Infratentorial (n=32)	Nystagmus (n=6 ST) Double vision (n=2 ST + 3 IT) LVA (n=4 ST) Strabismus (n=2 ST) Papilledema (n=1 ST + 1 IT)
Stocco et al., 2017, Italy⁽²⁹29 Stocco C, Pilotto C, Passone E, Nocerino A, Tosolini R, Pusiol A, et al. Presentation and symptom interval in children with central nervous system tumors. A single-center experience. Childs Nerv Syst. 2017;33(12):2109-16.⁾	75 (45 boys)	3.3-11.7	Supratentorial, midline, and infratentorial	Strabismus (n=16) Abnormal optic disc ( n=11) Nystagmus (n=11) Double vision (n=9) Papilledema (n=8) Visual field defects (n=7) Palpebral ptosis (n=6) Anisocoria (n=4) Abnormal pupillary light reflex (n=3) Blurred vision (n=3) "Eyes wide open" episodes (n=3) Loss of stereopsis (n=1) Abnormal eye movements (n=1) Sunset eyes (n=1) Visual loss (n=1)
Dotto et al., 2020, Brazil⁽⁵5 Dotto PF, Berezovsky A, Cappellano AM, da Silva NS, Sacai PY, Silva FA, et al. Visual function assessed by visually evoked potentials in optic pathway low-grade gliomas with and without neurofibromatosis type 1. Doc Ophthalmol. 2018;136(3):177-89.⁾	25 (13 boys)	0.2-7.9	Supratentorial/midline	Abnormal grating acuity (n=25) Nystagmus (n=17) Enlarged IAD (n=16) Abnormal optic nerve (n=13) Strabismus (n=12) Abnormal visual behavior (n=9)

[1] Corresponding author: Patrícia de Freitas Dotto Quaresma. E-mail: patdotto@gmail.com

Brasil

Brasil

Deficiência na acuidade visual de grades avaliada pelos potenciais visuais evocados de varredura em crianças com tumores da via óptica incapazes de informar a acuidade de optotipos

RESUMO

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