Visual loss in children with neurofibromatosis type 1 and optic pathway gliomas

Balcer, Laura J.; Liu, Grant T.; Heller, Gordon; Bilaniuk, Larissa T.; Volpe, Nicholas J.; Galetta, Steven; Molloy, Patricia T.; Phillips, Peter C.B.; Janss, Anna J.; Vaughn, Sheila; Maguire, Maureen G.

doi:10.1016/s0002-9394(00)00852-7

Cited by 182 publications

(113 citation statements)

References 9 publications

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“…2,[12][13][14] Reviewing the clinical records of 90 NF1 patients with OPG referred to two pediatric centers, neither housing a neuro-oncology program, King et al 7 found that, at the time of OPG diagnosis, 20 (22%) of these unselected patients had some decrease in VA (,20/20). More recently, Thiagalingam et al 15 retrospectively studied an unselected series of 54 patients with OPG and NF1 and documented that about 31% of them had some visual impairment at the time of OPG diagnosis and only 31.5% underwent treatment.…”

Section: Discussionmentioning

confidence: 99%

Visual outcome of a cohort of children with neurofibromatosis type 1 and optic pathway glioma followed by a pediatric neuro-oncology program

Via¹,

Opocher²,

Pinello³

et al. 2007

Neuro-Oncology

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We evaluated the visual outcome of a cohort of children with neurofibromatosis type 1 (NF1) and optic pathway glioma (OPG) treated according to standardized therapeutic guidelines. The study population consisted of all consecutive patients with NF1 and OPG referred to a specialized pediatric neuro-oncology program between 1994 and 2004. Treatment was instituted only in cases of progressive disease or clinical deterioration. Treatment modalities were chemotherapy (based on vincristine/ carboplatin) for children younger than 5 years and radiotherapy for all others. Ten boys and 10 girls (seven with a positive family history) entered the trial (median age at diagnosis of OPG, 29 months). At a median follow-up time of 78 months, seven patients had been treated with chemotherapy only, four with radiotherapy, and four with chemotherapy plus radiotherapy. Five patients were observed only. Currently, 18 are alive and two have died. Eight patients were treated for progressive visual loss in the face of stable disease, five for tumor volume increase without visual deterioration, and two for symptomatic tumor volume increase. At referral, six children had a visual acuity (VA) of , 30% in both eyes; eight children had 100% VA bilaterally. At referral, the visual field (VF) could be assessed in three children: One had VF loss in both eyes, one had VF loss in one eye, and one had normal VF. At last follow-up, eight children had VA , 20% in both eyes; only two children had 100% VA in both eyes. Among 11 children who had some visual function, three had VF loss in one eye and three in both eyes, and five had an intact VF. Contrast and color sensitivity were abnormal in seven and six patients, respectively. Thirteen children fell into the WHO hypovision category. In summary, among the 15 children treated, one had a definitive and two a mild improvement in VA. In conclusion, the visual outcome of this selected cohort of NF1 patients with OPG is unsatisfactory. A critical reappraisal of the therapeutic strategy adopted is needed.

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Section: Discussionmentioning

confidence: 99%

Visual outcome of a cohort of children with neurofibromatosis type 1 and optic pathway glioma followed by a pediatric neuro-oncology program

Via¹,

Opocher²,

Pinello³

et al. 2007

Neuro-Oncology

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“…The possibility of regression should be discussed when deciding the treatment plan. (13) Tumour location determines the prognosis. Optic nerve gliomas have fewer complications and a lower mortality compared to tumours in the optic chiasm, especially those involving postchiasmatic structures.…”

Section: Discussionmentioning

confidence: 99%

Neurofibromatose tipo I

Moraes¹,

Santos²,

Salomão³

2013

Rev. bras.oftalmol.

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RESUMOA neurofibromatose tipo I é uma doença autossômica dominante cujo diagnóstico presuntivo é feito com base em critérios clínicos. As três principais manifestações: neurofibromas, manchas café com leite e nódulos de Lisch ocorrem em mais de 90% dos pacientes até a puberdade. Relatamos o caso de um paciente jovem com diagnóstico de neurofibromatose tipo I e história familiar positiva para a doença, comentando seus aspectos clínicos e achados nos exames de imagem. Descritores: Neurofibromatose tipo I; Glioma; Imagem por ressonância magnética; Relatos de casos CASE REPORTThe authors declare no conflicts of interest

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“…5 Overall, a more favourable outcome with OPGs is seen in patients older than 2 years of age, with NF1, and with optic nerve and/or chiasmatic lesions. 2,6,10,13 Determining the best treatment for your next patient with progressive OPG is not currently defined in the medical literature. Comparing the success rates of the various treatment modalities, across the multiple publications on the subject, is fraught with pitfalls: many of the studies are retrospective, inclusion, and exclusion criteria are lacking, treatments are not standardized or randomized, and outcomes are not clearly stated.…”

Section: Treatmentmentioning

confidence: 99%

“…3,4 The clinical presentation of OPG is quite variable depending on the tumour location and extent. [4][5][6][7] Many patients are asymptomatic. 4,5 Patients with tumours within the orbit present with proptosis, strabismus, and/or visual loss.…”

Section: Introductionmentioning

confidence: 99%

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Optic glioma warranting treatment in children

Kaufman

Doroftei

2006

Eye

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Purpose To describe cases of optic pathway glioma (OPG) warranting treatment in children. Methods This is a retrospective review of pediatric patients treated for OPG. The clinical data and imaging studies were obtained from the medical records and radiology files of patients seen at the Pediatric NeuroOphthalmology Clinic at the University of Illinois, Chicago and the private office of the author (LMK). Results A total of seven cases with an age range of 3-48 months at presentation were reviewed. Three of the patients were also ultimately diagnosed with neurofibromatosis type 1. Presenting symptoms included proptosis, decreased vision, gaze deficit, and nystagmus. Four patients underwent biopsies that confirmed OPG. Six of the patients were treated with intravenous chemotherapy, with three patients requiring a second chemotherapy cycle. One patient was successfully treated with an en-bloc optic nerve excision. Two patients underwent unilateral enucleation owing to globe complications. Conclusion Although benign tumours, OPG can behave very aggressively in young children. Both chemotherapy and en-bloc excision can be employed for treatment.

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Visual loss in children with neurofibromatosis type 1 and optic pathway gliomas

Cited by 182 publications

References 9 publications

Visual outcome of a cohort of children with neurofibromatosis type 1 and optic pathway glioma followed by a pediatric neuro-oncology program

Visual outcome of a cohort of children with neurofibromatosis type 1 and optic pathway glioma followed by a pediatric neuro-oncology program

Neurofibromatose tipo I

Optic glioma warranting treatment in children

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