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A Journal on Pediatrics, Neonatology, Adolescent Medicine,
Child and Adolescent Psychiatry

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Minerva Pediatrica 2007 February;59(1):13-21


language: English

Neurofibromatosis type 1 and optic pathway glioma. A long-term follow-up

Lama G. 1, Esposito Salsano M. 1, Grassia C. 1, Calabrese E. 1, Grassia M. G. 2, Bismuto R. 1, Melone M. A. B. 3, Russo S. 4, Scuotto A. 3

1 Department of Paediatrics Second University of Naples, Naples, Italy 2 Department of Mathematics and Statistics University of Naples Federico II, Naples, Italy 3 Department of Neurologic Sciences Second University of Naples, Naples, Italy 4 Department of Ophthalmology Second University of Naples, Naples, Italy


Aim. Optic pathway gliomas (OPG) are the predominant intracranial tumours associated with neurofibromatosis type 1 (NF1). The aim of this study was to evaluate the prevalence and the outcome of OPG in 200 NF1 patients (122 males and 78 females, aged 1-25 years) followed up to 16 years (mean of 6 years).
Methods. All children were evaluated by a detailed physical, neurological and ophthalmological examination. Fifteen out of 200 (7.5%) of these patients (7 males, 8 females) were identified with evidence of optic pathway tumours.
Results. Nine children had symptoms such as endocranial hypertension, seizures, headache; 4 patients only showed anomalies at ophthalmological examination; 2 patients had no symptoms or signs. All children had evidence of optic pathway tumour on magnetic resonance imaging. Three had a prechiasmal tumour, 2 had a chiasmal tumour, 1 had prechiasmal/chiasmal tumour, 2 had a prechiasmal/chiasmal and postchiasmal tumour, 2 had a chiasmal and postchiasmal tumour, 4 had a massive involvement of the optic system, 1 child exhibited a bilateral involvement of the optic nerves with additional impairment of the chiasm. Four patients had partial and/or subtotal spontaneous regression.
Conclusion. Because optic pathway tumours arise in children younger than 6 years of age, all NF1 children should undergo yearly ophtalmologic examination and growth assessment to monitor signs of precocious puberty.

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