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J Neurol Surg A Cent Eur Neurosurg 2018; 79(03): 268-272
DOI: 10.1055/s-0037-1608869
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Optic Nerve Glioma in Two Sisters with Family History of Neurofibromatosis Type 1

Jivko Kolev Surchev
1   Department of Neurosurgery, UMHAT “St. Ivan Rilski”, Medical University, Sofia, Bulgaria
,
Sevdalin Nachev
2   Department of General and Clinical Pathology, MMA, Sofia, Bulgaria
,
Lyudmila Pavlova Todorova
3   Institute of Biophysics and Biomedical Engineering, Bulgarian Academy of Sciences, Sofia, Bulgaria
,
Marin Marinov
1   Department of Neurosurgery, UMHAT “St. Ivan Rilski”, Medical University, Sofia, Bulgaria
› Author Affiliations
Further Information

Publication History

17 March 2017

24 August 2017

Publication Date:
02 January 2018 (online)

Also available ateRefMedOne
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Abstract

Optic nerve glioma (ONG) is associated in 10% of patients with neurofibromatosis (NF) type 1. To date no consensus has been reached regarding the therapeutic approach and prevention of visual impairment in these patients. Reports in the literature vary from a conservative approach (observation) to the use of single treatment modalities or multimodality protocols of surgical removal, radiotherapy, and/or chemotherapy. We present our experience with two siblings with ONG whose mother carries cutaneous stigmata of NF type 1. The younger sister was diagnosed 3 years after the treatment of the older sibling following recommended imaging for screening. Postoperative follow-up for 11 and 15 years, respectively, demonstrated lack of tumor regrowth and preserved vision in the contralateral eye. We discuss the treatment strategy in pediatric patients with orbital ONG associated with NF type 1.

Keywords

optic nerve glioma - von Recklinghausen disease - heredity

Note

We had the informed consent of the mother and the children to publish the cases accompanied by photographs.