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|Year : 2016 | Volume
| Issue : 4 | Page : 835-836
Bilateral cranio-orbital neurofibromas in a 16-year-old child with neurofibromatosis 1
Peng Li, Zhijun Yang, Zhenmin Wang, Pinan Liu
Department of Neurosurgery, Beijing Tian Tan Hospital, Capital Medical University, Beijing, China
|Date of Web Publication||5-Jul-2016|
Dr. Pinan Liu
Department of Neurosurgery, Beijing Tian Tan Hospital, Capital Medical University, 6 Tiantan Xili, Dongcheng District, Beijing 100050
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Li P, Yang Z, Wang Z, Liu P. Bilateral cranio-orbital neurofibromas in a 16-year-old child with neurofibromatosis 1. Neurol India 2016;64:835-6
This 16-year-old boy had progressive bilateral exophthalmos and ocular movement restriction since birth. His ophthalmologic examination revealed a decreased visual acuity (left:right = 0.3:0.25). He had scattered café-au-lait spots and multiple subcutaneous pathologically confirmed neurofibromas. He was diagnosed with neurofibromatosis type 1 (NF1). His parents were healthy, and there was no family history of NF1 or other major diseases. Cranial magnetic resonance imaging showed bilateral cranio-orbital communicating tumors [Figure 1]. The tumors extended into the orbits through the superior orbital fissure and the hypoplastic greater sphenoidal wings. A right frontotemporal craniotomy along with orbitozygomatic osteotomy was performed to resect the right-sided tumor. The pathological diagnosis of the resected tumor was 'plexiform neurofibroma.' The postoperative course was uneventful, and his right-sided exophthalmos improved after the surgery. The ocular movement restriction and decreased visual acuity remained unchanged. His parents refused a second surgery to resect the left-sided tumor.
|Figure 1: Cranial magnetic resonance images show the tumor extending to bilateral parasellar regions and orbits; (a) T1-weighted image; (b) T2-weighted image; (c) enhanced T1-weighted image (no evident enhancement)|
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To the best of our knowledge, this is the first report of bilateral cranio-orbital communicating neurofibromas in NF1. Previously, only one patient with bilateral neurofibromas involving the cavernous sinuses has been reported. The tumors in our case were mostly located at the cavernous sinuses. They, therefore, in all likelihood took origin from the ocular or the trigeminal nerves of the cavernous sinuses. The pterional approach is suitable for the resection of cranio-orbital communicating tumors. The addition of orbitozygomatic osteotomy provides maximum exposure with minimum retraction. It is difficult to resect the plexiform neurofibromas totally. An early diagnosis and timely operation might help to improve the patient's prognosis.
Financial support and sponsorship
National Science and Technology Support Program of the 12th Five-Year of China (Grant number: 2012BAI12B03); Natural Science Foundation of Beijing (Grant number: 7112049).
Conflicts of interest
There are no conflicts of interest.
| » References|| |
Meersschaut VA, Kros JM, Catsman-Berrevoets CE, Lequin MH. Congenital bilateral plexiform neurofibromas of the cavernous sinuses. Pediatr Radiol 2003;33:272-4.
Jian T, Sun F, Tang D, Wang S, Wu T, Zhao L. Clinical analysis of transcranial orbitotomy approach on cranio-orbital tumors. J Craniofac Surg 2015;26:441-6.