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Case Report
 
Asymptomatic unilateral pulsatile eye: Clinical and therapeutic evaluation of sphenoid bone defect in neurofibromatosis type I
Ka Lung Chong1, Syeb Shoeb Ahmad1, Fatimah Hussin2, Norlina Mohd Ramli3, Shuaibah Abdul Ghani1
1Department of Ophthalmology, Queen Elizabeth Hospital, Kota Kinabalu, Sabah, Malaysia.
2Department of Radiology, Queen Elizabeth Hospital, Kota Kinabalu, Sabah, Malaysia.
3Department of Ophthalmology, University of Malaya Medical Centre, Malaysia.

doi:10.5348/ijcri-2013-11-395-CR-9

Address correspondence to:
Ka Lung Chong
Department of Ophthalmology, Queen Elizabeth Hospital
Kota Kinabalu, 88586
Sabah
MALAYSIA
Phone: +6088218684
Fax: +6088252827
Email: pbdckl@hotmail.com

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How to cite this article:
Chong KL, Ahmad SS, Fatimah H, Ramli NM, Ghani SA. Asymptomatic unilateral pulsatile eye: Clinical and therapeutic evaluation of sphenoid bone defect in neurofibromatosis type I. International Journal of Case Reports and Images 2013;(11):623–626.


Abstract
Introduction: Neurofibromatosis type I (NF-1) is a common inherited, multisystem, neurocutaneous disorder that predisposes to the development of benign and malignant tumors. Orbital-temporal manifestations of neurofibromatosis are not common.
Case Report: We report a rare case of atypical presentation of NF-1 which presented to us with incidental asymptomatic unilateral pulsatile eye. After investigation, the patient was found to have unilateral partial sphenoid bone defect. No other orbital-temporal manifestation of neurofibromatosis was noted.
Conclusion: The pathogenesis of unilateral greater wing defect in NF-1 is still unclear. Some authors have reported that the sphenoid bone defect may progress. If the defect does progress, we need to follow-up to monitor sphenoid bone defect manifestation in every orbital-temporal NF-1 patient.

Keywords: Sphenoid bone defect, Orbital-temporal neurofibromatosis, Greater wing defect, Pulsatile eye, Neurofibromatosis type I (NF-1)


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Author Contributions
Ka Lung Chong – Substantial contributions to conception and design, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Syeb Shoeb Ahmad – Substantial contributions to conception and design, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Fatimah Hussin – Substantial contributions to conception and design, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published.
Norlina Mohd Ramli – Substantial contributions to conception and design, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published.
Shuaibah Abdul Ghani – Substantial contributions to conception and design, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published.
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© Ka Lung Chong et al. 2013; This article is distributed the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any means provided the original authors and original publisher are properly credited. (Please see Copyright Policy for more information.)