Pulsatile Proptosis and Sphenoid Wing Dysplasia with no Evidence of Neurofibromatosis Type 1: A Case Report and Review of the Literature.

Item request has been placed! ×
Item request cannot be made. ×
loading   Processing Request
Read More Add to Saved list
  • Additional Information
    • Source:
      Publisher: Galenos Yayınevi Country of Publication: Turkey NLM ID: 101686048 Publication Model: Print Cited Medium: Internet ISSN: 2149-8709 (Electronic) Linking ISSN: 21498709 NLM ISO Abbreviation: Turk J Ophthalmol Subsets: MEDLINE
    • Publication Information:
      Original Publication: Fındıkzade-İstanbul, Türkiye : Galenos Yayınevi, [2015]-
    • Subject Terms:
      Exophthalmos*/diagnosis ; Exophthalmos*/etiology ; Tomography, X-Ray Computed* ; Neurofibromatosis 1*/complications ; Neurofibromatosis 1*/diagnosis ; Sphenoid Bone*/diagnostic imaging; Humans ; Male ; Adolescent ; Diagnosis, Differential
    • Abstract:
      In this study, we aimed to present a rare case of pulsatile proptosis due to sphenoid wing dysplasia without the features of neurofibromatosis type 1 (NF1). A 17-year-old male patient presented with swelling in the superotemporal region of the right eye. Physical examination revealed facial asymmetry with a pulsatile, ill-defined, soft lesion with in the superotemporal region of the right orbit associated with pulsatile proptosis, downward dystopia, and hypotropia. Computer tomography imaging to establish a differential diagnosis showed temporal lobe herniation secondary to sphenoid wing dysplasia. The patient was assessed for NF1, which is most commonly associated with sphenoid wing dysplasia, but no evidence supporting the diagnosis was found. Patients presenting with proptosis should be carefully examined for pulsation and murmurs, and a trauma history should be investigated. Radiological imaging should be used to facilitate the differential diagnosis, and the current clinical condition should be managed with a multidisciplinary approach.
      Competing Interests: Conflict of Interest: No conflict of interest was declared by the authors.
      (©Copyright 2024 by the Turkish Ophthalmological Association / Turkish Journal of Ophthalmology published by Galenos Publishing House.)
    • References:
      Childs Nerv Syst. 2024 Jan;40(1):27-39. (PMID: 37993698)
      PLoS One. 2017 Apr 12;12(4):e0175000. (PMID: 28403237)
      J Craniofac Surg. 2021 Oct 1;32(7):2539-2541. (PMID: 34406156)
      Childs Nerv Syst. 2021 Apr;37(4):1209-1217. (PMID: 33029727)
      Ophthalmic Plast Reconstr Surg. 2018 Mar/Apr;34(2):e65-e67. (PMID: 29346169)
      J Ayub Med Coll Abbottabad. 2022 Oct-Dec;34(Suppl 1)(4):S1013-S1020. (PMID: 36550665)
      JPRAS Open. 2021 Nov 10;31:67-71. (PMID: 34917731)
      Case Rep Radiol. 2017;2017:7047696. (PMID: 29435380)
      Indian J Ophthalmol. 2018 Jan;66(1):157-160. (PMID: 29283150)
      J Clin Neurosci. 2021 Apr;86:230-234. (PMID: 33775333)
      Craniomaxillofac Trauma Reconstr. 2016 Sep;9(3):255-9. (PMID: 27516843)
      Genet Med. 2021 Aug;23(8):1506-1513. (PMID: 34012067)
      Ann Plast Surg. 2014;72(6):S107-11. (PMID: 24835868)
      Skull Base. 2003 Nov;13(4):211-217. (PMID: 15912180)
      Clin Imaging. 2018 Sep - Oct;51:160-163. (PMID: 29787982)
      Anticancer Res. 2015 Dec;35(12):6813-8. (PMID: 26637901)
      In Vivo. 2011 Mar-Apr;25(2):287-90. (PMID: 21471549)
      Ulus Travma Acil Cerrahi Derg. 2008 Jul;14(3):247-52. (PMID: 18781424)
      Plast Reconstr Surg Glob Open. 2021 Mar 18;9(3):e3483. (PMID: 33758729)
      Clin Neurol Neurosurg. 2010 Dec;112(10):909-14. (PMID: 20702031)
      J Craniofac Surg. 1998 Nov;9(6):529-35. (PMID: 10029765)
      J Craniomaxillofac Surg. 2014 Dec;42(8):1937-41. (PMID: 25308959)
      J AAPOS. 2022 Aug;26(4):210-214.e1. (PMID: 35609740)
      Neuropediatrics. 2016 Aug;47(4):278-9. (PMID: 27280756)
      ANZ J Surg. 2018 Jul;88(7-8):E615-E616. (PMID: 27061833)
      BMC Ophthalmol. 2015 Oct 29;15:149. (PMID: 26514695)
      Medicine (Baltimore). 2021 Oct 22;100(42):e27575. (PMID: 34678901)
      World Neurosurg. 2022 Dec;168:207-208. (PMID: 36179763)
      Case Rep Ophthalmol. 2015 May 19;6(2):158-63. (PMID: 26078749)
      Neuroophthalmology. 2022 Feb 15;46(4):270-274. (PMID: 35859630)
      Cureus. 2024 Feb 6;16(2):e53728. (PMID: 38455798)
    • Contributed Indexing:
      Keywords: Dystopia; exophthalmos; neurofibromatosis type 1; pulsatile proptosis; sphenoid wing dysplasia
    • Publication Date:
      Date Created: 20241028 Date Completed: 20241028 Latest Revision: 20241128
    • Publication Date:
      20241202
    • Accession Number:
      PMC11589236
    • Accession Number:
      10.4274/tjo.galenos.2024.10662
    • Accession Number:
      39463178