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Research ArticlePEDIATRICS

Orbit Deformities in Craniofacial Neurofibromatosis Type 1

Claude Jacquemin, Thomas M. Bosley and Helena Svedberg
American Journal of Neuroradiology September 2003, 24 (8) 1678-1682;
Claude Jacquemin
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Thomas M. Bosley
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Helena Svedberg
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  • Fig 1.
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    Fig 1.

    T1-weighted MR images.

    A, Parasagittal image shows a temporal arachnoid cyst on the left that pushes the orbital contents, including the globe, forward (arrowheads), while a tumor mass in the superior quadrant of the orbit pushes the globe downward (arrow).

    B, Axial image of the same patient with a temporal arachnoid cyst on the left (white arrowheads) shows shortening of the lateral wall of the orbit (black arrowhead) and flattening of the orbital angle. Also note the abnormal concave posterior aspect of the lateral orbital wall and the large wing of the sphenoid.

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    Fig 2.

    Coronal CT scans.

    A, Image of the classic egg-shaped, orbital enlargement and deformity. Note the phthisical eye (asterisk) and tumoral infiltration of superior rectus muscle (arrow) and orbital septum (arrowheads). The left paranasal sinuses are underdeveloped. The nasal septum and crista galli are deviated.

    B, Image of the posterior orbit shows that the tumors in the pterygoid fossa (asterisk) infiltrates the left orbital apex, enlarging the inferior orbital fissure and distorting the posterior orbit.

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    Fig 3.

    A, Sagittal T1-weighted, contrast-enhanced, fat-suppressed MR image shows infiltration of the superior extraconal space (small asterisk), superior rectus muscle, and levator palpebrae muscle by PNFs. Posterior extension of the tumor into the cavernous sinus is marked by the large asterisk. Intraconal sensory nerves are contrast enhanced and thickened because of tumor infiltration. Ipsilateral maxillary sinus is small compared with that on the contralateral side.

    B, Coronal T1-weighted MR image of the same patient shows extension of the tumor into the cavernous sinus, with discrete remodeling of the sphenoid body (arrowheads).

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    Fig 4.

    Axial CT image of the skull base shows expanded cranial foramina (foramen ovale, rotundum, and lacerum) on the left (asterisk) due to tumor enlargement of branches of the trigeminal nerve.

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    Fig 5.

    Axial CT image through the orbits shows unilateral enlargement of the right optic canal due to tumor infiltration of the optic nerve sheath without an optic nerve glioma. The ipsilateral globe is buphthalmic, and the lateral rectus (asterisk) is enlarged as a result of tumoral infiltration. The greater wing of the sphenoid is partially decalcified and bowed forward (arrowheads), without an obvious arachnoid cyst.

Tables

  • Figures
  • Patients with NF1

    LocationNo. of Patients (n=31)
    Soft-tissue tumor without orbital involvement7 (22.6)
    Radiologic orbital changes24 (77.4)
    Distortion due to an enlarged middle cranial fossa13 (41.9)
    Orbital-rim enlargement18 (58.1)
     Associated with buphthalmos13
     Associated with a small maxillary sinus13
     Associated with tumor in the anterior orbit2
    Imaging evidence of tumor within the orbit19 (61.3)
     Intraconal tumor13
     Globe (buphthalmos with or without enucleation)13
     Lacrimal gland11
     Extraocular muscle13
     Posterior orbit16
     Orbital bone18
    Expansion of the orbital foramen16 (51.6)
     Expansion of the superior orbital fissure15
     Expansion of the inferior orbital fissure12
     Expansion of the optic canal8
      Optic nerve glioma only6
      PNF infiltration of the optic nerve sheath2
    • Note.—Data in parentheses are percentages.

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American Journal of Neuroradiology: 24 (8)
American Journal of Neuroradiology
Vol. 24, Issue 8
1 Sep 2003
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Cite this article
Claude Jacquemin, Thomas M. Bosley, Helena Svedberg
Orbit Deformities in Craniofacial Neurofibromatosis Type 1
American Journal of Neuroradiology Sep 2003, 24 (8) 1678-1682;

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Orbit Deformities in Craniofacial Neurofibromatosis Type 1
Claude Jacquemin, Thomas M. Bosley, Helena Svedberg
American Journal of Neuroradiology Sep 2003, 24 (8) 1678-1682;
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Cited By...

  • Posterior-Anterior Cephalometric Study of Neurofibromatosis Type 1 Patients With Facial Plexiform Neurofibroma: Analysis of Skeletal Symmetry Concerning Midfacial and Skull Base Reference Points (Zygomatic Arch, Mastoid, and Juga)
  • Sphenoid Bone Pneumatisation on Lateral Cephalograms of Patients With Neurofibromatosis Type 1
  • Periapical Cemento-osseous Dysplasia Is Rarely Diagnosed on Orthopantomograms of Patients with Neurofibromatosis Type 1 and Is Not a Gender-specific Feature of the Disease
  • Optic Pathway Glioma and Cerebral Focal Abnormal Signal Intensity in Patients with Neurofibromatosis Type 1: Characteristics, Treatment Choices and Follow-up in 134 Affected Individuals and a Brief Review of the Literature
  • Analysis of Orbital Plain Radiographs for Orbital Deformities in Neurofibromatosis Type 1 Patients, with Special Reference to Alterations of the Orbital Rim as Indicators of Adjacent Plexiform Neurofibroma
  • Reconstruction of the Sphenoid Wing in a Case of Neurofibromatosis Type 1 and Complex Unilateral Orbital Dysplasia with Pulsating Exophthalmos
  • Dysplasia of the Orbit and Adjacent Bone Associated with Plexiform Neurofibroma and Ocular Disease in 42 NF-1 Patients
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