Congenital orbital encephalocele, orbital dystopia, and exophthalmos

J Craniofac Surg. 2012 Jul;23(4):e343-4. doi: 10.1097/SCS.0b013e3182564d5a.

Abstract

We present here an exceedingly rare variant of a nonmidline basal encephalocele of the spheno-orbital type, and this was accompanied with orbital dystopia in a 56-year-old man. On examination, his left eye was located more inferolaterally than his right eye, and the patient said this had been this way since his birth. The protrusion of his left eye was aggravated when he is tired. His naked visual acuity was 0.7/0.3, and the ocular pressure was 14/12 mm Hg. The exophthalmometry was 10/14 to 16 mm. His eyeball motion was not restricted, yet diplopia was present in all directions. The distance from the midline to the medial canthus was 20/15 mm. The distance from the midline to the midpupillary line was 35/22 mm. The vertical dimension of the palpebral fissure was 12/9 mm. The height difference of the upper eyelid margin was 11 mm, and the height difference of the lower eyelid margin was 8 mm. Facial computed tomography and magnetic resonance imaging showed left sphenoid wing hypoplasia and herniation of the left anterior temporal pole and dura mater into the orbit, and this resulted into left exophthalmos and encephalomalacia in the left anterior temporal pole. To the best of our knowledge, our case is the second case of basal encephalocele and orbital dystopia.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Accidental Falls
  • Diagnosis, Differential
  • Diplopia / etiology*
  • Encephalocele / complications*
  • Encephalocele / diagnostic imaging
  • Exophthalmos / etiology*
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Orbit / abnormalities*
  • Orbit / diagnostic imaging
  • Orbital Diseases / complications*
  • Orbital Diseases / diagnostic imaging
  • Tomography, X-Ray Computed
  • Visual Acuity