Pediatric Macrophthalmia

  • Etiology:
    — Enlarged globe due to increased intraocular pressure due to obstruction of canal of Schlemm which is responsible for absorption of aqueous humor
    — Less rigid sclera in children
  • Imaging:
    — Enlarged globe that is generally enlarged and without mass or calcifications
  • DDX: Generalized enlargement of globe without mass
    — Staphyloma and severe axial myopia – defect is temporal to optic disc
    — Phakomatoses (neurofibromatosis Type 1, Sturge Weber syndrome), collagen vascular disease
  • Complications:
  • Treatment:
  • Clinical: Seen in neurofibromatosis Type 1 and Sturge Weber syndrome

Radiology Cases of Macrophthalmia

Radiology Cases of Macrophthalmia Due to Neurofibromatosis Type I

MRI of macrophtalmia and optic pathway glioma in neurofibromatosis type I
Axial (above) and coronal (below) T2 MRI without contrast of the brain shows the left globe is larger than the right globe. The optic nerves bilaterally are thickened and tortuous.