Septo-optic dysplasia

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aka DeMorsier’s Syndrome


Radiographic Appearance

  • Thinned optic tracts
  • Absent septum pellucidum
  • Boxlike appearance to the anterior
  • Thinning of corpus callosum


Clinically

Congenital anomaly, M:F=1:3, range of visual dysfunctions, nystagmus, seizures. There is also an association with hypothalamic hypopituitarism (66%). This may be manifested as diabetes insipidus or as growth retardation due to diminished growth hormone.